Extensive haemorrhagic-bullous skin manifestation of systemic AA-amyloidosis associated with IgGlambda-myeloma.
In an 86-year-old woman with a multiple myeloma of the IgG lambda subtype a coinciding systemic amyloidosis manifested as a macroglossia, diffuse alopecia and generalized cutaneous involvement. The skin was affected by milium-like papules, petechial haemorrhages and an increased tissue fragility with subsequent blister formation. The typical histology and immunohistology pattern revealed large intradermal amyloid masses, reacting positively with anti-amyloid A antibodies, which surrounded cuff-like dilatated blood capillaries. The abundance of these amyloid deposits led to significant deflexibilization and fragility of the capillaries and the dermal matrix eventually resulting in the haemorrhagic-bullous eruptions. The peculiar feature of the present case is the intensity of bullous-haemorrhagic skin damage due to amyloid A deposition without any detection of cutaneous IgGl as the myeloma-derived paraprotein assumed to be causative for the development of systemic AA amyloidosis.
Grundmann
J U
JU
Department of Dermatology and Venereology, Otto-von-Guericke University, Leipziger Stasse 44, D-39120 Magdeburg, Germany. Jens-Uwe.Grundmann@medizin.uni-magdeburg.de
Bonnekoh
B
B
Gollnick
H
H
D000368:Aged; D000369:Aged, 80 and over; D000505:Alopecia / Q000209:etiology; D000686:Amyloidosis / Q000150:complications* / Q000473:pathology; D002198:Capillary Fragility; D004438:Ecchymosis / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D008260:Macroglossia / Q000209:etiology; D009101:Multiple Myeloma / Q000150:complications*; D011693:Purpura / Q000209:etiology; D000685:Serum Amyloid A Protein / Q000032:analysis; D012867:Skin / Q000098:blood supply; D012871:Skin Diseases / Q000150:complications* / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology*
D007074:Immunoglobulin G; D007146:Immunoglobulin lambda-Chains; D000685:Serum Amyloid A Protein