II. Epidemiology

  1. Autosomal Dominant inherited condition

III. Pathophysiology

  1. Benign variant of Pemphigus Vulgaris
  2. Suprabasal cell Acantholysis
  3. Exacerbating factors
    1. Superficial Bacterial Infection
    2. Warm weather

IV. Signs

  1. Mouth mucosal lesions absent
  2. Large confluent vegetative Skin Erosions
    1. Center with moist granular tissue
    2. Border composed of Vesicles and Pustules
  3. Distribution
    1. Form in intertriginous areas (groin, axillae)
    2. Also affects neck folds and scalp or face

V. Labs

  1. Immunofluorescence shows no antibodies on biopsy

VI. Management

  1. Treat local infections
    1. Consider topical and systemic antibiotics
    2. Consider Antifungal agents for candida infection
  2. Surgical excision of large vegetative growths

VII. Course

  1. Much better prognosis than Pemphigus Vulgaris

VIII. References

  1. Parker in Goldman (2000) Cecil Medicine, p. 2284
  2. Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]

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Ontology: Familial benign pemphigus (C0085106)

Definition (MSH) 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.
Definition (NCI) An autosomal dominant disorder characterized by a history of multiple relapses and remissions of pemphigus lesions.
Concepts Disease or Syndrome (T047)
MSH D016506
ICD10 Q82.8
SnomedCT 79468000
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, ヘイリーヘイリービョウ, リョウセイカゾクセイテンポウソウ, リョウセイカゾクセイマンセイテンポウソウ, カゾクセイリョウセイテンポウソウ, 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
Czech pemfigus benigní familiární, Hailey-Haileyho nemoc, Benigní familiární pemfigus, Benigní familiární chronický pemfigus, Familiární benigní pemfigus, Haileyova-Haileyova nemoc
Finnish Hailey-Haileyn tauti
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

Ontology: Pemphigus vegetans (C0263316)

Concepts Disease or Syndrome (T047)
ICD10 L10.1
SnomedCT 81285006, 14800001
Dutch pemphigus vegetans, pemphigus; vegetans, vegetans; pemphigus, Pemphigus vegetans
French Pemphigus végétant
German Pemphigus vegetans
Italian Pemfigo vegetante
Portuguese Pênfigo vegetante
Spanish Pénfigo vegetante, pénfigo vegetante (trastorno), pénfigo vegetante
Japanese 増殖性天疱瘡, ゾウショクセイテンポウソウ
English pemphigus vegetans (diagnosis), pemphigus vegetans, pemphigus vegetan, Pemphigus vegetans, Pemphigus vegetans (disorder), pemphigus; vegetans, vegetans; pemphigus, Pemphigus, vegetans
Czech Pemphigus vegetans
Korean 증식성 천포창
Hungarian Pemphigus vegetans