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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; Familial Benign Chronic Pemphigus; Hailey-Hailey Disease
Networked: 180 relevant articles (27 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Darier Disease (Darier's Disease)
2. Pemphigus (Pemphigus Vulgaris)
3. Psoriasis (Pustulosis Palmaris et Plantaris)
4. Hidradenitis Suppurativa
5. Hidrocystoma

Experts

1. Kellermayer, Richard: 4 articles (11/2006 - 12/2004)
2. Szigeti, Réka: 4 articles (11/2006 - 09/2005)
3. Hovnanian, Alain: 4 articles (02/2006 - 02/2002)
4. Shimizu, H: 4 articles (01/2006 - 04/2000)
5. Hashimoto, Takashi: 3 articles (07/2014 - 07/2008)
6. Hamada, Takahiro: 3 articles (07/2014 - 07/2008)
7. Peltonen, Juha: 3 articles (08/2012 - 06/2009)
8. Peltonen, Sirkku: 3 articles (08/2012 - 06/2009)
9. Behne, Martin J: 3 articles (08/2007 - 10/2003)
10. Mauro, Theodora M: 3 articles (08/2007 - 10/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Benign Familial Pemphigus:
1. Tacrolimus (Prograf)FDA LinkGeneric
2. Cyclosporine (Ciclosporin)FDA LinkGeneric
3. Type A Botulinum Toxins (Botulinum A Toxin)FDA Link
4. Botulinum Toxins (Botulinum Toxin)IBA
5. HormonesIBA
6. AcitretinFDA Link
7. Doxycycline (Vibramycin)FDA LinkGeneric
8. pimecrolimus (Elidel)FDA Link
9. alitretinoinFDA Link
10. 1 alpha,24-dihydroxyvitamin D3 (Tacalcitol)IBA

Therapies and Procedures

1. Gas Lasers
2. Radiotherapy
3. Solid-State Lasers
4. Dermabrasion
5. Transplants (Transplant)