We report a 48 year-old man with a family history of hailey-hailey disease who had outbreaks of rashes and blisters of the neck, axillary (<xref ref-type="fig" rid="F1">Fig. 1</xref>) and inguinal folds. These lesions were recurrent every year. The histological examination of skin biopsy revealed intraepidermal and suprabasilar acantholysis (<xref ref-type="fig" rid="F2">Fig. 2</xref>). Elongated papillae extend into lacunae, and a single layer of basal cells lines the villi (<xref ref-type="fig" rid="F3">Fig. 3</xref>). The diagnosis of Hailey-Hailey disease was retained and the patient was treated by disulone. <fig id="F1"> <label>Figure 1</label> <caption> Erythematous and cracked plaques of the armpit. </caption> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="OURD-6-356-g001.tif"/> </fig> <fig id="F2"> <label>Figure 2</label> <caption> Intraepidermal and suprabasilar acantholysis, (HE x 20) </caption> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="OURD-6-356-g002.tif"/> </fig> <fig id="F3"> <label>Figure 3</label> <caption> Elongated papillae or villi lined by a single layer of basal cells, (HE x 40) </caption> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="OURD-6-356-g003.tif"/> </fig> Prior to the study, patient gave written consent to the examination and biopsy after having been informed. Hailey–Hailey disease, or familial benign chronic pemphigusor familial benign pemphigus is a rare hereditary blistering skin disease. It can occur at any age but usually appears in the third or fourth decade. Clinically, it typically begins as a painful erosive skin rash in the skin folds. The lesions may come and go and usually heal without scarring. Common sites include the armpits, groins, and neck, under the breasts and between the buttocks. The genetic defect responsible has now been identified on a gene called <italic>ATP2C1</italic> found on chromosome 3q21-24. Usually Hailey-Hailey disease is diagnosed by its appearance and the family history. Diagnosis may require a skin biopsy. There is no specific treatment and the underlying genetic defect cannot be altered; however, treatment does help and long remissions are common. </sec> <sec id="sec1-2"> <title>CONSENT

OURD

Our Dermatol Online

2081-9390

Our Dermatology Online

Poland

OURD-6-356

10.7241/ourd.20153.95

Clinical Image

A case of Hailey-Hailey disease

Attafi

Salsabil

Khayat

Olfa

Debbiche

Aschraf

Department of Pathology, Habib Thameur Hospital, Tunis, Tunisia

Corresponding author: Dr. Salsabil Attafi, E-mail: sehlisalsabil@hotmail.com

2015

6 3 356 357 23122014 03032015

2015

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

We report a 48 year-old man with a family history of hailey-hailey disease who had outbreaks of rashes and blisters of the neck, axillary (<xref ref-type="fig" rid="F1">Fig. 1</xref>) and inguinal folds. These lesions were recurrent every year. The histological examination of skin biopsy revealed intraepidermal and suprabasilar acantholysis (<xref ref-type="fig" rid="F2">Fig. 2</xref>). Elongated papillae extend into lacunae, and a single layer of basal cells lines the villi (<xref ref-type="fig" rid="F3">Fig. 3</xref>). The diagnosis of Hailey-Hailey disease was retained and the patient was treated by disulone. <fig id="F1"> <label>Figure 1</label> <caption> Erythematous and cracked plaques of the armpit. </caption> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="OURD-6-356-g001.tif"/> </fig> <fig id="F2"> <label>Figure 2</label> <caption> Intraepidermal and suprabasilar acantholysis, (HE x 20) </caption> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="OURD-6-356-g002.tif"/> </fig> <fig id="F3"> <label>Figure 3</label> <caption> Elongated papillae or villi lined by a single layer of basal cells, (HE x 40) </caption> <graphic xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="OURD-6-356-g003.tif"/> </fig> Prior to the study, patient gave written consent to the examination and biopsy after having been informed. Hailey–Hailey disease, or familial benign chronic pemphigusor familial benign pemphigus is a rare hereditary blistering skin disease. It can occur at any age but usually appears in the third or fourth decade. Clinically, it typically begins as a painful erosive skin rash in the skin folds. The lesions may come and go and usually heal without scarring. Common sites include the armpits, groins, and neck, under the breasts and between the buttocks. The genetic defect responsible has now been identified on a gene called <italic>ATP2C1</italic> found on chromosome 3q21-24. Usually Hailey-Hailey disease is diagnosed by its appearance and the family history. Diagnosis may require a skin biopsy. There is no specific treatment and the underlying genetic defect cannot be altered; however, treatment does help and long remissions are common. </sec> <sec id="sec1-2"> <title>CONSENT

The examination of the patient was conducted according to the Declaration of Helsinki principles.

Source of Support: Nil,

Conflict of Interest: None declared.