Autosomal recessive plasma cell panniculitis with morphea-like clinical manifestation

Abstract

Two siblings had hyperpigmented indurations over the inner aspects of both thighs extending to the lower abdomen. Skin biopsy showed plasma cell panniculitis favoring a diagnosis of morphea profundus. Family history of consanguinity was present, but both parents were unaffected. To our knowledge this is the first report of autosomal recessive plasma cell panniculitis with the clinical manifestations of morphea.