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Definition (MSH)
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An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and BULLAE mainly on the neck, axillae, and groin. Mutations in the ATP2C1 gene (encoding the secretory pathway Ca2++/Mn2++ ATPase 1 (SPCA1)) cause this disease. It is clinically and histologically similar to DARIER DISEASE - both have abnormal, unstable DESMOSOMES between KERATINOCYTES and defective CALCIUM-TRANSPORTING ATPASES. It is unrelated to PEMPHIGUS VULGARIS though it closely resembles that disease.
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Definition (NCI)
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An autosomal dominant disorder characterized by a history of multiple relapses and remissions of pemphigus lesions.
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| Concepts |
Disease or Syndrome
(T047)
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| MSH |
D016506
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| ICD10 |
Q82.8
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| SnomedCT |
79468000
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| English |
Familial Benign Chronic Pemphigus, Familial Pemphigus, Benign, Hailey Hailey Disease, Hailey-Hailey Disease, Pemphigus, Benign Familial, Benign famil.chronic pemphigus, HAILEY-HAILEY DISEASE, BENIGN CHRONIC PEMPHIGUS, BCPM, PEMPHIGUS, BENIGN FAMILIAL, HHD, HAILEY HAILEY DIS, familial benign chronic pemphigus (diagnosis), familial benign chronic pemphigus, Benign familial pemphigus, Familial Benign Pemphigus, Benign Familial Pemphigus, Benign familial pemphigus [Hailey-Hailey], Pemphigus, Benign Familial [Disease/Finding], Benign Chronic Pemphigus, hailey disease, hailey hailey disease, diseases hailey hailey, hailey-hailey disease, benign familial pemphigus, Familial benign chronic pemphigus, Hailey-Hailey disease, Benign familial chronic pemphigus, Familial benign pemphigus (disorder), benign; familial pemphigus, hailey-Hailey disease, pemphigus; benign familial, Familial benign pemphigus |
| French |
Pemphigus bénin familial, Pemphigus chronique familial bénin, Maladie de Hugh-Hailey, Pemphigus bénin chronique familial, Dermatose acantholytique familiale, Maladie de Hailey et Hailey, Maladie de Hailey-Hailey, Pemphigus chronique bénin familial |
| Dutch |
familiale benigne chronische pemphigus, ziekte van Hailey-Hailey, familiale benigne pemphigus, benigne; familiale pemphigus, pemphigus; benigne familiaal, benigne familiale pemphigus, Benigne pemphigus, familiale, Familiale benigne chronische pemphigus, Familiale benigne pemphigus, Hailey-Hailey-ziekte, Pemphigus, benigne familiale |
| German |
Pemphigus benignus familiaris chronisch, Pemphigus benignus familiaris, Hailey-Hailey-Krankheit, Pemphigus chronicus benignus familiaris, Familiärer benigner chronischer Pemphigus |
| Italian |
Pemfigo benigno familiare, Pemfigo familiare cronico benigno, Malattia di Hailey-Hailey, Pemfigo benigno cronico familiare, Malattia di Hailey Hailey, Pemfigo familiare benigno |
| Portuguese |
Pênfigo crónico familiar benigno, Pênfigo Familiar Benigno, Pênfigo Familiar Benigno Crônico, Pênfigo Benigno Familiar, Pênfigo Benigno Familiar Crônico, Pênfigo benigno familiar, Doença de Hailey-Hailey |
| Spanish |
Pénfigo crónico familiar benigno, Pénfigo benigno familiar, Enfermedad de Hailey y Hailey, enfermedad de Hailey - Hailey, pénfigo benigno familiar, pénfigo crónico benigno familiar (trastorno), pénfigo crónico benigno familiar, Pénfigo familiar benigno, Enfermedad de Hailey-Hailey, Pénfigo Crónico Benigno Familiar, Pénfigo Familiar Benigno |
| Japanese |
良性家族性慢性天疱瘡, ヘイリー・ヘイリー病, 家族性良性天疱瘡, ﾍｲﾘｰﾍｲﾘｰﾋﾞｮｳ, ﾘｮｳｾｲｶｿﾞｸｾｲﾃﾝﾎﾟｳｿｳ, ﾘｮｳｾｲｶｿﾞｸｾｲﾏﾝｾｲﾃﾝﾎﾟｳｿｳ, ｶｿﾞｸｾｲﾘｮｳｾｲﾃﾝﾎﾟｳｿｳ, Hailey-Hailey病, 天疱瘡-良性家族性, 家族性良性慢性天疱瘡, 良性家族性天疱瘡, 天疱瘡-家族性良性慢性 |
| Swedish |
Pemfigus, benign familjär
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| Czech |
pemfigus benigní familiární, Hailey-Haileyho nemoc, Benigní familiární pemfigus, Benigní familiární chronický pemfigus, Familiární benigní pemfigus, Haileyova-Haileyova nemoc |
| Finnish |
Hailey-Haileyn tauti
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| Russian |
KHARLEIA-KHARLEIA BOLEZN', PEMFIGUS DOBROKACHESTVENNYI SEMEINYI, SEMEINYI DOBROKACHESTVENNYI KHRONICHESKII PEMFIGUS, ПЕМФИГУС ДОБРОКАЧЕСТВЕННЫЙ СЕМЕЙНЫЙ, СЕМЕЙНЫЙ ДОБРОКАЧЕСТВЕННЫЙ ХРОНИЧЕСКИЙ ПЕМФИГУС, ХАРЛЕЯ-ХАРЛЕЯ БОЛЕЗНЬ |
| Polish |
Pęcherzyca łagodna przewlekła Haileya, Choroba Haileya, Pęcherzyca łagodna rodzinna |
| Hungarian |
Jóindulatú familiaris pemphigus, Hailey-Hailey betegség, Jóindulatú familiaris chronikus pemphigus |
| Norwegian |
Hailey-Hailey-sykdom, Familiær benign pemfigus, Pemphigus familiaris benignus, Hailey-Haileys sykdom |
