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Benign Familial Pemphigus in Skin of Color

To identify the key insights or developments described in this article
1 Credit CME

An African American man in his 40s presented with a 10-year history of pruritic intertriginous eruption. The patient had no improvement with treatment with triamcinolone, 0.1%, ointment. Physical examination showed friable erythematous plaques on the neck, inguinal folds, buttocks, bilateral axillae, and bilateral antecubital fossae. Punch biopsy results of the right axilla revealed acantholysis, confirming the diagnosis of benign familial pemphigus. Treatment with oral naltrexone, 4.5 mg, once daily, was initiated, which was followed by substantial clinical improvement within 6 weeks. An acute flare secondary to medication noncompliance prompted the addition of clobetasol, 0.05%, and mupirocin, 2%, external ointments to the treatment regimen for 4 weeks and on an as-needed basis after. Symptoms were controlled for 9 months until he developed a flare in the bilateral axillae and inguinal folds (Figure), at which point treatment with isotretinoin, 40 mg, once daily, was initiated in addition to continuing use of low-dose naltrexone.

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Article Information

Corresponding Author: Sami K. Saikaly, MD, Department of Dermatology, University of Florida College of Medicine, 4037 NW 86 Terr, 4th Floor, Gainesville, FL 32606 (ssaikaly13@dermatology.med.ufl.edu).

Published Online: January 11, 2023. doi:10.1001/jamadermatol.2022.5117

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Langenberg  A , Berger  TG , Cardelli  M , Rodman  OG , Estes  S , Barron  DR .  Genital benign chronic pemphigus (Hailey-Hailey disease) presenting as condylomas.   J Am Acad Dermatol. 1992;26(6):951-955. doi:10.1016/0190-9622(92)70140-B PubMedGoogle ScholarCrossref
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Oliveira  A , Arzberger  E , Pimentel  B , de Sousa  VC , Leal-Filipe  P .  Dermoscopic and reflectance confocal microscopic presentation of Hailey-Hailey disease: a case series.   Skin Res Technol. 2018;24(1):85-92. doi:10.1111/srt.12394 PubMedGoogle ScholarCrossref
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Rogner  DF , Lammer  J , Zink  A , Hamm  H .  Darier and Hailey-Hailey disease: update 2021.   J Dtsch Dermatol Ges. 2021;19(10):1478-1501.PubMedGoogle Scholar
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Michael  M , Benjamin M  W , Shannon C  T , Shah  BJ .  Recalcitrant cases of Hailey-Hailey disease successfully treated with low-dose naltrexone.   J Clin Aesthet Dermatol. 2020;13(11):19-21.PubMedGoogle Scholar
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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