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Journal articles on the topic 'Familial benign chronic pemphigus'

Author: Grafiati

Published: 3 June 2025

Last updated: 20 July 2025

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1

Sehgal, Virendra N., and Sanjiv Jain. "Chronic Familial Benign Pemphigus." Journal of Dermatology 21, no. 6 (1994): 382–88. http://dx.doi.org/10.1111/j.1346-8138.1994.tb01759.x.

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2

Galimberti, Ricardo Luis, Alicia Maria Kowalczuk, Oscar Bianchi, Maria Victoria Boning, and Adelina Garcia Garcia. "Chronic Benign Familial Pemphigus." International Journal of Dermatology 27, no. 7 (1988): 495–500. http://dx.doi.org/10.1111/j.1365-4362.1988.tb00929.x.

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3

Steffen, Charles G. "Familial Benign Chronic Pemphigus." American Journal of Dermatopathology 9, no. 1 (1987): 58–73. http://dx.doi.org/10.1097/00000372-198702000-00012.

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4

Kurwa, A. R., and H. R. Vickers. "(20) Benign familial chronic pemphigus." British Journal of Dermatology 113, s29 (1985): 64. http://dx.doi.org/10.1111/j.1365-2133.1985.tb13035.x.

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5

Falto-Aizpurua, Leyre, Harleen Arora, Fleta Bray, and Jessica Cervantes. "Management of familial benign chronic pemphigus." Clinical, Cosmetic and Investigational Dermatology Volume 9 (September 2016): 281–90. http://dx.doi.org/10.2147/ccid.s89483.

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6

Takami, Yoshihiro, Yutaka Akutsu, Masako Suzuki, and Makoto Takahashi. "Familial Benign Chronic Pemphigus Generalized byPseudomonasInfection." Journal of Dermatology 13, no. 6 (1986): 474–76. http://dx.doi.org/10.1111/j.1346-8138.1986.tb02979.x.

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7

Parajuli, Niraj. "A recurrent intertrigo: benign familial chronic pemphigus." Journal of Chitwan Medical College 8, no. 2 (2018): 63–65. http://dx.doi.org/10.3126/jcmc.v8i2.23741.

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Benign familial chronic pemphigus (BF CP) is an autosomal dominant disorder characterized by recurrent vesicles and plaques mostly over the intertriginous areas. The defect is in the AT P2C1 gene which leads to suprabasilar acantholysis. Pregnancy, physical trauma, excessive sweating, skin infections and exposure to ultraviolet radiation are the important trigger factors. Self-medications or over the counter treatment usually makes a delay in the proper diagnosis.

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8

Dadban, A., and B. Guillot. "Oesophageal Involvement in Familial Benign Chronic Pemphigus." Acta Dermato-Venereologica 86, no. 3 (2006): 252–53. http://dx.doi.org/10.2340/00015555-0053.

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9

Yordanova, Ivelina. "Familial benign chronic pemphigus (Hailey-Hailey disease)." Journal of IMAB - Annual Proceeding (Scientific Papers) 13, 1, no. 2007 (2008): 56–58. http://dx.doi.org/10.5272/jimab.2007131.56.

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10

Fairris, G. M., J. E. White, B. J. Leppard, and P. G. Goodwin. "Methotrexate for intractable benign familial chronic pemphigus." British Journal of Dermatology 115, no. 5 (1986): 640. http://dx.doi.org/10.1111/j.1365-2133.1986.tb05780.x.

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11

Kartamaa, Matti. "Familial Benign Chronic Pemphigus (Hailey-Hailey Disease)." Archives of Dermatology 128, no. 5 (1992): 646. http://dx.doi.org/10.1001/archderm.1992.01680150076009.

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12

Drozhdina, M. B., S. V. Koshkin, and P. V. Gorodnichev. "Treatment of benign familial chronic pemphigus with omalizumab." Russian Journal of Clinical Dermatology and Venereology 24, no. 1 (2025): 72. https://doi.org/10.17116/klinderma20252401172.

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Benign familial chronic pemphigus is a rare genodermatosis that has a significant impact on the quality of life of patients, currently incurable. Lack of unified clinical guidelines, diversity of methods of local and systemic therapy that do not give a pronounced long-lasting effect, cause researchers’ interest and necessity to find new therapeutic tactics for managing this disease. It is known that IgE-mediated pathological mechanisms promote degranulation of mast cells and basophils and stimulate Th2 immunity, that plays a leading role in creating protection against many diseases. It has bee

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13

Kadimella, Sai Maheswari. "A CASE SERIES OF HAILEY HAILEY DISEASE IN MALE AND FEMALE PATIENTS." YMER Digital 21, no. 08 (2022): 29–32. http://dx.doi.org/10.37896/ymer21.08/05.

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Hailey-Hailey disease was originally described by the Hailey brothers [Hugh Edward and William Howard] in 1939. It is a genetic disorder that causes blisters to from on the skin. The other synonyms of Hailey-Hailey is familial benign chronic, pemphigus. HHD is characterized by out breaks of rashes and blisters on the skin. Affected area of skin undergoes ruptured blistering and inflammation and may be painful to the touch. Area where the skin folds as well as armpits, groin, neck, buttocks and under the breasts are most commonly affected. Here, we discussed about the clinical and various diffe

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14

Wulf, Hans Christian, and Stine Regin Wiegell. "Treatment of Familial Benign Chronic Pemphigus With Superficial Radiotherapy." JAMA Dermatology 158, no. 3 (2022): 283. http://dx.doi.org/10.1001/jamadermatol.2021.5491.

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15

Flint, Ivan D., David M. Spencer, and Jonathan K. Wilkin. "Eczema herpeticum in association with familial benign chronic pemphigus." Journal of the American Academy of Dermatology 28, no. 2 (1993): 257–59. http://dx.doi.org/10.1016/s0190-9622(08)81146-2.

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16

Akiyama, Masashi, Makoto Sugiura, Hiroshi Shimizu, and Takeji Nishikawa. "Glycoconjugates in the epidermis of familial benign chronic pemphigus." Journal of Dermatological Science 2, no. 3 (1991): 248. http://dx.doi.org/10.1016/0923-1811(91)90236-q.

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17

Grabovskaya, O. V., N. P. Teplyuk, and Yuliya V. Kolesova. "GOUGEROT-HAILEY-HAILEY’S FAMILIAL BENIGN CHRONIC PEMPHIGUS: A CASE REPORT." Russian Journal of Skin and Venereal Diseases 22, no. 3-4 (2019): 86–92. http://dx.doi.org/10.17816/dv42942.

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The review of the literature on epidemiology, pathogenesis, diagnosis and treatment methods for chronic familial benign pemphigus Gougerot-Haley-Haley, as well as a clinical case of a patient with this disease with family history are presented. The manifestation of the disease occurred at the age of 24, after childbirth. Later there were numerous relapses. Remission was quickly achieved after treatment with antibiotics and oxygen-ozone therapy. In recent years, there has been an increase in the frequency of exacerbations of the disease, and a decrease in treatment effectiveness.

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18

BERTH-JONES, J., S. G. SMITH, and R. A. C. GRAHAM-BROWN. "Benign familial chronic pemphigus (Hailey-Hailey disease) responds to cyclosporin." Clinical and Experimental Dermatology 20, no. 1 (1995): 70–72. http://dx.doi.org/10.1111/j.1365-2230.1995.tb01290.x.

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19

Tyagi, S. P., Veena Maheshwari, Nirmala Tyagi, Seema Hakim, and Kamlesh Tewari. "Involvement of the Uterine Cervix in Benign Familial Chronic Pemphigus." International Journal of Dermatology 30, no. 6 (1991): 449–50. http://dx.doi.org/10.1111/j.1365-4362.1991.tb03907.x.

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20

Jitsukawa, K., J. Ring, U. Weyer, W. Kimmig, and H. Radloff. "Topical cyclosporine in chronic benign familial pemphigus (Hailey-Hailey disease)." Journal of the American Academy of Dermatology 27, no. 4 (1992): 625–26. http://dx.doi.org/10.1016/s0190-9622(08)80201-0.

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21

de Wit, F. S. "Familial benign chronic pemphigus (Hailey-Hailey disease) and contact allergies." Journal of the American Academy of Dermatology 23, no. 3 (1990): 532–33. http://dx.doi.org/10.1016/s0190-9622(08)81128-0.

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22

Oguz, Oya, Gökhan Gokler, Özcan Ocakoglu, Velittin Og̀uz, Cuyan Demirkesen, and Ertug̀rul H. Aydemir. "Conjunctival involvement in familial chronic benign pemphigus (Hailey-Hailey disease)." International Journal of Dermatology 36, no. 4 (2008): 282–85. http://dx.doi.org/10.1111/j.1365-4362.1997.tb03045.x.

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23

Bergman, Reuven, Rivka Levy, Zeev Pam, Chaim Lichtig, Bilha Hazaz, and Rachel Friedman-Birnbaum. "A Study of Keratin Expression in Benign Familial Chronic Pemphigus." American Journal of Dermatopathology 14, no. 1 (1992): 32–36. http://dx.doi.org/10.1097/00000372-199202000-00005.

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24

Siyuan, Chen, Huang Changzheng, and Li Jiawen. "Detection of ATP2C1 gene mutation in familial benign chronic pemphigus." Journal of Huazhong University of Science and Technology [Medical Sciences] 25, no. 5 (2005): 585–86. http://dx.doi.org/10.1007/bf02896025.

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25

Le Saché-de Peufeilhoux, L., E. Raynaud, A. Bouchardeau, S. Fraitag, and C. Bodemer. "Familial benign chronic pemphigus and doxycycline: a review of 6 cases." Journal of the European Academy of Dermatology and Venereology 28, no. 3 (2012): 370–73. http://dx.doi.org/10.1111/jdv.12016.

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26

Regnier, M., J. P. Ortonne, and M. Darmon. "Histological defects of chronic benign familial pemphigus expressed in tissue culture." Archives of Dermatological Research 281, no. 8 (1990): 538–40. http://dx.doi.org/10.1007/bf00412741.

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27

Mallen, John K. "Psoriasis, Chronic Benign Familial Pemphigus, and Dysplastic Naevus Syndrome in a Family." Australasian Journal of Dermatology 33, no. 1 (1992): 55. http://dx.doi.org/10.1111/j.1440-0960.1992.tb00059.x.

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28

Reitamo, Sakari, Anita Remitz, Antti I. Lauerma, and Lars Förström. "Contact allergies in patients with familial benign chronic pemphigus (Hailey-Hailey disease)." Journal of the American Academy of Dermatology 21, no. 3 (1989): 506–10. http://dx.doi.org/10.1016/s0190-9622(89)70216-4.

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29

Remitz, Anita, Antti I. Lauerma, Sakari Stubb, Lars Förström, and Sakari Reitamo. "Darier's disease, familial benign chronic pemphigus (Hailey-Hailey disease) and contact hypersensitivity." Journal of the American Academy of Dermatology 22, no. 1 (1990): 134. http://dx.doi.org/10.1016/s0190-9622(08)80018-7.

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30

Graham, Paul M., Allison Melkonian, and David Fivenson. "Familial benign chronic pemphigus (Hailey-Hailey disease) treated with electron beam radiation." JAAD Case Reports 2, no. 2 (2016): 159–61. http://dx.doi.org/10.1016/j.jdcr.2016.02.008.

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31

Shweta, Rai Vijetha, Ananda Vaidya Kuladeepa, and Sukesh. "Hailey-Hailey Disease: A Case Study Report with Review of Literature." International Journal of Health Sciences and Pharmacy (IJHSP) 2, no. 1 (2018): 13–17. https://doi.org/10.5281/zenodo.1214176.

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Hailey-Hailey disease (HHD), also called as familial benign chronic pemphigus, is a rare autosomal dominant blistering skin disease with waxing and waning in its clinical course. It is characterized by the presence of flaccid vesiculo-pustules, crusted erosions or expanding plaques in the areas of friction such as neck, axilla, groins, and perineum. Histopathologically shows suprabasal separations, inconspicuous dyskeratosis, acantholytic cells within the epidermis, giving a dilapidated brick wall appearance. Here we have discussed about the clinical and histopathological features of HHD and v

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32

Hayakawa and Shiohara. "Coexistence of psoriasis and familial benign chronic pemphigus: efficacy of ultraviolet B treatment." British Journal of Dermatology 140, no. 2 (1999): 374. http://dx.doi.org/10.1046/j.1365-2133.1999.02690.x.

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33

Ponyai, G., S. Karpati, É. Ablonczy, E. Temesvari, and A. Horvath. "Benign familial chronic pemphigus (Hailey-Hailey) provoked by contact sensitivity in 2 patients." Contact Dermatitis 40, no. 3 (1999): 168–69. http://dx.doi.org/10.1111/j.1600-0536.1999.tb06024.x.

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34

Kartamaa, M. "Familial benign chronic pemphigus (Hailey-Hailey disease). Treatment with carbon dioxide laser vaporization." Archives of Dermatology 128, no. 5 (1992): 646–48. http://dx.doi.org/10.1001/archderm.128.5.646.

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35

Al Qooz, Fahad, Mohammed Almuharraqi, Sajjad Salam, Veena Nagaraj, and Abdullah Darwish. "Recurrent Ulcerations in an 84-Year-Old Male Diagnosed with Hailey-Hailey Disease." Case Reports in Dermatology 12, no. 3 (2020): 209–12. http://dx.doi.org/10.1159/000510018.

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Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare inherited acantholytic dermatosis. It is an autosomal dominant disease affecting the intertriginous areas. HHD has been characterized by flaccid blisters, erosions, and macerations that are limited to flexural (friction-prone) areas. The painful blisters and erosions significantly decrease patients’ quality of life. There are multiple types of therapy related to this disorder. Many of the studies have suggested benefits from steroid therapy in addition to oral antibiotics.

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36

Kollman, Natalie, and Jonathan Bass. "Generalized familial benign chronic pemphigus (Hailey-Hailey disease) treated successfully with low-dose naltrexone." JAAD Case Reports 4, no. 7 (2018): 725–27. http://dx.doi.org/10.1016/j.jdcr.2018.07.002.

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37

Balogh, B., and G. Freilinger. "Familial benign chronic pemphigus Hailey-Hailey ?Surgical therapy by excision with simultaneous reduction mammoplasty." European Journal of Plastic Surgery 9, no. 3 (1986): 108–11. http://dx.doi.org/10.1007/bf00298950.

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38

Akiyama, M., M. Sugiura, H. Shimizu, and T. Nishikawa. "No significant change of glycoconjugates exists in the epidermis of familial benign chronic pemphigus." Archives of Dermatological Research 283, no. 8 (1991): 537–39. http://dx.doi.org/10.1007/bf00371931.

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39

Lipoff, Jules B., Adarsh V. Mudgil, Saryna Young, Paul Chu, and Steven R. Cohen. "Acantholytic Dermatosis of the Crural Folds with ATP2C1 Mutation is a Possible Variant of Hailey-Hailey Disease." Journal of Cutaneous Medicine and Surgery 13, no. 3 (2009): 151–54. http://dx.doi.org/10.2310/7750.2008.08005.

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Background: We describe a patient with acantholytic dermatosis of the crural folds (ADCF) that was misdiagnosed and treated as condyloma acuminata for 13 years. After many skin biopsies consistently showed epidermal acantholysis and negative human papillomavirus serotyping excluded condyloma acuminata, a diagnosis of ADCF was considered most likely. Objective and Conclusion: Acitretin effectively suppressed the symptomatic hyperkeratosis. Subsequent genetic testing revealed a deletion in the ATP2C1 gene that led us to conclude that this case of ADCF is probably a variant of familial benign chr

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40

Animesh, Saxena, Dey Vivek, Bhargava Akansha, Vinayak Garima, and Chandrakar Rohal. "Hailey Hailey Disease: Treated with Magnesium Glycine." PJSR 11, no. 2 (2018): 74–76. https://doi.org/10.5281/zenodo.8241833.

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Hailey hailey disease is an autosomal dominant disorder caused by mutation in geneATP2C1 leading to loss of cell adhesion and further acantholysis. The disease commonly involves the intertriginous areas and is characterized by erythematous, macerated, malodorous, fissured plaques.Traditional treatment modalities include steroids(topical&oral), retinoids , immunosupressants. We report successful use of magnesium glycine in a chronic non-responding case of HHD.

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41

Klimas, Natasha, Cameron West, and Michelle Tarbox. "A Bullous Flare of a Hyperkeratotic Affair." Journal of Cutaneous Medicine and Surgery 20, no. 6 (2016): 589–91. http://dx.doi.org/10.1177/1203475416660295.

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Background: Darier’s disease is an autosomal dominant genodermatosis typified by hyperkeratotic papules and plaques in a predominately seborrheic distribution. The vesiculo-bullous variant of Darier’s disease is rare. Several previously reported cases have demonstrated clinical and microscopic features resembling familial benign chronic pemphigus or Hailey-Hailey disease. Objectives: The objective of this report is to describe an uncommon presentation of Darier’s disease, which has been infrequently described in the literature. Methods: The authors present a case of bullous flare of long-stand

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42

SUEKI, H., K. SHANLEY, M. H. GOLDSCHMIDT, G. S. LAZARUS, and G. F. MURPHY. "Dominantly inherited epidermal acantholysis in dogs, simulating human benign familial chronic pemphigus (Hailey-Hailey disease)." British Journal of Dermatology 136, no. 2 (1997): 190–96. http://dx.doi.org/10.1046/j.1365-2133.1997.d01-1167.x.

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43

Santos-Juanes, J., P. Coto-Segura, J. Saavedra, S. Laviano, and C. Galache. "Development of familial benign chronic pemphigus in a patient undergoing treatment with efalizumab for psoriasis." Journal of the European Academy of Dermatology and Venereology 23, no. 5 (2009): 605–6. http://dx.doi.org/10.1111/j.1468-3083.2008.02979.x.

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44

SUEKI, H., K. SHANLEY, M. H. GOLDSCHMIDT, G. S. LAZARUS, and G. F. MURPHY. "Dominantly inherited epidermal acantholysis in dogs, simulating human benign familial chronic pemphigus (Hailey–Hailey disease)." British Journal of Dermatology 136, no. 2 (1997): 190–96. http://dx.doi.org/10.1111/j.1365-2133.1997.tb14894.x.

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45

Varada, Sowmya, Marigdalia K. Ramirez-Fort, Yahya Argobi, and A. David Simkin. "Remission of Refractory Benign Familial Chronic Pemphigus (Hailey-Hailey Disease) with the Addition of Systemic Cyclosporine." Journal of Cutaneous Medicine and Surgery 19, no. 2 (2015): 163–66. http://dx.doi.org/10.2310/7750.2014.14037.

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Background Benign chronic familial pemphigus (BFCP) is an autosomal dominant dermatosis characterized by flares of painful and often debilitating blistering lesions in high friction areas of the body such as the groin, axillae, lateral neck, and intergluteal cleft. Limited knowledge of its pathophysiology has made treatment of BFCP a considerable challenge and efficacy with current first line therapies, topical corticosteroids and antibiotics, is variable. Case Report We present a case of this disease in a 52 year old woman that has responded dramatically to the addition of oral cyclosporine t

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46

Tchernev, Georgi, and José Carlos Cardoso. "Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option." Revista médica de Chile 139, no. 5 (2011): 633–37. http://dx.doi.org/10.4067/s0034-98872011000500011.

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47

Kubanov, A. A., L. F. Znamenskaya, and T. V. Abramova. "Differential diagnostics of bullous dermatoses." Vestnik dermatologii i venerologii 92, no. 6 (2016): 43–56. http://dx.doi.org/10.25208/0042-4609-2016-92-6-43-56.

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Bullous dermatoses are the diseases characterized by vesication and erosion of skin and/or mucos coats, production of autoantibodies against the structural components of epidermis and/or derma. The most frequent diseases of these disorders are pemphigus, bullous pemphigoid, dermatitis herpetiformis, beningn familial chronic pemphigus (Gougerot Hailey-Hailey disease).Diagnostics of bullous dermatoses is based on assessement of clinical signs, results of cytological and histological studies and immunological tests. However none of the current diagnostic methods allows to reveal bullous dermatosi

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48

Li, X., D. Zhang, S. Xiao, and Z. Peng. "Four novel mutations of the ATP2C1 gene in Chinese patients are associated with familial benign chronic pemphigus." Clinical and Experimental Dermatology 37, no. 7 (2012): 797–99. http://dx.doi.org/10.1111/j.1365-2230.2012.04364.x.

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49

DON, PHILIP C., PATRICK S. CARNEY, WILLIAM S. LYNCH, M. TARIF ZAIM, and MEDHAT O. HASSAN. "Carbon Dioxide Laser abrasion: A New Approach to Management of Familial Benign Chronic Pemphigus (Hailey-Hailey Disease)." Journal of Dermatologic Surgery and Oncology 13, no. 11 (1987): 1187–98. http://dx.doi.org/10.1111/j.1524-4725.1987.tb02430.x.

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50

Lapa, Tatiana, and Maksym Breslavets. "Treatment of Hailey–Hailey disease with narrowband phototherapy and acitretin: A case report." SAGE Open Medical Case Reports 7 (January 2019): 2050313X1984522. http://dx.doi.org/10.1177/2050313x19845221.

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The Hailey–Hailey disease, or familial benign chronic pemphigus, is an autosomal dominant genodermatosis affecting mainly intertriginous areas. It manifests itself in painful blisters, erosions, and cracks and has a chronic course with frequent flares, significantly impacting patients’ quality of life. Presently, there is no cure, but multiple treatment modalities are available. Most evidence supports treatment with topical steroids and antimicrobials. Treatment of recalcitrant disease has been shown to benefit from the addition of oral antibiotics, Naltrexone, systemic retinoids, botulinum to

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