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Year : 2011  |  Volume : 2  |  Issue : 2  |  Page : 109-110  

Incidental Hailey-Hailey like acantholysis in a lesion of borderline tuberculoid leprosy

Consultant Dermatologist and Dermatopathologist, P. D. Hinduja Hospital, Mahim, Mumbai, India

Date of Web Publication14-Oct-2011

Correspondence Address:
Rajiv Joshi
14 Jay Mahal, A Road, Churchgate, Mumbai-400 020
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.86006

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How to cite this article:
Joshi R. Incidental Hailey-Hailey like acantholysis in a lesion of borderline tuberculoid leprosy. Indian Dermatol Online J 2011;2:109-10

How to cite this URL:
Joshi R. Incidental Hailey-Hailey like acantholysis in a lesion of borderline tuberculoid leprosy. Indian Dermatol Online J [serial online] 2011 [cited 2020 Sep 30];2:109-10. Available from: http://www.idoj.in/text.asp?2011/2/2/109/86006


Unusual finding of Hailey-Hailey like acantholysis was seen in the epidermis overlying granulomatous dermatitis in a hypoesthetic plaque on the left forearm in a 50-year-old male.

The clinical suspicion of borderline tuberculoid Hansen' disease was confirmed by the presence of several well-formed oval and slightly elongated epithelioid cell granulomas in periadnexal locations in the dermis. Clinically, there were no blisters or flexural crusted lesions suggestive of  Hailey-Hailey disease More Details.

Hailey-Hailey like acantholysis as an incidental microscopic finding without any clinical evidence or family history of the disease has been described in several unrelated diseases but never before in a lesion of Hansen's disease.

The finding of intraepidermal acantholysis resembling Pemphigus or Hailey-Hailey disease has been described in a case of bullous pemphigoid [1] as well as in other non-blistering diseases. [2],[3],[4],[5] The patients described did not have clinical findings suggestive of either pemphigus or Hailey-Hailey disease and the microscopic findings were considered incidental and not related to the primary disease.

We report one such case where a biopsy from a patient of BT Hansen's disease showed acantholysis of the epidermis resembling Hailey-Hailey disease in several foci .

A 50-year-old male presented with an erythematous, hypoesthetic plaque, 4 by 6 cm in size, on the left extensor forearm with mild thickening of the left ulnar nerve. A clinical diagnosis of borderline tuberculoid Hansen's disease was made and a 4 mm punch biopsy was obtained from the center of the plaque. The biopsy showed several small and medium sized oval and elongated epithelioid cell granulomas surrounded by few lymphocytes. The granulomas were in the upper, mid, and lower dermis and surrounded and infiltrated adnexal structures including an arrectores pilorum muscle in the mid dermis [Figure 1].
Figure 1: Low power view showing dermal granulomas and foci of acantholysis in the epidermis (H and E, ×40)

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The epidermis, however, showed in several foci acantholysis that involved almost the full thickness of the moderately acanthotic epidermis with suprabasal clefting with several partially acantholytic keratinocytes of the spinous layers in the blister cavity [Figure 2] and [Figure 3]. Also in one focus incomplete acantholysis of the epidermal spinous layers giving rise to the "dilapidated brick wall" appearance that is typical of Hailey-Hailey disease was seen [Figure 4].
Figure 2: Medium power view showing dermal granulomas and epidermal acantholysis (H and E, ×100)

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Figure 3: High power view of granuloma and epidermal acantholysis with many partially acantholytic keratinocytes (H and E, ×400)

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Figure 4: Incomplete acantholysis of full thickness of epidermis (H and E, ×400)

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On enquiry the patient did not have any personal or family history of recurrent blisters or crusted lesions in flexural areas, nor any findings to suggest Hailey-Hailey disease.

The incidental microscopic finding of dyskeratotic acantholysis resembling Darier's disease is not uncommon and is often seen in epidermal nevi and keratotic palmo-plantar lesions. It has also been described to occur along with epidermolytic hyperkeratosis in the same biopsy specimen; [6] however, descriptions of other types of incidental acantholysis resembling Pemphigus or Hailey-Hailey disease are few in literature. [2]

This is to the best of our knowledge, the first description of a case of Hansens disease with incidental histological finding of Hailey-Hailey like acantholysis.

We present this case for awareness of this condition as incidental acantholysis may rarely be seen as an isolated microscopic finding in non-blistering unrelated diseases and should not be confused with a primary acantholytic blistering disease by the pathologist. And finally this should also be a lesson to the practicing dermatologist to be aware of such unusual incidental findings and not to take all histology reports as gospel!

   References Top

1.Mehregan DA, Umbert IJ, Peters MS. Histological findings of Hailey-Hailey disease in a patient with bullous pemphigoid. J Am Acad Dermatol 1989;21:1107-12.  Back to cited text no. 1
2.Sánchez Yus E, Requena L, Simón P, de Hijas CM. Incidental acantholysis. J Cutan Pathol 1993;20:418-23.  Back to cited text no. 2
3.Howe K, Foresman P, Griffin T, Johnson W. Pityriasis rubra pilaris with acantholysis. J Cutan Pathol 1996;23:270-4.  Back to cited text no. 3
4.Sebastian A, Koff AB, Goldberg LJ. Pityriasis rubra pilaris with subcorneal acantholysis. J Cutan Pathol 2010;37:99-101.  Back to cited text no. 4
5.Yamamoto T. Incidental acantholysis of the overlying epidermis of dermatofibroma. J Eur Acad Dermatol Venereol 2009;23:735-6.  Back to cited text no. 5
6.Ackerman AB, Goldman G. Combined epidermolytic hyperkeratosis and focal acantholytic dyskeratosis. Arch Dermatol 1974;109:385-6.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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