title: Bullous amyloidosis in a hemodialysis patient is myeloma-associated rather than hemodialysis-associated amyloidosis.
abstract: We report a 78-year-old woman on hemodialysis who presented with refractory multiple pruritic vesicles and bullae on her trunk and extremities for 2 months. Histopathologic examination of skin biopsy specimen showed subepidermal bullae with many amyloid deposits in the papillary dermis. No evidence of systemic amyloidosis could be found on physical examination. While the initial clinical diagnosis was bullous pemphigoid, the histopathology and direct immunofluorescence result favored hemodialysis-associated amyloidosis. However, immunochemical study for beta(2)-microglobulin was negative. Further hematologic and immunologic work-up revealed the presence of multiple myeloma and that the deposit was AL amyloid. This is the first case of bullous amyloidosis in a hemodialysis patient and should remind dermatologists that bullous amyloidosis should be considered in addition to the usual presentation of porphyria cutanea tarda and pseudoporphyria for bullous dermatosis in the hemodialysis patient. We also suggest that hemodialysis-associated amyloidosis should not be taken for granted in the hemodialysis patient with cutaneous amyloidosis without systemic signs and symptoms. Further testing for other types of amyloid should be performed.
authors.affiliation: Department of Dermatology, Chang-Gung Memorial Hospital, No. 199 Tunghaw N. Road, Taipei, Taiwan.
authors.forename: Shyue-Luen
authors.initials: SL
authors.lastname: Chang
authors.forename: Ping-Chin
authors.initials: PC
authors.lastname: Lai
authors.forename: Chien-Jui
authors.initials: CJ
authors.lastname: Cheng
authors.forename: Li-Cheng
authors.initials: LC
authors.lastname: Yang
authors.forename: Hong-Shang
authors.initials: HS
authors.lastname: Hong
mesh_terms: D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D001768:Blister / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D009101:Multiple Myeloma / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism; D006435:Renal Dialysis / Q000009:adverse effects*; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D001613:beta 2-Microglobulin / Q000378:metabolism
chemical_list: D000682:Amyloid; C035222:amyloid protein AL; D001613:beta 2-Microglobulin