Hypodermite à plasmocytes, variante histologique de la morphée profonde

Morphea profunda is a deep-localized variant of scleroderma. The presence of predominant plasma cell infiltrate suggests a rare type of morphea profunda named plasma cell panniculitis. Subcutaneous plasma cell infiltrate has been described in many diseases such as lupus profundus and lymphoplasmocytic panniculitis of Lyme disease. The family history of consanguinity, similar age of onset and identical clinicopathological presentation suggest a genetic role in this disorder. This is the second report of plasma cell panniculitis in two siblings with a history of consanguinity.