Childhood Vulvar Mucous Membrane Pemphigoid.

BACKGROUND: Autoimmune bullous diseases in childhood are a diagnostic challenge. CASE: We present the case of an 11-year-old girl with recurrent vulvar erosions since early childhood. She had been referred to a child abuse unit under the suspicion of sexual abuse. She responded well to dapsone and topical corticosteroids. SUMMARY AND CONCLUSION: Our review focuses on previously reported cases of pemphigoid (bullous or mucous membrane) in childhood with exclusively genital involvement. We also summarize mucous membrane pemphigoid cases diagnosed during childhood. There seems to be a differentiated form of pemphigoid predominantly affecting girls with exclusively vulvar involvement and with good prognosis. Dermatologic evaluation and a skin biopsy with direct immunofluorescence are key to diagnosing a mucous membrane pemphigoid. Further antigenic studies are needed to nosologically classify the disease properly.

High-Frequency Ultrasound to Assess Activity in Connective Tissue Panniculitis.

Determining disease activity from clinical signs in patients with connective tissue panniculitis (CTP) is often challenging but is essential for therapeutic decision making, which largely relies on immunosuppressant treatment. High-frequency ultrasound (HFUS) may be useful in supporting such decisions by accurately determining CTP activity. This study aimed to investigate the accuracy of HFUS in identifying signs of CTP activity or inactivity and assess its usefulness in therapeutic decision making. A prospective cohort study of consecutive patients with biopsy-proven CTP receiving HFUS was conducted in a tertiary university hospital (2016-2020). HFUS was performed at inclusion and at each 3- or 6-month follow-up visit, depending on disease activity. Twenty-three patients with CTP were included, and 134 HFUSs were performed. In 59.7% (80) of the evaluations, the clinical presentation did not show whether CTP was active or not. In these cases, HFUS showed activity in 38.7% (31) and inactivity in 61.3% (49). In 71.25% (57) of the visits, HFUS was the determinant for therapeutic decisions. Further follow-up showed consistent clinical and HFUS responses in all unclear cases after treatment modification. HFUS appears to be a useful adjunct to the clinical examination for CTP to assess activity and make therapeutic decisions.

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases.

The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.

A facility and community-based assessment of scabies in rural Malawi.

BACKGROUND: Scabies is a neglected tropical disease of the skin, causing severe itching, stigmatizing skin lesions and systemic complications. Since 2015, the DerMalawi project provide an integrated skin diseases clinics and Tele-dermatology care in Malawi. Clinic based data suggested a progressive increase in scabies cases observed. To better identify and treat individuals with scabies in the region, we shifted from a clinic-based model to a community based outreach programme. METHODOLOGY/PRINCIPAL FINDINGS: From May 2015, DerMalawi project provide integrated skin diseases and Tele-dermatological care in the Nkhotakota and Salima health districts in Malawi. Demographic and clinical data of all patients personally attended are recorded. Due to a progressive increase in the number of cases of scabies the project shifted to a community-based outreach programme. For the community outreach activities, we conducted three visits between 2018 to 2019 and undertook screening in schools and villages of Alinafe Hospital catchment area. Treatment was offered for all the cases and school or household contacts. Scabies increased from 2.9% to 39.2% of all cases seen by the DerMalawi project at clinics between 2015 to 2018. During the community-based activities approximately 50% of the population was assessed in each of three visits. The prevalence of scabies was similar in the first two rounds, 15.4% (2392) at the first visit and 17.2% at the second visit. The prevalence of scabies appeared to be lower (2.4%) at the third visit. The prevalence of impetigo appeared unchanged and was 6.7% at the first visit and 5.2% at the final visit. CONCLUSIONS/SIGNIFICANCE: Prevalence of scabies in our setting was very high suggesting that scabies is a major public health problem in parts of Malawi. Further work is required to more accurately assess the burden of disease and develop appropriate public health strategies for its control.

Skin Manifestations in COVID-19: Prevalence and Relationship with Disease Severity.

BACKGROUND: Data on the clinical patterns and histopathology of SARS-CoV-2 related skin lesions, as well as on their relationship with the severity of COVID-19 are limited. METHODS AND MATERIALS: Retrospective analysis of a prospectively collected cohort of patients with SARS-CoV-2 infection in a teaching hospital in Barcelona, Spain, from 1 April to 1 May 2020. Clinical, microbiological and therapeutic characteristics, clinicopathological patterns of skin lesions, and direct immunofluorescence and immunohistochemical findings in skin biopsies were analyzed. RESULTS: Fifty-eight out of the 2761 patients (2.1%) either consulting to the emergency room or admitted to the hospital for COVID-19 suspicion during the study period presented COVID-19 related skin lesions. Cutaneous lesions could be categorized into six patterns represented by the acronym "GROUCH": Generalized maculo-papular (20.7%), Grover's disease and other papulo-vesicular eruptions (13.8%), livedo Reticularis (6.9%), Other eruptions (22.4%), Urticarial (6.9%), and CHilblain-like (29.3%). Skin biopsies were performed in 72.4%, including direct immunofluorescence in 71.4% and immunohistochemistry in 28.6%. Patients with chilblain-like lesions exhibited a characteristic histology and were significantly younger and presented lower rates of systemic symptoms, radiological lung infiltrates and analytical abnormalities, and hospital and ICU admission compared to the rest of patients. CONCLUSION: Cutaneous lesions in patients with COVID-19 appear to be relatively rare and varied. Patients with chilblain-like lesions have a characteristic clinicopathological pattern and a less severe presentation of COVID-19.

Mácula eritemato-violácea en la pierna de una turista / Violaceous patch in the leg of a tourist

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Úlceras y erosiones orales simulando penfigoide de mucosas: la importancia de asegurar la toma adecuada de alendronato / An uncommon cause of oral ulcers: Inappropriate intake of altendronate

No disponible

Generalized Necrobiotic Palisading Granulomatous Follicular Eruption: A Peculiar Pustular Variant of Perforating Granuloma Annulare or an Individualized Disease?

Herein, we report a case of an adult male patient with a chronic and recurrent papulopustular eruption mainly involving the trunk and lower extremities. A dense superficial perifollicular inflammatory infiltrate with palisading necrobiotic granuloma formation and infundibular perforation was observed at the histological examination, with no granulomatous inflammatory infiltrates in deeper areas. The possibility that this peculiar clinicopathological presentation constitutes a case of generalized perforating granuloma annulare (PGA) or an individualized skin condition is discussed. The observation of a pustular follicular generalized PGA represents an exceedingly rare phenomenon and constitutes an infrequent subtype of PGA that can mimic pustular eruptions secondary to many different etiologies. The clinicopathological features of this rare variant may represent a diagnostic challenge, often requiring multiple biopsies to establish a definite diagnosis.

Diagnosis and management of pemphigus: Recommendations of an international panel of experts.

BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.

Nonclassical pemphigus with exclusively IgG anti-desmocollin 3-specific antibodies.

We describe a patient with nonclassical clinical and histopathological features of pemphigus with exclusively IgG antibodies against desmocollin (Dsc) 3 detected by enzyme-linked immunosorbent assay of recombinant eukaryotic protein of Dsc1-Dsc3. The absence of antibodies against other known targets, such as desmogleins, reinforces the role of anti-Dsc antibodies in the pathophysiology of atypical pemphigus.

Assessment of Treatment of Refractory Granuloma Faciale With Intralesional Rituximab.

Importance: Granuloma faciale (GF) is a rare, benign inflammatory dermatosis of unknown cause. Some reports have suggested that it could be part of the spectrum of IgG4-related sclerosing diseases. Granuloma faciale is characterized by single or multiple red-brown nodules, most frequently occurring on the face, and it can produce severe disfigurement. Treatment is difficult, and poor outcomes are often seen. Rituximab is a monoclonal antibody against CD-20 approved by the US Food and Drug Administration for treatment of some autoimmune and tumoral diseases. Objective: To evaluate the treatment of refractory GF with intralesional rituximab. Design, Setting, and Participants: In this case series, 3 patients with biopsy-proved refractory GF who underwent treatment at a dermatology outpatient clinic of a tertiary referral hospital were evaluated. The study was conducted from August 2015 to December 2017. Interventions: Doses of 0.5 to 1 mL/cm2 of intralesional rituximab, 10 mg/mL, were administered monthly for 6 months and thereafter depending on clinical response. In 2 patients, peripheral blood B-cell counts were monitored before and during treatment, and in 1 patient, only during treatment. Main Outcomes and Measures: Reduction in size of the lesions, ultrasonography evaluation, subjective improvement, and adverse events were monitored throughout the course of therapy. Results: All 3 of the patients were men (ages from 30s to 60s). They showed a significant reduction in the size and thickness of GF both clinically and on ultrasonography evaluation. Two patients had a complete response and the third, a partial response. A reduction in peripheral blood B-cell count was observed in the 3 patients, suggesting that the action of rituximab could be systemic. No severe adverse reactions were reported. Conclusions and Relevance: Intralesional rituximab may represent a novel and well-tolerated therapy for refractory GF.