Perniosis are inflammatory cutaneous lesions, located on acral skin, which present in association with cold exposure. They can appear as an idiopathic dermatosis or with an underlying autoimmune disease. The use of cutaneous biopsy to distinguish between both types is controversial. We analyze the histological findings in 9 cases of idiopathic perniosis (IP) and compare them with those obtained from 11 cases of perniosis associated with an autoimmune disease (autoimmune perniosis). The most frequent histopathological features observed in cases of IP were a lymphocytic infiltrate with perivascular (8 cases, 89%) and perieccrine distribution (6 cases, 66%), dermal edema (5 cases, 55%), and necrotic keratinocytes (5 cases, 55%), whereas those found in perniosis associated with an autoimmune disease were lymphocytic infiltrate with perivascular distribution (11 cases, 100%) but without perieccrine distribution (3 cases, 27%), vacuolation of the basal layer (7 cases, 63%), and necrotic keratinocytes (5 cases, 45%). The only significant difference between both groups was the perieccrine distribution of the lymphocytic infiltrate in cases of IP, which, as mentioned in previous studies, could be considered a histopathological clue to differentiate both types of perniosis.

Deep morphea encompasses a variety of clinical entities in which inflammation and sclerosis are found in the deep dermis, panniculus, fascia, or superficial muscle. Morphea profunda, eosinophilic fasciitis, and disabling pansclerotic morphea of children are included in this group, but overlapping of the extent and depth of cutaneous involvement in these various conditions precludes their distinction on the sole basis of clinical or even histologic examination. Furthermore, the limits between morphea profunda and generalized morphea, which usually are classified outside this group, are not clear. Histologically, all these disorders show similar inflammatory and sclerotic findings, the primary difference being the depth of these changes. Associated clinical findings, including arthralgias, arthritis, contractures, or carpal tunnel syndrome, are frequent. Although visceral complications are uncommon, pulmonary, esophageal, and even cardiac abnormalities have been reported. Eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimentation rate may be present with disease activity. Laboratory studies may demonstrate autoantibody production. Treatment is nonstandardized but UVA irradiation and antiinflammatory or immunosuppressive drugs (mainly antimalarial agents and corticosteroids) may be beneficial.

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