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Riegert-Johnson DL, Boardman LA, Hefferon T, et al., editors. Cancer Syndromes [Internet]. Bethesda (MD): National Center for Biotechnology Information (US); 2009-.

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Cancer Syndromes [Internet].

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Appendix: The original description of perifollicular fibromas (Birt-Hogg-Dubé syndrome.)

and .

Published as “Multiple subcutaneous peripilar fibromas of the neck” by Burnier and Rejsek in 1925. Translated from French ( 1 ).

Mrs. Charlotte M…, 23 years old, came for a visit in September 1924 for several papular elements, sitting for 6 years on the neck and skin of the back of the neck. Their number is about 100, their size varies from the size of a grain of couscous to that of a pea (picture 1), a couple sit as far as the chest, others on the cheeks.

Picture 1

Figure

Picture 1.

These small granulations, non pruriginous, covered by a dermis of normal color, are hard and resist when palpated with a finger: no liquid comes out if poked with a needle. Therefore, these are solid (not hollow) granulations/bodies.

A biopsy of one of these papular elements shows, above a normal epidermis, small, epidermal nodes made of conjunctive fibers and separated from each other by dermis and hypoderm collagen fibers (picture 2). As we examine the series of slices/scans, we realize that these fibromas develop exclusively around the hair follicles (picture 3). In the center of the tumor, by the exterior epithelial sheath of the hair, the conjunctive fibers form a light network which becomes denser on the external part of the tumor. These tumors are round or piroform, and they completely surround the hair follicle and its sebaceous glands (picture 4).

Picture 2

Figure

Picture 2.

Picture 3

Figure

Picture 3.

Picture 4

Figure

Picture 4.

Some of these hair follicles are, at their lower extremity, curbed at a right or obtuse angle; the epithelium of the follicle does not show any pathological alteration/modification. The tumors do not have any xanthomatous elements, nor any elastic fibers. The blood vessels are normal.

The patient had wished to get rid of these indolent but unaesthetic tumors, which got destroyed via electro-coagulation/clotting.

In this case, we are talking about small multiple under-skin fibromas of the neck and back of the neck exclusively developed around the hair follicle.

These types of tumors are usually relatively rare. Some small xanthomatous tumors look like these fibromas; but in the case of this patient, one could not detect, by the appropriate means, any hint of fat or cholesterol. The cholesterol level in the blood was also normal.

References

1.
Rejsek and Burnier, Fibromes sous-cutanes peripilares multiples du cou. Bull Soc francq dermat et syph, 1925. 32: p. 242-243.
Copyright © 2009-, Douglas L Riegert-Johnson.
Bookshelf ID: NBK45327

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