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Journal of the American Academy of Dermatology
Volume 48, n° 4
pages 508-516 (avril 2003)
Doi : 10.1067/mjd.2003.98
accepted : 17 September 2002
Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: Relationship to symptomatology, tryptase levels, and bone marrow pathology

Knut Brockow, MD, Cem Akin, MD, PhD, Mary Huber, RN, Dean D. Metcalfe, MD
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health. Bethesda, Maryland 


Background: Cutaneous involvement occurs in most patients with systemic mastocytosis. Objective: We sought to determine whether the extent of cutaneous involvement is predictive of systemic disease. Methods: In a prospective survey of 48 adults and 19 children, the extent and density of cutaneous lesions were compared with patient history, symptoms, internal organ involvement, serum total mast cell tryptase level, and bone marrow pathology. Results: Cutaneous lesions in children were of a greater mean and maximum diameter, but similar in extent and density compared with lesions in adults. In adults with skin lesions, the extent of lesions correlated to disease duration. Adults with extensive cutaneous disease experienced more pruritus and flushing. Fatigue, splenomegaly, and hepatomegaly were more frequent in adults without cutaneous involvement; and in those with a greater density of lesions and disease duration. Increased tryptase levels were found in children and adults with systemic disease and correlated to skin lesion density and bone marrow pathology. Conclusion: An examination of the extent and density of cutaneous lesions in adults helps identify those with more extensive extracutaneous disease and, thus, requiring a more thorough evaluation. (J Am Acad Dermatol 2003;48:508-16.)

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Abbreviations : BSA, CM, ISM, MCL, SM, SM-AHNMD, SSM, UP

 Supported by the National Institute of Allergy and Infectious Diseases Intramural Program.
 Conflict of interest: None identified.
 Reprint requests: Dean D. Metcalfe, MD, National Institutes of Health, National Institute of Allergy and Infectious Diseases, Laboratory of Allergic Diseases, Bldg 10, Room 11C205, 10 Center Dr, MSC 1881, Bethesda, MD 20892-1881. E-mail:

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