Keratosis pilaris syndrome (overview) Q82.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 22.08.2022

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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 syndromes in varying manifestation and severity, which may occur in different combinations. The classification presented here is based on morphological-clinical and histological data. Valid genetic data are currently lacking.

Vellus hair follicular keratoses

Long hair follicular keratoses

Remark: The long hair follicular keratoses are very often associated with keratosis pilaris hereditaria simplex.

Histology
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The main histologic and dermoscopic findings of KP are hyperkeratosis, hypergranulosis, mild lymphocytic inflammation dominated by type 1 T helper cells, plugging of follicular orifices, conspicuous absence of sebaceous glands, and abnormalities of the hair shaft in KP lesions but not in unaffected skin sites. Changes in barrier function and abnormal paracellular permeability can be detected in both the interfollicular and follicular stratum corneum of lesional KP, which correlated ultrastructurally with impaired maturation and organization of the extracellular lamellar bilayer. All of these features were independent of filaggrin genotype. Ultrastructurally, the corneodesmosomes and tight junctions appear normal (Gruber R et al. 2015).

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 dose reduction to 10 mg/day or to 5 mg/day depending on skin findings. Caveat. Contraception! In case of extensive symptoms (severe itching), low-dose glucocorticoid treatment can be combined with acitretin therapy, e.g. prednisolone (e.g. Decortin H) 20-40 mg/day with rapid gradual dose reduction.

Glucocorticoids: If possible, long-term internal therapy with glucocorticoids should be avoided because of the chronicity of the clinical picture. If necessary, short-term shock therapy at medium-high doses with rapid dose reduction. In combination with acitretin, a rapid therapeutic success can usually be observed, 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.
  3. Gruber R et al (2015) Sebaceous gland, hair shaft, and epidermal barrier abnormalities in keratosis pilaris with and without filaggrin deficiency. Am J Pathol 185:1012-1021

Disclaimer

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

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Last updated on: 22.08.2022