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Fatigue, macroglossia, xanthomatous papules and bullae
C-9 New Medical Enclave, Banaras Hindu University, Varanasi - 221 005
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Singh S, Kumar S, Chaudhary R. Fatigue, macroglossia, xanthomatous papules and bullae. Indian J Dermatol Venereol Leprol 2010;76:216
A 45-year-old man had fatigue, nail dystrophy (thinning, striations) (13 months); diffuse scalp alopecia, macroglossia, dysphagia (4 months); xanthomatous lesions, hoarseness, generalized pruritus (3 months); and bullae (6 weeks). Except pallor and expressionless face, general and systemic examination was normal. Itchy, xanthomatous papules were present on the back [Figure - 1], and tense vesicles and bullae, some with hemorrhagic fluid, were on the limbs and abdomen.
Hemoglobin was 7.8 g/dl, and ESR was 31 mm. Blood counts, routine urinalysis, and serum biochemistry including calcium level, X-rays and ultrasonography (abdomen) were normal. The 24-h urinary protein was 360 mg/dl. Skin biopsy [Figure - 2] and bone-marrow examination [Figure - 3] were performed.
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Nyirady J, Schwartz RA. Amyloidosis, primary systemic. Available from: http://emedicine.medscape.com/article/1093258-overview . [last accessed on 2009 May 10][Google Scholar]
Lachmann HJ, Hawkins PN. Amyloidosis and the skin. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editors. Fitzpatrick′s dermatology in general medicine. 7 th edition. New York: McGraw-Hill; 2008. p. 1257-65.[Google Scholar]
Silverstein SR. Primary systemic amyloidosis and the dermatologist: Where classic skin lesions may provide the clue for early diagnosis. Dermatol Online J 2005;11:5.[Google Scholar]
Palladini G, Perfetti V, Obici L, Caccialanza R, Semino A, Adami F, et al. The association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis ineligible for stem cell transplantation. Blood 2004;103:2936-8.[Google Scholar]
Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, et al. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: An 8-year study. Ann Intern Med 2004;140:85-93.[Google Scholar]
Kyle RA, Rajkumar SV. Treatment of multiple myeloma: a comprehensive review. Clin Lymphoma Myeloma 2009;9:278-88.[Google Scholar]