Familial poikylodermic cutaneous amyloidosis.

Among the less common variants of primary cutaneous amyloidosis are both the poikylodermic and the familial types. The case of two sisters of thirteen and seventeen years old with extensive asymptomatic lesions with a poikylodermic aspect is reported. The girls' father was also affected and a paternal aunt had consulted at our department some years previously for similar lesions. Thus a new case of familial cutaneous amyloidosis with an autosomal dominant hereditary pattern is described. The possible relationship between multiple endocrine neoplasia type 2A (MEN-2A) and familial cutaneous amyloidosis is discussed.