Perniosis

Perniosis consists of the development of inflammatory, erythematous and frequently pruriginous lesions on acral sites after contact with cold. These lesions can occur as an isolated disorder in an otherwise healthy individual or in the context of various disorders such as anorexia nervosa or other diseases associated with low body weight, hematological processes — especially myelodysplastic syndrome — the presence of antiphospholipid antibodies and even some genetic diseases in which there is immune dysregulation, such as Aircadi-Goutières disease. The term idiopathic perniosis is used to designate patients with no underlying systemic disease.

Diagnosis is clinical, although microscopic study of a biopsy sample can be useful in specific circumstances. Perniosis should be distinguished from other, very different processes that may nevertheless cause lesions with a similar appearance. Among these are chilblain lupus, which still lacks a precise definition and is difficult to distinguish from idiopathic perniosis. Treatment is disappointing and is essentially based on topical corticosteroids and oral calcium channel blockers such as nifedipine and felodipine