Resumo

MAQUEDA-ZAMORA, Gloria  e  SIERRA-SANTOS, Lucía. Multiplepilomatrixomas in children: syndromic or chance association?. Rev Clin Med Fam [online]. 2019, vol.12, n.3, pp.143-146.  Epub 21-Out-2019. ISSN 2386-8201.

Pilomatrixomas are infrequent neoplasms of the follicular tissue. In 70% of cases they usually appear in the first two decades of life, and are most often distributed over the head and neck. They usually occur in isolation, so multiple pilomatrixomas are very rare. These are connected with genetic syndromes, especially Steinert myotonic dystrophy which may occur some time after the appearance of the pilomatrixomas. Therefore these require follow-up. A correct identification in primary care is essential, as well as referral to a dermatology specialist, since treatment involves their removal and the ruling out and follow-up of possible associated syndromes. There are case reports of malignant pilomatrixomas (pilomatrix carcinoma).

We present a case of multiple pilomatrixomas seen in primary care, which reviews the pathology and considers associated syndromes. Although accurate diagnosis is histological and treatment is surgical, the family doctor is the first physician who cares for the patient. Thus, their correct diagnostic orientation based on clinical examination, correlation and referral can speed up the process and prevent the possible complications of associated syndromes.

Palavras-chave : Pilomatrixoma; Skin Neoplasms; Neoplasms; Adnexal and Skin Appendage.

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