Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients.

Introduction: This study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies. Methods: Among a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed. A Cox proportional hazards model was used to identify factors associated with poor outcomes, i.e., death or progressive disease at the last follow-up. Results: Among the 70 patients, 45 were women, and 54 were Caucasian. A dermatologic involvement was observed in 58 (83%) patients, including 40 with MDA5 vasculopathy-related skin lesions. Muscular involvement was observed in 39 (56%) patients. Interstitial lung disease (ILD) was observed at baseline in 52 (74%) patients, including 23 (44%) who developed rapidly progressive (RP) ILD. Seven (10%) patients showed thromboembolic complications within the first weeks of diagnosis, and eight (11%) other patients developed a malignancy (4 before the diagnosis of anti-MDA5 disease). Poor outcomes were observed in 28 (40%) patients, including 13 (19%) deaths. Among the 23 patients with RP-ILD, 19 (79%) showed poor outcomes, including 12 (63%) who died. In multivariate analyses, RP-ILD (hazard ratio (HR), 95% CI: 8.24 [3.21-22], p<0.0001), the occurrence of thromboembolic events (HR: 5.22 [1.61-14.77], p=0.008) and the presence of any malignancy (HR: 19.73 [6.67-60], p<0.0001) were the three factors independently associated with poor outcomes. Discussion: This new independent cohort confirms the presence of different clinical phenotypes of anti-MDA5 diseases at baseline and the poor prognosis associated with RP-ILD. Thromboembolic events and malignancies were also identified as prognostic factors.

Cutaneous calciphylaxis of the glans penis presenting as a gangrenous ulceration.

Report _Case Presentation X Photo Vignette _Letter Authors declare that the contents of this article are their own original unpublished findings. Title: Cutaneous calciphylaxis of the glans penis presenting as a gangrenous ulceration Authors: Marie Danset, Cécile Lesort, Denis Jullien, Jean Kanitakis Affiliations: Dermatology Department, Edouard Herriot Hospital, Hospices Civils de Lyon, Claude Bernard Lyon I University, Lyon, France Corresponding Author: Jean Kanitakis, Department of Dermatology, Edouard Herriot Hospital Group, 69437 Lyon Cedex 03, France, Tel: 33-472110301, Email: jean.kanitakis@univ-lyon1.fr Abstract: Calciphylaxis is a rare microvascular disorder causing necrotic skin ulcers. It is characterized by deposits of calcium within vascular walls but its precise pathogenesis remains poorly understood. A major risk factor is end-stage renal disease on dialysis. We report a 67-year-old man with calciphylaxis revealed by an unusual necrotic ulcer of the glans penis. The patient also presented with bilateral panniculitis of the thighs and a calf ulcer. All those lesions were painful, highlighting the value of pain as a diagnostic clue. Penile involvement of calciphylaxis is rare and biopsy is often avoided in this area. However, rapid diagnosis of calciphylaxis is important because early treatment has a better chance of being successful. Our patient's condition deteriorated rapidly with development of bilateral retinal artery occlusion and he died shortly thereafter. This case further highlights the fact that calciphylaxis is a systemic vascular disease with an ominous prognosis.

Chilblain-like acral lesions during the COVID-19 pandemic ("COVID toes"): Histologic, immunofluorescence, and immunohistochemical study of 17 cases.

BACKGROUND: During the coronavirus disease 2019 pandemic, several acral chilblain-like lesions were observed in young patients with suspected, but mostly unconfirmed, infection with severe acute respiratory syndrome coronavirus 2. The histopathologic aspect of these lesions is as yet poorly known. OBJECTIVE: To investigate the pathologic features of chilblain-like lesions. METHODS: Biopsies were obtained from 17 cases of chilblain-like lesions during the coronavirus disease 2019 pandemic in France and were studied by routine histologic examination, immunohistochemistry, and direct immunofluorescence. The patients had suspected but unconfirmed infection with severe acute respiratory syndrome coronavirus 2 (negative nasopharyngeal polymerase chain reaction and serologic test results). RESULTS: Chilblain-like lesions showed many features in common with those reported in idiopathic and autoimmune-related chilblains, including epidermal necrotic keratinocytes, dermal edema, perivascular and perieccrine sweat gland lymphocytic (predominantly CD3/CD4+) inflammation, and frequent vascular changes (endothelialitis, microthromboses, fibrin deposition, and immunoreactant deposits on vessels). CONCLUSIONS: Chilblain-like lesions show histopathologic features similar to those of idiopathic and autoimmune-related chilblains, with a high rate of vascular changes and direct immunofluorescence positivity. The role of severe acute respiratory syndrome coronavirus 2 in the development of these puzzling lesions remains to be elucidated.

Dermoscopic Features of Onychomatricoma: A Study of 34 Cases.

BACKGROUND: Onychomatricoma is a benign tumor of the nail matrix with a precise histopathological definition but many different clinical features and differential diagnoses. OBJECTIVES: The diagnosis of onychomatricoma is based on the pathological examination of a surgical specimen. The objective of this study was to define preoperative diagnostic criteria using noninvasive investigations: observation and dermoscopy. METHODS: A total of 34 cases of onychomatricoma were studied. In detail, 6 observers evaluated 12 clinical and 12 dermoscopical criteria as present or absent. RESULTS: For clinical criteria, the highest mean values were leuconychia (3.21), splinter hemorrhages (3.45), and thickening of the plate (3.0). Dermoscopical criteria were more often found present, such as longitudinal parallel white lines (4.33), parallel lesion edges (4.61), splinter hemorrhages (4.48), dark dots (3.96), free-edge nail pitting (4.5), and thickening of the free edge (5.27). CONCLUSION: Dermoscopical criteria for onychomatricoma are more frequently present and less subject to divergent interpretation. This offers new and original preoperative reliable diagnostic criteria and should be used in the evaluation of nail tumors.