An Unusual Presentation of Mastocytosis: Unilateral Swelling of the Vulva

Authors

  • Joydip Roy M.D.,

    1. Department of Pediatrics, Endocrinology, Dermatology, and Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas
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  • Denise W. Metry M.D.,

    1. Department of Pediatrics, Endocrinology, Dermatology, and Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas
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  • John Hicks M.D.,

    1. Department of Pediatrics, Endocrinology, Dermatology, and Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas
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  • Alice J. Morgan R.N.,

    1. Department of Pediatrics, Endocrinology, Dermatology, and Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas
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  • Rubina A. Heptulla M.D.

    1. Department of Pediatrics, Endocrinology, Dermatology, and Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas
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  • Dr Heptulla is the recipient of the National Institute of Health grant DK065059.

Address correspondence to Rubina A. Heptulla, M.D., Texas Children's Hospital, 6621 Fannin St, Houston, TX 77030, or e-mail: heptulla@bcm.tmc.edu.

Abstract

Abstract: Mastocytosis is a primary, abnormal accumulation of mast cells associated with a broad range of local and systemic symptoms. We report two female adolescents with episodic, unilateral, swelling of the labia majora that was discovered to be an unusual presentation of mastocytosis. Mastocytosis is frequently misdiagnosed because of its rarity and variable clinical presentation, which often mimics other conditions. In the appropriate setting, mastocytosis should be included in the differential diagnosis of labium majus swelling.

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