eMedicine Specialties > Dermatology > Bullous Diseases

Familial Benign Pemphigus (Hailey-Hailey Disease): Differential Diagnoses & Workup

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

Differential Diagnoses

Erythrasma
Pemphigus, Drug-Induced
Extramammary Paget Disease
Pemphigus, IgA
Intertrigo
Pemphigus, Paraneoplastic
Pemphigoid Gestationis
Pyoderma Vegetans
Pemphigus Erythematosus
Tinea Corporis
Pemphigus Foliaceus
Tinea Cruris
Pemphigus Herpetiformis
Pemphigus Vulgaris

Workup

Laboratory Studies

  • Microscopic examination reveals intraepidermal and suprabasilar acantholysis (see Media File 7). Elongated papillae (villi) extend into lacunae, and a single layer of basal cells lines the villi.
  • Many layers of detached keratinocytes (acantholysis) exist and appear similar to a dilapidated brick wall (clumped acantholytic cells have only a few intact intercellular bridges holding the keratinocytes together).
  • Unlike pemphigus vulgaris, direct immunofluorescence in familial benign pemphigus s negative. The Nikolsky sign is negative, and Tzanck preparation fails to reveal giant cells and syncytia formation characteristic of herpes virus infection.
  • Serologic studies for familial benign pemphigus fail to reveal circulating autoantibodies, unlike pemphigus vulgaris in which antibody titers correlate with disease activity.

Histologic Findings

Histologic findings in familial benign pemphigus are suprabasilar and widespread acantholysis.

More on Familial Benign Pemphigus (Hailey-Hailey Disease)

Overview: Familial Benign Pemphigus (Hailey-Hailey Disease)
Differential Diagnoses & Workup: Familial Benign Pemphigus (Hailey-Hailey Disease)
Treatment & Medication: Familial Benign Pemphigus (Hailey-Hailey Disease)
Follow-up: Familial Benign Pemphigus (Hailey-Hailey Disease)
Multimedia: Familial Benign Pemphigus (Hailey-Hailey Disease)
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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