title: An unusual presentation of mastocytosis: unilateral swelling of the vulva.
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.
authors.affiliation: Department of Pediatrics, Endocrinology, Dermatology, and Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.
authors.forename: Joydip
authors.initials: J
authors.lastname: Roy
authors.forename: Denise W
authors.initials: DW
authors.lastname: Metry
authors.forename: John
authors.initials: J
authors.lastname: Hicks
authors.forename: Alice J
authors.initials: AJ
authors.lastname: Morgan
authors.forename: Rubina A
authors.initials: RA
authors.lastname: Heptulla
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D001707:Biopsy, Needle; D017332:Cetirizine / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D004487:Edema / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D034701:Mastocytosis, Cutaneous / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D011899:Ranitidine / Q000627:therapeutic use; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014845:Vulvar Diseases / Q000175:diagnosis / Q000473:pathology*
chemical_list: D011899:Ranitidine; D017332:Cetirizine