Fibrofolliculoma - Altmeyers Encyclopedia

Definition
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Very rare, benign epithelial adnexal tumor of the skin with follicular differentiation, originating from connective tissue and epithelial parts of the hair shaft.

Manifestation
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Mainly occurring in adulthood.

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Clinical features
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Multiple, very rarely solitary, 2-5 mm large, yellowish-white, firm papules, usually in combination with other tumors originating from the hair shaft connective tissue (e.g. trichodiscoma in Birt-Hogg-Dubé syndrome) or with a connective tissue nevus.

Histology
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Sharply defined tumour located in the dermis with numerous thin strands anastomosing with each other and extending from the upper part of the follicular epithelium, which may also contain small sebaceous gland parts. These epithelial strands protrude finger-shaped into a thick mantle of basophilic, mucoid, connective tissue stromas.

Differential diagnosis
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Trichofolliculoma; mantleoma; neurofollicular hamartoma.

Therapy
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If necessary, excision (without safety distance).

Literature
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Gotz G et al (2001) Multiple fibrofolliculomas. Dermatologist 52: 824-827
Hey K et al (1996) Multiple fibrofolliculomas and glandular cysts of the stomach. Act dermatol 22: 71-73
Kupres KA et al (2003) Numerous asymptomatic facial papules and multiple pulmonary cysts: a case of Birt-Hogg-Dubé syndrome. Cutis 72: 127-131
Weintraub R et al (1977) Multiple fibrofolliculomas (Birt-Hogg-Dubé) associated with a large connective tissue naevus. J Cutan Pathol 4: 289-299