Keratosis lichenoides chronica L85.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Kaposi Bureau Barrière Group Syndrome; Keratosis lichenoid striae; <lichenoid triceratosis; Lichenoid trikeratosis; Lichen ruber monileformis; lichen verrucosus et reticularis; Nekam's disease; striated porokeratosis; triceratosis lichenoid

History
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Kaposi, 1886; Wise and Rein, 1936; Nekam, 1938

Definition
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Rare, eminently chronic inflammatory skin disease of unclear etiology characterized by hyperkeratotic, considerably itchy papules, plaques and excoriations. Variant of a "hyperkeratotic" Lichen planus verrucosus? The first descriptor Moritz Kaposi described the clinical picture under the name: Lichen ruber acuminatus and Lichen ruber planus and thus created more confusion than a clear definition of the clinical picture.

Etiopathogenesis
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Unclear, variant of Lichen planus or hyperkeratosis follicularis et parafollicularis in cutem penetrans is discussed. A coincidence with anaplastic lymphoma has been described (single case report). Furthermore, infections (hepatitides, glomerulonephritides, tuberculosis) were discussed as triggers.

Manifestation
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Usually occurring around the age of 20 or 50; but in principle always possible (two-peaked incidence maximum curve). The clinical picture is also described for children.

Men are slightly more frequently affected than women (2:1?).

Localization
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Mostly spreading from a focus to large parts of the integument; trunk and extremity extensor sides are preferred.

Clinical features
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Generalized, hyperkeratotic, reddish-brown itchy, lichenoid papules confluent to linear and reticular formations, more rarely to psoriasiform plaques.

The facial involvement is reminiscent of seborrheic eczema or atypical rosacea.

Infection of the oral mucosa ( aphthae, laryngitis) and eyes (keratoconjunctivitis, synechieformation) possible.

Palmoplantar keratoses are not uncommon. Furthermore, nail changes (raised hyperkeratotic nail fold, increased curvature of the nail plate).

Histology
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Acanthosis and orthohyperkeratosis, more rarely columnar parakeratosis over sunken epidermis, also follicular keratosis, vacuolar degeneration of the stratum basale, lichenoid lymphocytic infiltrate (interface dermatitis) with focal accumulations of cytoid corpuscles and plasma cells

External therapy
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Usually complete resistance to local therapeutic agents. Temporary improvement can be achieved with 5-10% urea-containing external agents (e.g. R102, basodexan ointment). Glucocorticoids lead to a temporary improvement.

Alternative: Vitamin A acid ointment/cream (e.g. Cordes VAS).

Alternative: Tacrolimus ointment.

Radiation therapy
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Sustained success has been described for systemic PUVA or PUVA bath therapy.

Internal therapy
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Retinoids: Acitretin (Neotigason) initial 0.5 mg/kg bw/day, maintenance therapy according to clinic. Possibly combination of PUVA therapy and retinoids according to RePUVA therapy. A combination of acitretin and methotrexate has also proved successful in one individual case (Li AW et al. 2017).

A positive response to an Efalizumab therapy was described as an individual case.

Note(s)
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The entity of this disease, described by Kaposi since 1895, is still disputed. Most authors assume that it is a variant of lichen planus, especially since neither histologically nor immunohistologically clear differences can be detected.

Literature
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  1. Avermaete A et al (2001) Keratosis lichenoides chronica: characteristics and response to acitretin. Br J Dermatol 144: 422-424
  2. Kaposi M (1895) Lichen ruber acuminatus and Lichen ruber planus. Arch Dermatol Syphilol 31: 1-32
  3. Kaposi M (1886) Lichen ruber moniliformis. Dermatol Syphil (Vienna) 13: 571-582
  4. Li AW et al (2017) Keratosis lichenoides chronica successfully treated with isotretinoin andmethotrexate
    .JAAD Case Rep 3:205-207.
  5. Muñoz-Santos C et al. (2009) Response of keratosis lichenoides chronica to efalizumab therapy. Arch Dermatol 145:867-869
  6. Nekam L (1938) Sur la question du lichen moniliforme. Press Med 51: 1000-1003
  7. Oyama N et al (2011) Juvenile-onset keratosis lichenoides chronica treated successfully with topical tacrolimus: a safe and favourable outcome. Eur J Dermatol 21:595-596
  8. Remling R et al (2002) Keratosis lichenoides chronica. Bath PUVA therapy. dermatologist 53: 550-553
  9. Skorupka M et al (1992) Keratosis lichenoides chronica. dermatologist 43: 97-99
  10. Wise F, Pure CR (1936) Lichen ruber moniliformis (morbus moniliformis lichenoides). Report of a case and description of a hitherto unrecorded histological structure. Arch Dermatol Syphilol (Chicago) 34: 830-849
  11. Zhou P et al (2014) Keratosis lichenoides chronica in association with primary cutaneous anaplastic large cell lymphoma. Int J Dermatol 53:e109-12

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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: 29.10.2020