Tags

Type your tag names separated by a space and hit enter

Clinical findings, cutaneous pathology, and response to therapy in 21 patients with keratosis pilaris atrophicans.
Arch Dermatol 1994; 130(4):469-75AD

Abstract

BACKGROUND

Keratosis pilaris atrophicans defines a group of cutaneous disorders characterized by follicular hyperkeratosis and scarring. X-linked dominant inheritance has recently been reported in a Dutch family with a form of keratosis pilaris atrophicans defined as keratosis follicularis spinulosa decalvans, with males more severely affected and having corneal involvement. The clinical manifestations observed in different families by others and ourselves did not follow that pattern, suggesting genetic heterogeneity. We report our experience with 21 unrelated individuals.

RESULTS

There were 15 male and six female patients whose onset of the skin disease was in early childhood but with scalp involvement occurring in the teen years. The cutaneous lesions consisted of follicular papules with scalp involvement present in eight individuals. Half the women had scalp involvement, and one female and one male had eye changes. Familial involvement was observed in three patients and was compatible with dominant inheritance. Histopathologic examination revealed hyperkeratosis of the upper follicle with an inflammatory response that resulted in follicular destruction. Response to therapy including keratolytics, antibiotics, corticosteroids and retinoids was limited.

CONCLUSIONS

Our findings support the hypothesis that there is genetic and clinical heterogeneity among the disorders represented by the term keratosis pilaris atrophicans. The cause of these diseases may be a disorder of the keratinocyte, which is responsible for inducing both the hyperkeratosis and inflammatory changes.

Authors+Show Affiliations

Department of Dermatology, Harvard Medical School, Massachusetts General Hospital, Boston.No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

8166484

Citation

Baden, H P., and H R. Byers. "Clinical Findings, Cutaneous Pathology, and Response to Therapy in 21 Patients With Keratosis Pilaris Atrophicans." Archives of Dermatology, vol. 130, no. 4, 1994, pp. 469-75.
Baden HP, Byers HR. Clinical findings, cutaneous pathology, and response to therapy in 21 patients with keratosis pilaris atrophicans. Arch Dermatol. 1994;130(4):469-75.
Baden, H. P., & Byers, H. R. (1994). Clinical findings, cutaneous pathology, and response to therapy in 21 patients with keratosis pilaris atrophicans. Archives of Dermatology, 130(4), pp. 469-75.
Baden HP, Byers HR. Clinical Findings, Cutaneous Pathology, and Response to Therapy in 21 Patients With Keratosis Pilaris Atrophicans. Arch Dermatol. 1994;130(4):469-75. PubMed PMID: 8166484.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical findings, cutaneous pathology, and response to therapy in 21 patients with keratosis pilaris atrophicans. AU - Baden,H P, AU - Byers,H R, PY - 1994/4/1/pubmed PY - 1994/4/1/medline PY - 1994/4/1/entrez SP - 469 EP - 75 JF - Archives of dermatology JO - Arch Dermatol VL - 130 IS - 4 N2 - BACKGROUND: Keratosis pilaris atrophicans defines a group of cutaneous disorders characterized by follicular hyperkeratosis and scarring. X-linked dominant inheritance has recently been reported in a Dutch family with a form of keratosis pilaris atrophicans defined as keratosis follicularis spinulosa decalvans, with males more severely affected and having corneal involvement. The clinical manifestations observed in different families by others and ourselves did not follow that pattern, suggesting genetic heterogeneity. We report our experience with 21 unrelated individuals. RESULTS: There were 15 male and six female patients whose onset of the skin disease was in early childhood but with scalp involvement occurring in the teen years. The cutaneous lesions consisted of follicular papules with scalp involvement present in eight individuals. Half the women had scalp involvement, and one female and one male had eye changes. Familial involvement was observed in three patients and was compatible with dominant inheritance. Histopathologic examination revealed hyperkeratosis of the upper follicle with an inflammatory response that resulted in follicular destruction. Response to therapy including keratolytics, antibiotics, corticosteroids and retinoids was limited. CONCLUSIONS: Our findings support the hypothesis that there is genetic and clinical heterogeneity among the disorders represented by the term keratosis pilaris atrophicans. The cause of these diseases may be a disorder of the keratinocyte, which is responsible for inducing both the hyperkeratosis and inflammatory changes. SN - 0003-987X UR - https://www.unboundmedicine.com/medline/citation/8166484/Clinical_findings_cutaneous_pathology_and_response_to_therapy_in_21_patients_with_keratosis_pilaris_atrophicans_ L2 - https://jamanetwork.com/journals/jamadermatology/fullarticle/vol/130/pg/469 DB - PRIME DP - Unbound Medicine ER -