Plum-colored tender plaques, palmar xanthoma and IgM gammapathy

presented by

Omid Zargari MD (1) and

Catherine Kiavash MD (2), Rasht, Iran

on December 19, 2004

(1) Assistant Professor of Dermatology (2) Resident, Department of Dermatology, Razi Hospital, Guilan University of Medical Sciences, Rasht, Iran

Abstract

1. Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349

2. Von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994;31(4):557-60

Acute febrile neutrophilic dermatosis, first described in 1964 by Robert Douglas Sweet, has been termed Sweet's syndrome. Classic Sweet's syndrome occurs in middle-aged women after a nonspecific infection of the respiratory or gastrointestinal tract. Raised erythematous plaques with pseudoblistering and occasionally pustules occur on the face, neck, chest, and extremities, accompanied by fever and general malaise. Involvement of the eyes, joints, and oral mucosa as well as internal manifestations of Sweet's syndrome in the lung, liver, kidneys, and central nervous system has been described. The disease is thought to be a hypersensitivity reaction. Parainflammatory (e.g., infections, autoimmune disorders, vaccination) and paraneoplastic (e.g., hemoproliferative disorders, solid malignant tumors) occurrence is found in approximately 25% of the cases and 2% are associated with pregnancy. Sweet's syndrome responds rapidly to systemic therapy with corticosteroids but recurs in about 25% of the cases. Alternative treatment modalities (e.g., potassium iodide, colchicine, dapsone, clofazimine, cyclosporine) have also been used. This article presents data from 38 patients with Sweet's syndrome and reviews its epidemiology, clinical spectrum, histologic features, laboratory results, differential diagnosis, pathogenic mechanisms, associated diseases, and treatment.
(J Am Acad Dermatol 1994 Oct;31(4):535-56; quiz 557-60)

3. Modiano P. et al. [Normolipemic plane xanthoma, monoclonal gammopathy, anti-lipoprotein activity, hypocomplementemia]
Ann Dermatol Venereol. 1995;122(8):507-8.