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Epidermolysis bullosa simplex with circinate migratory erythema

ORPHA:158681DiseaseAutosomal dominantInfancy, Neonatal

Ассоциированные гены (1)

KRT5
keratin 5
Disease-causing germline mutation(s) in
OMIM: 148040

Фенотипы (19)

Частый (30–79%)11
HP:0000953Hyperpigmentation of the skin
HP:0000989Pruritus
HP:0001036Parakeratosis
HP:0003341Junctional split
HP:0005585Spotty hyperpigmentation
HP:0007513Generalized hypopigmentation
HP:0007585Skin fragility with non-scarring blistering
HP:0008066Abnormal blistering of the skin
HP:0031045Acral blistering
HP:0031180Erythema migrans
HP:0200037Skin vesicle
Периодический (5–29%)4
HP:0000464Abnormality of the neck
HP:0002815Abnormality of the knee
HP:0007599Generalized reticulate brown pigmentation
HP:0012221Pretibial blistering
Исключён (0%)4
HP:0000478Abnormality of the eye
HP:0000972Palmoplantar hyperkeratosis
HP:0008404Nail dystrophy
HP:0200097Oral mucosal blisters

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы