CLINICAL FINDINGS, CUTANEOUS PATHOLOGY, AND RESPONSE TO THERAPY IN 21PATIENTS WITH KERATOSIS PILARIS ATROPHICANS

Authors
Citation
Hp. Baden et Hr. Byers, CLINICAL FINDINGS, CUTANEOUS PATHOLOGY, AND RESPONSE TO THERAPY IN 21PATIENTS WITH KERATOSIS PILARIS ATROPHICANS, Archives of dermatology, 130(4), 1994, pp. 469-475
Citations number
22
Categorie Soggetti
Dermatology & Venereal Diseases
Journal title
ISSN journal
0003987X
Volume
130
Issue
4
Year of publication
1994
Pages
469 - 475
Database
ISI
SICI code
0003-987X(1994)130:4<469:CFCPAR>2.0.ZU;2-9
Abstract
Background: Keratosis pilaris atrophicans defines a group of cutaneous disorders characterized by follicular hyperkeratosis and scarring. X- linked dominantinheritance has recently been reported in a Dutch famil y with a form of keratosis pilaris atrophicans defined as keratosis fo llicularis spinulosa decalvans, with males more severely affected and having corneal involvement. The clinical manifestations observed in di fferent families by others and ourselves did not follow that pattern, suggesting genetic heterogeneity. We report our experience with 21 unr elated individuals. Results: There were 15 male and six female patient s whose onset of the skin disease was in early childhood but with scal p involvement occurring in the teenyears. The cutaneous lesions consis ted of follicular papules with scalp involvement present in eight indi viduals. Half the women had scalp involvement, andone female and one m ale had eye changes. Familial involvement was observed inthree patient s and was compatible with dominant inheritance. Histopathologic examin ation revealed hyperkeratosis of the upper follicle with an inflammato ryresponse that resulted in follicular destruction. Response to therap y including keratolytics, antibiotics, corticosteroids and retinoids w as limited. Conclusions: Our findings support the hypothesis that ther e is genetic and clinical heterogeneity among the disorders represente d by the term keratosis pilaris atrophicans. The cause of these diseas es may be a disorder of the keratinocyte, which is responsible for ind ucing both the hyperkeratosis and inflammatory changes.