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Epidermolysis bullosa simplex with mottled pigmentation

ORPHA:79397DiseaseAutosomal dominantInfancy, Neonatal

Ассоциированные гены (2)

KRT14
keratin 14
Disease-causing germline mutation(s) in
OMIM: 148066
KRT5
keratin 5
Disease-causing germline mutation(s) in
OMIM: 148040

Фенотипы (19)

Очень частый (80–99%)6
HP:0001070Mottled pigmentation
HP:0003341Junctional split
HP:0007427Reticulated skin pigmentation
HP:0007585Skin fragility with non-scarring blistering
HP:0008066Abnormal blistering of the skin
HP:0009123Mixed hypo- and hyperpigmentation of the skin
Частый (30–79%)8
HP:0001034Hypermelanotic macule
HP:0002164Nail dysplasia
HP:0005590Spotty hypopigmentation
HP:0007556Plantar hyperkeratosis
HP:0008404Nail dystrophy
HP:0009719Hypomelanotic macule
HP:0010765Palmar hyperkeratosis
HP:0045059Hyperkeratotic papule
Периодический (5–29%)5
HP:0000164Abnormality of the dentition
HP:0000992Cutaneous photosensitivity
HP:0001056Milia
HP:0001596Alopecia
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