Search

Leprosy is a chronic infectious disease caused by the bacillus Mycobacterium leprae. The disease may evolve for inflammatory reactions, reversal reaction (RR) and erythema nodosum leprosum (ENL), the major cause of irreversible neuropathy in leprosy, which occur in 1 in 3 people with leprosy, even with effective treatment of M. leprae. Leprosy remains persistently endemic in our region where it predominantly affects lowest socioeconomic conditions people, as Toxoplasma gondii infection in the municipality studied. Previously, we have shown T. gondii coinfection as a risk marker for leprosy, mainly in its severe form. This present study assessed whether T. gondii infection is also a risk factor for leprosy reactions and the predictive value of immunoglobulin production prior to development of leprosy reactions. Patients with leprosy (n = 180), co-infected or not with T. gondii, had their serum investigated for levels of IgA, IgE, IgG1, IgG2, IgG3 and IgG4 anti-PGL-1 by ELISA prior to development of leprosy reactions. The serologic prevalence for T. gondii infection was 87.7% in leprosy reaction patients reaching 90.9% in those with ENL. The leprosy reaction risk increased in T. gondii seropositive individuals was two-fold ([OR] = 2.366; 95% confidence interval [CI 95%]: 1.024–5.469) higher than those seronegative, and considering the risk of ENL, this increase was even more evident (OR = 6.753; 95% CI: 1.050–72.85) in coinfected individuals. When evaluated the prediction of anti-PGL-1 immunoglobulin levels for development of leprosy reactions in patients coinfected or not with T. gondii, only the increase IgE levels were associated to occurrence of reactional episodes of leprosy, specifically ENL type, in patients coinfected with T. gondii, compared to those not coinfected or no reaction. Thus, the immunomodulation in co-parasitism T. gondii–M. leprae suggest increased levels of IgE as a biomarker for early detection of these acute inflammatory episodes and thereby help prevent permanent neuropathy and disability in leprosy patients. [ABSTRACT FROM AUTHOR]

Coccidioidomycosis occurs after inhalation of airborne spores of the endemic, dimorphic fungus, Coccidioides. While the majority of individuals resolve the infection without coming to medical attention, the fungus is a major cause of community-acquired pneumonia in the endemic region, and chronic pulmonary and extrapulmonary disease poses significant personal and economic burdens. This review explores the literature surrounding human susceptibility to coccidioidomycosis, including chronic pulmonary and extrapulmonary dissemination. Over the past century of study, themes have emerged surrounding factors impacting human susceptibility to severe disease or dissemination, including immune suppression, genetic susceptibility, sex, pregnancy, and genetic ancestry. Early studies were observational, frequently with small numbers of cases; several of these early studies are highly cited in review papers, becoming part of the coccidioidomycosis "canon". Specific genetic variants, sex, and immune suppression by TNF inhibitors have been validated in later cohort studies, confirming the original hypotheses. By contrast, some risk factors, such as ABO blood group, Filipino ancestry, or lack of erythema nodosum among black individuals, are repeated in the literature despite the lack of supporting studies or biologic plausibility. Using examination of historical reports coupled with recent cohort and epidemiology studies, evidence for commonly reported risk factors is discussed. [ABSTRACT FROM AUTHOR]

Introduction: Behcet's disease (BD) is classified among inflammatory vascular diseases, affecting vessels of all kinds and sizes thus affecting every tissue and organ, with unknown aetiology. Considering low frequency with which this condition occurs, variation with the signs of symptoms, lack of specific test for diagnosis, this study was done to understand the clinical behaviour of patients with BD, which will aid in diagnosing and managing patients with BD and also relapses and other associated conditions. Methodology: A hospital-based study was done in 50 adult patients of either sex, diagnosed with BD in a tertiary care hospital after obtaining ethical committee clearance during February 2020 to January 2023. The diagnosis was based on the diagnostic criteria laid down by the International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). Results of laboratory tests, including HLA-B5, HLA-B51, and HLA-B27, were recorded. NSAIDS, steroids and immunomodulating drugs were prescribed, either individually or in combination, to treat the disease. Data analysis was done using SPSS 20. Data represented as frequency percentage and bar diagrams. Results: Majority of the patients with BD belong to age group of 41 - 60 years (84%) with mean age of the patients being 42.3±8.4. BD was seen more in males (68%). Oral apthous (94%) ulcers were the most common manifestations followed by skin lesions (52% includes erythema nodosum, pseudo folliculitis and others) and ocular involvement (50% includes anterior uveitis, posterior uveitis, pan uveitis and retinal vasculitis). Pathergy test was positive in 62% of patients. Out of 50 patients 62% had positive HLA B5, followed by HLA B51 (40%) and HLA B27 (8%). Conclusion: BD is more common in younger age group with mean age 42.3 years and in males. Most of the patients were positive for HLAB5. [ABSTRACT FROM AUTHOR]

Background: Previous studies have demonstrated the coexistence of erythema nodosum (EN) and inflammatory bowel disease (IBD), while the exact etiology of the co‐occurrence of the two disorders remains uncertain. Methods: A bidirectional two‐sample Mendelian randomization (MR) design was employed to determine the causal link between EN and IBD. Genetic variations associated with Crohn's disease (CD) and ulcerative colitis (UC) were derived from accessible genome‐wide association studies pertaining to European ancestry. The FinnGen database was used to find the genetic variations containing EN. In the forward model, IBD was identified as the exposure, whereas in the reverse model, EN was identified as the exposure. The causal link between IBD and EN was examined using a range of different analysis techniques, the primary one being the inverse variance weighted (IVW) method, including inverse variance weighted‐fixed effects (IVW‐FE) and inverse‐variance weighted‐multiplicative random effects (IVW‐MRE). To strengthen the results, assessments of sensitivity, heterogeneity, and pleiotropy were also conducted. Results: MR results showed that IBD increased the risk of EN (IVW‐MRE: OR = 1.242, 95% CI = 1.068–1.443, p = 0.005). Furthermore, there was a strong correlation found between CD and a higher risk of EN (IVW‐FE: OR = 1.250, 95% CI = 1.119–1.396, p = 8.036 × 10−5). However, UC did not appear to be linked to EN (IVW‐FE: OR = 1.104, 95% CI = 0.868–1.405, p = 0.421). The reverse MR analysis findings did not imply that EN was linked to IBD. Horizontal pleiotropy did not appear to exist, and the robustness of these findings was confirmed. Conclusion: The current investigation found that in European populations, IBD and its subtype CD could raise the incidence of EN. [ABSTRACT FROM AUTHOR]

Background: Leprosy is the most ancient and widely spread global disease and was described in various articles of ancient civilizations. In 1990, the goal of eliminating leprosy by the end of the 20th century was proposed by the World Health Organization but still leprosy is highly prevalent in India. Leprosy is categorized in various types depending upon clinical features, histopathological findings and the host immunity. Various clinical features and acid fast bacilli demonstration in slit skin smears can help in diagnosis of leprosy but histopathological examination can help to classify and aid in the definitive diagnosis of leprosy and also prognosis of the disease and assessment of regression of the disease in patient undergoing treatment. Aim: To classify all skin biopsies having clinical diagnosis of leprosy in to various types according to the Ridley Jopling's classification of leprosy, also to find out the correlation between histopathological diagnosis of skin biopsies with clinical diagnosis of leprosy and to study various clinical presentations of leprosy with respect histopathological diagnosis. Materials and Methods: It was a prospective study done in the Department of Pathology, Shri Bhausaheb Hire Government Medical College Dhule over a period of 24 months i.e. from 1st January 2022 to 31st December 2023. Skin punch biopsies from 288 clinically diagnosed patients of leprosy were subjected for routine processing and routine staining with Haematoxyline & Eosin stain as well as Fite-Faraco staining. The lesions were classified using the Ridley-Jopling's classification into Tuberculoid Leprosy, Borderline Tuberculoid, Mid Borderline, Borderline Lepromatous and Lepromatous Leprosy. Few of the lesions were categorized as Histioid leprosy and Erythema Nodosum Leprosum Result: There was a male predominance (59.02%) seen in our study. Most common age group affected was 21to 30yrs followed by 31to 40yrs. Most common histopathological diagnosis was Borderline Tuberculoid leprosy followed by Indeterminate leprosy. Most common clinical presentation was hypopigmented patches, hypoasthetic patches, erythematous patches and tingling and numbness. 100% Fite-Faraco stain positivity was noted in Lepromatous and Histioid leprosy. Clinicohistopathological concordance was 100% in Erythema Nodosum Leprosum, followed by Tuberculoid (94%) and Borderline Tuberculoid (90%). The concordance was minimum in Mid Borderline leprosy(33%). Conclusion: Despite of implementation several government projects to control leprosy, leprosy still remains to be highly prevalent disease in India, also seen in this study, as we got 188 cases of leprosy over a period of 24 months. In our study we got high clinicohistopathological concordance in the diagnosis of Tuberculoid, Borderline Tuberculoid and Erythema Nodosum Leprosum cases. But it is low in the diagnosis of Midborderline and Indeterminate leprosy. There is overlapping of clinical features in various types of leprosy, so histopathological examination should be strongly recommended and considered as Gold standard for the diagnosis of leprosy. [ABSTRACT FROM AUTHOR]

Mycoplasma pneumoniae is an atypical bacterium causing respiratory tract infections mainly in the pediatric population. As a superantigen, it dysregulates the immune system and promotes immunological reactions. Dermatological symptoms occur in approximately one-fourth of the patients infected with this bacterium. This review describes skin diseases occurring during Mycoplasma pneumoniae infection. Differences in the course of these diseases compared to their presentation associated with other etiological factors, are also discussed. Among the cutaneous manifestations of Mycoplasma pneumoniae infection, unspecific rashes and urticaria are the most common. This bacterium is also a frequent cause of erythema multiforme, Stevens-Johnson syndrome, Mycoplasma-induced rash and mucositis, and erythema nodosum. Less frequently toxic epidermal necrolysis, leukocytoclastic vasculitis, IgA vasculitis, subcorneal pustular dermatosis, Gianotti-Crosti syndrome, and Sweet syndrome are described. Familiarity with Mycoplasma-induced entities is important and can be useful in dermatological practice in determining the etiology and implementing appropriate treatment. [ABSTRACT FROM AUTHOR]

Erythema nodosum leprosum (ENL) is an immunological complication of leprosy characterized by acute inflammation of the skin, nerves, and other organs. Identifying laboratory parameters is important for early diagnosis of leprosy reactions. Various cytokine biomarkers have been examined and only a few studies have reported on angiogenesis in leprosy. This study aims to understand the pathomechanism of ENL by examining IL-7 and plateletderived growth factor (PDGF)-BB mRNA expression that can be the development and consideration of new effective therapies to prevent reactions, recurrences, and defects in leprosy. The study used a cross-sectional analytic design. Sampling was done by peripheral blood from the patient and measuring mRNA expression with specific primers RT-PCR. The expression of mRNA IL-7 and PDGF-BB was significantly different between multibasilar patients without reaction and with ENL reaction, where there was an increased expression in ENL patients. This could be used as the development of potential biomarkers in ENL and development of new therapeutic intervention pathways in ENL. [ABSTRACT FROM AUTHOR]

Tuberculosis (TB) is an important and topical problem at global level, so it remains a challenge for public health policies. According to the World Health Organization (WHO), in 2018, about 10 million new TB cases worldwide were estimated. Of these, 11% are children under the age of 15. In addition to these alarming data, TB is responsible for 130,000 deaths per year in children and it is estimated that there are approximately 1 million TB-cases in the global population of children, being one of the top 10 causes of death in children worldwide. According to the WHO, there are economic, social, cultural, ethnic/racial, psychological and behavioral factors that interfere with the occurrence of diseases, and these factors are attributed to the persistence of infectious diseases such as TB in the population, even today. Numerous specialized studies strengthen the hypothesis of associating social vulnerability with TB emergence and distribution in the community. Therefore, the development of more accurate estimates of pediatric TB cases is an urgent need for public policy scheduling and resource allocation and, consequently, for reducing morbidity and mortality from TB among children. [ABSTRACT FROM AUTHOR]

Introduction: Lupus erythematosus tumidus (LET) is a rare photosensitive dermatosis that is categorized as intermittent cutaneous lupus erythematosus. It shares clinical similarities and histopathological features with other skin disorders, such as erythema nodosum, lymphocytic infiltrate of Jessner, and reticular erythematous mucinosis, thus making diagnosis quite challenging. We present a patient with LET whose diagnosis was confirmed after seeing several doctors. Case Presentation: A 52-year-old Hispanic female presented with tender erythematous nodules on her thighs for approximately 1 month. She was suspected of having erythema nodosum secondary to coccidioidomycosis and was prescribed fluconazole 200 mg for 30 days but showed no improvement. However, histopathological and direct immunofluorescence tests later confirmed a diagnosis of LET. The patient was treated with hydroxychloroquine, and the lesions improved remarkably after 2 weeks. Conclusion: LET is a rare dermatosis that closely resembles other dermatologic conditions such as erythema nodosum, lymphocytic infiltrate of Jessner, and reticular erythematous mucinosis. Diagnosis based on clinical features alone should be avoided, and ideally, treatment should only be initiated after confirmatory histopathological testing. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Erythema nodosum (EN) is a type of panniculitis occurring due to various conditions. It can be associated with certain malignancies or manifest as a side effect of drugs. This article presents a unique case of EN in a patient with chronic myeloid leukemia (CML‐blast phase) following dasatinib and chemotherapy. Timely recognition and appropriate management are crucial to alleviate symptoms and consider potential drug‐induced etiology. [ABSTRACT FROM AUTHOR]

Morbus Adamantiades–Behçet (MAB) is an inflammatory disease typically manifesting with oral and genital aphthosis, erythema nodosum, and vasculopathy, and in only around 2%, cardiac involvement. Its prevalence is usually higher along the historic Silk Road, but rarer in Scandinavia where 0.64–4.9 in 100,000 people are affected. We herein present two Swedish patients with cardiac manifestations of Morbus Adamantiades–Behçet. Along with the intracardial thrombi, which both patients presented with, one patient also had cerebrovascular insults leading to visual field deficits as well as involvement of peripheral nerves. Being of Scandinavian origin and showing uncommon symptoms as their initial manifestations of MAB, the 62- and 35-year-old patients presenting herein constitute rare cases. [ABSTRACT FROM AUTHOR]

Background: Increasing evidence has suggested that Corynebacterium kroppenstedtii is associated with some cases of granulomatous mastitis, mostly based on pathology or microbiology. We aimed to identify the clinical characteristics and treatment regimens for granulomatous mastitis with Corynebacterium kroppenstedtii infection. Understanding these clinical features is essential for patient care. Methods: We retrospectively collected data on 201 patients who were pathologically diagnosed with granulomatous mastitis and had microbiological results of either Corynebacterium kroppenstedtii or no bacterial growth and recorded and analysed their demographics, clinical characteristics, and clinical outcomes. Results: There were 107 patients in the CK group and 94 patients in the negative group. Sinus formation (x2 = 13.028, p = 0.000), time to complete remission at the first treatment period (Z = -3.027, p = 0.002), diameter of breast mass at first-time medical consultancy (Z = -2.539, p = 0.011) and recurrence (x2 = 4.953, p = 0.026) were statistically significant. Age (Z = -1.046, p = 0.295), laterality (x2 = 4.217, p = 0.121), time to presentation since the last delivery (x2 = 0.028, p = 0.868), BMI (Z = -0.947, p = 0.344), lactation time (Z = -1.378, p = 0.168), parity (x2 = 1.799, p = 0.180), gravida (Z = -0.144, p = 0.885), history of lactational mastitis or abscess (x2 = 0.115, p = 0.734), local trauma (x2 = 0.982, p = 0.322), hyperprolactinemia (x2 = 0.706, p = 0.401), erythema nodosum (x2 = 0.292, p = 0.589), and nipple discharge (x2 = 0.281, p = 0.596) did not demonstrate statistical significance. Regarding recurrence related to therapeutic strategy, except for surgery combined with immunosuppressants (x2 = 9.110, p = 0.003), which was statistically significant, none of the other treatment regimens reached statistical significance. The recurrence rate of patients in the CK group using rifampicin in their treatment course was 22.0% (x2 = 4.892, p = 0.027). Conclusions: Granulomatous mastitis accompanied by Corynebacterium kroppenstedtii more easily forms sinuses and has a higher recurrence rate. Both of the clinical characteristics may indicate that Corynebacterium kroppenstedtii plays an important role in the development and progression of granulomatous mastitis. Lipophilic antibiotics may be essential for granulomatous mastitis with Corynebacterium kroppenstedtii infection. [ABSTRACT FROM AUTHOR]

Background: Primary immunodeficiencies are immunological disorders caused by gene mutations involved in immune system development and activation. Recently, activated phosphoinositide 3-kinase delta syndrome (APDS) due to mutations in the phosphoinositide 3-kinase (PI3K), phosphatidylinositol-4, 5-bisphosphate 3-kinase, catalytic subunit delta gene (PIK3CD), and phosphoinositide 3-kinase regulatory subunit 1 (PIK3R1) genes have been reported to induce a combined immunodeficiency syndrome leading to senescent T cells, lymphadenopathy, and immunodeficiency. The exact diagnosis of these deficiencies is essential for treatment and prognosis. In recent years, targeted treatment with selective PI3Kd inhibitors has had a significant effect on controlling the symptoms of these patients. Case presentation: In this case report, we represent a 27-month-old girl with recurrent fever, an increased level of inflammatory markers, and erythema nodosum, who was referred to the rheumatology clinic. In the course of evaluations, because of the lack of clinical improvement with usual treatments, and a history of frequent respiratory infections, combined immunodeficiency was diagnosed in the immunological investigations. Moreover, whole-exome sequencing was performed for her. Conclusion: The genetic analysis found a novel variant of PIK3CD (c.1429 G > A) in the patient. Following daily antibiotic prophylaxis and monthly IV therapy, the patient's frequent infections and fevers were controlled. [ABSTRACT FROM AUTHOR]

Erythema nodosum (EN) is acute nodular septal panniculitis characterized by firm nodule or erythematous plaque which is pain on palpation and commonly localized on lower extremities. EN cases related to various vaccinations have been reported with the onset of <24 hours to 30 days of first or following dose. Some studies have reported EN case after COVID-19 vaccination in adult. We reported a case of 25 years old male presented with tender, erythematous nodules on the bilateral lower extremities that started one day after receiving second dose of COVID-19 vaccination. Based on patient history, physical examination, laboratory, and histopathologic investigation all pointed to EN related to vaccination. The patient was given nonsteroidal antiinflammatory agent and clinical improvement was noticed. [ABSTRACT FROM AUTHOR]

Tuberculids are a group of dermatoses with tuberculoid histology and the absence of tubercle bacilli. They are considered to be hypersensitivity reactions to circulating Mycobacterium tuberculosis (M. tb) or its antigens in individuals with good immunity. The objective of the review is to provide a detailed literature review of all available articles on tuberculids in the past 10 years and provide an update on epidemiology, etiopathogenetic mechanisms, clinical manifestations, and treatment. A search was performed on PubMed using the keywords lichen scrofulosorum, papulonecrotic tuberculid, erythema induratum, and erythema nodosum tuberculosis for all articles, with no restrictions on age, gender, or nationalities. An update on tuberculids, including some new concepts in pathogenesis, atypical presentations, new investigative modalities, and treatments are reviewed. [ABSTRACT FROM AUTHOR]

Introduction: Leprosy reactions (LRs) are inflammatory responses observed in 30%-50% of people with leprosy. First-line treatment is glucocorticoids (GCs), often administered at high doses with prolonged courses, resulting in high morbi-mortality. Methotrexate (MTX) is an immunomodulating agent used to treat inflammatory diseases and has an excellent safety profile and worldwide availability. In this study, we describe the efficacy, GCs-sparing effect and safety of MTX in LRs. Methods: We conducted a retrospective multicentric study in France consisting of leprosy patients receiving MTX for a reversal reaction (RR) and/or erythema nodosum leprosum (ENL) since 2016. The primary endpoint was the rate of good response (GR) defined as the complete disappearance of inflammatory cutaneous or neurological symptoms without recurrence during MTX treatment. The secondary endpoint was the GCs-sparing effect, safety and clinical relapse after MTX discontinuation. Results: Our study included 13 patients with LRs (8 men, 5 women): 6 had ENL and 7 had RR. All patients had had at least one previous course of GCs and 2 previous treatment lines before starting MTX. Overall, 8/13 (61.5%) patients had GR, allowing for GCs-sparing and even GCs withdrawal in 6/11 (54.5%). No severe adverse effects were observed. Relapse after MTX discontinuation was substantial (42%): the median relapse time was 5.5 months (range 3–14) after stopping treatment. Conclusion: MTX seems to be an effective alternative treatment in LRs, allowing for GCs-sparing with a good safety profile. Furthermore, early introduction during LRs may lead to a better therapeutic response. However, its efficacy seems to suggest prolonged therapy to prevent recurrence. Author summary: Leprosy reactions (LRs) are acute inflammatory episodes that complicate 30% to 50% of Mycobacterium leprae infections. Type 1 is known as reversal reaction (RR), and type 2 is known as erythema nodosum leprosum (ENL). The two types have relatively distinct pathogenesis and clinical features; furthermore, they can advance to become severe and progress to irreversible nerve damage and deformities. Beyond leprosy infection, LR can bring about neurological burden, poor quality of life, and risk of further descent into poverty for often young patients. Despite effective standardized treatment of leprosy infection with multidrug therapy (MDT) since 1981, treating LRs prove to be challenging. First-line treatment of LR is based on prolonged systemic glucocorticoids (GCs) therapy. The current challenge in leprosy is now to develop and evaluate new therapeutic alternatives to reduce not only the sequelae and morbidity of LR but also the lasting side effects of GCs. Thalidomide is an effective alternative in moderate to severe ENL, but its use is limited given its numerous adverse effects. Methotrexate (MTX) is an immunomodulating agent used to treat inflammatory diseases and has an excellent safety profile and worldwide availability. Here, we describe the efficacy, GCs-sparing effect, and safety of MTX in LR. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a chronic granulomatous disease of unknown etiology involving multiple organs of body. Cutaneous sarcoidosis varied in morphology with follicular, papular, nodular plaque, lupus pernio and erythema nodosum are the commonest forms. We herein present a case of a middle age male who presented with micropapular lesion on photo exposed areas which turn out to be cutaneous sarcoidosis on histopathology. There was no systemic involvement. Multidisciplinary and comprehensive management approach is required in such cases. Patients' clinical features improved considerably after treatment with topical and systemic corticosteroids. [ABSTRACT FROM AUTHOR]

Erythema Nodosum (EN) is the most common panniculitis, presented as acutely eruptive painful erythematous nodules on lower extremities symmetrically, with a wide spectrum etiology from infection to malignancy. The aim of this case report is to comprehend the diagnosis and the underlying treatable origin of EN. A 24-year-old female presented with painful red bumps on both legs a week after an episode of fever and sore throath. She also noted tenderness on both ankle joints. She was diagnosed with vasculitis by the internist and was treated with Methylprednisolone (MP) 16mg/day for 2 weeks. The symptomps were resolved but re-emmerged 1 week after the MP was stopped. Physical examination revealed erythematous nodules on both legs with tenderness on palpation. ASTO test was positive. Septal panniculitis and Miescher's granuloma was identified through histopathology examination. Exclusion of inflammatory subcutaneous differential diagnosis such as EN, nodular vasculitis (NV), and cutaneous polyarteritis nodosa (CPAN) require clinical and histopathologic consideration. Etiology identification of EN was done through a relevant supporting examination based on epidemiology, history, and physical examination. Poststreptococcal EN possibly related to molecular mimicry and immune complex deposition leading to reactive inflammation. Hereby we reported one case of post-streptococcal EN that was confirmed by laboratory examination and skin biopsy. The clinical response was good towards the etiologybased treatment. [ABSTRACT FROM AUTHOR]

Background: Behçet disease may be confused with sarcoidosis because they are both multisystem inflammatory disorders presenting with uveitis, polyarthritis, cardiovascular disease, erythema nodosum, and other cutaneous lesions. The coexistence of Behçet disease and sarcoidosis is extremely rare but not impossible. Vascular involvement is a significant pathological finding in Behçet disease, and arterial aneurysm formation is more common than occlusion. However, large-vessel vasculitis associated with sarcoidosis is extremely rare. Case Report: A 23-year-old man was admitted to the hospital because of swelling and pain in the right groin. The patient, who had predominant Behçet disease, was also diagnosed with sarcoidosis during the examination. He had inguinal distention associated with femoral artery aneurysm and underwent successful surgery without complication. Conclusion: Assessment of the coexistence of Behçet disease and sarcoidosis is critical because of their potentially fatal effects by triggering systemic inflammation. Furthermore, arterial diseases, especially those developing at young ages, can be a sign of immunoinflammatory disease. [ABSTRACT FROM AUTHOR]

Vitamin D deficiency is endemic in India. Hypovitaminosis D not only results in musculoskeletal disorders but also affects extraskeletal systems. In this article, we describe three interesting patients suffering from hypovitaminosis D with dermatologic manifestations of erythema nodosum, heel fissures, and palmar hyperhidrosis. All the three cases responded well to treatment with vitamin D. Similar cases have not been reported earlier. [ABSTRACT FROM AUTHOR]

Erythema nodosum (EN), the most common form of panniculitis, is a reactive inflammation of the subcutaneous fat clinically presented with a sudden onset of painful, erythematous, nodular, subcutaneous lesions, typically localized to the pretibial area. EN is commonly caused by numerous infections (especially beta‐haemolytic streptococcal infections), autoimmune diseases (sarcoidosis), inflammatory bowel conditions and drugs. EN induced by Covid‐19 vaccines is rarely reported. We describe an original clinical observation of a 75‐year‐old woman who presented with EN after receiving the second dose of BNT162b2, an mRNA vaccine. [ABSTRACT FROM AUTHOR]

Intraocular tuberculosis should always be deemed as a differential diagnosis due to its high importance, in addition to the high morbidity of systemic infection. This condition may be associated with extraocular manifestations that are not prevalent in tuberculosis. The author reported a case of ocular tuberculosis vasculitis associated with erythema nodosum and Poncet’s disease, with resolution of the medical findings and symptoms after the standard treatment for tuberculosis. [ABSTRACT FROM AUTHOR]

Background Only 0.2% of coccidioidomycosis (CM) diagnoses were made in patients (pts) with pneumonia (PNA) in urgent care (UC), because they were not being tested for CM. Our objective in this study was to improve CM testing rates. Methods This was a time series of clinician practice before and after an intervention that occurred at UC clinics in Phoenix and Tucson Arizona. All patients in UC were >18 years old. We included information about CM in periodic educational activities for clinicians. Coccidioidal serologic testing (CST), CST results, and their relation to International Classification of Diseases, Tenth Revision (ICD-10) codes were extracted from medical records. Results Urgent care received 2.1 million visits from 1.5 million patients. The CST orders per 104 visits increased from 5.5 to 19.8 (P <.0001). Percentage positive CSTs were highest for August, November, and December (17.0%) versus other months (10.6%). Positive CSTs were associated with PNA ICD-10 codes, and, independently, for Erythema nodosum (EN) which had the highest positivity rate (61.4%). Testing of PNA pts increased on first visits and on second visits when the first CST was negative. Yearly rates of PNA due to CM ranged from 17.3% to 26.0%. Despite this improvement, CST was still not done for over three quarters of pts with PNA. This was a noncomparative study. Conclusions Routine quality improvement activities have significantly but only partially improved rates of testing pts with PNA for CM in UC clinics located in a highly endemic area. Innovative strategies may be needed to improve current practice. Also in our region, EN, independent of PNA, is a strong predictor of CM. [ABSTRACT FROM AUTHOR]

Granulomatous lobular mastitis (GLM) is a benign inflammatory disease of the mammary gland with unknown etiology. Erythema nodosum (EN) is a rare, extramammary symptom of GLM. The purpose of this article was to investigate the clinical features of EN associated with GLM. We recruited 102 GLM patients diagnosed between December 2018 and December 2021 at Renmin Hospital of Wuhan University. The clinical characteristics and laboratory indices of the EN group (n = 12) and the non-EN group (n = 90) were compared. The proportion of GLM patients with lesions involving ≥2 quadrants and high peripheral white blood cell count, absolute neutrophil count and neutrophil-to-lymphocyte ratio (NLR) was larger in the EN group than in the non-EN group (p = 0.002, 0.025, 0.014, 0.002, respectively). The duration of EN was longer in GLM patients with EN than in GLM patients without EN (p = 0.005). GLM patients with EN had more abscesses and sinus tracts than those without EN (p = 0.003, 0.038). Lesions involving ≥2 quadrants and the NLR were positively associated with the occurrence of EN (R = 0.304, 0.0302, p = 0.002, 0.002). Receiver operating characteristic curve analysis revealed that the area under the curve of the NLR was 0.770. When NLR > 5.73, the sensitivity and specificity of predicting EN were 66.67% and 87.78%, respectively. Our findings suggest that GLM concomitant with EN suggests the presence of a more severe condition and extensive lesions. [ABSTRACT FROM AUTHOR]

Background Erythema Nodosum leprosum (ENL) is a type 2 hypersensitive reaction mostly affecting multibacillary leprosy types. Understanding the risk factors of ENL progression is necessary to prevent complications and further damage. Methods A case-control descriptive study on leprosy patients visiting the Dermatovenereology outpatient clinic of Dr. Moewardi Hospital, Surakarta, Indonesia from January 2016 to December 2020. The data taken from medical records of patients were sex, age, education, job, type of leprosy based on WHO or Ridley-Jopling, ENL onset, the length of being on multidrug therapy (MDT), and bacterial index. We analyzed the data statistically with the Chi-Square test and Logistic regression analysis, and the significance was determined with P <0.05. Results We obtained 98 subjects who were dominated by males (69.4%). Leprosy mostly occurred in subjects aged between 26 and 45 years old (41.8%). Most of our subjects were primary school graduates (47%). Farmers predominated our subjects. Bivariate analysis showed no significant relationship between sex as well as age and ENL (p=0.738 and p=0.244, respectively). ENL significantly related to WHO-based leprosy type, LL as well as BL type, onset of ENL, length of MDT consumption, and bacterial index (p=0.000,p=0.000,p=0.013,p=0.009, and p=0.014, respectively). Conclusion There was a significant correlation between the occurrence of ENL and WHO type of leprosy, Ridley-Jopling type of leprosy, the onset of ENL, duration of MDT, and Bacterial Index. The most significant relationship was between Ridley-Jopling type of leprosy and the occurrence of leprosy. [ABSTRACT FROM AUTHOR]

This 38‐year‐old man has a familial BRCA2 mutation. He presented with skin erythema, polyarthritis, dactylitis, and febrile erythema nodosum; a biopsy of a liver metastase revealed acinar cell carcinoma of the pancreas. After FOLFIRINOX, olaparib was initiated, and 24 months after, the patient was PS 0 and asymptomatic. [ABSTRACT FROM AUTHOR]

Sarcoidosis is an inflammatory and granulomatous disorder, developed due to dysregulation between immune response and certain environmental antigens. We hereby report an interesting case of sarcoidosis following COVID‐19 vaccination (COVIran Barekat), which presented with inflammation of previous tattoo sites as well as the development of erythema nodosum and systemic lymphadenopathy, suggested a possible link between the COVID vaccination and dysregulation of the inflammatory process and served as a reminder for clinicians to have enough vigilance before proposing a vaccine booster to these patients. [ABSTRACT FROM AUTHOR]

Background: Behçet's disease (BD) is a chronic, multi-systemic, recurrent condition that affects the vascular, ocular, mucocutaneous, and central nervous systems. The diagnosis of this disease depends on its clinical features, which are similar to those observed in several diseases, such as Parkinson's disease, pemphigus vulgaris, systemic lupus erythematosus, Crohn ҆s disease, and Sjӧgren's syndrome. Lysosome-mediated autophagy is a catabolic, cytoprotective mechanism that maintains cell homeostasis by degrading undesired long-lived proteins and recycling nutrients. The aim of this study was to evaluate the correlations between some autophagy-related genes (ATG5, ATG7, ATG12, LC3b, mTOR) and the pathogenesis and immunopathology of BD. The expression levels of the genes were evaluated by quantitative polymerase chain reaction (qPCR) in 101 individuals that are classified into two groups. Group 1: (n = 71) BD patients, Group 2: (n = 30) healthy controls. Results: Patients with BD had lower mRNA expression levels of ATG5 and mTOR and higher levels of LC3b mRNA than the controls. No significant differences in the levels of both ATG7 and ATG12 were observed between the two groups. According to the area under the curve analysis, LC3b was considered the best candidate biomarker among the selected markers for the diagnosis of BD. The mRNA expression of ATG5 was significantly correlated with patient age and the presence of oral ulcers. The mRNA expression of ATG7 was significantly associated with age and the presence of erythema nodosum and vascular lesions, whereas that of LC3b was significantly correlated with the presence of pustules. Conclusion: These findings indicated that elevated levels of LC3b were strongly associated with BD. Likewise, the levels of ATG5 and ATG7 were associated with the complications and outcomes of this disease. Additional assessments of the mRNA expression levels of these autophagy-related genes might prove beneficial in diagnosing this autoimmune disorder. [ABSTRACT FROM AUTHOR]

Background: Leprosy is a chronic granulomatous infection and is one of the neglected diseases caused by Mycobacterium leprae. ENL is a complex syndrome, that caused inflammation of the skin, nerves, and other organs due to an inflammatory immune response to Mycobacterium leprae antigens. Acute ENL was defined as the first episode of ENL with a duration of less than 24 weeks. Chronic ENL was defined as ENL that persisted for more than 24 weeks. These types of ENL can have different risk factors and require different therapeutic interventions. Purpose: The onset of ENL is acute, but may progress to a chronic or recurrent phase and require long-term therapy. Early detection of leprosy is very important, because the infection is curable and prompt treatment can reduce nerve damage and associated stigma. Chronic ENL patients require prolonged high doses of corticosteroids to control inflammation in ENL and cause severe complications and side effects associated with morbidity and mortality. Methods: This retrospective study was conducted using a non-probability sampling technique consecutively using a casecontrol formula in leprosy patients with ENL in the Leprosy Division of the Outpatient Dermatology and Venereology Unit RSUD Dr. Soetomo Surabaya for the period 2015 - 2020, using secondary data in the form of medical record data. Result: The results of this study obtained leprosy patients with ENL as many as 234 patients, 56 patients with acute ENL and 89 patients with chronic ENL. 45 patients with acute ENL and 45 patients with chronic ENL were obtained from the case-control minimal sample size formula. Conclusion: The results of the bivariate analysis test showed that there was a relationship between risk factors for coinfection and steroid therapy with the type of ENL. The presence of coinfection and steroid therapy showed that patients tend to be chronic ENL, conversely, in the absence of coinfection and without steroid therapy, patients tend to be acute ENL. The multivariate logistic regression analysis test showed a significant association between risk factors for ENL onset before MDT and the presence of coinfection with chronic ENL, whereas ENL onset after MDT and absence of coinfection were associated with acute ENL. [ABSTRACT FROM AUTHOR]

Rheumatoid arthritis is a systemic inflammatory disease that has many extra‐articular manifestations. Cardiovascular involvement, including coronary vasculitis and aortitis and skin lesion of erythema nodosum are uncommon findings of patients with rheumatoid arthritis, and thus, it is challenging for diagnosis of this case from those unusual extra‐articular presentation. [ABSTRACT FROM AUTHOR]

The coexisting of sarcoidosis and Sjögren's syndrome (SS) has long been neglected since sarcoidosis is considered as an exclusion criterion for SS. We described a 55-year-old woman, who was diagnosed with coexisting neurosarcoidosis and Sjögren's syndrome for 16 years. She presented with erythema nodosum, progressive sensory and motor impairment of the extremities, dry mouth, and dry eyes. High-resolution computed tomography (HRCT) of the chest showed symmetrical pulmonary micronodules, interstitial changes, and enlarged mediastinal lymph nodes. Spine magnetic resonance imaging (MRI) showed syringomyelia and thickening of the T3-9 spinal cord. She was with positive ANA and anti-SSA antibodies, impaired function of the lacrimal, salivary gland and renal tubules. Biopsy of skin and lung nodules revealed non-caseous granuloma. Salivary gland biopsy showed focal lymphocyte infiltration. Classification criteria for sarcoidosis and Sjogren's syndrome were fulfilled in this patient based on clinical and laboratory features. This case extends our understanding of overlapped Sjogren's syndrome with sarcoidosis and provides a referential value for clinical diagnosis. [ABSTRACT FROM AUTHOR]

This is a case of subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) was diagnosed by skin biopsy in a patient who presented with fever and erythema nodosum in the umbilicum following mRNA‐1273 COVID‐19 vaccination. COVID‐19 vaccines may cause SPTCL and skin biopsy may help in the diagnosis of erythema nodosum. [ABSTRACT FROM AUTHOR]

Acquired hypertrichosis can occur in local inflammation. Erythema nodosum (EN) is a hypersensitivity reaction to various underlying antigenic stimuli including Mycobacterium tuberculosis, which causes inflammation in the septa of subcutaneous fat. There were several case reports that describe the association of localized hypertrichosis (LH) with traumatic panniculitis and lupus panniculitis. To our knowledge, this is the first reported case of acquired LH associated with EN. Thus, EN can be added to the list of causes of localized hypertrichosis. [ABSTRACT FROM AUTHOR]

We report the case of a 69-year-old woman who underwent 18F-FDG PET/CT due to prolonged fever. One month before, the patient was diagnosed with COVID-19 infection. The 18F-FDG PET/CT showed several subcutaneous nodules with 18F-FDG uptake on the thorax and upper extremities and bilateral lung infiltrates due to organizing pneumonitis. Clinical examination revealed multiple tender nodules on thorax, arms, and legs, consistent with erythema nodosum (EN) induced by COVID-19 infection. The woman was treated with prednisone with a good effect on EN. To our knowledge, this is the first report on EN secondary to COVID-19 infection diagnosed on 18F-FDG PET/CT. [ABSTRACT FROM AUTHOR]

Background: The numbers of circulating regulatory T cells (Tregs) are increased in lepromatous leprosy (LL) but reduced in erythema nodosum leprosum (ENL), the inflammatory complication of LL. It is unclear whether the suppressive function of Tregs is intact in both these conditions. Methods: A longitudinal study recruited participants at ALERT Hospital, Ethiopia. Peripheral blood samples were obtained before and after 24 weeks of prednisolone treatment for ENL and multidrug therapy (MDT) for participants with LL. We evaluated the suppressive function of Tregs in the peripheral blood mononuclear cells (PBMCs) of participants with LL and ENL by analysis of TNFα, IFNγ and IL-10 responses to Mycobacterium leprae (M. leprae) stimulation before and after depletion of CD25+ cells. Results: 30 LL participants with ENL and 30 LL participants without ENL were recruited. The depletion of CD25+ cells from PBMCs was associated with enhanced TNFα and IFNγ responses to M. leprae stimulation before and after 24 weeks treatment of LL with MDT and of ENL with prednisolone. The addition of autologous CD25+ cells to CD25+ depleted PBMCs abolished these responses. In both non-reactional LL and ENL groups mitogen (PHA)-induced TNFα and IFNγ responses were not affected by depletion of CD25+ cells either before or after treatment. Depleting CD25+ cells did not affect the IL-10 response to M. leprae before and after 24 weeks of MDT in participants with LL. However, depletion of CD25+ cells was associated with an enhanced IL-10 response on stimulation with M. leprae in untreated participants with ENL and reduced IL-10 responses in treated individuals with ENL. The enhanced IL-10 in untreated ENL and the reduced IL-10 response in prednisolone treated individuals with ENL was abolished by addition of autologous CD25+ cells. Conclusion: The findings support the hypothesis that the impaired cell-mediated immune response in individuals with LL is M. leprae antigen specific and the unresponsiveness can be reversed by depleting CD25+ cells. Our results suggest that the suppressive function of Tregs in ENL is intact despite ENL being associated with reduced numbers of Tregs. The lack of difference in IL-10 response in control PBMCs and CD25+ depleted PBMCs in individuals with LL and the increased IL-10 response following the depletion of CD25+ cells in individuals with untreated ENL suggest that the mechanism of immune regulation by Tregs in leprosy appears independent of IL-10 or that other cells may be responsible for IL-10 production in leprosy. The present findings highlight mechanisms of T cell regulation in LL and ENL and provide insights into the control of peripheral immune tolerance, identifying Tregs as a potential therapeutic target. Author summary: Leprosy is complicated by episodes of inflammation called leprosy reactions. Leprosy reactions are important causes of nerve damage and illness. Erythema Nodosum Leprosum (ENL) also called type 2 reaction is a severe systemic immune-mediated complication of borderline and lepromatous leprosy. ENL causes high morbidity and thus requires immediate medical attention. We recruited 60 untreated patients with lepromatous leprosy (30 patients with ENL reactions and 30 patients without ENL reactions) in Ethiopia to better understand the loss of immune regulation in ENL. We took blood samples at 2 time points before and after prednisolone treatment and assessed if the regulatory T-cells in these patients are functionally competent to control inflammation. Previously we described that the proportion of Tregs are reduced in ENL. In the present study we found that despite the reduction in the proportion of Tregs, their functional integrity is intact and competent which confirms that ENL is associated with reduction of Tregs proportion but not with loss of their function. This is an important finding which suggests that future studies should focus on ways of increasing the proportion of Tregs in ENL to control the inflammation. [ABSTRACT FROM AUTHOR]

Leprosy is a chronic disease with clinical presentations according to the immunologic spectrum. Lepromatous form is the most advanced, with the highest transmissibility and risk of causing disabilities. Lucio’s phenomenon is a rare manifestation among lepromatous patients with a rapid and severe evolution and high mortality. It is difficult to differentiate from ulcerative/necrotic erythema nodosum leprosum and has no consensus on how it should be treated. This article is a qualitative review of the literature after the introduction of multidrug therapy, aiming to bring consensus related to the clinical, laboratory and histopathological diagnostic criteria of the disease and its management. [ABSTRACT FROM AUTHOR]

Introduction Erythema nodosum (EN) is a subcutaneous fat tissue inflammation (pannicullitis) characterized by diffuse erythematous nodules symmetrically distributed in the anterior part of the lower extremities. EN is considered a reactive process that can be triggered by a wide variety of factors including mycobacterium (M.) tuberculosis infection. Case A 21-year-old woman, presented in our department with a 9-month-history of painful erythematous nodules on both legs accompanied by ankle swelling and joint pain. Previous treatments with amoxicillin-clavulanic acid, steroid, and nonsteroidal anti-inflammatory drugs (NSAIDs) did not yield satisfactory results. On examination, we found multiple tender erythematous nodules symmetrically distributed on both legs. On laboratory examination, Creactive protein and erythrocyte sedimentation rate were increased, interferon-gamma release assay (IGRA) test was found positive for M. tuberculosis infection. The patient was treated with a standard anti-tuberculosis regimen. At 3-month follow-up, all the skin lesions had disappeared. The patient did not report any recurrence after 1 year of follow-up. [ABSTRACT FROM AUTHOR]

Background: Leprosy reactions, Type-1 and erythema nodosum leprosum, are immune-mediated complications of leprosy, which play a significant role in the morbidity associated with the disease. A considerable amount of literature has been published on the impact of leprosy in general but few studies focus specifically on leprosy reactions. This study aimed to investigate the impact of leprosy reactions on physical, psychological, and social aspects of the lives of people affected by analysing their life experiences and perspectives about leprosy reactions. Methods/Principal findings: This qualitative study involved people affected by leprosy reactions and their family members in two leprosy endemic countries. The data were collected through 66 interviews and 9 focus group discussions (4–6 participants each) in Surabaya, Indonesia, and Purulia, India. Content analysis and conversational analysis were performed. This study found that both types of leprosy reactions were perceived as an unpredictable and painful condition. Leprosy reactions restricted physical activities of the participants, such as going to bathroom, sleeping, eating, and cooking. In the interviews, the respondents expressed a range of emotions and feelings including confusion, sadness, anxiety, and anger. Some recounted that they felt stigmatized and lost opportunities to socialise and earn money. Differences between the two settings were identified. The majority of Indonesian participants preferred to stay at home, and some concealed the diagnosis of leprosy, while most of the Indian respondents continued working up to the time of hospitalization. Conclusion: Leprosy reactions are a distressing complication of leprosy and adversely affect the lives of those affected. Individuals reported physical discomfort, distress, anxiety, stigma, and financial hardship and these negative impacts in the physical, psychological, and social spheres reinforced each other. These findings provide important information about a need for early detection and sustained commitment to follow-up care for people with a history of leprosy reactions. More research on new drugs for reactional episodes, tools to measure knowledge, attitude, and practice, and costing study on leprosy reactions treatment are needed. We recommend the development and testing of holistic strategies to improve the management of leprosy reactions. Author summary: Leprosy reactions are immune-mediated complications of leprosy. There are two types of reaction. To provide a deep understanding about experiences and perceptions of people with both types of reactions, we interviewed the individuals affected and conducted group discussions with their family members in two leprosy endemic countries: Indonesia and India. The results of this study show that both types of reactions significantly affect the participants' lives. People with leprosy reactions in both countries experienced persistent pain which hampers their daily activities. A majority of participants reported that this makes them feel more often sad, that they isolate themselves, rely upon their family members and have less income. There is a need for more support for people with leprosy reactions and we recommend testing of different interventions including (peer) counselling. [ABSTRACT FROM AUTHOR]

Background: Granulomatous mastitis is a rare inflammatory disease of the breast, typically seen in woman of child-bearing age. No definitive etiology has been described. In rare instances, this condition has been reported to be associated with extramammary manifestations such as erythema nodosum and arthritis. We describe this rare condition in an adolescent female. Case presentation: A 16-year-old, Hispanic female presented with right-sided painful breast swelling, polyarthritis, and erythema nodosum on bilateral shins and lower thighs. Physical examination was negative for lymphadenopathy and pulmonary, gastrointestinal, and cardiovascular findings. Ophthalmologic examination for uveitis and serologic tests for autoimmune diseases were negative. Diagnosis of idiopathic granulomatous mastitis was made by exclusion of other etiologies and conditions. Confirmation was made by histopathologic examination demonstrating noncaseating granuloma within breast lobules with neutrophils and microabscess formation. After wide local excision and a short course of trimethoprim–sulfamethoxazole, our patient was placed on naproxen and prednisone, the latter being tapered off over 3 months, with steady and complete resolution of all symptoms. Conclusion: This is the first reported case of idiopathic granulomatous mastitis in a pediatric patient who also had extramammary manifestations, including erythema nodosum and polyarthritis. In this case-based review, we summarize the phenotype, risk factors, prognosis, and treatment options of this rare condition, chiefly to make the readers cognizant of such a diagnostic possibility in similar clinical presentation in the future. [ABSTRACT FROM AUTHOR]

OBJECTIVE: The objective of the study was to assess the efficacy and safety profiles of combined treatment of prednisolone with thalidomide (Gr-A) and prednisolone with clofazimine (Gr. B) in patients with erythema nodosum leprosum (ENL) or type 2 lepra reactions. MATERIALS AND METHODS: Efficacy of both regimens was assessed on the basis of clinical recovery of recurrent ENL measured by reaction severity score (RSS), Visual Analog Scale (VAS), and recurrence of type 2 lepra reaction. The causality assessment of adverse drug reactions was done using the WHO UMC causality assessment scale. RESULTS: The average age of patients with recurrent ENL was 42.8 years (male) and 51.8yrs (female) and had mean duration of leprosy and recurrent ENL 2.4 years and 2.09 years, respectively. 80% of nonrecurrence was observed in Gr-A versus 66% in Gr-B. Significant (P < 0.05) lower RSS and VAS was found in both the treatment groups as compared to pretreatment value. The reduction in RSS and VAS was statistically significant (P < 0.05) in Gr-A compared to Gr-B treatment. CONCLUSION: Thalidomide combination with steroid was found to be more efficacious than clofazimine combination with steroid in the treatment of ENL both the treatment regimens showed few tolerable side effects. Improved strategies for the treatment and management of these reactions need to be developed. [ABSTRACT FROM AUTHOR]

Summary: Background and aims: Extra‐intestinal manifestations (EIMs) are a common complication of inflammatory bowel diseases (IBD), affecting up to half of the patients. Despite their high prevalence, information on standardised definitions, diagnostic strategies, and treatment targets is limited. Methods: As a starting point for a national EIM study network, an interdisciplinary expert panel of 12 gastroenterologists, 4 rheumatologists, 3 ophthalmologists, 6 dermatologists, and 4 patient representatives was assembled. Modified Delphi consensus methodology was used. Fifty‐four candidate items were derived from the literature review and expert opinion focusing on five major EIMs (erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis) were rated in three voting rounds. Results: For use in a clinical practice setting and as part of the creation of a prospective registry of patients with EIMs, the panel developed definitions for erythema nodosum, pyoderma gangrenosum, uveitis, peripheral arthritis, and axial arthritis; identified the appropriate and optimal subspecialists to diagnose and manage each; provided methods to monitor disease course; offered guidance regarding monitoring intervals; and defined resolution and recurrence. Conclusions: Consensus criteria for appropriate and optimal means of diagnosing and monitoring five EIMs have been developed as a starting point to inform clinical practice and future trial design. Key findings include straightforward diagnostic criteria, guidance regarding who can appropriately and optimally diagnose each, and monitoring options that include patient and physician‐reported outcomes. These findings will be used in a national multicenter study network to optimise the management of EIMs. [ABSTRACT FROM AUTHOR]

During the process of revision of IAL Textbook of Leprosy (3 SP rd sp Edition) we had the occasion to look more carefully at the 10-item ENLIST-ENL severity scale for erythema nodosum leprosum (ENL) which is now being followed by many researchers in many countries (Available from: https://bit.ly/3uc1pYt) [[1]]. Pain: The "pain" at item No.1 probably means "pain in the skin or pain in the nodules of ENL". Moreover, the authors themselves while referring to a study on ENL from Nepal [[3]] where joint pain, nerve pain and muscle pain were itemized separately, had commented "it is not clear how these were defined nor whether these symptoms were solely attributable to ENL"?. [Extracted from the article]

Erythema nodosum (EN) is the most common type of septal panniculitis which causes inflammation of the subcutaneous fat, being the result of a hypersensitivity reaction to specific triggers. It usually presents with erythematous painful rounded lumps symmetrically on the anterior surface of the lower limbs. Rarely, it may occur in other areas such as thighs, neck and arms. This is a retrospective study describing a cohort of patients hospitalized in the University Hospital of Heraklion, Heraklion, Greece. The present research compares characteristics between patients with and without relapse and identifies independent factors associated with relapse. All patients with EN hospitalized during a 17-year period were included. Data regarding epidemiology, current or recent infections, symptoms, laboratory values and relapses were all recorded and evaluated. In total, 138 patients, of which 27 (19.6%) males, with a median age of 46.5 years, were evaluated. Clinical presentation involved multiple lesions in 115 (83.3%) patients, while 12 (8.7%) of them were febrile. Relapse was noted in 27 (19.6%) subjects. Multivariate logistic regression analysis showed that male gender was associated with a higher risk of relapse, while cases with multiple lesions were associated with a lower risk. [ABSTRACT FROM AUTHOR]

Granulomatous mastitis (GM) is a chronic inflammatory disease of the breast that usually occurs in women of reproductive age. However, GM during pregnancy is unusual and only one case of GM in the accessory breast has been reported so far. Here, we report an extremely rare case of GM in the accessory axillary breast of a pregnant woman. A 24-year-old pregnant woman had persistent pain and swelling in the right axilla that did not improve with antibiotic administration. Despite incision and drainage for subcutaneous abscess, the incised skin gradually became ulcerated, exposing the subcutaneous granulomatous tissue. Corynebacterium species were isolated in the bacterial culture of drained pus. Lower back pain, pain in several joints, and erythema nodosum on the lower legs appeared later. Based on the result of bacterial culture and the above disease course, the patient was clinically suspected of having GM. The axillary mass was surgically removed after childbirth, and the excised mass was histopathologically confirmed as GM. Treatment for GM should be considered individually and carefully in accordance with the patients' condition. Unnecessary surgery should be avoided. However, early addition of surgical interventions may yield good outcomes, especially for pregnant women because of limited treatment options. [ABSTRACT FROM AUTHOR]

Keywords: erythema; nodosum; panniculitis; ultrasound EN erythema nodosum panniculitis ultrasound 361 364 4 03/25/22 20220301 NES 220301 To the Editor, Erythema nodosum (EN) is an acute, septal panniculitis, presumably resulting from massive deposition of immune complexes in the venules of subcutaneous fat.1 It represents a stereotypical reaction pattern to a wide variety of triggering factors - such as infections, autoimmune diseases, drugs, and classically manifests with painful, rounded to oval, slightly raised, non-ulcerative erythematous nodules on the extensor aspect of the lower limbs.1 Although the execution of a biopsy may be spared in classic cases, due to its particularly painful nature and the intrinsic risk of sequelae, often obtaining histopathological confirmation is unavoidable. Herein, we present a five-patient case series, illustrating corresponding ultrasonographic and histopathological findings of EN and corroborating the role of US in the approach to EN. In conclusion, we reported five patients with EN and described the typical US features of septal panniculitis, that is, the presence of hypoechoic, not compressible septa, in the context of a hyperechoic hypodermis. [Extracted from the article]

Background: Erythema nodosum and erythema induratum of Bazin are similar inflammatory diseases of the lower extremities. These are clinically distinguishable entities, though overlap can occur. Both diseases are reported to be related to Mycobacterium tuberculosis infection, but it is very difficult to identify Mycobacterium tuberculosis in skin lesions. Aim: This study aimed to develop a new nested polymerase chain reaction targeting the IS6110 insertion sequence of M. tuberculosis to improve the M. tuberculosis detection rate in skin lesions of erythema nodosum or erythema induratum of Bazin. Methods: From May 2016 to Jan 2018, 14 patients with clinically suspicious erythema nodosum or erythema induratum were enrolled in the study. Two cases were classified as erythema nodosum and 12 as erythema induratum. Individual patients were subjected to a 4-mm punch biopsy, and their venous whole blood was sampled immediately after diagnosis. Results: Eight patients were tested for M. tuberculosis using QuantiFERON, of which seven (87.5%) were positive. IS6110-nested polymerase chain reaction on all 14 patients identified 11 (78.6%) positive cases. Four of the eight (50%) individuals tested with QuantiFERON were also positive in the IS6110 nested polymerase chain reaction. The difference between the outcomes of the QuantiFERON and the IS6110-nested polymerase chain reaction tests was not statistically significant. There was also no significant agreement between the results of both assays. Sequencing the IS6110-nested polymerase chain reaction products showed a 97%-100% nucleotide sequence identity with the H37Rv genome. Conclusion: It is important to test for tuberculosis in patients with multiple tender subcutaneous nodules on their lower extremities in high-burden tuberculosis countries like Korea. [ABSTRACT FROM AUTHOR]

Background: Necrobiosis lipoidica located to the breast; without evidence of glucose intolerance, is extremely rare, and its association to Crohn's disease is not usual. Case presentation: We report an interesting case of an association of necrobiosis lipoidica of the breast and Crohn's disease in a 54-year-old Moroccan woman. Skin necrobiotic changes are a characteristic feature in necrobiosis lipoidica, but they are exceptional in metastatic Crohn's disease, since there are only three published cases of necrobiotic skin lesions on the lower leg resembling erythema nodosum in metastatic Crohn's disease. Conclusions: On the basis of this rare observation, necrobiosis lipoidica without evidence of glucose intolerance should be recognized as a possible cutaneous manifestation or association of Crohn's disease. [ABSTRACT FROM AUTHOR]

Hemophagocytic lymphohistiocytosis (HLH) and erythema nodosum leprosum (ENL) result from a complex agent–host interaction and form a continuum of the same spectrum. A 30-year-old multi-gravida presented at 36 weeks gestation with fever and erythematous raised lesions over the face and upper and lower limbs after defaulting treatment for borderline lepromatous leprosy. Skin biopsy confirmed ENL, hence multi-drug therapy (MDT) and oral steroids were restarted. However, her condition worsened and she developed icterus, periorbital puffiness, pleural effusion, ascites and splenomegaly. Laboratory investigations showed pancytopenia, conjugated hyperbilirubinemia, transaminitis, elevated lactate dehydrogenase, hypertriglyceridemia, hyperferritinemia and hypofibrinogenemia. Dapsone was stopped on the suspicion of dapsone hypersensitivity but hyperbilirubinemia progressed. Diagnosis of HLH was clinched after bone marrow aspirate showed florid hemophagocytosis and subsequently, intravenous immunoglobulin (2 g/kg) over 5 days and dexamethasone were administered. The patient improved gradually with normalization of laboratory parameters and restarted MDT. This case depicts a rare and potentially catastrophic complication of ENL and emphasizes a vigil for HLH syndrome in such cases. [ABSTRACT FROM AUTHOR]

Purpose: Although it is worth to investigate seasonality in a disease that has a recurrent course, this issue has not been dealt with sufficiently for Behçet’s disease. The aim of the study was to evaluate whether or not frequencies of its mucocutaneous attacks varied between months of the year. Materials and Methods: One hundred eight patients with Behçet’s disease were retrospectively evaluated in terms of attacks of oral aphthae, genital ulcers, and erythema nodosum. For their each visit, a four-week period preceded this visit and dates of attack onsets in this period were taken into consideration. Then, by using them, mean monthly attack numbers were calculated for time series analysis. Results: Although mean monthly attack numbers were higher in May and October for oral aphthae and in August for genital ulcers, seasonalities were not verified by time series analysis. Conclusion: After a literature search on PubMed, there is only one study that detected no seasonality for mucocutaneous lesions of Behçet’s disease, and only one study detected a seasonality for oral aphthae. However, it is worth noting that time series analysis is not utilized in these studies. Since the present study is retrospective, its results should be verified by diary-based prospective studies that include time series analysis. [ABSTRACT FROM AUTHOR]

Background: Over the course of the pandemic, cutaneous manifestations of SARS-CoV-2 infections have been increasingly characterized, yet only a few cases of erythema nodosum (EN) are reported in the literature and international registries.Case Presentation: In this report, we describe a case of tender, erythematous nodules that appeared acutely on the distal legs in a 48-year-old female with renal transplant following SARSs-CoV-2 infection complicated by prolonged fevers and pneumonia. The patient was diagnosed with a classic presentation of EN arising from a new and emerging trigger-COVID-19 infection. The cutaneous lesions resolved with conservative management.Conclusions: This report highlights the importance of clinician awareness of the potential association of COVID-19 with a classic clinical presentation of EN and underscores that these cases can be managed with the same therapeutic repertoire as EN due to other aetiologies. Of note, use of systemic agents was not employed in this case, as our patient improved with conservative therapy alone. [ABSTRACT FROM AUTHOR]