Number of the records: 1
pemfigus benígny familiárny
Record number d016506 Date 06.06.2025 Type M - MESH Topical term pemfigus benígny familiárny Other term English (Pseudonym) Chronic Benign Familial Pemphigus
English (Pseudonym) Familial Benign Chronic Pemphigus
English (Pseudonym) Hailey-Hailey Disease
Slovak (Pseudonym) pemfigus benígny chronický
Slovak (Pseudonym) pemfigus benígny familiárny chronický
Slovak (Pseudonym) Haileyova-Haileyova choroba
Slovak (Pseudonym) Hailey-Haileyova choroba
UDC C16.320.850.700C17.800.827.700C17.800.865.858 Note 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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Number of the records: 1