Lichen planus follicularis L66.10

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 03.08.2023

Synonym(s)

acuminate lichen; lichen acuminatus; Lichen plan péripilaire; lichen ruber acuminatus

Definition
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Variant of lichen planus linked to the hair follicles. The disease can occur ubiquitously, but manifests itself preferentially in the neck region and on the capillitium(lichen planus follicularis capillitii). In case of conically pointed follicular hyperkeratosis, it is also called lichen planus acuminatus (lichen ruber acuminatus) (see Fig.).

The occurrence of lichen planus follicularis on the trunk with lichen planus of the main hair (with pseuopelade condition) as well as dystrophic nail changes is also called Graham-Little syndrome.

Manifestation
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Occurring in middle age, women are more frequently affected than men.

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Localization
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Occurs mainly on the capillitium. More rarely, the upper trunk, extremities in joint bends, neck, inner thighs or sacral region are affected. S.a.u. Lichen planus follicularis capillitii.

Clinical features
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Diffusely distributed over a larger area, pinhead-sized, follicular, delicately to strongly red colored, ruffle-like erythema arranged around the hair follicle, which gives way in the course of months to 0.1-0.2 cm large, reddened papules with pointed hyperkeratosis without central hair. Older burnt areas show an atrophically scarred, smooth reflecting surface without erythema or follicular structures.

Histology
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Dense adnexotropic perifollicular lymphocytic infiltrate. Vacuolar degeneration of the basal, adnexal keratinocytes. Acanthosis of the follicular epithelium with amphora-like widening of the excretory duct. In this case, hair structures are often no longer detectable, but only ortho- and parakeratotic horn masses.

Differential diagnosis
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Lichen planopilaris as a partial symptom of keratosis pilaris

Tuberculosis cutis lichenoides: Very rare clinical picture; symmetrically arranged, disseminated or also grouped, always follicular or perifollicular, pointed conical, yellow-brown, reddish or skin-colored, lichenoid papules, sometimes solitary, sometimes also confluent.

Pityriasis rubra pilaris (pityriasis rubra pilaris type 1/classic form): extensive pityriasiform siliceous, often marginal, sharply demarcated, follicular ("nutmeg grater"), erythema, also lichenified papules or plaques on the capillitium, face (especially eyebrows, nasolabial folds), also on the trunk.

Keratosis follicularis

Ulerythema ophryogenes.

External therapy
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Glucocorticoid ointments 1-2 times/day on the capillitium. S.u. Lichen planus follicularis capillitii.

Internal therapy
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According to the Lichen planus.

Progression/forecast
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Quoad sanationem bad. Years of chronically creeping progress.

Note(s)
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The lichen planus follicularis can be nosologically distinguished from the lichen planopilaris, which is etiopathogenetically more likely to be counted among the follicular cornification disorders (variant of keratosis follicularis). To what extent the Lassueur-Graham-Little syndrome can be classified as a variant of lichen planus remains unclear at present.

Literature
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Chieregato C et al (2003) Lichen planopilaris: report of 30 cases and review of the literature. Int J Dermatol 42: 342-345
Gupta SN, Palceski D (2003) Lichen planopilaris presenting as truncal alopecia: a case presentation and review of the literature. Cutis 72: 63-66
Mirmirani P et al (2003) Short course of oral cyclosporine in lichen planopilaris. J Am Acad Dermatol 49: 667-671
Vaisse V et al (2003) Postmenopausal frontal fibrosing alopecia: 20 cases. Ann Dermatol Venereol 130: 607-610