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Benign Familial Pemphigus (Hailey Hailey Disease) Summary

Description: 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 Show All >>

Networked: 180 relevant articles (27 outcomes, 8 trials/studies) for this Disease

Key Drugs and Agents for Benign Familial Pemphigus

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Tacrolimus (Prograf) : 3 outcomes in 13 results : FDA 7 Generic
  2. Cyclosporine (Ciclosporin) : 2 outcomes in 7 results : FDA 20 Generic
  3. Type A Botulinum Toxins (Botulinum A Toxin) : 2 outcomes in 4 results : FDA 2
  4. Botulinum Toxins (Botulinum Toxin) : 2 outcomes in 4 results : IBA
  5. Hormones : 2 outcomes in 3 results : IBA
  6. Acitretin : 1 outcome in 2 results : FDA 1
  7. Doxycycline (Vibramycin) : 1 outcome in 2 results : FDA 23 Generic
  8. pimecrolimus (Elidel) : 1 outcome in 2 results : FDA 2
  9. alitretinoin : 1 outcome in 2 results : FDA 1
  10. 1 alpha,24-dihydroxyvitamin D3 (Tacalcitol) : 1 outcome in 2 results : IBA
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Diseases Related to Benign Familial Pemphigus

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

Efficacy Chart >>
  1. Gas Lasers : 4 outcomes in 13 results
  2. Radiotherapy : 2 outcomes in 4 results
  3. Solid-State Lasers : 2 outcomes in 2 results
  4. Dermabrasion : 1 outcome in 8 results
  5. Transplants (Transplant) : 1 outcome in 4 results
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