HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Benign Familial Pemphigus (Hailey Hailey Disease)

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.
Also Known As:
Hailey Hailey Disease; Pemphigus, Benign Familial; Benign Chronic Pemphigus; Familial Pemphigus, Benign; Chronic Benign Familial Pemphigus; Familial Benign Chronic Pemphigus; Hailey-Hailey Disease
Networked: 307 relevant articles (47 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Darier Disease (Darier's Disease)
2. Pemphigus (Pemphigus Vulgaris)
3. Benign Familial Pemphigus (Hailey Hailey Disease)
4. Lichen Planus (Lichen Ruber Planus)
5. Atopic Dermatitis (Atopic Eczema)

Experts

1. Hamada, Takahiro: 5 articles (10/2017 - 07/2008)
2. Hashimoto, Takashi: 5 articles (10/2017 - 07/2008)
3. Kellermayer, Richard: 5 articles (11/2006 - 12/2004)
4. Szigeti, Réka: 5 articles (11/2006 - 09/2005)
5. Nakano, Hajime: 4 articles (06/2022 - 03/2014)
6. Akiyama, M: 4 articles (01/2018 - 04/2000)
7. Wuytack, Frank: 4 articles (01/2017 - 07/2003)
8. Hovnanian, Alain: 4 articles (02/2006 - 02/2002)
9. Shimizu, H: 4 articles (01/2006 - 04/2000)
10. Cialfi, Samantha: 3 articles (12/2019 - 01/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Benign Familial Pemphigus:
1. Naltrexone (ReVia)FDA LinkGeneric
2. Type A Botulinum Toxins (Botox)FDA Link
3. Tacrolimus (Prograf)FDA LinkGeneric
4. Magnesium ChlorideFDA LinkGeneric
5. Botulinum Toxins (Botulinum Toxin)IBA
6. Cyclosporine (Ciclosporin)FDA LinkGeneric
7. AcitretinFDA Link
8. Hormones (Hormone)IBA
9. apremilastIBA
10. Doxycycline (Periostat)FDA LinkGeneric

Therapies and Procedures

1. Gas Lasers
2. Therapeutics
3. Radiotherapy
4. Solid-State Lasers
5. Photochemotherapy (Photodynamic Therapy)