Vesicular amyloidosis - Altmeyers Encyclopedia

Synonym(s)

blistering amyloidosis; bullous amyloidosis; cutaneous amyloidosis blistering

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

External therapy
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Symptomatic, primarily astringent (later antipruriginous) treatment with synthetic tannic acid (e.g. Tannolact), aqueous dye solutions such as 0.5% methylrosanilinium chloride solution. Trials with external glucocorticoids such as 0.1% triamcinolone cream (e.g. Triamgalen, R259 ), if necessary under occlusion, and antipruriginosa such as Polidocanol shaking mixtures R200 are possible, see also amyloidosis, macular.

Case report(s)
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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.

Literature
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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.