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MULTIPLE CUTANEOUS GRANULAR CELL TUMORS WITH SYSTEMIC DEFECTS: A DISTINCT ENTITY?
Author(s) -
BAKOS LUCIO
Publication year - 1993
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4362.1993.tb02815.x
Subject(s) - medicine , pathology , dermatology
Background. The association between cutaneous granular cell tumors and systemic defects Is extremely rare, this being the tenth case reported in the literature. The reported defects in the literature include lentiginosis, face and skull alterations, heart defects, muscular and neural pathologies, among other sporadic defects in other organs. The patients do not present visceral granular cell tumors. Case Report. An 11year‐old girl had 124 skin granular cell tumors associated with face and skull defects, pulmonary stenosis, EEG and other neurologic alterations, diffuse muscle hypotonia, and an excess of finger joint mobility. Conclusions. The combination of multiple cutaneous granular cell tumors with repeated alterations in other organs seems to be more than simply coincidental, thus suggesting a syndromic separate entity.