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Keratosis pilaris atrophicans

Authors
Megan N Landis, MD
Jennifer Perryman, MD
Section Editor
Joseph Fowler, MD
Deputy Editor
Rosamaria Corona, MD, DSc

INTRODUCTION

Keratosis pilaris atrophicans is a group of related disorders characterized by inflammatory keratotic papules that may result in alopecia and scarring. They include keratosis pilaris atrophicans faciei (also called ulerythema ophryogenes), atrophoderma vermiculatum, and keratosis follicularis spinulosa decalvans [1].

This topic will review the clinical presentation and management of keratosis pilaris atrophicans. Keratosis pilaris is discussed separately. (See "Keratosis pilaris".)

PATHOGENESIS

Keratosis pilaris atrophicans is caused by abnormal keratinization of the follicular infundibulum, resulting in obstruction of the growing hair shaft and inflammation. Chronic inflammation leads to fibrosis, atrophy, shrinkage of the hair bulb, and alopecia. The association with several congenital syndromes due to partial monosomy or deletion in chromosome arm 18p suggests that the genes regulating the follicular keratinization may be located on chromosome 18p. (See 'Associated syndromes' below.)

PATHOLOGY

The histopathologic features of keratosis pilaris atrophicans are nonspecific. In the early stages, there is an orthokeratotic keratin plug that blocks and dilates the orifice and upper portion of the follicular infundibulum (picture 1) [2]. A twisted hair shaft may be trapped within this keratotic material and a mild perivascular mononuclear cell infiltrate is usually present in the adjacent dermis. In later stages, dermal fibrosis and loss of hair follicles may be seen.

CLINICAL VARIANTS

There are three clinical variants of keratosis pilaris atrophicans: ulerythema ophryogenes, also called keratosis pilaris atrophicans faciei, atrophoderma vermiculatum, and keratosis follicularis spinulosa decalvans [3]. Clinical features common to all forms include involvement of the face, in particular of the lateral eyebrows and cheeks; prominent erythema; and progression to an atrophic stage with loss of hairs in the involved areas [4].

       

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Literature review current through: Nov 2016. | This topic last updated: Tue Oct 04 00:00:00 GMT 2016.
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