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Figure 1. Left, Rash of right axilla. Right, Rash of right lateral area of the neck with global view and close-up view (inset).
Diagnosis unlikely to vary with season. A crusted rash with seasonal variation
Hailey-Hailey disease (benign familial pemphigus) could suggest seborrheic dermatitis or candidal intertrigo, which
can be treated with ketoconazole. Seborrheic dermatitis, however,
What to Do Next usually involves the face or scalp and worsens during winter. Lack
C. Prescribe a topical corticosteroid and a topical antimicrobial of satellite lesions makes candidal intertrigo unlikely.
Hailey-Hailey disease is a rare autosomal dominant acantholytic
Discussion disease with variable penetrance.3,4 The disease was first described in
The key to the correct diagnosis is the presence of an intertriginous 1939bybrothersWilliamandHughHailey,whowerebothafflictedwith
rash with report of blistering, summertime exacerbation, and fam- the condition.3 Hailey-Hailey disease is often misdiagnosed and is part
ily history of a similar rash. First-line treatment for Hailey-Hailey dis- of a broad differential diagnosis that includes common diseases such
ease is topical and should include a topical corticosteroid with or with- as intertrigo, inverse psoriasis, seborrheic dermatitis, erythrasma, and
out a topical antimicrobial.1,2 Hailey-Hailey disease can have an acanthosis nigricans, as well as rare diseases such as Darier disease,
appearance similar to that of acanthosis nigricans in dark-skinned Galli-Galli disease, and autoimmune pemphigus variants.3-5 Accurate
patients, but acanthosis nigricans is typically asymptomatic and diagnosis and proper treatment are essential, because Hailey-Hailey
jama.com (Reprinted) JAMA April 10, 2018 Volume 319, Number 14 1499
ARTICLE INFORMATION 2. Arora H, Bray FN, Cervantes J, Falto Aizpurua LA. 8. Tomaszewska KA, Gerlicz-Kowalczuk Z, Kręgiel
Author Affiliations: Center for Clinical Studies, Management of familial benign chronic pemphigus. M, et al. The coexistence of Darier’s disease and
Webster, Texas (Haley, Mui, Tyring); Department of Clin Cosmet Investig Dermatol. 2016;9:281-290. Hailey-Hailey disease symptoms. Postepy Dermatol
Dermatology, University of Texas Health Science 3. Engin B, Kutlubay Z, Çelik U, Serdaroğlu S, Tüzün Y. Alergol. 2017;34(2):180-183.
Center at Houston, Houston (Tyring). Hailey-Hailey disease: a fold (intertriginous) 9. de Aquino Paulo Filho T, deFreitas YK, da Nóbrega
Corresponding Author: Christopher Haley, MD, dermatosis. Clin Dermatol. 2015;33(4):452-455. MT, et al. Hailey-Hailey disease associated with
Center for Clinical Studies, 451 N Texas Ave, 4. Chiaravalloti A, Payette M. Hailey-Hailey disease herpetic eczema-the value of the Tzanck smear test.
Webster, TX 77598 (chaley1029@gmail.com). and review of management. J Drugs Dermatol. Dermatol Pract Concept. 2014;4(4):29-31.
Conflict of Interest Disclosures: All authors have 2014;13(10):1254-1257. 10. Ibrahim O, Hogan SR, Vij A, Fernandez AP.
completed and submitted the ICMJE Form for 5. Wolf R, Oumeish OY, Parish LC. Intertriginous Low-dose naltrexone treatment of familial benign
Disclosure of Potential Conflicts of Interest and eruption. Clin Dermatol. 2011;29(2):173-179. pemphigus (Hailey-Hailey disease). JAMA Dermatol.
none were reported. 2017;153(10):1015-1017.
6. Missiaen L, Dode L, Vanoevelen J, Raeymaekers
Additional Contributions: We thank the patient for L, Wuytack F. Calcium in the Golgi apparatus. Cell
providing permission to share his information. Calcium. 2007;41(5):405-416.
7. Burge SM, Millard PR, Wojnarowska F.
REFERENCES
Hailey-Hailey disease: a widespread abnormality of
1. Burge SM. Hailey-Hailey disease: the clinical cell adhesion. Br J Dermatol. 1991;124(4):329-332.
features, response to treatment and prognosis. Br J
Dermatol. 1992;126(3):275-282.
1500 JAMA April 10, 2018 Volume 319, Number 14 (Reprinted) jama.com