Keratosis follicularis Q80.0

Authors: Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Martina Bacharach-Buhles, Julian Baur

All authors of this article

Last updated on: 18.12.2020

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Synonym(s)

Follicular keratoses; ichthyosis anserina; Ichthyosis scrophulosorum; keratosis pilaris; lichen pilaris; suprafollicular keratosis

Definition
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Common, completely harmless cornification disorder of the hair follicles, probably autosomal dominant inheritance. Occasionally combined with mild or pronounced ichthyosis vulgaris and in atopic diathesis.

Occurrence/Epidemiology
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The prevalence of keratosis follicularis is reported to be almost 50% of the population.

Manifestation
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Especially in young girls but also in boys at puberty age. The "disease" usually extends over the entire life span. In the area of the cheeks, persistent erythema(erythema perstans faciei - type rusticanus) can manifest itself. Largely unnoticed over the course of years, a complete loss of vellus hair can occur on the extensor side of the extremities, including the facial region. This is perceived by women as an attractiveness enhancing rather than a "disease". The situation is different with the loss of eyebrows or frontal forehead hairs. This manifestation is also possible. Here, the follicle-destroying process runs chronically and insidiously over years and cannot be influenced by therapy.

Localization
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Mainly extensor sides of the upper arms, outer side of the upper and lower legs, glutaeal region are affected.

Clinical features
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Innumerable horn droplets attached to the follicles, mostly skin-coloured, pointed conical, protruding above the skin level. Keratosis follicularis (synonym = keratosis pilaris) can affect body regions covered with vellus hair as well as those with bristle hair (e.g. eyebrows) and long hair (capillitium).

The follicle environment may be skin-coloured without reaction, but may also be characterised by a ring-shaped erythema (keratosis follicularis rubra).

There is a rubbing sensation when rubbing over the follicle.

Regular acrocyanosis, also perniosis follicularis in the affected areas (type rusticanus).

When scraping out the keratoses, a curled hair often appears.

See also the explanations under the name"Keratosis pilaris syndrome".

Histology
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Hyperkeratosis of the follicular ostium and the supraseboglandular follicular epithelium mostly without perifollicular inflammatory reaction. Occasionally an upturned vellus hair enclosed by horny masses is found.

Differential diagnosis
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Therapy
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  • Nursing and keratolytic external measures. Lubricating lotions (base lotion (DAC)) or creams; possibly addition of urea in a 5-20% concentration (e.g. Nubral cream or 5% urea in excipial hydro- or lipolotio, see recipes), salicylic acid (2-3%) or common salt (5-10%), possibly combinations thereof. Even highly diluted (0.005%) tretinoin creams can improve the skin condition.
  • It is important to use cleansing agents such as syndets or soaps sparingly in personal hygiene. Instead of detergents use hydrophilic body oils as a substitute for washing (e.g. hydrophilic body oil or ready-to-use oil baths which are usually used as oil baths). Use oil or saline baths for bathing.
  • Internal treatment measures are not indicated.

Progression/forecast
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Significant improvement with increasing age. The cause of this is the follicular atrophy that sets in with the years and at the same time leads to the loss of terminal hair on the affected skin areas.

Notice!

This gradual loss of follicles is not, however, perceived as a "symptom of illness"! S.a. Keratosis follicularis spinulosa decalvans.

Note(s)
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Keratosis follicularis is more frequently combined with a light-skinned skin type as well as varying degrees of obesity.

Literature
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  1. Gruber R et al (2015) Sebaceous gland, hair shaft, and epidermal barrier abnormalities in keratosis pilaris with and without filaggrin deficiency. On J cathetus 185:1012-1021
  2. Ibrahim O et al (2014) Treatment of keratosis pilaris with 810-nm diode laser: a randomized clinical trial. JAMA Dermatol 151:187-19
  3. Ma H et al (2015) Unilateral keratosis pilaris occurring on linear hypopigmentation patches: a new variant of keratosis pilaris in an Asian? J Dermatol 42:437-438
  4. Shimizu A et al (2016) Generalized keratosispilaris-like eruptions in a chronic myelogenous leukemia patient treated with nilotinib. J Dermatol doi: 10.1111/1346-8138.13336.

Disclaimer

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