Lichen planopilaris L66.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 01.02.2022

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Definition
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Chronic primary lymphocytic folliculitis, which is bound to the hair follicles and leads to creeping follicular atrophy and alopecia.

Etiopathogenesis
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Unknown. Probably programmed chronic cornification disorder of the follicular epithelium with consecutive destruction of the follicle and hair shaft. A loss of CK15 (cytokeratin 15)-positive stem cells was detected. The relationships to the following diseases are not clarified: lichen planus (follicularis), keratosis pilaris, folliculitis decalvans.

Manifestation
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Occurring in middle age. f:m = 8:2.

Localization
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Localized at the capillitium, especially in the parietal and frontal region. Rarely occipital involvement.

Clinical features
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Diffusely distributed over a smaller or also larger area, pinhead-sized, follicular, pale to strong red colored, ruff-like erythema arranged around the hair follicle, giving way in the course of months to 0.05 - 0.1 cm large, reddened papules with pointed hyperkeratosis/follicular hyperkeratosis ("pilar casts") with or without central hair.

Older "burned out" areas show atrophic scarring, smooth mirroring (scarring) alopecia without erythema or follicular structures(pseudopelade).

In some patients, there is concomitant evidence of a still present or already burned out keratosis follicularis (atrophicans) on the extensor sides of the upper arms or thighs and/or an ulerythema ophryogenes.

In other patients, evidence of lichen planus (oral mucosa, nails) is found.

Histology
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Interface dermatitis with lymphocytic infiltration of the infundibulum and the isthmus of the follicle

External therapy
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Symptomatic: Topical glucocorticoids.

Internal therapy
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Experimental approaches in smaller studies:

  • Application of oral tetracyclines.
  • Moderately good clinical results have been described with the use of hydroxychloroquine and oral retinoids.
  • There are reports of systemic use of low dose methotrexate or cyclosporine A.

Operative therapie
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In the burnt out stage hair transplants can be recommended.

Note(s)
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The term "lichen planopilaris" is used inconsistently. In the Anglo-American language it is used synonymously with"lichen planus follicularis". This equation would make the term "lichen planopilaris" superfluous and rather lead to confusion.

Terminologically clearer is the term lichen (ruber) planus follicularis (capillitii).

The clinical picture described here characterizes lichen planopilaris as an atrophic, follicular keratinization disorder. The relationship of lichen planopilaris to Graham-Little syndrome (in which a combination of lichen planus capillitii and characteristic skin or mucosal changes is a prerequisite) and to keratosis pilaris decalvans (syn: keratosis follicularis spinulosa decalvans) and postmenopausal frontal fibrosing alopecia has not been clarified, although there is no clear histological difference between the latter and lichen planopilaris (Gálvez-Canseco A et al. 2018).

It can be assumed that the clinical picture described here is a disparate symptom complex(keratosis pilaris syndrome), to which other clinical pictures described as entities belong, in particular keratosis follicularis, ulerythema ophryogenes, folliculitis ulerythematosa reticulata, folliculitis decalvans.

Literature
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  1. Cevasco NC et al (20007) A case-series of 29 patients with lichen planopilaris: The Ceveland Clinic Foundation experience on evaluation, diagnosis and treatment. J Am Acad Dermatol 57: 47-53
  2. Chieregato C et al (2003) Lichen planopilaris: report of 30 cases and review of the literature. Int J Dermatol 42: 342-345
  3. Gálvez-Canseco A et al (2018) Lichen planopilaris and frontal fibrosing alopecia cannot be differentiated by histopathology. J Cutan Pathol doi: 10.1111/cup.13112.
  4. Gupta SN, Palceski D (2003) Lichen planopilaris presenting as truncal alopecia: a case presentation and review of the literature. Cutis 72: 63-66
  5. Habashi-Daniel A et al.(2014) Absence of catagen/telogen phase and loss of cytokeratin 15 expression in hair follicles in lichen planopilaris. J Am Acad Dermatol 71:969-972
  6. Lyakhovitsky A et al (2014) A case series of 46 patients with lichen planopilaris: Demographics, clinical evaluation, and treatment experience. J Dermatologist Treat 1:1-5
  7. Mirmirani P et al (2003) Short course of oral cyclosporine in lichen planopilaris. J Am Acad Dermatol 49: 667-671
  8. Vaisse V et al (2003) Postmenopausal frontal fibrosing alopecia: 20 cases. Ann Dermatol Venereol 130: 607-610

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

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Last updated on: 01.02.2022