Vesicular amyloidosis E85.3

Rare clinical presentation of systemic amyloidosis (AL amyloidosis) with vesicular, usually hemorrhagic, detachment of the skin. A poikilodermic aspect may develop.

Casuistry (published by Winzer M et al 1992).

A 75-year-old patient presented with bullae, poilocilodermal skin and nail dystrophy as signs of systemic amyloidosis, one year before a lambda-type IgG myeloma was diagnosed.

The skin lesions occurred at mechanically irritated sites on the trunk and at the tension sites of the extremities.

Hist: Subepidermal blister and necrotic keratinocytes in the epidermis. Evidence of amyloid deposits in the papillary dermis perivascularly and in the deep dermis around the eccrine glands and in the musculi arrectores pilorum. Polyclonal antibodies allowed classification of the deposits as amyloid composed of immunoglobulins of the light lambda chain.

Electron microscopy: globoid deposits of nonbranched filaments typical of amyloid.

1. Winzer M et al.(1992) Bullous poikilodermic amyloidosis of the skin with junctional blistering in IgG light chain plasmacytoma of the lambda type. Histology, immunohistology, and electron microscopy. Dermatologist 43:199-204.