Dermoscopy of Hailey Hailey Disease.

A 50-year-old female presented with a 2-year history of fluid-filled and raised hyperpigmented lesions over her groin and left thigh. No significant past or family history was present. On examination, multiple, well-defined flaccid vesicles and pustules arranged in an arciform pattern along with raw erosions were present over the inguinoperineal region and the medial aspect of left thigh. Multiple disseminated, dirty looking, greasy, hyperpigmented, keratotic papules forming plaques were also present over the perineal area [Figure 1a and b]. Examination of nails showed longitudinal white bands on the right thumbnail [Figure 1c]. Palms, soles, and oral mucosa were normal. Dermoscopic examination (DermLite II hybrid m; 3Gen; polarized mode, 10 × magnification) of the lesions was done which showed irregular pinkish white areas separated by pink furrows along with white areas in a cloud-like arrangement at places. We also noted whitish areas which were seen as irregularly raised wavy folds giving it a crumpled fabric pattern. Erosions with a few dotted vessels and crusted areas were also seen occasionally [Figure 2]. Dermoscopy of the keratotic papules surrounding the vesiculopustules and erosions showed irregular brownish black to greyish black areas [Figure 3]. Histopathological examination from the pustule revealed hyperkeratosis and acanthosis with a subcorneal bulla showing intraepidermal clefting and acantholytic cells giving a dilapidated brick wall appearance, which was consistent with the diagnosis of Hailey Hailey disease (HHD) [Figure 4].

Figure 1

(a) Vesiclopustules arranged in an annular configuration, multiple greasy hyperpigmented keratotic papules along with raw erosions over the inginoperineal area. (b) Multiple flaccid vesicles and pustules over an erythematous base over the medial aspect of left thigh. (c) Longitudinal white bands on the right thumbnail

Figure 2

(a) Irregular pinkish white areas separated by pink furrows (black arrow) with whitish areas seen in a crumpled fabric pattern (blue diamond). (b) Pink furrows (black arrow) and white areas present in a cloud-like arrangment (black diamond). (c) Crumpled fabric appearance (circle) of the vesiclopustules. Erosions with few dotted vessels also seen. (DermLite II hybrid m; 3Gen; polarized mode, ×10 magnification)

Figure 3

Dermoscopy of the keratotic papules surrounding the vesiculopustules and erosions showing irregular brownish black to greyish black areas

Figure 4

(a) Hyperkeratosis and acanthosis with a subcorneal bulla showing intraepidermal clefting and acantholytic cells giving a dilapidated brick wall appearance (H and E, ×100). (b) Closer view showing acanthoytic cells (H and E, ×400)

HHD is an uncommon autosomal dominant genodermatosis characterized by the development of flexural erosions, blisters, and warty papules.[1] To the best of our knowledge, dermoscopic findings of HHD have been described only once previously and ours is the second report highlighting the dermoscopic features of this rare disease.[2] Dermoscopic findings in this case are in line with those described by Kelati et al.[2] who found similar combination of pink and white areas. In addition, we also describe the crumpled fabric appearance which represents the flaccid vesiclopustules present in HHD. This dermoscopic crumpled fabric appearance has not been described in any blistering disorder hitherto; however, we would like to believe that it can be elicited in blistering disorders where grouped flaccid vesiclopustules form the presentation, as seen typically in HHD. Although the classic presentation and nail findings made the diagnosis straightforward in our case, dermoscopy can be a useful clue in the diagnosis of HHD when faced with an intertriginous rash. Other differentials of HHD include subcorneal pustular dermatosis (SCPD), pustular psoriasis, Dariers disease, and Galli galli disease. We could not find the dermoscopic findings of SCPD, pustular psoriasis, and Galli Galli disease in the literature, and more studies are required in this area to delineate the dermoscopic differences of these disorders. Dermoscopic findings of other flexural disorders are tabulated in Table 1.

Table 1

Dermoscopic findings of flexural disorders

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.