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Giornale Italiano di Dermatologia e Venereologia 2001 August;136(4):287-91

Copyright © 2001 EDIZIONI MINERVA MEDICA

language: Italian

Keratosis follicularis spinulosa decalvans. A clinical case

Ridolfi R., Paolinelli M., Mattutini G., Giangiacomi M., Cataldi I.

Università degli Studi - Ancona Clinica Dermatologica *Istituto di Anatomia Patologica


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The case described refers to a 23 years old man who came to our attention, because of a serious alopecia of the scalp, which had flared up two years previously. From the objective examination of the scalp, erythemato-pustolosis lesions over wide scarred areas were observed, while keratosis follicularis was present on limbs, torso and eyebrows. During the anamnesis, it turned out that his younger brother presented similar lesions. Histological examination of a biopsy specimen of the scalp showed hyperkeratosis on the upper part of the follicle and an inflammatory infiltration of the derma with partially disrupted follicles. The clinical features and the histological examination lead us to diagnose keratosis follicularis spinulosa decalvans (KFSD). KFSD, described for the first time by Siemens in 1925, is a rare kind of atrophic pilaris keratosis, which is transmitted in an X-linked recessive mode. The manifestation starts in infancy with photophobia, and usually improves during adolescence; then keratosic follicularis papulas appear on the scalp, torso and limbs. Corneal dystrophy, keratodermie and atopia can also be present. The disease has a progressive and chronic course; it does not respond well to any treatment. In the case, presented, a therapeutic cycle with etetrinate leads to fairly good improvement of the inflammatory active lesions. The rarity of this disease, still paradigmatic in its clinical expression, induced us to describe this case.

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