Cutaneous manifestations of the eosinophilia-myalgia syndrome.
We report the cutaneous manifestations of the eosinophilia-myalgia syndrome in 10 patients, with specific reference to their clinical course, histopathological features, and immunogenetic studies. Cutaneous manifestations could be classified into three groups: morphoea-like sclerosis, urticarial and papular lesions, and generalized sclerosis. Despite this polymorphic clinical presentation, the histopathological abnormalities in all cases were strikingly similar, and consisted of superficial and deep perivascular lymphocytic dermal infiltrates, mucin deposition, and fascial inflammation (often in the absence of sclerosis). Immunoperoxidase studies revealed increased numbers of factor XIIIa- and MAC 387-positive cells in the inflammatory infiltrate. Immunogenetic studies demonstrated that 77% (7/9) of patients possessed the HLA-DR3 or HLA-DR4 phenotypes. Mean follow-up of 24 months after discontinuation of L-tryptophan revealed the presence of persistent severe disabling disease in 30% of patients.
Oursler
J R
JR
Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, MD.
Farmer
E R
ER
Roubenoff
R
R
Mogavero
H S
HS
Watson
R M
RM
D000328:Adult; D000368:Aged; D016603:Eosinophilia-Myalgia Syndrome / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D008875:Middle Aged; D012594:Scleroderma, Localized / Q000473:pathology; D012595:Scleroderma, Systemic / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology / Q000473:pathology*; D014581:Urticaria / Q000473:pathology
D006680:HLA Antigens