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An otherwise healthy 4‐week‐old term female of Japanese heritage presented with a 1‐week history of asymptomatic progressive, generalized skin lesions. The lesion morphology, distribution, and dermatopathology result was consistent with Sweet syndrome. The patient was found to have a congenital type H rectovestibular fistula. This case highlights the rare association of rectovestibular fistula in neonatal Sweet syndrome which has only been described in neonates of Japanese heritage. [ABSTRACT FROM AUTHOR]

Histiocytoid Sweet syndrome (H‐SS) is a histopathological variant of Sweet syndrome (SS) defined by cutaneous infiltration of immature myeloid cells morphologically resembling histiocytes. The association of H‐SS with underlying malignancy, particularly myelodysplastic syndromes, is well‐established. Myelodysplasia cutis (MDS‐cutis) has been proposed to describe cases historically diagnosed as H‐SS but characterized by shared clonality of the myeloid infiltrate in skin and bone marrow. Therefore, identifying patients who might have MDS‐cutis is critical for the management of the associated hematologic malignancy. VEXAS syndrome, an adult‐onset autoinflammatory disease, should also be included in the histopathologic differential diagnosis of H‐SS, as it shares clinical and pathologic features with MDS‐cutis. Through the presentation of two cases, we aim to highlight the defining features and key clinical implications of MDS‐cutis and VEXAS syndrome. [ABSTRACT FROM AUTHOR]

Background: Sweet syndrome (acute febrile neutrophilic dermatosis) is an uncommon inflammatory disorder characterized by the abrupt appearance of painful, edematous, and erythematous papules, plaques, or nodules on the skin. There are various subtypes, such as classical, drug-induced, malignancy-associated, and the less common variant giant cellulitis-like Sweet syndrome. This case is unique due to its presentation of the giant cellulitis-like variant of Sweet syndrome in a patient from Ethiopia. The unusual distribution of the skin lesions and the initial lack of response to antibiotics make this case particularly noteworthy. It underscores the importance of considering Sweet syndrome in differential diagnoses when faced with atypical skin manifestations and ineffective antibiotic treatment. This contribution adds valuable insights to the scientific literature by highlighting the need for heightened awareness of this rare variant and improving diagnostic accuracy in similar clinical scenarios. Case presentation: A 60-year-old Ethiopian male patient who presented to the accident and emergency department with a 5-day history of fever, chills, sweating, and rigor accompanied by a reddish skin color change around the anterolateral region of the right chest wall. On physical examination, there were erythematous, indurated tender plaques with ill-defined borders over the right antero- and posterolateral chest wall with extension to the lateral part of the right neck and medial aspect of the right arm. Subsequently, the patient was started on antibiotics, but there was a suboptimal response. Skin biopsy revealed features suggestive of giant cellulitis-like Sweet syndrome. He was then started on steroids, which significantly improved his symptoms. Conclusion: A cautious stance is essential when identifying Sweet syndrome in individuals displaying erythematous plaque-like skin lesions in atypical areas of the body with uneven distribution. Such presentation may signal Sweet syndrome rather than a common infection. If conventional treatments, such as antibiotics, fail to resolve symptoms, consider Sweet syndrome as a potential diagnosis. This approach ensures timely and appropriate treatment, preventing treatment delay and misdiagnosis. [ABSTRACT FROM AUTHOR]

Abstract Background Sweet syndrome (acute febrile neutrophilic dermatosis) is an uncommon inflammatory disorder characterized by the abrupt appearance of painful, edematous, and erythematous papules, plaques, or nodules on the skin. There are various subtypes, such as classical, drug-induced, malignancy-associated, and the less common variant giant cellulitis-like Sweet syndrome. This case is unique due to its presentation of the giant cellulitis-like variant of Sweet syndrome in a patient from Ethiopia. The unusual distribution of the skin lesions and the initial lack of response to antibiotics make this case particularly noteworthy. It underscores the importance of considering Sweet syndrome in differential diagnoses when faced with atypical skin manifestations and ineffective antibiotic treatment. This contribution adds valuable insights to the scientific literature by highlighting the need for heightened awareness of this rare variant and improving diagnostic accuracy in similar clinical scenarios. Case presentation A 60-year-old Ethiopian male patient who presented to the accident and emergency department with a 5-day history of fever, chills, sweating, and rigor accompanied by a reddish skin color change around the anterolateral region of the right chest wall. On physical examination, there were erythematous, indurated tender plaques with ill-defined borders over the right antero- and posterolateral chest wall with extension to the lateral part of the right neck and medial aspect of the right arm. Subsequently, the patient was started on antibiotics, but there was a suboptimal response. Skin biopsy revealed features suggestive of giant cellulitis-like Sweet syndrome. He was then started on steroids, which significantly improved his symptoms. Conclusion A cautious stance is essential when identifying Sweet syndrome in individuals displaying erythematous plaque-like skin lesions in atypical areas of the body with uneven distribution. Such presentation may signal Sweet syndrome rather than a common infection. If conventional treatments, such as antibiotics, fail to resolve symptoms, consider Sweet syndrome as a potential diagnosis. This approach ensures timely and appropriate treatment, preventing treatment delay and misdiagnosis.

Abstract Neutrophilic dermatoses are autoinflammatory disorders characterised by sterile cutaneous inflammatory neutrophilic infiltrates. This group of diseases is rare in infants and children, where sepsis is the diagnosis of exclusion. This report focuses on the clinical presentation, investigation and management of infants with idiopathic pyoderma gangrenosum. It should allow for a better appreciation of the disease and a more patient‐focused approach to its diagnosis and management.

Background: Sweet syndrome (SS) is a rare dermatological adverse reaction caused by granulocyte colony-stimulating factor (G-CSF), but the characteristics of G-CSF-induced SS are unclear. This study aims to elucidate the characteristics of G-CSF-induced SS and offer guidance for its prevention and management. Methods: We collected relevant case reports of G-CSF-induced SS by searching pertinent databases until June 30, 2024, and synthesized the data for retrospective analysis. Results: A total of fifty patients were analyzed, with a median age of 44 years (1.7–77). The onset of SS occurred between 2 and 90 days post-administration, with a median onset time of 7 days. The predominant cutaneous manifestations included papules/plaques (74.0%), nodules (32.0%), and vesicles/bullae (24.0%). Fever presented in 74.0% of cases, while extra-cutaneous symptoms appeared in 32% of patients. Skin biopsy revealed key findings such as dermal diffuse neutrophil infiltration (97.8%), leukocytoclasis (19.1%), and dermal papillary edema (27.7%). Following both the cessation of G-CSF and systemic corticosteroids treatment, patients showed symptomatic improvement at a median interval of 7 days (2–70). Conclusion: Clinicians should remain vigilant for the risk of SS during G-CSF administration. Skin biopsy plays a crucial role in confirming SS diagnosis. G-CSF-induced SS exhibits a favorable response to corticosteroids, and re-administration of G-CSF should be avoided due to the risk of symptom recurrence. [ABSTRACT FROM AUTHOR]

Objective To explore the oral mucosal manifestations of Sweet’s syndrome and provide a reference for its early detection and correct diagnosis. Methods The oral mucosal manifestations of a 60-year-old female patient with Sweet’s syndrome are described in detail, followed by a discussion of the related literature. Results The patient had skin erythema of both lower extremities, which was accompanied by oral mucosal ulceration and pain for 3 days. The patient presented with mild cutaneous lesions and diffuse large-scale erosion in the oral mucosa with obvious pain. During the onset of the disease, the patient was accompanied by fever with a temperature of 38.5°C. After visiting the Department of Stomatology, laboratory tests showed an increase in C-reactive protein (35.2 mg/L) and an accelerated erythrocyte sedimentation rate (77.00 mm/h). Scattered red plaques and mild tenderness were observed in the knees and lower limbs. Histopathological examination of the skin lesions revealed scattered infiltration of immature neutrophils across the entire dermis. The patient responded well to glucocorticoid therapy. According to the clinical signs and laboratory examination, combined with the lesion histopathological results, a diagnosis of Sweet’s syndrome was given. The patient was administered 1 mL compound Betamethasone injection only once intramuscularly. In the meantime, the patient was asked to gargle with compound chlorhexidine solution and topically apply recombinant bovine basic fibroblast growth factor solution to the damaged mucosa three times a day for 1 week. After 4 days of medication, the patient’s body temperature had returned to normal and the oral lesions were significantly reduced. After 2 weeks, the erythema in the leg and knee had almost all subsided, and the oral mucosal lesions had disappeared. The patient was followed up 6 months after treatment, with no recurrence of skin lesions. After 2 years of follow-up, the disease was stable with no recurrence. A review of the relevant literature shows that Sweet’s syndrome is a rare inflammatory reactive dermatosis with unknown etiology, which can be divided into three clinical types: specific, tumor-related, and drug-induced. The male/female prevalence ratio is 1:4. The salient clinical manifestations are abrupt onset of painful erythematous plaques or nodules most commonly involving the extremities, often accompanied by pyrexia, elevated neutrophil count, elevation of the erythrocyte sedimentation rate, and positive C-reactive protein. The use of glucocorticoids is the most common treatment for this disease, and most patients see a rapid improvement in skin lesions; however, some may experience infection or recurrence after withdrawal. Some patients with Sweet’s syndrome are accompanied by oral lesions, but cases of oral mucosal damage have been rarely reported, and this condition is easily misdiagnosed. Conclusion Oral mucosal lesions may be extraterritorial manifestations of Sweet’s syndrome, and the patient’s systemic condition should be comprehensively considered. Skin biopsy should be completed as soon as possible to make a clear diagnosis, so as not to delay the disease.

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.

Copyright of Journal of Prevention & Treatment For Stomatological Diseases is the property of Journal of Prevention & Treatment For Stomatological Diseases Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The aim of this review is to increase obstetrician awareness of pregnancy‐associated Sweet syndrome. Patients present with fever, leukocytosis, and skin eruption, which can mimic other infectious or inflammatory conditions, but do not respond to antibiotics. A search using PubMed, EMBASE, and Web of Science Core Collection was conducted to review all reported cases of pregnancy‐associated Sweet syndrome, an acute febrile neutrophilic dermatosis occurring during pregnancy or postpartum. A total of 33 episodes among 30 patients were identified, with the majority (54.5% [18]) of cases occurring within the second trimester. Among the 30 patients, skin lesions most commonly affected the head and neck (73.3% [22]), with rare oral or ocular involvement. Leukocytosis was the most common laboratory finding, reported in 96.7% [29] of patients, with neutrophil predominance noted in 70.0% [21]. The diagnosis was confirmed for all patients with pathognomonic results of skin biopsies. Of the 27 cases detailing treatment, systemic corticosteroids were most frequently used (19 cases), followed by conservative management (seven cases), and dapsone (one case). The dapsone‐treated patient and 15 of the 19 steroid‐treated patients experienced resolution, but additional management strategies were required in the remaining four individuals. Spontaneous resolution occurred during pregnancy in six of the seven conservatively managed individuals, with one patient experiencing spontaneous abortion shortly after skin eruption at 10 weeks of gestation. No associated maternal deaths were reported. Obstetric complications of pregnancy‐associated Sweet syndrome included endomyometritis, sterile placental abscesses, and abdominal wall necrosis. Delivery of healthy infants occurred in 24 of the 25 cases that presented fetal outcome, which included two infants who underwent medically indicated preterm deliveries. Synopsis: There are 30 documented cases of pregnancy‐associated Sweet syndrome. One early miscarriage, delayed diagnosis, and extracutaneous neutrophil infiltration contributing to maternal morbidity have been reported. [ABSTRACT FROM AUTHOR]

Copyright of Chinese Journal of Dermatovenereology is the property of Xi'an Jiaotong University Periodicals Center and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Sweet syndrome is an acute febrile neutrophilic dermatosis characterized by the infiltration of neutrophils into the skin. It may occur idiopathically or be linked to malignancies, inflammatory or autoimmune diseases. Leukocyte adhesion deficiency type I (LAD-I) is an inborn error immunity wherein leukocytes lack adhesion molecules necessary for migration to infection sites due to mutations in the CD18 gene encoding β2 integrins. We present a case of a 16-month-old female initially diagnosed and treated for Sweet syndrome based on histopathological findings with recurrent flare episodes. Subsequent workup revealed LAD-I, making this case the first documented association between Sweet syndrome and LAD-I. Moreover, we reviewed the pertinent literatures detailing the concurrence of neutrophilic dermatosis and immunodeficiency disorders. This case underscores the significance of comprehensive evaluation for Sweet syndrome patients who are refractory to conventional treatments. [ABSTRACT FROM AUTHOR]

Introduction: Sweet syndrome (SS) is well known to be associated with underlying hematologic malignancies. The incidence and qualities of SS among novel targeted therapies for acute myeloid leukemia (AML) have not yet been described. Methods: Through retrospective review of 19,432 patients diagnosed with acute/chronic leukemia or myelodysplastic syndromes/myeloproliferative neoplasms (MDS+/−MPN) over 28 years, we calculated the incidence of SS in the setting of select hematologic malignancies and described the clinicopathologic characteristics of SS in patients with onset of SS after initiation of novel AML-targeted therapies. Results: Overall incidence of SS was 0.36% (95% CI: 0.27–0.45%), which was significantly higher among patients with AML (50/5,248, 0.94%; 95% CI: 0.71–1.25%). Nine AML patients were on 4 classes of novel targeted treatments – IDH1/2 inhibitor alone, FLT3 inhibitor, IDH2 and DOT1L inhibitor, and anti-CD33 therapy. In therapies inducing myeloid blast differentiation, SS occurred at later onset following treatment. Conclusions: In AML patients with fever and unusual skin lesions, physicians may consider SS earlier, which may shorten time to diagnosis. Future assessments of SS among patients treated with novel therapies for AML and molecular studies of biopsies may help further explain this dermatologic adverse event with earlier diagnosis and management of neutrophilic dermatoses in these patients. [ABSTRACT FROM AUTHOR]

This article explores a rare skin condition called cryptococcoid Sweet syndrome, which can be triggered by infection, cancer, or certain medications. The case discussed involves a patient who had previously used hydralazine and had a history of cocaine use. The article emphasizes the connection between hydralazine use and autoimmune disorders like Sweet syndrome. It also stresses the importance of distinguishing between true cryptococcosis and cryptococcoid Sweet syndrome, and calls for more research on this condition and its potential causes. [Extracted from the article]

VEXAS syndrome is a rare entity secondary to UBA1 gene mutations, located on the X chromosome. This mutation generates, as a consequence, a characteristic vacuolation on haematopoietic stem-cells. It is characterized by multiple autoinflammatory and haematologic manifestations, which respond and end up being dependent on corticosteroid treatment. In this publication we present a 2-case series diagnosed at our hospital and make a brief literature review of the published evidence so far. [ABSTRACT FROM AUTHOR]

Cryptococcoid Sweet syndrome (cSS) is a recently described clinical and histological variant of Sweet syndrome (SS). Its cutaneous presentation is similar to the classical form of SS but it includes atypical findings, such as capsular and yeast-like structures on microscopy that are reminiscent of Cryptococcus species. However, in cSS, fungal staining and cultural examination are negative, whereas myeloperoxidase (MPO) staining on biopsy specimens is typically positive. Due to the rarity and the diagnostic challenge represented by this disease, its extracutaneous involvement, and the latency in its diagnosis, this condition is frequently associated with poor prognosis. In this study, we report the case of a cSS patient with a positive outcome.

Neutrophilic dermatosis, or Sweet syndrome, is a cutaneous disorder caused by neutrophilic infiltration in the upper dermis. It has been associated with medications, infections and malignancies but to date it has not been associated with femoral arterial angioplasty or stenting. We present the case of a 75-year-old female who, after angioplasty and stent placement of the right superficial femoral artery, developed right heel pain with ulceration that did not respond to broad antibiotics. She underwent incision and drainage twice without improvement; both times produced negative cultures. She then underwent a punch biopsy by dermatology, which was consistent with acute spongiotic and other neutrophilic dermatoses. She was started on prednisone with immediate improvement of her symptoms. She was discharged to a rehabilitation centre with a prednisone taper and antibiotics. This report highlights the importance of maintaining Sweet syndrome on the differential for cellulitis as it is a rare mimicry of other infectious and non-infectious aetiologies, which are common in the perioperative space. Early treatment is crucial to improve symptoms, outcomes, healthcare cost and potentially the length of stay.

ABSTRACTBackground: Acute febrile neutrophilic dermatosis, also commonly referred to as Sweet syndrome, is often associated with tumors, infections, immune disorders and medications. FLT3 inhibitor-induced Sweet syndrome is a rare complication.Methods and results: We report a patient with relapsed and refractory acute monocytic leukemia harboring high-frequency FLT3-ITD and DNMT3a mutations. The FLT3 inhibitor gilteritinib was administered for reinduction therapy after failure of chemotherapy with a combination of venetoclax, decitabine, aclarubicin, cytarabine and granulocyte colony-stimulating factor. The leukemia patient achieved remission after 1 month of treatment. However, Sweet syndrome induced by gilteritinib, which was confirmed by skin biopsy, developed during induction therapy. Similar cases of Sweet syndrome following FLT3 inhibitor therapy for acute myeloid leukemia were reviewed.Conclusion: Attention should be given to this rare complication when FLT3 inhibitors are used for acute myeloid leukemia therapy, and appropriate treatments need to be administered in a timely manner.

Neutrophilic dermatosis of the dorsal hands (NDDH) is a variant of Sweet syndrome that presents with erythematous bullae, papules/plaques, or pustules on the dorsal hands. It is most commonly associated with hematologic and solid organ malignancies, though cases of NDDH associated with inflammatory bowel disease, rheumatologic disorders, and medication exposure have also been described in the literature. Felty syndrome is a rare complication of long-standing rheumatoid arthritis characterized by neuropathy, splenomegaly, and neutropenia. Granulocyte colony stimulating factors (e.g., filgrastim) can be utilized to rescue the neutropenia observed in Felty syndrome, but this treatment may subsequently cause Sweet syndrome. Herein, we present a 64-year-old man with Felty syndrome and a complex medical history who presented with sudden onset, painful blisters located on the dorsal and palmar aspects of his bilateral hands. Given the patient's past medical history, a broad differential diagnosis, including disseminated fungal and viral infection was initially considered. A punch biopsy of the skin lesion disclosed neutrophilic dermatosis, which together with laboratory data satisfied the von den Driesch criteria for Sweet syndrome. As the lesions were localized exclusively on the patient's hands, the qualification of NDDH was also endorsed.

VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is an inflammatory disorder with hematological and systemic features. A recent study demonstrated that the dermal infiltrate in neutrophilic dermatosis from VEXAS patients is derived from the pathological UBA1-mutated myeloid clone. Neutrophilic dermatosis is, however, only one of the various skin involvements observed in VEXAS syndrome. We analyzed 10 formalin-fixed paraffin-embedded skin biopsies from genetically confirmed VEXAS syndrome. UBA1 mutation was found in the biopsies related to neutrophilic dermatitis but in none of the other histological patterns (leukocytoclastic vasculitis and septal panniculitis). This could lead to a distinction between clonal and paraclonal cutaneous involvements in VEXAS syndrome, which could in turn improve therapeutic outcomes.

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare disorder typically characterized by the clinical triad including a sudden onset of fever, painful skin lesions, and neutrophilia. The histopathological findings are a dense neutrophilic infiltrate and oedema of the dermis and epidermis without evidence of a vasculitis. Besides treatment of the underlying cause, sweet syndrome is typically treated with high‐dose corticosteroids leading to a relapse‐free response in 70% of patients. However, if left unrecognized or untreated, the condition may lead to serious complications. Here, we report on the case of a 38‐year‐old patient in whom, under the assumption of the presence of necrotizing fasciitis, exarticulation of the right arm was performed. In the absence of pathogen detection and insufficient response to anti‐infective therapies, the diagnosis of a sweet syndrome was assumed and, later, confirmed by an excellent response to high‐dose administration of systematic glucocorticoids. The case emphasizes the need to be aware of this rare syndrome, which can be easily misdiagnosed due to its close resemblance to infection and stresses the need of further research to define distinct diagnostic tools. [ABSTRACT FROM AUTHOR]

Background: A dysregulated immune response has been implicated in Sweet syndrome (SS) pathogenesis; however, cytokine profiles across different conditions associated with SS -- including adult-onset immunodeficiency (AOID) due to anti-interferon (IFN)-γ autoantibodies -- remain unknown. Objective: To investigate alterations in inflammatory cytokines in skin lesions of distinct subtypes of SS. Methods: Skin biopsies were collected from 42 AOID- and 52 non-AOID-associated SS patients and 18 healthy controls. The comparative immunohistochemical study was conducted using monoclonal antibodies against interleukin (IL)-1β, IL-6, IL-17, IFN-γ, and tumor necrosis factor-α on paraffin-embedded sections. The quantitative percentage positivity and intensity were calculated using computer-based image analysis. Results: The results showed stronger and more diffuse dermal immunoreactivity for IFN-γ and IL-17 in the AOID-associated (p < 0.001 and p < 0.001, respectively) and non-AOID-associated SS (p < 0.001 and p < 0.001, respectively) groups. However, no significant differences in the levels of these two cytokines were observed between the AOID- and non-AOID-associated SS groups. Increased expression of IFN-γ together with IL-17 was also noted in almost all subtypes among non-AOID-associated SS. Conclusions: These results demonstrate that IFN-γ and IL-17 are implicated in immunopathology of all SS subtypes, including AOID-associated SS, despite the presence of anti-IFN-γ autoantibodies. [ABSTRACT FROM AUTHOR]

Key clinical message: A suspicious malignant lung nodule with cutaneous reaction is not always cancer, especially in low risk for malignancy patients. A lung biopsy should be taken into consideration. The associated cause of Sweet's syndrome directs the treatment in each patient. [ABSTRACT FROM AUTHOR]

Mevalonate kinase deficiency is a group of rare metabolic autoinflammatory disorders that present with recurrent fevers, abdominal pain, arthralgias, adenopathy, and a variety of cutaneous manifestations. The skin findings may mimic cellulitis, erythema elevatum diutinum, IgA vasculitis, and Sweet syndrome, and there is often a morbilliform or urticarial rash and aphthous stomatitis. Mevalonate kinase deficiency is one of the identified monogenic variants that can cause very early onset inflammatory bowel disease (IBD). We present a rare case of a patient with mevalonate kinase deficiency, neonatal Sweet syndrome, and infantile‐onset IBD, who has been successfully treated with canakinumab therapy. [ABSTRACT FROM AUTHOR]

Purpose: Two cases are described of necrotizing Sweet syndrome (nSS), a rare variant of acute febrile neutrophilic dermatosis that mimics necrotizing soft tissue infections. Observation: A 74-year-old female with myelodysplastic syndrome (MDS) presented with isolated periorbital nSS that closely mimicked necrotizing fasciitis (NF); she displayed pathergy to debridement, was exquisitely responsive to corticosteroids, and underwent successful first-stage reconstruction of the eyelid with full-thickness skin grafting. A second 40-year-old female patient with relapsed acute myelogenous leukemia (AML) presented with multifocal nSS most prominently involving the eyelid. Positive herpes zoster virus (HSV) PCR and bacterial superinfection complicated the diagnosis. She improved with chemotherapy for AML and corticosteroid therapy. Conclusion: nSS is rare and a high level of clinical suspicion as well as an understanding of its distinguishing features is necessary to avoid undue morbidity. Identification of pathergy, histopathology, microbiology, and clinical context are critical to avoid misdiagnosis of infection.

A significant threat to patients’ well-being, mental health, capacity to function, and social participation- a measure of disability- is posed by skin diseases, resulting in significant mortality and morbidity worldwide. Despite the fact that the majority of rare diseases are complex, disabling, and life-threatening, little knowledge has been gained in this area. The diagnosis and classification of these rare skin syndromes with pre-determined sets of symptoms present a challenge. To diagnose a rare skin syndrome, one frequently has to correlate histologic findings with clinical symptoms, as there is a vast range of skin disorders, many of whose histologic traits overlap with just slight variances. However, histologic knowledge alone makes it difficult to diagnose these; proper diagnosis demands appropriate clinical knowledge. In the present study institute, 675 skin biopsies were performed over the course of five years, from 2018 to 2022. Based on clinico-pathological analysis, various rare skin syndromes were diagnosed, as depicted in present case series of five cases (10 years old boy, 40 years old male, 27 years old male, 44 years old male, 52 years old male patients) for: a) Griscelli Syndrome; b) Gougerot-Carteaud Syndrome, considered rare as it is one of the underdiagnosed and misdiagnosed syndromes due to its similarity with Pityriasis versicolour; c) Kyrle’s disease, a rare perforating dermatosis occurring in 10% of chronic renal failure patients who are on dialysis; d) Nekam’s disease; e) Sweet syndrome, an uncommon syndrome occurring in about 10-20% of malignancies.

A significant threat to patients' well-being, mental health, capacity to function, and social participation- a measure of disability- is posed by skin diseases, resulting in significant mortality and morbidity worldwide. Despite the fact that the majority of rare diseases are complex, disabling, and life-threatening, little knowledge has been gained in this area. The diagnosis and classification of these rare skin syndromes with pre-determined sets of symptoms present a challenge. To diagnose a rare skin syndrome, one frequently has to correlate histologic findings with clinical symptoms, as there is a vast range of skin disorders, many of whose histologic traits overlap with just slight variances. However, histologic knowledge alone makes it difficult to diagnose these; proper diagnosis demands appropriate clinical knowledge. In the present study institute, 675 skin biopsies were performed over the course of five years, from 2018 to 2022. Based on clinico-pathological analysis, various rare skin syndromes were diagnosed, as depicted in present case series of five cases (10 years old boy, 40 years old male, 27 years old male, 44 years old male, 52 years old male patients) for: a) Griscelli Syndrome; b) Gougerot-Carteaud Syndrome, considered rare as it is one of the underdiagnosed and misdiagnosed syndromes due to its similarity with Pityriasis versicolour; c) Kyrle's disease, a rare perforating dermatosis occurring in 10% of chronic renal failure patients who are on dialysis; d) Nekam's disease; e) Sweet syndrome, an uncommon syndrome occurring in about 10-20% of malignancies. [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]

Sweet syndrome (SS) is an acute febrile neutrophilic dermatosis. Many factors such as inflammatory and autoimmune diseases have been reported in its etiology. In this article, SS, which developed in a pediatric patient with ulcerative colitis, was presented and discussed in the light of the literature. SS can be seen both due to ulcerative colitis and azathioprine (AZT) treatment. Our case presented to the emergency department with high fever and erythematous plaques. Laboratory examination revealed leukocytosis, neutrophilia, high levels of both of erythrocyte sedimentation rate and C-reactive protein. Histopathological examination of biopsies taken from skin lesions revealed neutrophilic dermatosis consistent with SS. AZT treatment was discontinued in order to exclude the diagnosis of AZT-associated SS, however drug-related SS was excluded due to the absence of relapses after oral challenge with AZT. With systemic glucocorticoid therapy, the patient's fever and rash completely resolved and did not recur in the follow-up. Patients with acute onset erythematous plaques or nodules and high fever who have been diagnosed with SS based on histopathological analysis and clinical findings should be started on systemic glucocorticoids as soon as possible. [ABSTRACT FROM AUTHOR]

El síndrome de VEXAS (Vacuolas, enzima E1, ligado al X, Autoinflamatorio, Somático) es un síndrome autoinflamatorio de inicio en la edad adulta que se caracteriza por mutaciones somáticas en el gen UBA1 y se considera el prototipo de enfermedad hematoinflamatoria. Los pacientes con síndrome de VEXAS exhiben manifestaciones inflamatorias y hematológicas que pueden conducir a diagnósticos clínicos como policondritis recidivante, poliarteritis nodosa, síndrome de Sweet y síndrome mielodisplásico. El diagnóstico requiere la evaluación de la médula ósea en búsqueda de vacuolas citoplásmicas en precursores mieloides y eritroides. Sin embargo, la confirmación genética de las mutaciones en UBA1 es necesaria. El tratamiento es un desafío y a menudo incluye glucocorticoides e inmunosupresores, con respuestas variables. Las terapias hipometilantes y el trasplante alogénico de células progenitoras hematopoyéticas se consideran terapias prometedoras. El pronóstico es influido por factores genéticos y clínicos. El objetivo de esta revisión es proporcionar una visión general sobre la patogénesis, la presentación clínica, el tratamiento y el pronóstico del síndrome de VEXAS para la comunidad médica latinoamericana. VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is an adult-onset autoinflammatory syndrome characterized by somatic mutations in the UBA1 gene and is considered the prototype of hematoinflammatory diseases. Patients with VEXAS syndrome exhibit inflammatory and hematological manifestations that can lead to clinical diagnoses such as relapsing polychondritis, polyarteritis nodosa, Sweet syndrome, and myelodysplastic syndrome. Diagnosis requires bone marrow evaluation to identify cytoplasmic vacuoles in myeloid and erythroid precursors. However, genetic confirmation of mutations in UBA1 is necessary. Treatment is challenging and often involves glucocorticoids and immunosuppressants with variable responses. Hypomethylating agents and allogenic haemopoietic stem cell transplant are considered promising therapies. Prognosis is influenced by genetic and clinical factors. The aim of this review is to provide an overview of the pathogenesis, clinical presentation, treatment, and prognosis of VEXAS syndrome for the Latin American medical community. [ABSTRACT FROM AUTHOR]

Sweet syndrome (SS) is a neutrophilic dermatosis that may be associated with malignancy, especially hematologic malignancy. It may be a warning sign of malignancy or its recurrence. SS is typically a dermal process. Rare variants of SS are infrequently reported and the diagnosis is often delayed. Here, we describe three cases of unusual and atypical presentation of SS occurring in acute myeloid leukemia and myelodysplastic syndromes. Accurate diagnosis, would facilitate early detection of underlying malignancy and adequate therapy. [ABSTRACT FROM AUTHOR]

Sweet syndrome (SS) is a rare neutrophilic dermatosis. It is characterized by acute onset of fever and painful erythematous papules, plaques and nodule on skin. SS may affect other organs. Pemphigus Vulgaris is an immune mediated condition which is characterized by the flaccid blisters affecting mainly trunk and mucosae with painful erosions. A 59 years old male, diagnosed case of Diabetes Mellitus and Pemphigus vulgaris, presented to our outpatient department with fever and multiple painful erythematous plaques, nodules and ulcers on both lower limbs. He was in remission phase of pemphigus vulgaris with immunosuppressants namely steroids and azathioprine. Patient fulfilled diagnostic criteria of Sweet syndrome. During his stay in the department of dermatology, he developed neurological manifestations in the form of weakness of the left proximal upper limb with altered sensorium. Cerebrovascular infarct was confirmed on computed tomography (CT) scan. Cerebrovascular infarct is a very rare manifestation of neuro-Sweet disease. SS is associated with many conditions. It is reported in various autoimmune conditions. But as far as pemphigus vulgaris is concerned SS is reported very rarely with pemphigus vulgaris. [ABSTRACT FROM AUTHOR]

CD30+ cells are typically part of lymphoproliferative disorders but can also be seen in inflammatory dermatoses. We present a case of 47‐year‐old man with a history of B‐lymphoblastic leukemia (B‐ALL) who presented with fever, leukocytosis, and papulonodular skin lesions, involving the extremities and trunk. A punch biopsy specimen demonstrated papillary dermal edema with a neutrophilic and histiocytic infiltrate extending into the subcutis. The infiltrate also harbored scattered large cells that were positive for CD30 and demonstrated the immunohistochemical profile of monocytes. A diagnosis of histiocytoid Sweet syndrome with CD30+ cells was made. The case is unique, demonstrating a combination of Sweet syndrome variants with subcutis involvement, histiocytoid morphology, and large CD30+ cells. A prior history of B‐ALL and immunohistochemical profile of monocytes with immature morphology broadened the differential diagnosis and added to the diagnostic challenge. [ABSTRACT FROM AUTHOR]

In 2020, Beck et al 1 described a novel adult autoinflammatory syndrome entitled VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic), a newly-discovered disorder that connected previously unrelated inflammatory syndromes and a prototype for a new class of hematoinflammatory diseases. 2 Eighty-nine percent of published cases have documented skin involvement, but despite the high incidence and diagnostic accessibility of skin manifestations, there has been little focus on the dermatological features of VEXAS syndrome thus far. A PubMed search of all published case reports of VEXAS syndrome to date was performed, with inclusion of all cases confirmed by genetic sequencing, and this review summarizes the reported dermatological signs. There have already been 141 confirmed published cases since original publication, 126 of which had documented cutaneous signs. 1-34 A wide range of skin presentations are reported, including Sweet-like urticated and tender erythematous nodules, cartilaginous involvement with chondritis, cutaneous vasculitis, and periorbital angiodema. 1-34 Many patients had been diagnosed with Sweet syndrome, relapsing polychondritis, polyarteritis nodosa, or erythema nodosum. 1-34 Hallmarks of skin histopathology are a neutrophilic dermatosis with coexisting or exclusive leukocytoclastic vasculitis. 1 The new classification therefore helps link previously disparate inflammatory skin conditions into a unifying pathophysiological pathway. [ABSTRACT FROM AUTHOR]

Key clinical message A suspicious malignant lung nodule with cutaneous reaction is not always cancer, especially in low risk for malignancy patients. A lung biopsy should be taken into consideration. The associated cause of Sweet's syndrome directs the treatment in each patient.

BackgroundA dysregulated immune response has been implicated in Sweet syndrome (SS) pathogenesis; however, cytokine profiles across different conditions associated with SS — including adult-onset immunodeficiency (AOID) due to anti-interferon (IFN)-γ autoantibodies — remain unknown.ObjectiveTo investigate alterations in inflammatory cytokines in skin lesions of distinct subtypes of SS.MethodsSkin biopsies were collected from 42 AOID- and 52 non-AOID-associated SS patients and 18 healthy controls. The comparative immunohistochemical study was conducted using monoclonal antibodies against interleukin (IL)-1β, IL-6, IL-17, IFN-γ, and tumor necrosis factor-α on paraffin-embedded sections. The quantitative percentage positivity and intensity were calculated using computer-based image analysis.ResultsThe results showed stronger and more diffuse dermal immunoreactivity for IFN-γ and IL-17 in the AOID-associated (p < 0.001 and p < 0.001, respectively) and non-AOID-associated SS (p < 0.001 and p < 0.001, respectively) groups. However, no significant differences in the levels of these two cytokines were observed between the AOID- and non-AOID-associated SS groups. Increased expression of IFN-γ together with IL-17 was also noted in almost all subtypes among non-AOID-associated SS.ConclusionsThese results demonstrate that IFN-γ and IL-17 are implicated in immunopathology of all SS subtypes, including AOID-associated SS, despite the presence of anti-IFN-γ autoantibodies.

Sweet syndrome, also called acute febrile neutrophilic dermatosis, is a rare disorder characterized by skin lesions accompanied by high fever and elevated inflammatory markers.

Abstract Background Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. Histiocytoid Sweet syndrome (H-SS) is a histopathologic variant of SS. While SS most commonly occurs in adults, this case report discusses an infant patient who presented with H-SS. Case presentation Through a multidisciplinary approach, this patient was also found to have very early onset inflammatory bowel disease (VEO-IBD) and Mevalonate kinase-associated disease (MKAD). While prior case studies have characterized an association between VEO-IBD and MKAD, there is no literature describing the association of all three diagnoses this case: H-SS, VEO-IBD and MKAD. Initiation of canakinumab in this patient resulted in successful control of the disease. Conclusions This case highlights the importance of a multidisciplinary approach to rare diagnoses, and collaboration during cases with significant diagnostic uncertainty.

Sweet syndrome is a neutrophilic dermatosis associated with multiple disorders. This retrospective case-series study of patients with Sweet syndrome in a tertiary hospital in Spain from 2001 to 2021, explores clinicopathological characteristics of Sweet syndrome and variables associated with malignancy, presence of autoinflammatory disorders and differences between histological subtypes. A total of 93 patients were identified: 30% idiopathic, 34% malignancy-associated, 29% reactive to infections or drug-associated, and 6% with an autoimmune/inflammatory condition. Acute myeloid leukaemia was the most common malignancy (16/93) followed by myelodysplastic syndrome (7/93). Patients with acute myeloid leukaemia presented isolated flares, marked cytopaenia and rapid response to treatment, whereas myelodysplastic syndrome followed a chronic-recurrent course. The most frequent associated medications and inflammatory disorders were filgrastim and hydroxyurea (n = 2); and inflammatory bowel disease (n = 4). In addition, 3 patients were diagnosed with VEXAS syndrome. Male sex (p = 0.006), fever (p = 0.034), increased erythrocyte sedimentation rate (p

Sweet syndrome is a rare complication of azathioprine treatment with unelucidated clinical features. The purpose of this study was to investigate the clinical characteristics of azathioprine-induced Sweet syndrome (AISS) and provide a reference for diagnosis, treatment and prognosis. We collected relevant case reports of AISS by searching Chinese and English databases from 1960 to December 31, 2022, extracted the data and carried out a retrospective analysis. The median age of the 44 patients was 50 (range 9–89) years, and they included 32 males (72.7%). Fever (86.4%) and arthralgia (31.8%) were the most common clinical symptoms. The skin lesions were mainly pustules (54.5%), papules (40.9%), plaques (40.9%) and nodules (31.8%), which were mainly distributed on the extremities (54.5%), face (38.6%) and hands (36.4%). Laboratory examination revealed neutropenia (65.9%) as well as elevated C-reactive protein (63.6%) and erythrocyte sedimentation (40.9%) rates. Histopathology of the lesioned skin showed neutrophil infiltration (93.2%) and dermal edema (38.6%). Symptom relief was achieved at a median time of 7 days (range 2–28 days) after azathioprine discontinuation in all patients. Nine patients (20.5%) had skin lesions that recurred within 24 h after taking azathioprine again. Clinicians and pharmacists should grasp the regularity and characteristics of AISS and should not recommend the readministration of azathioprine, to avoid the recurrence of Sweet syndrome. [ABSTRACT FROM AUTHOR]