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Most viral infections can be self-limited, with no requirement for medical intervention. However, the same viruses can cause severe diseases in patients with compromised immunity due to single-gene diseases, acquired immune deficiency syndrome, or hematologic malignancies or those receiving immunosuppressive drugs. Occasionally, these immunocompromised patients harbor >1 infectious agent, requiring several concomitant diagnostic tests. We have developed, to our knowledge, a previously unreported whole-transcriptome sequencing–based pipeline that allows virome profiling, quantitation, and expression pattern analysis of 926 distinct viruses by sequencing of RNA isolated from a single lesional skin biopsy. This pipeline can also explore host genetics if there is a Mendelian predisposition to infection. We applied this pipeline to 6 Iranian patients with viral-induced skin lesions associated with immune deficiency secondary to HIV, human T-lymphotropic virus 1, chronic lymphocytic leukemia, and post transplant immunosuppression. In 5 cases, definitive human papillomavirus infections were identified, some caused by multiple viral types. In addition to human papillomavirus, coinfection with other viruses (Merkle cell polyomavirus, cytomegalovirus, and human herpesvirus 4) was detected in some lesions. In 1 case, whole-transcriptome sequencing validated the clinical diagnosis of adult T-cell leukemia/lymphoma in a patient with an initial diagnosis of mycosis fungoides/Sézary syndrome. These findings attest to the power of whole-transcriptome sequencing in profiling the cutaneous virome in the context of compromised immunity.

Abstract Objectives Inborn errors of immunity (IEIs) are monogenic diseases of the immune system. Despite the increasing genetic advancements, the diagnosis of IEIs still lean on clinical diagnosis. Dermatological manifestations are observed in a large number of IEI patients and can lead to proper approach and prompt intervention. Methods This cross‐sectional study was carried out between 2018 and 2020 on IEIs at a Children's tertiary care center in Tehran, Iran. Demographic details and age at onset of symptoms of IEI were recorded. Results Overall, 212 patients were included. Cutaneous findings were reported in 95 (44.8%) patients, and 61 of 95 (64.2%) reported skin lesions as the first clinical presentation. Skin infection (69, 72.6%) was the most frequent cutaneous manifestation, followed by eczematous rash (24, 25%). Conclusions Skin manifestations are a common feature in IEI patients and are readily recognizable by healthcare providers. This study tried to provide information on prognostic consequences.

Hereditary progressive mucinous histiocytosis (HPMH) is an extremely rare progressive non-Langerhans cell histiocytic disorder presenting with only cutaneous manifestations. Patients typically present with multiple asymptomatic dome-shaped erythematous papules, usually involving the face and upper extremities. Twenty-six cases have been reported worldwide, with no spontaneous regression. Treatment with thalidomide stopped the progression of the disease in two cases. We report a case of progressive mucinous histiocytosis in a 31-year-old female patient with a history of tuberculosis who presented papular lesions on the face that later extended to the hands. She was treated with isoniazid for tuberculosis and isotretinoin for the skin lesions; the improvement was minimal during the next two months, with new lesions appearing on both hands. Thalidomide stopped the progression of the disease. The cause and pathogenesis of HPMH are undetermined. The pathogenesis of HPMH may be similar to that of lysosomal storage disease, considering the intra-cytoplasmic phospholipid deposition in both diseases, in addition to the likelihood of a role of macrophages in triggering the disease. In our patient, tuberculosis may have contributed.

Key Clinical Message Pyoderma gangrenosum (PG) is a neutrophilic dermatosis associated with underlying disorders. The association between PG and solid organ tumors (SM), including gynecologic cancers, has been previously reported. Here, we report a case of a 61‐year‐old woman with pyoderma gangrenosum on the posterior auricular region associated with an underlying fallopian tube carcinosarcoma: a rare and aggressive gynecologic malignancy. The patient's ulcer responded favorably to treatment, and surgical resection of the tumor was performed. The patient was then referred for further cancer management. No new lesions or recurrences were found over the 18 months of routine follow‐up.

Abstract Background Bullous pemphigoid is the most common autoimmune subepidermal blistering disorder with a low incidence in childhood. Combined immunodeficiencies (CIDs) are a group of monogenic inborn errors of immunity (IEIs) characterized by T- and B-cell dysfunction leading to recurrent infections, lymphoproliferation, predisposition to malignancy, and autoimmunity. Here, we report two Afghan siblings with a diagnosis of CID and extremely rare manifestation of diffuse bullous pemphigoid skin lesions. Case presentation The older sibling (patient 1) was a 32-month-old male with facial dysmorphism, protracted diarrhea, failure to thrive, recurrent oral candidiasis, recurrent otitis media with tympanic membrane perforation, who had been previously diagnosed with CID. While he was under treatment with intravenous immunoglobulin (IVIg), he developed extensive blistering lesions, which were diagnosed as childhood bullous pemphigoid. Methylprednisolone and azathioprine were added to the regimen, which resulted in a remarkable improvement of the skin lesions and also the feeding condition. However,2 weeks later, he was re-admitted to the intensive care unit (ICU) and eventually died due to fulminant sepsis. Later, his 12-month-old sister (patient 2) with similar facial dysmorphism and a history of developmental delay, food allergy, recurrent oral candidiasis, and respiratory tract infections also developed blistering skin lesions. She was under treatment for occasional eczematous lesions, and had been receiving IVIg for 3 months due to low levels of immunoglobulins. Further immunologic workup showed an underlying CID and thus treatment with IVIg continued, gradually improving her clinical condition. The genetic study of both siblings revealed a novel homozygous mutation in exon 7 of the PGM3 gene, c.845 T > C (p.Val282Ala). Conclusions Dermatologic disorders may be the presenting sign in patients with CID and mutated PGM3. This case report further extends the spectrum of skin manifestations that could be observed in PGM3 deficiency and emphasizes the importance of considering CIDs during the assessment of skin disorders, particularly if they are extensive, recurrent, refractory to treatment, and/or associated with other signs of IEIs.

COVID-19 vaccination, although is a promising tool to overcome the pandemic, has side effects. There are increasing reports of oral lesions after COVID-19 vaccination. The aim of this review is to identify the occurrence of some oral lesions after COVID-19 vaccination, and highlight the underlying immune mechanisms involved. A narrative literature review was performed by searching electronic databases including PubMed, Scopus and Web of Science to investigate the oral lesions after COVID-19 vaccination. The inclusion criteria were original studies, including the case reports, case series, letter to the editor, and cross-sectional studies. The exclusion criteria included the studies which examined the oral lesions caused by COVID-19 infection. The information, including the number of participant(s) receiving vaccine, type of vaccine, dose number, side effect(s), time of onset following vaccination, healing time, treatment strategies for the existing lesions, and related mechanisms were then summarized in a data extraction sheet. The results of this review showed that some vaccines had side effects with oral involvement such as pemphigus vulgaris, bullous pemphigoid, herpes zoster, lichen planus, Stevens-Johnson syndrome and Behçet's disease. Future research needs to elucidate the physiopathology of oral manifestations after the COVID-19 vaccination, and better understand the risk factors associated with such responses. Sometimes vaccine's side effects may be due to the nocebo effect, which means that the person expects some adverse events to occur following the vaccine administration.

Key Clinical Message As dermatologists, we must be aware that even limited localized lesions may signal a life‐threatening condition, for which early diagnosis and treatment can improve the prognosis. Abstract Bullous pemphigoid is an autoimmune disorder characterized by blister formation. Hypereosinophilic syndrome is a myeloproliferative disorder featuring papules, nodules, urticarial lesions, and blisters. The coexistence of these disorders may highlight the involvement of common molecular and cellular factors. Here, we describe a 16‐year‐old patient with hypereosinophilic syndrome and bullous pemphigoid.

Abstract Nowadays, cold atmospheric plasma shows interesting results in dermatology. In the present study, a new portable cold plasma was designed for plasma skin rejuvenation (PSR) purposes. This device is safe and easy to use at beauty salons and homes. The effects of this device were investigated on the rat skins. Also, as a new method to improve PSR results, vitamin C ointment was combined with plasma. In this study, there were four groups of 5 Wistar rats. The first group received vitamin C ointment, the second received 5 min of high-voltage plasma, and the third and the fourth groups received 5 min of high- and low-voltage plasma and vitamin C ointment. This process was done every other day (3 sessions per week) for 6 weeks. To evaluate the thermal effect of plasma, the skin temperature was monitored. Also, the presence of reactive species was demonstrated by the use of optical spectroscopy. In addition, mechanical assays were performed to assess the effect of plasma and vitamin C on the tissue’s mechanical strength. The mechanical assays showed a positive impact of plasma on the treated tissue compared to the control group. Also, changes in the collagen level and thickness of the epidermal layer were examined in histological studies. The results indicated an increase in collagen levels after using plasma alone and an accelerated skin reaction after using vitamin C combined with plasma therapy. The epidermal layer’s thickness increased after applying high-voltage plasma, which indicates an increase in skin elasticity. This study demonstrates the positive effect of using the portable plasma device with vitamin C ointment on effective parameters in skin rejuvenation.

Abstract Arrhythmogenic right ventricular cardiomyopathy (ARVC), with skin manifestations, has been associated with mutations in JUP encoding plakoglobin. Genotype–phenotype correlations regarding the penetrance of cardiac involvement, and age of onset have not been well established. We examined a cohort of 362 families with skin fragility to screen for genetic mutations with next-generation sequencing-based methods. In two unrelated families, a previously unreported biallelic mutation, JUP: c.201delC; p.Ser68Alafs*92, was disclosed. The consequences of this mutation were determined by expression profiling both at tissue and ultrastructural levels, and the patients were evaluated by cardiac and cutaneous work-up. Whole-transcriptome sequencing by RNA-Seq revealed JUP as the most down-regulated gene among 21 skin fragility-associated genes. Immunofluorescence showed the lack of plakoglobin in the epidermis. Two probands, 2.5 and 22-year-old, with the same homozygous mutation, allowed us to study the cross-sectional progression of cardiac involvements in relation to age. The older patient had anterior T wave inversions, prolonged terminal activation duration (TAD), and RV enlargement by echocardiogram, and together with JUP mutation met definite ARVC diagnosis. The younger patient had no evidence of cardiac disease, but met possible ARVC diagnosis with one major criterion (the JUP mutation). In conclusion, we identified the same biallelic homozygous JUP mutation in two unrelated families with skin fragility, but cardiac findings highlighted age-dependent penetrance of ARVC. Thus, young, phenotypically normal patients with biallelic JUP mutations should be monitored for development of ARVC.

Background: Placenta extract has been shown to improve androgenetic alopecia (AGA) by inducing the anagen phase and increasing hair follicle density and size. Objective: This study aimed to evaluate the safety and efficacy of cow placenta extract lotion compared with topical minoxidil 2% as a gold standard treatment for female pattern AGA. Methods: In this double-blind, randomized controlled trial, a total of 90 women with AGA were enrolled and randomly assigned to receive either topical minoxidil 2% or cow placenta solutions. At the end of the sixth month, the number of hair follicles was evaluated using a trichoscope and compared with the baseline. Global photographic review was also conducted by a blinded dermatologist. Results: By the end of the sixth month, there was an increase in total hair count in the specified area in both groups. The mean increase in hair count was 10.9 ± 5.74 and 10.2 ± 6.5 for minoxidil and cow placenta groups, respectively (p = .63). The percentage of patients who were rated as having moderate or marked growth was 44.2% and 32.2% in the cow placenta and minoxidil groups, respectively (p = .90). Limitations: The study limitations were the limited number of cases, short duration of treatment, and the fact that none of our participants volunteered to undergo a biopsy to evaluate microscopic changes. Conclusions: Cow placenta hair-tonic lotion can be as effective as minoxidil 2% for female pattern AGA.

Abstract Background Benign joint hypermobility syndrome (BJHS) is one of the most common hereditary connective tissue disorders in children in which autonomic nervous system involvement has been reported. This study aimed to evaluate the frequency of primary focal hyperhidrosis in children with BJHS. Methods This observational-analytical study was conducted in a case-control setting on children aged 3 to 15 years in 2018 at Mofid Children’s Hospital, Tehran, Iran. Benign joint hypermobility syndrome was diagnosed according to the Brighton criteria; then, the patients referred to a dermatologist for evaluation of hyperhidrosis. Results In total, 130 eligible patients with confirmed BJHS and 160 age- and sex-matched healthy subjects were enrolled in this study. Primary focal hyperhidrosis (PFH) was seen in 56.2 and 16.3% of the cases and controls, respectively, indicating a significant difference (P

Abstract A 34‐year‐old female patient presented with recurrent bilateral hypopigmented macules on the labia majora. The lesions were treated with topical steroids, which led to mild improvement, but erosive plaques developed after discontinuing the treatment. Histopathological findings were compatible with extramammary Paget disease (EMPD), which was treated with radical vulvectomy with no recurrence in the next months of follow‐up.

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm of the sweat glands. It is believed to arise from the malignant transformation of syringocystadenoma papilliferum (SCAP). The majority of cases present on the head and neck and up to 17% of cases show metastatic progression. These tumors seldom occur in the anogenital area and, to date, only one case has been reported on the penis. Here, we report a rare case of SCACP in a 65-year-old man who presented with an erythematous, non-healing, ulcerated lesion on the penis.

Abstract COVID‐19 should be considered as a new triggering factor for autoimmune disorders like DM‐lupus overlap syndrome. We recommend that patients presenting with dermatomyositis during this pandemic be screened for COVID‐19.

Abstract During the COVID‐19 pandemic, physicians must maintain a high index of suspicion for COVID‐19 in cases of urticarial vasculitis or other forms of urticaria. This is particularly important for acute presentations in otherwise asymptomatic individuals and pregnant women, where a prompt approach to the patient can prevent undesirable complications.

Introduction: The novel coronavirus is rapidly spreading around the world. Since the public announcement of the COVID-19 outbreak, several concerns have been raised by dermatologists as well as pemphigus patients who take immunosuppressive drugs. In this paper, we review the literature about the common treatment of pemphigus with a focus on the lessons from similar epidemics to find a proper suggestion to manage pemphigus in the COVID-19 pandemic era. The effect of many of the drugs used for treatment of Pemphigus vulgaris (PV) on COVID-19 is not clear. We also do not have data on the impact of this autoimmune disease, which may involve the mucous membranes, on the acquisition or course of COVID-19. We are currently in the midst of a pandemic and evaluating the effect of COVID-19 on the population of susceptible patients suffering from auto-immune diseases like pemphigus is essential. The evidence on best ways to manage patients with underlying conditions, such as pemphigus, during the outbreak of COVID-19 is evolving and the data is updated every day.

Inflammatory myofibroblastic tumor (IMT) is a peculiar low-grade neoplasm of spindle cell fibroblasts and myofibroblasts in an inflammatory background. The lung is the most common site of involvement. Here, we report a case of paraneoplastic pemphigus (PNP)-associated with an extensive retroperitoneal IMT. The patient had a favourable response following treatment with a low dose of systemic steroid, mycophenolate mofetil, and intravenous immunoglobulin (IVIG). He subsequently underwent surgery for resection of the tumor with nephrectomy and five courses of IVIG were administered after surgery due to a minor relapse. He was in remission in her last follow-up visit 16 months after surgery. The occurrence of PNP with IMT is notable; early detection and treatment are crucial for this tumor-associated autoimmune disease.

Incontinentia pigmenti (IP) is an X-linked dominant neurocutaneous syndrome with ophthalmologic, neurologic, cutaneous, and dental manifestations and in most cases antenatally lethal in boys. Occasionally, typical IP may occur in boys due to Klinefelter syndrome or a genomic mosaicism. Skin lesions are observed in 4 stages: blistering, verrucous linear plaques, swirling macular hyperpigmentation, followed by linear hypopigmentation that develop during adolescence and early adulthood. Neonatal herpes simplex virus (HSV) infection can be manifested in 3 forms: localized, disseminated, and central nervous system (CNS) involvement. Timely diagnosis and treatment of neonatal HSV infection is critical. In this case report, we present a 12-day female newborn with a history of maternal genital HSV in second trimester and vesicular lesions on the upper and lower limbs that was appeared at first hours of life. She was admitted in the maternity hospital that was born and was treated by antibiotic and acyclovir for 11 days. Then, she readmitted for her distributed vesicular lesions. The results of blood and CSF for HSV PCR were negative. Eventually the diagnosis for incontinentia pigmenti was made by consultation with a dermatologist, and skin biopsy confirmed the diagnosis.

Background. Morphea is an inflammatory disease of the connective tissue that may lead to thickening and hardening of the skin due to fibrosis. The aim of this study was to document magnetic resonance imaging (MRI) changes in patients with linear morphea who were treated with methotrexate (MTX) and high-dose corticosteroid. Methods. This study was conducted on 33 patients from the outpatient’s dermatology clinic of our institute, who fulfilled the inclusion criteria. Patients received 15 mg/week of MTX and monthly pulses of methylprednisolone for three days in six months. The effectiveness of the treatment was evaluated by MRI, modified LS skin severity index (mLoSSI), and localized scleroderma damage index (LoSDI). Results. All parameters of mLoSSI and LoSDI including erythema, skin thickness, new lesion/lesion extension, dermal atrophy, subcutaneous atrophy, and dyspigmentation were also noticeably improved after treatment. Subcutaneous fat enhancement was the most common finding in MRI. MRI scores were significantly associated with clinical markers both before and after the treatment with the exception of skin thickness and new lesion/lesion extension which were not associated with MRI scores before and after the treatment, respectively. Limitations. The lack of correlative laboratory disease activity markers, control group, and clearly defined criteria to judge the MRI changes. Conclusion. MRI could be a promising tool for the assessment of musculoskeletal and dermal involvement and also monitoring treatment response in patients with morphea.

Introduction: History taking and physical examination help clinicians identify the patient’s problem and effectively treat it. This study aimed to evaluate the descriptors of dyspnea in patients presenting to emergency department (ED) with asthma, congestive heart failure (CHF), and chronic obstructive pulmonary disease (COPD). Method: This cross-sectional study was conducted on all patients presenting to ED with chief complaint of dyspnea, during 2 years. The patients were asked to describe their dyspnea by choosing three items from the valid and reliable questionnaire or articulating their sensation. The relationship between dyspnea descriptors and underlying cause of symptom was evaluated using SPSS version 16. Results: 312 patients with the mean age of 60.96±17.01 years were evaluated (53.2% male). Most of the patients were > 65 years old (48.7%) and had basic level of education (76.9%). "My breath doesn’t go out all the way" with 83.1%, “My chest feels tight " with 45.8%, and "I feel that my airway is obstructed" with 40.7%, were the most frequent dyspnea descriptors in asthma patients. "My breathing requires work" with 46.3%, "I feel that I am suffocating" with 31.5%, and "My breath doesn’t go out all the way" with 29.6%, were the most frequent dyspnea descriptors in COPD patients. "My breathing is heavy" with 74.4%, "A hunger for more air” with 24.4%, and "I cannot get enough air" with 23.2%, were the most frequent dyspnea descriptors in CHF patients. Except for “My breath does not go in all the way”, there was significant correlation between studied dyspnea descriptors and underlying disease (p = 0.001 for all analyses). Conclusion: It seems that dyspnea descriptors along with other findings from history and physical examination could be helpful in differentiating the causes of the symptom in patients presenting to ED suffering from dyspnea.

Introduction: History taking and physical examination help clinicians identify the patient’s problem and effectively treat it. This study aimed to evaluate the descriptors of dyspnea in patients presenting to emergency department (ED) with asthma, congestive heart failure (CHF), and chronic obstructive pulmonary disease (COPD). Method: This cross-sectional study was conducted on all patients presenting to ED with chief complaint of dyspnea, during 2 years. The patients were asked to describe their dyspnea by choosing three items from the valid and reliable questionnaire or articulating their sensation. The relationship between dyspnea descriptors and underlying cause of symptom was evaluated using SPSS version 16. Results: 312 patients with the mean age of 60.96±17.01 years were evaluated (53.2% male). Most of the patients were > 65 years old (48.7%) and had basic level of education (76.9%). "My breath doesn’t go out all the way" with 83.1%, “My chest feels tight " with 45.8%, and "I feel that my airway is obstructed" with 40.7%, were the most frequent dyspnea descriptors in asthma patients. "My breathing requires work" with 46.3%, "I feel that I am suffocating" with 31.5%, and "My breath doesn’t go out all the way" with 29.6%, were the most frequent dyspnea descriptors in COPD patients. "My breathing is heavy" with 74.4%, "A hunger for more air†with 24.4%, and "I cannot get enough air" with 23.2%, were the most frequent dyspnea descriptors in CHF patients. Except for “My breath does not go in all the wayâ€, there was significant correlation between studied dyspnea descriptors and underlying disease (p = 0.001 for all analyses). Conclusion: It seems that dyspnea descriptors along with other findings from history and physical examination could be helpful in differentiating the causes of the symptom in patients presenting to ED suffering from dyspnea.  Â