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 Table of Contents  
Year : 2019  |  Volume : 6  |  Issue : 2  |  Page : 117-118

A late presentation of Hailey–Hailey disease with clinical-dermoscopic-histological correlation

1 Bhojani Clinic, Mumbai, Maharashtra, India
2 Dr. Save's Clinic, Mumbai, Maharashtra, India

Date of Web Publication28-Nov-2019

Correspondence Address:
Sushrut Save
Dr. Save's Clinic, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijdpdd.ijdpdd_34_19

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How to cite this article:
Vasani R, Save S. A late presentation of Hailey–Hailey disease with clinical-dermoscopic-histological correlation. Indian J Dermatopathol Diagn Dermatol 2019;6:117-8

How to cite this URL:
Vasani R, Save S. A late presentation of Hailey–Hailey disease with clinical-dermoscopic-histological correlation. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2022 Jan 24];6:117-8. Available from: https://www.ijdpdd.com/text.asp?2019/6/2/117/271942


Hailey–Hailey disease (HHD) is a blistering disease caused by mutation in ATP2C1 gene with autosomal dominant inheritance. The usual age of presentation is late teenage or in adulthood (30s and 40s).[1] Delayed diagnosis is common, especially if the patient's lesions respond to topical steroids and antifungals.[1] We present a clinically misdiagnosed case in an elderly female and attempt a clinic-dermoscopic-histological correlation.

A 77-year-old female presented with intensely pruritic lesions over the groins and perianal area since 2 years. She was previously diagnosed as a case of tinea cruris et corporis and had received repeated courses of oral and topical antifungals along with antihistamines and intermittent topical steroids for a variable period. She was a known case of rheumatoid arthritis since 15 years and was on prednisolone 10 mg daily, along with oral methotrexate 7.5 mg/week since 3 years. There was no other significant past, personal, or family history.

The clinical examination revealed ill-defined erythematous to violaceous macerated plaques over bilateral groins and labia majora extending onto its inner aspect [Figure 1]a and [Figure 1]b. Similar plaques were noted over the perianal area [Figure 1]c. Few erosions were noted over the plaques, which were attributable to the intense pruritus. The remaining dermatological examination was normal. Considering the violaceous appearance of the lesions, a clinical diagnosis of inverse lichen planus with genital involvement was made.
Figure 1: (a) Erythematous to violaceous plaques over the inner aspects of the labia majora. (b) Erythematous plaque on the groin with areas showing minute erosions and hemorrhagic crusting in the periphery. (c) Hyperpigmented to violaceous macerated plaque in the perianal area

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Dermoscopy of the lesions performed using DermLite DL4N in polarized mode showed linear red to brown linear ulcers with sharp angulated margins along with whitish macerated edges on a pinkish-white background. Arborizing telangiectasia were seen in the periphery [Figure 2]. Histopathology showed acantholysis of the whole epidermis with classical dilapidated brick wall appearance with acanthosis and dyskeratosis, features diagnostic of HHD [Figure 3]a and [Figure 3]b.
Figure 2: Black arrow: Linear ulcers with sharp angulated borders blue arrow: White margins of ulcers red arrow: Arborizing telangiectasias

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Figure 3: (a) Histopathology – H and E, ×4 – Hyperkeratosis, acanthosis with full-thickness acantholysis of the epidermis giving a dilapidated brick wall appearance. Papillary dermis shows lymphocytic infiltrate. (b) Histopathology – H and E, ×40 – Full-thickness acantholysis of the epidermis with the presence of acantholytic cells. Dermal papillae with dilated blood vessels are seen projecting in the suprabasal separation

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The diagnosis was intriguing considering the late age of onset, absent family history, ongoing immunosuppressive treatment, and absence of seasonal variation.

HHD (familial benign pemphigus) is an autosomal dominant genetic disorder characterized by painful blistering and erosions affecting the flexural areas.[1] The onset is usually between third and fourth decade of life; however, it may manifest in early childhood or just after puberty.

Although the diagnosis is straight forward in most cases, the presentation was quite unusual in our case considering the age of onset being over 70 years.[2]

The dermoscopic finding of pink and white areas in a cloud-like pattern described in HHD [3] was not a prominent feature in our case; however, some interesting points in dermoscopic-histopathological correlation are listed in [Table 1].[4],[5]
Table 1: Dermoscopic-histopathological correlation of Hailey-Hailey disease

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The sharp angulated borders of the linear ulcers are considered a marker of dermoscopic Nikolsky.[5] The whitish margins of the fissures can be attributed to the hyperkeratosis and acanthosis along with the associated maceration owing to the intertriginous location of the lesion.[6] Hence, the characteristic clinical feature of Hailey and Hailey-fissures or rhagades which may not be visible to the naked eye, as in this case, can be better visualized using a dermatoscope can aid the diagnosis earlier.[7]

In conclusion, we present a rare case of late HHD with no associated family history in a patient of rheumatoid arthritis on immunosuppressive therapy. Although the characteristic histopathological picture clinched the diagnosis, we wish to highlight the importance of noninvasive dermoscopy in helping to diagnose the condition early.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We would like to acknowledge Dr. Subrata Malakar for the help with the dermoscopic description of this entity.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Vasudevan B, Verma R, Badwal S, Neema S, Mitra D, Sethumadhavan T, et al. Hailey-Hailey disease with skin lesions at unusual sites and a good response to acitretin. Indian J Dermatol Venereol Leprol 2015;81:88-91.  Back to cited text no. 1
[PUBMED]  [Full text]  
Wick MR. Bullous, pseudobullous, & pustular dermatoses. Semin Diagn Pathol 2017;34:250-60.  Back to cited text no. 2
Kelati A, Argenziano G, Mernissi FZ. Dermoscopic presentation of Hailey-Hailey disease. J Am Acad Dermatol 2017;76:S31-3.  Back to cited text no. 3
Giacomel J, Zalaudek I, Argenziano G. Dermatoscopy of Grover's disease and solitary acantholytic dyskeratoma shows a brown, star-like pattern. Australas J Dermatol 2012;53:315-6.  Back to cited text no. 4
Specchio F, Argenziano G, Tiodorovic-Zivkovic DM, Moscarella E, Lallas A, Zalaudek I, et al. Dermoscopic clues to diagnose acantholytic dyskeratosis. Dermatol Pract Concept 2015;5:59-60.  Back to cited text no. 5
Apalla Z, Lallas A, Sotiriou E, Lazaridou E, Vakirlis E, Ioannides D, et al. Stellate erosion: The dermoscopic Nikolsky sign? Eur J Dermatol 2017;27:659-60.  Back to cited text no. 6
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 2017. p. 1-8.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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