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August 1978

Acute Febrile Neutrophilic Dermatosis Associated With Sjögren's Syndrome

Author Affiliations

From the Departments of Dermatology (Drs Prystowsky and Goette) and Oral Medicine (Dr Daniels), and the Division of Rheumatology (Dr Fye), University of California School of Medi-; cine, San Francisco.

Arch Dermatol. 1978;114(8):1234-1235. doi:10.1001/archderm.1978.01640200086030

Sweet's syndrome (acute febrile neutrophilic dermatosis)1,2 is a dermatologic syndrome that is characterized by dull red, tender plaques with a predilection for the extremities and trunk. The lesions usually resolve within a month but recurrences are not infrequent. Histological features consist of extensive perivascular polymorphonuclear (PMN) infiltrates with prominent leukocytoclasis in the papillary and upper reticular dermis. The syndrome is frequently preceded by a febrile illness and is often associated with an elevated ESR, leukocytosis, fever, and a prompt response to corticosteroids. Conjunctivitis, episcleritis, arthralgia, and arthritis may occur.2

Leukemia,3,4 seminoma,5 and metastatic adenocarcinoma6 have occurred in association with Sweet's syndrome. We report the case of a patient in whom both Sweet's syndrome and Sjögren's syndrome developed simultaneously.

Report of a Case  This 53-year-old woman had a four-year history of arthralgia and myalgia and a three-year history of a "sandy" sensation in the eyes, polydipsia,