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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 36  |  Issue : 2  |  Page : 57-59

A rare case of isolated extragenital bullous lichen sclerosus


Department of Dermatology, Swami Dayanand Hospital, New Delhi, India

Date of Submission17-Oct-2015
Date of Acceptance27-May-2016
Date of Web Publication21-Mar-2017

Correspondence Address:
Kajal Manchanda
Department of Dermatology, Swami Dayanand Hospital, New Delhi 110093
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-6530.202640

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  Abstract 


Lichen sclerosus et atrophicus is a rare, chronic, mucocutaneous disorder of unknown etiology predominantly involving the anogenital skin and mucosa. It is rarely seen exclusively at an extragenital site. A 40-year-old man presented with multiple asymptomatic clear fluid-filled vesicles with shiny atrophic papules over bilateral thighs and the calf region for a period of 4 months. Histopathology confirmed the diagnosis of bullous lichen sclerosus. The patient showed significant improvement with topical clobetasol propionate 0.05% and tacrolimus 0.1% ointment.

Keywords: bullous, extragenital, lichen sclerosus


How to cite this article:
Manchanda K, Rohatgi PC, Mohanty S. A rare case of isolated extragenital bullous lichen sclerosus. Egypt J Dermatol Venerol 2016;36:57-9

How to cite this URL:
Manchanda K, Rohatgi PC, Mohanty S. A rare case of isolated extragenital bullous lichen sclerosus. Egypt J Dermatol Venerol [serial online] 2016 [cited 2020 May 28];36:57-9. Available from: http://www.ejdv.eg.net/text.asp?2016/36/2/57/202640




  Introduction Top


Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory disorder of unknown etiology predominantly affecting the anogenital skin and mucosa in both sexes. It was first reported by Hallopeau in 1887, and Darier first reported the characteristic histopathological changes in 1892 [1]. It preferentially affects women in the fifth or sixth decade of life and children younger than 10 years of age [2]. It is estimated that in 15–20% of cases there is also extragenital involvement, whereas in only 2.5% of cases it is found exclusively at an extragenital site [3]. Here, we report a rare case of isolated, extragenital, nonhemorrhagic bullous lichen sclerosus in an adult male patient.


  Case report Top


A 40-year-old man presented with multiple asymptomatic fluid-filled lesions over both legs for 4 months. Lesions were gradually increasing in size and number. All Patients gave their formal consent. The protocol was approved the Ethical committee of the Swami Dayanand Hospital. There was no history suggestive of genital mucosal involvement. There were no other systemic complaints or similar history in family members. On examination, there were clear fluid-filled vesicles ranging from 1 to 4 mm. Most of the vesicles were present over shiny atrophic skin and few over normal skin. These vesicles were arranged in a linear pattern at multiple places, suggestive of isomorphic phenomenon of Koebner. Multiple skin-colored erythematous shiny papules along with atrophy were also present. Lesions were localized to the lateral aspect of bilateral thighs and the calf region ([Figure 1]a and [Figure 1]b). Mucosal (both oral and genital), hair, and nail examination were normal. On the basis of clinical examination, differential diagnosis of subepidermal blistering disorders such as epidermolysis bullosa aquisita, variant of bullous pemphigoid, bullous LSA, and bullous lichen planus were considered. Routine hematological investigations were within normal limits. Histopathological examination of the biopsy specimen from the vesicular lesion over the lateral aspect of the left thigh showed moderately dense superficial and deep perivascular infiltrate of lymphocytes and histiocytes with edema and sclerosis of the papillary dermis. The papillary dermal collagen was thickened and closely packed giving hyalinized appearance. The epidermis was separated from underlying dermis and showed focal interface vacuolar change. The stratum corneum was thick and lamellated ([Figure 2]a and [Figure 2]b).
Figure 1 (a) Multiple, discrete, clear, fluid-filled vesicles over the posterior aspect of the calf along with few atrophic areas. (b) Clinical picture showing few vesicles, multiple areas of erythematous, and shiny atrophic papules in a linear pattern along with postinflammatory changes.

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Figure 2 (a) Hyalinization of collagen in upper papillary dermis along with separation of the epidermis from the dermis (H&E, ×40). (b) Perivascular lymphocytic infiltrate with edema and sclerosis of the papillary dermis (H&E, ×100).

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Direct immunoflourescence from the perilesional skin was negative for IgG, IgA, and C3, which excluded the diagnosis of subepidermal blistering disorders. A final diagnosis of bullous LSA was thus established. The patient was started on topical clobetasol 0.05% along with topical tacrolimus 0.1% ointment.


  Discussion Top


LSA is a rare, chronic, mucocutaneous disorder of unknown etiology. It is predominantly present in women, with a female : male ratio of 5 : 1 and may occur at any age. However, the maximum incidence is between the fifth and sixth decade of life and there is a second peak in girls between the ages of 8 and 13 years [2],[4].

It is found only at an extragenital site in roughly 2.5% of the cases [3]. The etiology is unknown, but genetic susceptibility and autoimmune mechanisms have been suggested. Various other factors, including low levels of androgens, chronic infections, and trauma, have been implicated [3],[5].

Extragenital LSA is usually asymptomatic and commonly involves the neck and shoulders, axilla, upper arms, flexor aspects of wrists, and around the umbilicus. Lesions have been described along the Blaschko’s lines, and disseminated forms have also been reported [5],[6],[7].

In our case, lesions were confined to the thighs and calves, which is a rare site of involvement.

Lesions have been found to be induced at the sites of trauma (Koebner phenomenon), and extragenital lesions commonly occur in pre-existing scars and damaged areas [8]. In our case, subclinical trauma and pressure might have resulted in the lesions over the thighs and calves.

The lesions in LSA are small, ivory or porcelain-white, shiny, round macules or papules, coalescing to form sclerotic plaques. Blisters (occasionally hemorrhagic) may rarely develop when the lichenoid infiltrate separates epidermis from the sclerotic dermis [2]. Our case presented with nonhemorrhagic blisters along with shiny atrophic papules.

Histologically, a well-developed lesion of LSA is characterized by marked orthohyperkeratosis, keratotic plugging of the hair follicles and dermal appendages, atrophy of the stratum malpighii with flattening, and loss of rete ridges. The basal layer shows liquefactive degeneration. Beneath the epidermis, there is a broad zone of pronounced edema and homogenization of the collagen.

Extensive vacuolar degeneration of the basal layer and edema may lead to fragility of the dermal–epidermal junction resulting in bulla formation [4]. Our case showed characteristic findings on histopathology.There is no definitive treatment for LSA, although various therapeutic options have been tried. Numerous therapies have been used, including topical corticosteroids, ointments containing estrogens or testosterone, retinoids, topical calcineurin inhibitors, systemic corticosteroids, systemic hydroxychloroquine, phototherapy, and surgery [9]. Our patient was prescribed topical clobetasol 0.05% once daily along with topical tacrolimus 0.1% ointment daily. There was a significant improvement after 6 weeks of follow-up.

Isolated extragenital nonhemorrhagic bullous morphology of LSA is a rare presentation and often misleads the physician. The authors here want to highlight that the diagnosis of LSA must be considered in such a presentation and confirmed by histopathological examination for appropriate treatment of the patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol 1995; 32:393–416.  Back to cited text no. 1
    
2.
Hengge UR. Lichen sclerosus. In: Fitzpatrick TB, Wolff K, Goldsmith AL, Gilchrest AB, Paller SA, Leffell JD, editors. Dermtology in general medicine. 7th ed. New York, NY: Mc-Graw-Hill; 2008. 546–550.  Back to cited text no. 2
    
3.
Heymann WR. Lichen sclerosus. J Am Acad Dermatol 2007; 56:683–684.  Back to cited text no. 3
    
4.
Ballester I, Bañuls J, Pérez-Crespo M, Lucas A. Extragenital bullous lichen sclerosus atrophicus. Dermatol Online J 2009; 15:6.  Back to cited text no. 4
    
5.
Trinh TV, Parr K, Butler DF. Disseminated extragenital bullous lichen sclerosus. Indian Dermatol Online J 2014; 5:66–68.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Khatu S, Vasani R. Isolated, localised extragenital bullous lichen sclerosus et atrophicus: a rare entity. Indian J Dermatol 2013; 58:409.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Choi SW, Yang JE, Park HJ, Kim CW. A case of extragenital lichen sclerosus following Blaschko’s lines. J Am Acad Dermatol 2000; 43(Pt 2): 903–904.  Back to cited text no. 7
    
8.
Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet 1999; 353:1777–1783.  Back to cited text no. 8
    
9.
Neill SM, Tatnall FM, Cox NH. Guidelines for the management of lichen sclerosus. Br J Dermatol 2002; 147:640–649.  Back to cited text no. 9
    


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