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CONGKNITAL MACULAR URTICARIA PIGMENTOSA COMPLICATED BY MASSIVE NODULAR MASTOCYTOSIS WITH SYSTMETIC INVOLVEMENT
Author(s) -
BAKOS LUCIO,
STORCK RONILDO,
NETTO ERNESTO SILVEIRA
Publication year - 1972
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1972.tb07455.x
Subject(s) - urticaria pigmentosa , medicine , malaise , dermatology , surgery , pathology
Summary.— A case of congenital macular urticaria pigmentosa ran a normal course untii puberty, when large cutaneous tumours appeared, associated with episodic flushing, malaise and gastrointestinal symptoms. There were repeated episodes of profuse bleeding after local trauma. Systemic mastocyte infiltrate was found in liver, lymph nodes and bones. Review of the literature on miastocytosis failed to show any similar case. Althoush the patient is now aged 37 and has survived surgical removal of cutaneous tumour masses, it is suggested that her prognosis is poor.