eMedicine Specialties > Dermatology > Bullous Diseases

Familial Benign Pemphigus (Hailey-Hailey Disease): Follow-up

Author: Thomas N Helm, MD, Clinical Associate Professor, Departments of Dermatology and Pathology, State University of New York at Buffalo; Director, Buffalo Medical Group Dermatopathology Laboratory
Coauthor(s): Thomas C Lee, MD, Intern, Department of Internal Medicine, New York University School of Medicine
Contributor Information and Disclosures

Updated: Aug 13, 2008

Follow-up

Further Outpatient Care

  • Almost all patients with familial benign pemphigus can be treated successfully on an outpatient basis, but many patients may need to be excused temporarily from manual labor so that affected body folds can heal promptly under treatment.
  • Familial benign pemphigus patients require regular evaluation to be sure that secondary infection is brought under control and that the adverse effects of topical corticosteroids (eg, cutaneous atrophy) are avoided.
  • Individuals who receive intermittent courses of systemic corticosteroids should be evaluated for possible decrease in bone density and should be instructed regarding diet and therapy that may maintain bone density.
  • One report describes squamous cell carcinoma arising in the setting of familial benign pemphigus. Biopsy specimens should be taken from any suspicious infiltrated areas.

Inpatient & Outpatient Medications

  • Intracutaneous botulinum toxin A injection may be of benefit by inhibiting sweating. Remissions of up to at least 12 months have been achieved using only botulinum A toxin injection. Only a few isolated reports have been published,8,9 and further work is needed to explore the benefits of this off-label procedure.

Deterrence/Prevention

  • Maintaining a healthy weight and keeping the body folds cool and dry as much as possible help prevent flares of the disease.

Complications

  • Cellulitis, abscess formation, scarring, and depression concerning the chronic and refractory nature of the skin condition are possible complications.
  • Systemic corticosteroid therapy may result in the adverse effects of steroids, such as osteoporosis, cataracts, striae, ulcers, and others.

Prognosis

  • Patients live long and productive lives. The skin disorder is more of a nuisance than a serious health threat.

Patient Education

  • Patients with familial benign pemphigus must be instructed to recognize flares in the disease promptly and to seek treatment for secondary infection before it becomes severe.

Miscellaneous

Medicolegal Pitfalls

  • Failure to monitor adverse effects of systemic therapy is the most significant pitfall. Systemic corticosteroids should be used intermittently and as a last resort when topical therapy fails.
 


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References
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References

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Further Reading

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Keywords

pemphigus, benign familial pemphigus, familial benign pemphigus, bullous disease, Hailey-Hailey disease

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Contributor Information and Disclosures

Author

Thomas N Helm, MD, Clinical Associate Professor, Departments of Dermatology and Pathology, State University of New York at Buffalo; Director, Buffalo Medical Group Dermatopathology Laboratory
Thomas N Helm, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for Dermatologic Surgery, and American Society of Dermatopathology
Disclosure: Nothing to disclose.

Coauthor(s)

Thomas C Lee, MD, Intern, Department of Internal Medicine, New York University School of Medicine
Disclosure: Nothing to disclose.

Medical Editor

David Woodley, MD, Co-Chair, Professor, Department of Medicine, Division of Dermatology, University of Southern California
David Woodley, MD, Co-Chair is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American College of Emergency Physicians, American College of Physicians, American Federation for Medical Research, American Society for Clinical Investigation, New York Academy of Medicine, Society for Investigative Dermatology, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Julia R Nunley, MD, Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center
Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society
Disclosure: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex  sub-investigator

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

 
 
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