HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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.
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EtiopathogenesisThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
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?).
LocalizationThis section has been translated automatically.
Mostly spreading from a focus to large parts of the integument; trunk and extremity extensor sides are preferred.
Clinical featuresThis section has been translated automatically.
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).
HistologyThis section has been translated automatically.
Differential diagnosisThis section has been translated automatically.
External therapyThis section has been translated automatically.
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 therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
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)This section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- Avermaete A et al (2001) Keratosis lichenoides chronica: characteristics and response to acitretin. Br J Dermatol 144: 422-424
- Kaposi M (1895) Lichen ruber acuminatus and Lichen ruber planus. Arch Dermatol Syphilol 31: 1-32
- Kaposi M (1886) Lichen ruber moniliformis. Dermatol Syphil (Vienna) 13: 571-582
- Li AW et al (2017) Keratosis lichenoides chronica successfully treated with isotretinoin andmethotrexate
.JAAD Case Rep 3:205-207.
- Muñoz-Santos C et al. (2009) Response of keratosis lichenoides chronica to efalizumab therapy. Arch Dermatol 145:867-869
- Nekam L (1938) Sur la question du lichen moniliforme. Press Med 51: 1000-1003
- 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
- Remling R et al (2002) Keratosis lichenoides chronica. Bath PUVA therapy. dermatologist 53: 550-553
- Skorupka M et al (1992) Keratosis lichenoides chronica. dermatologist 43: 97-99
- 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
- Zhou P et al (2014) Keratosis lichenoides chronica in association with primary cutaneous anaplastic large cell lymphoma. Int J Dermatol 53:e109-12
Incoming links (11)Eye diseases, skin changes; Interface dermatitis; Kaposi bureau barrière group syndrome; Keratosis lichenoid striae; Lichenoid trikeratosis; Lichen verrucosus et reticularis; Nekam's disease (lichen ruber monileformis); Repuva therapy; Striated porokeratosis; Triceratosis, lichenoid; ... Show all
Outgoing links (21)Acanthosis; Acitretin; Aphthae (overview); Dyskeratosis follicularis; Efalizumab; Glucocorticosteroids; Hyperkeratosis follicularis et parafollicularis in cutem penetrans; Lichen planus classic type; Lichen ruber verrucosus; Lupus erythematosus (overview); ... Show all
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