Pathology of Chilblains (Perniosis) and Equestrian Perniosis

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Dermpath-India

Dr Sampurna Roy MD

Syn: Pernio

Exposure to cold climate can produce a wide range of injuries as a result of inability to adapt to the low temperature.

Chilblains (perniosis) is a localized disease occurring after prolonged exposure to moderate to cold temperature.

Perniosis presents as inflammatory, erythematous or purple, intensely pruritic or painful conditions on the hands and feet.

Other locations include the ear, nose, thighs, and buttocks.

Chilblains is seen in all age.

It is more common in a younger age group, usually in the second and third decade.

These lesions appear approximately 12-24 hours after exposure to cold and spontaneous resolution is usually seen in one to three weeks.

Chilblains can present as a secondary feature of diseases such as systemic lupus erythematosus, Behçet disease, chronic myelomonocytic leukemia, metastatic breast carcinoma and cryoglobulinemia.

Histologically, there is prominent perivascular mononuclear cell infiltrate (lymphocytic vasculitis) in the upper dermis.

There is variable edema of the papillary dermis. In some cases there is intense papillary edema.

The lymphocytic vasculitis is associated with "fluffy edema" of the vessel wall.

In deep perniosis the lesion extends throughout the dermis.

Atypical lymphocytes with CD30 positive cells in classical clinical perniosis (chilblains), has been reported by Massey et al.

Equestrian perniosis is a rare condition in which the patient develops tender, burning, nodular plaques on both thighs and buttocks after riding in the cold.

It is not associated with systemic and autoimmune connective tissue disease.

The histology of Equestrian Perniosis is similar to perniosis.

One distinct feature is that the perivascular lymphocytic infiltrate prominently involves the fat.