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

  • Author: Thomas N Helm, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 26, 2012
 

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.
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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,[10, 11] and further work is needed to explore the benefits of this off-label procedure.
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Deterrence/Prevention

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

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

  • Patients with familial benign pemphigus live long and productive lives. The skin disorder is more of a nuisance than a serious health threat.
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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.
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Contributor Information and Disclosures
Author

Thomas N Helm, MD  Clinical Professor of Dermatology and Pathology, University of Buffalo, State University of New York School of Medicine and Biomedical Sciences; 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.

Specialty Editor Board

David Woodley, MD  Co-Chair, Professor, Department of Medicine, Division of Dermatology, University of Southern California

David Woodley, MD 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: Lotus Tissue Repair Ownership interest owner and advisor

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

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: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Hailey H, Hailey H. Familial benign chronic pemphigus. Arch Dermatol. 1939;39:679-85.

  2. Fairclough RJ, Dode L, Vanoevelen J, et al. Effect of Hailey-Hailey Disease mutations on the function of a new variant of human secretory pathway Ca2+/Mn2+-ATPase (hSPCA1). J Biol Chem. Jul 4 2003;278(27):24721-30. [Medline].

  3. Foggia L, Aronchik I, Aberg K, Brown B, Hovnanian A, Mauro TM. Activity of the hSPCA1 Golgi Ca2+ pump is essential for Ca2+-mediated Ca2+ response and cell viability in Darier disease. J Cell Sci. Feb 15 2006;119:671-9. [Medline].

  4. Leinonen PT, Hagg PM, Peltonen S, et al. Reevaluation of the normal epidermal calcium gradient, and analysis of calcium levels and ATP receptors in Hailey-Hailey and Darier epidermis. J Invest Dermatol. Jun 2009;129(6):1379-87. [Medline].

  5. Zhu YG, Yang S, Gao M, et al. Two novel mutations of the ATP2C1 gene in Chinese families with Hailey-Hailey disease. J Dermatol Sci. May 2006;42(2):125-7. [Medline].

  6. Lipoff JB, Mudgil AV, Young S, Chu P, Cohen SR. Acantholytic dermatosis of the crural folds with ATP2C1 mutation is a possible variant of Hailey-Hailey Disease. J Cutan Med Surg. May-Jun 2009;13(3):151-4. [Medline].

  7. Hunt MJ, Salisbury EL, Painter DM, Lee S. Vesiculobullous Hailey-Hailey disease: successful treatment with oral retinoids. Australas J Dermatol. Nov 1996;37(4):196-8. [Medline].

  8. Rabeni EJ, Cunningham NM. Effective treatment of Hailey-Hailey disease with topical tacrolimus. J Am Acad Dermatol. Nov 2002;47(5):797-8. [Medline].

  9. Ruiz-Rodriguez R, Alvarez JG, Jaen P, Acevedo A, Cordoba S. Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease). J Am Acad Dermatol. Nov 2002;47(5):740-2. [Medline].

  10. Konrad H, Karamfilov T, Wollina U. Intracutaneous botulinum toxin A versus ablative therapy of Hailey-Hailey disease--a case report. J Cosmet Laser Ther. Dec 2001;3(4):181-4. [Medline].

  11. Lapiere JC, Hirsh A, Gordon KB, Cook B, Montalvo A. Botulinum toxin type A for the treatment of axillary Hailey-Hailey disease. Dermatol Surg. Apr 2000;26(4):371-4. [Medline].

  12. Berger EM, Galadari HI, Gottlieb AB. Successful treatment of Hailey-Hailey disease with acitretin. J Drugs Dermatol. Jul 2007;6(7):734-6. [Medline].

  13. Hurd DS, Johnston C, Bevins A. A case report of Hailey-Hailey disease treated with alefacept (Amevive). Br J Dermatol. Feb 2008;158(2):399-401. [Medline].

  14. Tang MB, Tan ES. Hailey-Hailey disease: effective treatment with topical cadexomer iodine. J Dermatolog Treat. Oct 2011;22(5):304-5. [Medline].

  15. Don PC, Carney PS, Lynch WS, Zaim MT, Hassan MO. Carbon dioxide laserabrasion: a new approach to management of familial benign chronic pemphigus (Hailey-Hailey disease). J Dermatol Surg Oncol. Nov 1987;13(11):1187-94. [Medline].

  16. Fisher GH, Geronemus RG. Improvement of familial benign pemphigus after treatment with pulsed-dye laser: a case report. Dermatol Surg. Jul 2006;32(7):966-8. [Medline].

  17. Kartamaa M, Reitamo S. Familial benign chronic pemphigus (Hailey-Hailey disease). Treatment with carbon dioxide laser vaporization. Arch Dermatol. May 1992;128(5):646-8. [Medline].

  18. Fernandez Guarino M, Ryan AM, et al. Experience with photodynamic therapy in Hailey-Hailey disease. J Dermatolog Treat. 2008;19(5):288-90. [Medline].

  19. Berth-Jones J, Smith SG, Graham-Brown RA. Benign familial chronic pemphigus (Hailey-Hailey disease) responds to cyclosporin. Clin Exp Dermatol. Jan 1995;20(1):70-2. [Medline].

  20. Burge SM. Hailey-Hailey disease: the clinical features, response to treatment and prognosis. Br J Dermatol. Mar 1992;126(3):275-82. [Medline].

  21. Cooley JE, Briggaman RA, Cronce DJ, Banes AJ, O'Keefe EJ. Hailey-Hailey disease keratinocytes: normal assembly of cell-cell junctions in vitro. J Invest Dermatol. Dec 1996;107(6):877-81. [Medline].

  22. Dadban A, Guillot B. Oesophageal involvement in familial benign chronic pemphigus. Acta Derm Venereol. 2006;86(3):252-3. [Medline].

  23. De Dobbeleer G, De Graef C, M'Poudi E, Gourdain JM, Heenen M. Reproduction of the characteristic morphologic changes of familial benign chronic pemphigus in cultures of lesional keratinocytes onto dead deepidermized dermis. J Am Acad Dermatol. Nov 1989;21(5 Pt 1):961-5. [Medline].

  24. Fisher BK, Margesson LJ. Hailey-Hailey disease (familial benign chronic pemphigus). In: Genital Skin Disorders: Diagnosis and Treatment. St. Louis, Mo: Mosby-Year Book; 1998:68, 117.

  25. Flint ID, Spencer DM, Wilkin JK. Eczema herpeticum in association with familial benign chronic pemphigus. J Am Acad Dermatol. Feb 1993;28(2 Pt 1):257-9. [Medline].

  26. Galimberti RL, Kowalczuk AM, Bianchi O, Bonino MV, Garcia Garcia A. Chronic benign familial pemphigus. Int J Dermatol. Sep 1988;27(7):495-500. [Medline].

  27. Gokdemir G, Gonen S, Kivanc-Altunay I, Koslu A. Familial pemphigus vulgaris: two siblings with unusual localized variant. Int J Dermatol. Feb 2006;45(2):170-2. [Medline].

  28. Hernandez-Perez E. Familial benign chronic pemphigus. Cutis. Jan 1987;39(1):75-7. [Medline].

  29. Ikeda S, Suga Y, Ogawa H. Successful management of Hailey-Hailey disease with potent topical steroid ointment. J Dermatol Sci. Jun 1993;5(3):205-11. [Medline].

  30. Ishibashi Y, Kajiwara Y, Andoh I, Inoue Y, Kukita A. The nature and pathogenesis of dyskeratosis in Hailey-Hailey's disease and Darier's disease. J Dermatol. Aug 1984;11(4):335-53. [Medline].

  31. Metze D, Hamm H, Schorat A, Luger T. Involvement of the adherens junction-actin filament system in acantholytic dyskeratosis of Hailey-Hailey disease. A histological, ultrastructural, and histochemical study of lesional and non-lesional skin. J Cutan Pathol. Jun 1996;23(3):211-22. [Medline].

  32. Oguz O, Gokler G, Ocakoglu O, Oguz V, Demirkesen C, Aydemir EH. Conjunctival involvement in familial chronic benign pemphigus (Hailey-Hailey disease). Int J Dermatol. Apr 1997;36(4):282-5. [Medline].

  33. Remitz A, Lauerma AI, Stubb S, Forstrom L, Reitamo S. Darier's disease, familial benign chronic pemphigus (Hailey-Hailey disease) and contact hypersensitivity. J Am Acad Dermatol. Jan 1990;22(1):134. [Medline].

  34. Sehgal VN, Jain S. Chronic familial benign pemphigus. J Dermatol. Jun 1994;21(6):382-8. [Medline].

  35. Starzycki Z, Chorzelski TP, Jablonska S. Familial pemphigus vulgaris in mother and daughter. Int J Dermatol. Mar 1998;37(3):211-4. [Medline].

  36. Welsh EA, Ikeda S, Peluso AM, et al. Hailey-Hailey disease is not allelic to Darier's disease. J Invest Dermatol. Jun 1994;102(6):992-3. [Medline].

 
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Right axilla with erosive erythematous plaques.
Lumbar back with erythematous plaques with impetiginized crust.
Left axilla with tender erythematous plaques.
Eroded and crusted plaques in right groin at base of penis.
Erythema of scrotum and erosive plaques in left groin (simulated intertrigo).
Central groin with yellow crust over cleaned plaques.
Acantholysis at all levels of the epidermis (hematoxylin and eosin stain, original magnification X20).
 
 
 
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