DIAGNOSIS: Birt-Hogg-Dube syndrome.

Discussion

Histologically, a distorted hair follicle surrounded by a thickened, fibrotic stroma with eosinophilic collagen bundles is visible. Several thin strands of basaloid cells extend from the basal layer of the hair follicle into the stroma in a pattern typical of that seen in fibrofolliculoma.

Although solitary fibrofolliculomas can occur, they are seen relatively uncommonly and when present, the Birt-Hogg-Dube syndrome should be considered. This syndrome consists of multiple hamartomas of the perifollicular connective tissue including fibrofolliculomas, trichodiscomas and, acrochordons. There is commonly a history of other family members being affected as it is transmitted in autosomal dominant fashion although spontaneous mutations may occur. The lesions are asymptomatic and usually of cosmetic significance only. Association with medullary thryoid carcinoma, intestinal polyposis and bilateral renal cell carcinoma have been reported although these are rare. Lesions may be destroyed in a number of different fashions and recently, the use of copper vapor laser has been described.

References

Scully K, et al. Solitary fibrofolliculoma. J Am Acad Dermatol 1984;11:361-3.

Birt AR, Hogg GR, Dube WJ. Hereditary multiple fibrofolliculomas with Trichodiscomas and acrochordons. Arch Dermatol 1977;113:1674-7

Roth JS, et al. Bilateral renal cell carcinoma in the Birt-Hogg-Dube syndrome. J Am Acad Dermatol 1993;29: 1055-6.

Rongioletti F, et al. Fibrofolliculoma, tricodiscomas and acrochordons(Birt-Hogg-Dube) associated with intestinal polyposis. Clin Exp Dermatol 1989;14:72 -4.

Giroux JM, et al. Fibrofolliculoma. Treatment with copper vapor laser. Ann Dermatol Venereol 1994;121:130-3.