Idiopathic perniosis presenting as acral purpuric lesions: Clustering of cases before COVID ‐19 pandemic and their comparison with chilblain like lesions reported in the literature

Abstract

Perniosis/chilblains are the acral inflammatory skin lesions developing in susceptible individuals as an abnormal reaction to cold. In the absence of a discernible cause, it is labeled as idiopathic perniosis (IP). With the ongoing COVID‐19 pandemic, there was an upsurge of reports of chilblain like lesions (CLL) especially in young patients possibly implicated to the SARS‐CoV‐2 virus. Twelve clinically suspected and histopathologically confirmed cases of IP seen from November 2019 through February 2020 were retrospectively recruited. Clinical, dermoscopic, and histopathological characteristics of these were reviewed and compared with CLL reported in the literature. Mean age of patients was 26.58 ± 15.18 years with an equal male to female ratio. Characteristic histopathology findings were spongiosis (100%), dermal edema (100%), perivascular lymphocytic infiltrate (100%) with peri‐eccrine accentuation (66.7%), keratinocyte necrosis (50%), focal basal vacuolar damage (58.3%), and lymphocytic vasculitis (58.3%). Significant dermoscopy findings were variable background color ranging from dull red and violaceous to copper red and brown orange, coiled vessels (44.4%) and orange‐red structureless areas (63.9%). Lesions over palms and soles preferentially had white dots/clods and lines (38.9%). There appears no exclusive histopathological as well as dermoscopy features of CLL and IP, yet certain clues can be appreciated. Keratinocyte necrosis and severe dermal edema favors IP, whereas fibrin thrombi with involvement of both superficial and deep dermal vessels favor CLL. Dermoscopically presence of irregular, linear or branching vessels, red/purple dots and clods and gray brown reticule supports CLL while white dots/clods and lines supports IP.

A COVID-19 e as manifestações em pé e tornozelo: uma revisão integrativa

“…It can be idiopathic, hereditary, or associated with diseases such as leukemia, viral infections, and rheumatoid arthritis. The perniosis-like lesions observed in COVID-19 cases are clinically and histopathologically similar to perniosis, except for the definition of its triggering by cold (26) . A possible explanation for the perniosis-like lesions are more prevalent in young, healthy patients with mild symptoms of COVID-19 or asymptomatic patients is that these patients have an exuberant immune reaction mediated by interferon I, containing the infection early and favorably (27) .…”

A Case of Longstanding Idiopathic Pernio/Chilblain Disease

“…There have been case reports demonstrating that pernio may be a cutaneous manifestation of celiac disease, which this patient did not suffer from [ 2 ]. Histological clues that favor idiopathic pernio include keratinocyte necrosis, severe dermal edema, and dermoscopic white dots and lines [ 3 ]. Pernio is a disease of clinical diagnosis and skin biopsies are not necessary, unless a physician suspects a different disorder, as it can occur in patients with hematologic disorders, such as paraproteinemia, Raynaud’s, systemic lupus erythematosus, viral hepatitis or malignancy, and must be ruled out before suspecting pernio [ 4 ].…”

“…Lesions may also blister and ulcerate and there is a poorly understood pathophysiology of the disease; however, it seems to be caused by vasospasm of the vessels [ 2 ]. There have also been numerous studies showing a possible connection between pernio and the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2); however, no concrete relationship has been established thus far [ 3 ]. Pernio mainly affects young and middle-aged women, usually before the age of 40, with a prevalence of 0.9-2.1 per 1000 in women and 0.6 per 1000 in men [ 4 ].…”

COVID ‐19‐related chilblain‐like lesions and idiopathic perniosis: Additional variables possibly influencing dermoscopic pattern

“…In this study, the authors concluded that the presence of keratinocyte necrosis and severe dermal edema would be indicative of IP, while fibrin thrombi with involvement of both superficial and deep dermal vessels would favor CLLs. 1 Additionally, the evidence of irregular, linear or branching vessels, red/ purple dots or clods, and gray brown reticule on dermoscopy would support CLLs, whereas white dots/clods and lines would be associated with IP. 1 However, according to published data (not reported by Jindal et al), 4,5 dermoscopy of CLLs commonly reveals white lines (likely related to altered collagen in the dermis), especially in acute lesions, whereas IP may display linear-irregular and dotted vessels (corresponding to subpapillary and papillary vessels dilation, respectively) as well as purpuric spots (representing erythrocytes extravasation).…”

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I read with great interest the paper by Jindal et al in which dermoscopic and histological features of idiopathic perniosis (IP) observed in a cohort of Indian patients before SARS-CoV-2 emergence were retrospectively analyzed to explore possible differences with chilblain-like lesions (CLLs) reported in the literature over the ongoing COVID-19 pandemic. [1][2][3] Although several limitations were clearly highlighted by the authors, mainly including the lack of a statistical comparative analysis due to the absence of a CLLs control group and heterogeneity of available literature data, 1 I would like to bring to the reader's attention some additional issues that should be considered in the interpretation of dermoscopy of IP and CLLs.

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