eMedicine Specialties > Dermatology > Bullous Diseases
Familial Benign Pemphigus (Hailey-Hailey Disease): Multimedia
Updated: Aug 13, 2008
Multimedia
![]() | Media file 1: Right axilla with erosive erythematous plaques. |
![]() | Media file 2: Lumbar back with erythematous plaques with impetiginized crust. |
![]() | Media file 3: Left axilla with tender erythematous plaques. |
![]() | Media file 4: Eroded and crusted plaques in right groin at base of penis. |
![]() | Media file 5: Erythema of scrotum and erosive plaques in left groin (simulated intertrigo). |
![]() | Media file 6: Central groin with yellow crust over cleaned plaques. |
![]() | Media file 7: Acantholysis at all levels of the epidermis (hematoxylin and eosin stain, original magnification X20). |
More on Familial Benign Pemphigus (Hailey-Hailey Disease) |
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| 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
Hailey H, Hailey H. Familial benign chronic pemphigus. Arch Dermatol. 1939;39:679-85.
Fairclough RJ, Dode L, Vanoevelen J, Andersen JP, Missiaen L, Raeymaekers L, 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].
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(Pt 4):671-9. [Medline].
Zhu YG, Yang S, Gao M, Chen JJ, Li W, Wang PG, 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].
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].
Rabeni EJ, Cunningham NM. Effective treatment of Hailey-Hailey disease with topical tacrolimus. J Am Acad Dermatol. Nov 2002;47(5):797-8. [Medline].
Ruiz-Rodriguez R, Alvarez JG, Jaén P, Acevedo A, Córdoba 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].
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].
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].
Berger EM, Galadari HI, Hassan I, Gottleib AB. Successful treatment of Hailey-Hailey disease with acitretin. Journal of Drugs in Dermatology. July 2007;6:734-6. [Medline].
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].
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].
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].
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].
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].
Burge SM. Hailey-Hailey disease: the clinical features, response to treatment and prognosis. Br J Dermatol. Mar 1992;126(3):275-82. [Medline].
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].
Dadban A, Guillot B. Oesophageal involvement in familial benign chronic pemphigus. Acta Derm Venereol. 2006;86(3):252-3. [Medline].
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].
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.
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].
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].
Gokdemir G, Gonen S, Kivanç-Altunay I, Köslü A. Familial pemphigus vulgaris: two siblings with unusual localized variant. Int J Dermatol. Feb 2006;45(2):170-2. [Medline].
Hernández-Pérez E. Familial benign chronic pemphigus. Cutis. Jan 1987;39(1):75-7. [Medline].
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].
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].
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].
Oguz O, Gökler 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].
Remitz A, Lauerma AI, Stubb S, Förström 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].
Sehgal VN, Jain S. Chronic familial benign pemphigus. J Dermatol. Jun 1994;21(6):382-8. [Medline].
Starzycki Z, Chorzelski TP, Jablonska S. Familial pemphigus vulgaris in mother and daughter. Int J Dermatol. Mar 1998;37(3):211-4. [Medline].
Welsh EA, Ikeda S, Peluso AM, Bonifas JM, Bare JW, Woodley DT, et al. Hailey-Hailey disease is not allelic to Darier's disease. J Invest Dermatol. Jun 1994;102(6):992-3. [Medline].
Further Reading
Keywords
pemphigus, benign familial pemphigus, familial benign pemphigus, bullous disease, Hailey-Hailey disease














Multimedia: Familial Benign Pemphigus (Hailey-Hailey Disease)