Clustered cases of acral perniosis: Clinical features, histopathology, and relationship to COVID-19

Abstract

Background/objectives: A recent marked increase in pediatric and adult patients presenting with purpuric acral lesions concerning for ischemia, thrombosis and necrosis has been observed in COVID-19 prevalent regions worldwide. The clinical and histopathological features and relationship to COVID-19 have not been well described. The objective of this case series is to describe the clinical features and determine the histopathologic findings and clinical implications of the clusters of acral perniosis cases identified in pediatric patients.

Methods: We describe six otherwise healthy adolescents-three siblings per family from two unrelated families-presented within a 48-hour period in April, 2020, with acral perniosis-like lesions in the context of over 30 similar patients who were evaluated within the same week.

Results: Affected patients had mild symptoms of viral upper respiratory infection (URI) or contact with symptomatic persons 1-2 weeks preceding the rash. They all presented with red to violaceous macules and dusky, purpuric plaques scattered on the mid and distal aspects of the toes. Skin biopsies performed on each of the six patients demonstrated near identical histopathologic findings to those of idiopathic perniosis, with a lymphocytic inflammatory infiltrate without evidence of thromboembolism or immune complex vasculitis. While SARS-CoV-2 polymerase chain reaction was negative, testing was performed 1-2 weeks after URI symptoms or sick contact exposure.

Conclusion: We offer a clinical approach to evaluation of patients with this presentation and discuss the possibility that these skin findings represent a convalescent-phase cutaneous reaction to SARS-CoV-2 infection.

Figure 1

Clinical features of acral perniosis in two adolescent patients: dusky purpuric patches on the dorsal toes (A, E and G) with focal lesions on the dorsum of the foot and clustered along Wallace's line and heels (A, B, D, E and F). Net‐like vascular pattern (livedo reticularis) on the dorsal hands and feet (A, C and E)

Figure 2

Histopathologic sections show a superficial and deep lymphocytic infiltrate (A) that is both perivascular and perieccrine in distribution (B–D). In the perivascular component of the infiltrate, some lymphocytes are present in the walls of small vessels (C and D). There are also perijunctional lymphocytes with vacuolar change (B). Mucin deposition can be seen in both the reticular and periadnexal dermis (B–D)