Lichen planus pigmentosus
Definicija
Lichen planus (LP) pigmentosus is a rare variant of cutaneous lichen planus (see this term) characterized by the presence of hyperpigmented lichenoid lesions in sun-exposed or flexural areas of the body.
Pretraga
Pun naziv
Lichen planus pigmentosus
Kratki naziv
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Sinonimi
Lichen planus pigmentosa_x000D_Lichen planus pigmentosus inversus_x000D_LP pigmentosus_x000D_LP pigmentosa
Orpha broj
254463
Kategorija
Podkategorija
Naziv na stranom jeziku
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Prevalenca
Unknown
Nasleđivanje
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Period početka bolesti
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ICD 10
L43.8
OMIM
UMLS
C0406366
GARD
10816
MEDDRA
Tekstualni opis
The disease usually appears in the third and fourth decade of life. The lesions are asymptomatic or mildly pruritic. Skin changes are dark brown or slate grey macules or papules with, in most cases, a diffuse pigmentation pattern. They most commonly affect the face, neck and upper limbs. Predominantly a intertriginous disease, usually in the axillae and groin (described mostly in Caucasians), it has also been termed as lichen planus pigmentosus inversus. The scalp, nails or mucosa are not affected. LP pigmentosus can exist in association with typical LP lesions.
Etiologija
Etiology is unknown but various factors (e.g. viral infections and certain topical agents including mustard oil, amla oil and henna hair dyes) can trigger the disease. One case has been reported in association with Bazex syndrome (see this term).
Prognoza
bolest_prognoza
Diferencijalna dijagnoza
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Tretman
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Dijagnostičke metode
Histological features of LP pigmentosus are similar but milder than classic LP, with a lichenoid interface reaction, vacuolar change and apoptotic keratinocytes. Melanin incontinence is prominent, and may be the only feature of older lesions; it may extend deeper into the dermis than typical post-inflammatory pigmentation.
Antenatalna dijagnoza
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Epidemiologija
LP pigmentosus is a rare disease in Europe but it is common in Indian populations and in the Middle East. The overall prevalence is unknown. There is no difference in distribution between males and females.
Genetsko savetovanje
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Terapija
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Klinička istraživanja
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