Dermatology: Practical and Conceptual Observation | Dermatol Pract Concept 2014;4(4):4 29 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Introduction Herpetic eczema (HE), also known as Kaposi varicelliform eruption, is the dissemination of the herpes simplex virus in the same area of a pre-existing skin disease. Hailey-Hailey disease (HHD), or benign familial pemphigus, is autosomal dominant and manifests through erythematous plaques with flaccid blisters that rupture easily. It results in superficial linear erosions with crusts and maceration in intertriginous areas. The coexistence between HE and HHD is a rare condi- Hailey-Hailey disease associated with herpetic eczema—the value of the Tzanck smear test Thomás de Aquino Paulo Filho1, Yara Kelly Rodrigues deFreitas1, Mylenne Torres Andrade da Nóbrega1, Carlos Bruno Fernandes Lima1, Barbara Luiza Medeiros Francelino Carriço1, Maria Aurora Pinto Leite e Silva1, Filipe Lauria Paulo1, Pedro Bezerra da Trindade Neto1 1 Department of Clinical Medicine, Dermatology Center, Rio Grande do Norte University, Natal, Brazil Keywords: benign familial pemphigus, Kaposi varicelliform eruption, Hailey-Hailey disease Citation: Paulo Filho TA, deFreitas YK, da Nóbrega MT, Lima CB, Carriço BF, Leite Silva MA, Lauria Paulo F, Trindade Neto P. Hailey- Hailey disease associated with herpetic eczema—the value of the Tzanck smear test. Dermatol Pract Concept. 2014;4(4):4. http://dx.doi. org/10.5826/dpc.0404a04 Received: August 8, 2014; Accepted: September 11, 2014; Published: October 31, 2014 Copyright: ©2014 Paulo Filho et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Thomas de Aquino Paulo Filho, MD, 240, Deodoro Avenue, 59012600, Natal, RN, Brazil. Email: thomasfi@uol.com.br Background: Herpetic eczema is a herpetic superinfection of a preexisting skin disease. Hailey-Hailey disease is an autosomal dominant dermatosis that is clinically characterized by flaccid vesicles and rashes in intertriginous areas. The coexistence of those findings is a rare condition; only five cases have been published in literature. Purpose: To report the rare coexistence between Hailey-Hailey disease and herpetic eczema and to highlight the importance of cytology for a quick diagnosis. Case report: A 38-year-old man had been diagnosed with Hailey-Hailey disease for 13 years. His condition evolved into what could be herpetic eczema, which was later confirmed by skin cytology and histopathology. The man showed remission in the infection after 10 days under treatment with acyclovir. Conclusion: Research on the concomitance of infection by the herpes virus must be performed in the exacerbations of Hailey-Hailey disease, and, in those situations, the quick diagnosis through skin cytology makes the early treatment possible. ABSTRACT 30 Observation | Dermatol Pract Concept 2014;4(4):#4 tion with only five cases that have been described in literature [1,2]. The diagnosis should be clinically suspected as the show- ing up of monomorphic eruption of vesiculopustules in the areas that had previously been affected by the underlying disease. The diagnosis is confirmed by viral culture, direct immunofluorescence, polymerase chain reaction (PCR) proce- dure or by skin cytology (Tzanck test); the last one is a rapid and practical method that shows giant viral multinucleated cells, which is an indirect piece of evidence of the presence of herpetic infection [1,2,3,6]. Case report A 38-year-old male single driver has been diagnosed with Hailey-Hailey disease, which has affected his axillary and inguinal regions for 13 years. Three months prior, he was being treated with acitretin 50 mg/day and desonide 0.05% alternating with tacrolimus 0.1% topically in the areas of exacerbation of lesions. A burning sensation, followed by redness, vesicles and subsequent ulcers in the inguinal region for about two days was reported. Physical examination showed vesiculopustules and areas with ulcerations in the inguinal region (Figure 1). Skin cytology showed giant viral multinucleated cells and characteristic changes of the underly- ing disease—acantholytic cells, not only parabasal ones, but also those from upper layers of the epidermis, giving them the aspect of “crumbling wall” (Figure 2). Histopathology confirmed the diagnosis of Hailey-Hailey disease and herpetic eczema (Figure 3). The treatment with acyclovir 400 mg three times daily for ten days was recommended with complete remission of the herpetic condition (Figure 4). Discussion HHD was first described in 1939. It normally appears in the third or fourth decade of a person’s life, but it may occur at any age. It is a rare entity, and a person may have multiple Figure 1. Presence of vesicopustules and ulcerations in an erythe- matosus base in the inguinal region. (Copyright: ©2014 Paulo Filho et al.) Figure 2. Giant multinucleated cells and acantholytic cells in the same slide. Giemsa stain, at 400x. (Copyright: ©2014 Paulo Filho et al.) Figure 3. (A) Intraepidermal suprabasal acantholytic blisters with dyskeratosis of keratinocytes. (B) Giant multinucleated cells. H&E, at 400x. (Copyright: ©2014 Paulo Filho et al.) Observation | Dermatol Pract Concept 2014;4(4):4 31 relapses and remissions of the disease [1,4]. It occurs due to mutations on the ATPC21 gene that causes failure in kerati- nocyte adhesion. The epidermal defect leads to spontaneous acantholysis or it might occur as a result of friction or infec- tions [1]. Infections caused by herpes simplex virus may lead to exacerbation of the lesions [2]. HE is a viral infection caused by herpes simplex (poten- tially fatal) that appears in preexistent conditions such as atopic and contact dermatitis, Darier disease, pemphigus, pityriasis rubra pilaris, skin lymphoma, and benign familial pemphigus among others. The risk factors in the pathogen- esis include rupture of the epidermal barrier and the use of topical calcineurin inhibitors; some authors also describe the topical use of corticosteroids [2,5]. Our patient appeared to be susceptible to the development of the condition presented since he had the main risk factors. The diagnosis of coexistence of those findings may be reached in a reliable way through skin cytology, which is a simple, quick and viable method that shows sensitivity higher than 80%, making the establishment of early treatment pos- sible. To confirm HE, the presence of giant viral multinucleated cells that might contain inclusion bodies is necessary. On the other hand, the parabasal and upper layers of the skin acan- tholytic cells may be observed, having the aspect of “crumbling wall” [1,3]. The current therapeutic strategies for HHD aim to reduce outbreaks and improve the patients’ quality of life. Oral retinoids, antibiotics, corticosteroids, cyclosporine, topical vitamin D analogues, calcineurin inhibitors, botulinum toxin, surgical excision and CO2 lasers may be utilized [4]. Upon the occurrence of HE, the treatment must be promptly pro- vided. The early use of antiviral drugs—acyclovir, famciclovir and valacyclovir—is extremely important in order to avoid potentially fatal complications [1,2]. Thus, it is important to stress the possibility of concomi- tant infection by the herpes virus in the frequent exacerba- tions of Hailey-Hailey disease and, in those cases, the quick diagnosis through skin cytology will allow the initiation of early treatment in order to avoid complications that can be potentially fatal. References 1. Lee GH, Kim YM, Lee SY, et al. A case of eczema herpeticum with Hailey-Hailey disease. Ann Dermatol. 2009; 21:311-4. 2. Olson J, Robles DT, Kirby P, Colven R. Kaposi varicelliform erup- tion (eczema herpeticum). 2008;14:18. 3. Ruocco E, Brunetti G, Del Vecchio M, Ruocco V. The practical use of cytology for diagnosis in dermatology. JEADV. 2011; 25:125–9. 4. Berger EM, Galadari HI, Gottlieb AB. Successful treatment of Hai- ley-Hailey disease with acitretin. J Drugs Dermatol. 2007; 6:734-6. 5. Wollenberg A, Zoch C, Wetzel S, Plewig G, Przybilla B. Predispos- ing factors and clinical features of eczema herpeticum: a retrospec- tive analysis of 100 cases. J Am Acad Dermatol 2003;49:198-205. 6. Paulo Filho TA. Citodiagnóstico prático em dermatologia. A Pele. 2006;37:6. Figure 4. Post-treatment (presence of erythema with no signs of vesi- copustules). (Copyright: ©2014 Paulo Filho et al.)