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Multiple perifollicular fibromas: report of a case and analysis of the literature
J M Junkins-Hopkins1, P H Cooper
1Department of Dermatology, University of Virginia Health Sciences Center, Charlottesville 22908.
Abstract:
Perifollicular fibroma is a cutaneous hamartomatous proliferation of the pilar connective tissue sheath. We describe a patient with multiple perifollicular fibromas and analyze the literature on this topic. Histologically, perifollicular fibroma is characterized by a concentric arrangement of collagen fibers surrounding a generally unaltered hair follicle. Clinically, it is usually multiple and occurs predominantly on the face and upper trunk. This clinical presentation is similar to that observed in patients with the Birt-Hogg-Dubé syndrome where, in addition to perifollicular fibromas, fibrofolliculomas, trichodiscomas, and acrochordons are found. Several reports of multiple perifollicular fibroma prior to the recognition of this syndrome may, in fact, represent cases of the Birt-Hogg-Dubé syndrome.
Insights
Multiple perifollicular fibromas, a skin growth, may indicate Birt-Hogg-Dubé syndrome. Further analysis of existing cases is recommended for accurate diagnosis.
Area of Science:
- Dermatology
- Genetics
Background:
- Perifollicular fibroma is a benign skin tumor originating from the hair follicle's connective sheath.
- Multiple occurrences suggest a potential underlying genetic predisposition.
Observation:
- A case of multiple perifollicular fibromas is presented.
- Clinical and histological features are analyzed in the context of existing literature.
Findings:
- Perifollicular fibromas exhibit a characteristic concentric collagen fiber arrangement around hair follicles.
- The clinical presentation of multiple lesions, primarily on the face and trunk, overlaps with Birt-Hogg-Dubé syndrome.
Implications:
- This overlap suggests that some previously reported cases of multiple perifollicular fibromas might be undiagnosed Birt-Hogg-Dubé syndrome.
- Accurate diagnosis is crucial for appropriate patient management and genetic counseling.
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