Scleroderma Profunda

Abstract

Scleroderma profunda represents an unusual clinical entity. There are no unified criteria for its diagnosis. Seven patients with scleroderma profunda were studied. The clinical features can be summarised in three groups:

• 1. hyperpigmented plaques with subcutaneous induration;

• 2. erythematous plaques with induration of the skin and subcutis;

• 3. pale indurated plaques with atrophy and sometimes ulcerobullous lesions.

Histology showed sclerodermiform changes in the deep dermis and subcutis and vasculitis in one case. Dermal lymphocytic infiltration was well pronounced in 5 cases and consisted of predominantly CD3+ T cells. Differential diagnosis from panniculitis can be difficult. Mucin deposition was established in all cases.