Keratosis pilaris syndrome (overview) Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 21.04.2021

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Synonym(s)

Atrophying keratosis pilaris; keratosis pilaris atrophicans; keratosis pilaris rubra

Definition
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Term that summarizes the different, mostly genetically determined clinical pictures of the "atrophizing keratosis pilaris group", which are characterized by a uniform pathogenetic principle of chronic creeping follicular atrophy. The atrophizing follicular keratosis disorders are characterized by:

  • follicular hyperkeratosis (friction skin)
  • perifollicular and interfollicular erythema (keratosis pilaris rubra, see also erythema perstans faciei)
  • follicular atrophies leading to alopecia in different hair types (vellus hair, bristly hair, long hair)

The keratosis pilaris syndrome can present itself in different mono- or polytopic forms.

Classification
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Keratosis pilaris syndrome includes the following clinical pictures in different manifestations and degrees of severity, which can occur in different combinations:

External therapy
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Internal therapy
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  • Acitretin (Neotigason): Start with 25 mg/day p.o. for 2-4 weeks, then reduce the dose to 10 mg/day or to 5 mg/day depending on the skin condition. Cave! Contraception! In the case of extensive complaints (severe itching), a low-dose glucocorticoid treatment can be combined with acitretin therapy, e.g. prednisolone (e.g. Decortin H) 20-40 mg/day with rapid stepwise dose reduction.
  • Glucocorticoids: If possible, a long-term internal therapy with glucocorticoids should be avoided due to the chronicity of the clinical picture. If necessary, short-term shock therapy at medium-high dosage with rapid dose reduction. In combination with acitretin, rapid therapeutic success is usually achieved, especially at the beginning of therapy.

Operative therapie
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Dermabrasion or cryosurgery (open spray method) are also suitable for the treatment of circumscribed areas when used with practice.

Note(s)
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In the literature of the past century, different terms circulate for one and the same clinical picture or for the different manifestations (keratosis pilaris, keratosis follicularis, lichen pilaris, lichen spinulosus, lichen spinulosis decalvans, etc.). To be excluded is the genodermatosis first described by Siemens, the "keratosis follicularis spinulosa decalvans". Furthermore, acquired clinical pictures characterized by follicular keratoses as e.g. the vitamin A-deficient phrynoderm.

In addition, atrophic keratosis pilaris is frequently confused with lichen planus follicularis, which also leads to follicular atrophy but is based on a fundamentally different pathogenetic principle.

Literature
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  1. Azambuja R et al (1987) Ulerythema ophryogenes and Folliculitits ulerythemtosa reticulata. dermatologist 38: 411-413
  2. Ehsani A et al (2003) Unilaterally generalized keratosis pilaris. J Eur Acad Dermatol Venereol 17: 361-362

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 21.04.2021