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[Keratosis follicularis spinulosa decalvans. Therapy with isotretinoin and etretinate in the inflammatory stage].
Hautarzt 1993; 44(8):529-34H

Abstract

Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked disorder of keratinization of the hair follicle associated with corneal dystrophy. The clinical picture is characterized by solid follicular hyperkeratosis, especially on the exposed skin, sparse eyebrows/eyelashes, follicular scaling and scarring alopecia of the scalp, dry skin and ocular symptoms with keratitis and photophobia. We describe the three stages of the disease: onset, inflammation and partial remission and the treatment appropriate in each. Two patients in the inflammatory stage of KFSD, with recurrent deep, fibrosing folliculitis and perifolliculitis followed by spreading and scarring alopecia on the scalp, responded to oral therapy with retinoids. In both cases there was a distinct and lasting remission of the inflammation and stabilization of the spreading alopecia after treatment with etretinate (Tigason), up to 0.8 mg/kg body weight, or isotretinoin (Roaccutan), 0.5 mg/kg body weight, for 12 weeks. The follicular spinulous hyperkeratosis became softer, but persisted. Thus, oral therapy with retinoids appears helpful in the inflammatory stage of KFSD, even though there is little improvement in the follicular hyperkeratosis.

Authors+Show Affiliations

Haut- und Poliklinik, Medizinischen Hochschule Erfurt.No affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

ger

PubMed ID

8376108

Citation

Richard, G, and W Harth. "[Keratosis Follicularis Spinulosa Decalvans. Therapy With Isotretinoin and Etretinate in the Inflammatory Stage]." Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete, vol. 44, no. 8, 1993, pp. 529-34.
Richard G, Harth W. [Keratosis follicularis spinulosa decalvans. Therapy with isotretinoin and etretinate in the inflammatory stage]. Hautarzt. 1993;44(8):529-34.
Richard, G., & Harth, W. (1993). [Keratosis follicularis spinulosa decalvans. Therapy with isotretinoin and etretinate in the inflammatory stage]. Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete, 44(8), pp. 529-34.
Richard G, Harth W. [Keratosis Follicularis Spinulosa Decalvans. Therapy With Isotretinoin and Etretinate in the Inflammatory Stage]. Hautarzt. 1993;44(8):529-34. PubMed PMID: 8376108.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Keratosis follicularis spinulosa decalvans. Therapy with isotretinoin and etretinate in the inflammatory stage]. AU - Richard,G, AU - Harth,W, PY - 1993/8/1/pubmed PY - 1993/8/1/medline PY - 1993/8/1/entrez SP - 529 EP - 34 JF - Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete JO - Hautarzt VL - 44 IS - 8 N2 - Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked disorder of keratinization of the hair follicle associated with corneal dystrophy. The clinical picture is characterized by solid follicular hyperkeratosis, especially on the exposed skin, sparse eyebrows/eyelashes, follicular scaling and scarring alopecia of the scalp, dry skin and ocular symptoms with keratitis and photophobia. We describe the three stages of the disease: onset, inflammation and partial remission and the treatment appropriate in each. Two patients in the inflammatory stage of KFSD, with recurrent deep, fibrosing folliculitis and perifolliculitis followed by spreading and scarring alopecia on the scalp, responded to oral therapy with retinoids. In both cases there was a distinct and lasting remission of the inflammation and stabilization of the spreading alopecia after treatment with etretinate (Tigason), up to 0.8 mg/kg body weight, or isotretinoin (Roaccutan), 0.5 mg/kg body weight, for 12 weeks. The follicular spinulous hyperkeratosis became softer, but persisted. Thus, oral therapy with retinoids appears helpful in the inflammatory stage of KFSD, even though there is little improvement in the follicular hyperkeratosis. SN - 0017-8470 UR - https://www.unboundmedicine.com/medline/citation/8376108/[Keratosis_follicularis_spinulosa_decalvans__Therapy_with_isotretinoin_and_etretinate_in_the_inflammatory_stage]_ L2 - http://www.diseaseinfosearch.org/result/3982 DB - PRIME DP - Unbound Medicine ER -