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Journal of the American Academy of Dermatology
Volume 38, n° 6
pages 919-928 (juin 1998)
Doi : 10.1016/S0190-9622(98)70588-2
accepted : 7 February 1998
Familial eosinophilic cellulitis, dysmorphic habitus, and mental retardation
 

Mark D.P. Davis, MB, MRCPI a, A.C. Brown, MDd, R.Dwain Blackston, MDe, Claudia Gaughf, MDc, Ellen A. Peterson, ASa, Gerald J. Gleich, MDb, Kristin M. Leiferman, MD a
Rochester, Minnesota, and Augusta, Atlanta, Georgia 
From the Departments of Dermatology,a Immunology and Medicine, b Mayo Clinic and Mayo Foundation, Rochester ; the Section of Dermatology, Medical College of Georgia, Augusta c ; the Atlanta Skin and Cancer Clinic, PCd; and the Department of Pediatrics, Division of Medical Genetics, Emory University, Atlanta.e 

Abstract

Background: Eosinophilic cellulitis is a polymorphous, chronic disease characterized by eosinophil infiltration and granulomatous inflammation. Objective: Our purpose was to describe the clinical, histologic, and immunohistologic findings in three family members who have had eosinophilic cellulitis since childhood associated with mental retardation and abnormal body habitus. Methods: Family members were evaluated. Multiple skin biopsy specimens were obtained and examined after hematoxylin-and-eosin staining, by immunofluorescence and by electron microscopy. Blood specimens were analyzed by immunoassays for eosinophil granule proteins and eosinophil active cytokines. Results: Three short-statured, mentally retarded family members with abnormal body habitus in at least two generations had recurrent eosinophilic cellulitis. Peripheral blood and bone marrow eosinophilia was present. Plasma eosinophil granule major basic protein and eosinophil-derived neurotoxin levels were elevated with normal plasma eosinophil cationic protein levels. Eosinophil survival in culture was increased by patients’ plasma and was blocked with monoclonal interleukin-5 antibody. The level of plasma interleukin-5 was elevated. Lesional skin biopsy specimens showed massive staining for three eosinophil granule proteins. Electron microscopy showed eosinophil disruption. Conclusion: Eosinophilic cellulitis, mental retardation, and abnormal body habitus were likely inherited as a dominant syndrome in this family in which eosinophil involvement was striking. (J Am Acad Dermatol 1998;38:919-28.)

The full text of this article is available in PDF format.

 Supported in part by National Institutes of Health grants AR 36008, AI 15231, AI 34577; the Kieckhefer Foundation (Prescott, Ariz.), and the Mayo Foundation.
 Reprint requests: Kristin M. Leiferman, MD, Department of Dermatology, Mayo Clinic, 200 First St. SW, Rochester, MN 55905.
 0190-9622/98/$5.00 + 0   16/1/89375



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