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CASE REPORT
Year : 2016  |  Volume : 36  |  Issue : 1  |  Page : 20-22

Isolated extragenital lichen sclerosus et atrophicans in a middle-aged male: a case report


Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad, Gujarat, India

Date of Submission24-Dec-2015
Date of Acceptance04-May-2016
Date of Web Publication22-Nov-2016

Correspondence Address:
Pragya A Nair
Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad 388325, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-6530.194158

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  Abstract 

Lichen sclerosus et atrophicans (LSA) is an idiopathic inflammatory dermatosis. It mainly affects the anogenital area. Extragenital sites are affected rarely in isolation. It presents as ivory or porcelain-white, shiny, round macules or papules, coalescing to form sclerotic plaques with prominent dilated pilosebaceous or sweat duct orifices. Histopathology is indicated to differentiate it from previtiligo, morphea, and atrophic lichen planus. There is no known cure for LSA. Standard treatment modality includes topical corticosteroid and calcineurin inhibitors, such as tacrolimus. We report a case of a middle-aged man with isolated extragenital LSA.

Keywords: Csillag’s disease, extragenital site, lichen sclerosus et atrophicans


How to cite this article:
Kota RS, Jivani NB, Nair PA. Isolated extragenital lichen sclerosus et atrophicans in a middle-aged male: a case report. Egypt J Dermatol Venerol 2016;36:20-2

How to cite this URL:
Kota RS, Jivani NB, Nair PA. Isolated extragenital lichen sclerosus et atrophicans in a middle-aged male: a case report. Egypt J Dermatol Venerol [serial online] 2016 [cited 2020 Jan 26];36:20-2. Available from: http://www.ejdv.eg.net/text.asp?2016/36/1/20/194158


  Introduction Top


Lichen sclerosus et atrophicans (LSA) is an idiopathic inflammatory dermatosis affecting women 10 times more commonly than men and is usually seen over 50 years of age with a worldwide prevalence of 0.03% [1].

Genetic factors and autoimmunity have been described as possible etiologies. It mainly affects the anogenital area and can also involve extragenital sites occasionally, but rarely isolated extragenital involvement is seen. Extragenital LSA is most commonly located on the neck, upper arms, and flexor surfaces of the wrist, presenting as small, porcelain-white, shiny macules [2]. Symptomatic treatment with emollients, antihistaminics, and topical corticosteroid ointments form the mainstay of treatment. Here, we present the case of a middle-aged man with isolated extragenital LSA unilaterally over the right side of the upper arm.


  Case report Top


A 41-year-old male patient presented to the skin outpatient department with complaints of asymptomatic lesions over the right arm and elbow since 6 months. The patient initially developed red-colored papule over the dorsal aspect of the right arm, which gradually increased in size and also involved the elbow. Lesions in due time became hypopigmented. There was no history of similar complaints in the past or no other associated comorbidities. There was a family history of vitiligo in the mother. On cutaneous examination, multiple hypopigmented macules and papules coalescing to form patches with few plaques and atrophy over the extensor aspect of the right arm [Figure 1]a and the elbow [Figure 1]b were present in a linear pattern [Figure 1]c. Oral cavity, nails, and scalp were normal. No lymphadenopathy was noted. Systemic examination was unremarkable. Clinical diagnosis of extragenital LSA, morphea, and previtiligo was made.
Figure 1: Multiple hypopigmented macules and papules coalescing to form plaques and few patches with atrophy over (a) the extensor aspect of the right arm, (b) the elbow, and (c) the arm and elbow.

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Wood’s lamp examination did not show any accentuation, which ruled out vitiligo. A 4 mm punch biopsy was taken from the lesion over the right arm, which on histopathology showed compact hyperkeratosis with epidermal atrophy. The superficial dermis showed pronounced edema and collagenization with moderate lymphoplasmocytic infiltrate in mid dermis [Figure 2]a and [Figure 2]b. Changes were consistent with diagnosis of LSA. The patient was advised topical steroids twice a day and called after a month, but was lost to follow-up.
Figure 2: (a,b) Compact hyperkeratosis with epidermal atrophy. Pronounced edema and collagenization in superficial dermis and lymphoplasmocytic infiltrate in mid dermis (hematoxylin and eosin stain, ×4 and ×10).

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  Discussion Top


Hallopeau, in 1887, first described LSA as a benign chronic inflammatory dermatosis with unclear pathogenesis affecting both the epidermis and the dermis [3]. It is more commonly seen in women, with a female : male ratio varying from 10 : 1 to 6 : 1 [4]. The maximum incidence occurs between the fifth and sixth decade of life, in the perimenopausal and the prepubertal group [5].

Although the etiopathogenesis is not exactly known, Borrelia infections [1], hepatitis C infections, genetic factors, androgen level irregularities, hereditary, and autoimmune factors are thought to be associated. Immunogenetic studies have demonstrated links with human leukocyte antigen A29, B44, and DQ-7, 8, and 9 [1]. Autoantibodies to glycoprotein extracellular matrix protein 1 has also been demonstrated in few cases [6]. According to the literature, there is 21.5–34% rate of association between this entity and autoimmune diseases, and 79% of cases had autoantibodies [4]. Vitiligo, thyroid, alopecia areata, lichen planus, morphea, pernicious anemia, and systemic lupus erythematosus are the most commonly associated disorders. In our case, association seen was in the form of family history of vitiligo in mother.

Although commonly seen in the anogenital area (85–98%), it can be seen over extragenital sites in 15–20% of patients [7]. Extragenital involvement along with anogenital involvement occurs in few cases, but in isolation it occurs only in 2.5% [8],[9], as was seen in our case. The exact prevalence of extragenital LSA may be underestimated because of its asymptomatic nature. The lesions are small, ivory or porcelain-white, shiny, round macules or papules, coalescing to form sclerotic plaques. An individual lesion is usually slightly raised, or level with the surface of the skin and shows prominent dilated pilosebaceous or sweat duct orifices, which often contain yellow or brown horny plugs. Lichenification may occur secondarily as a result of rubbing.

Common extragenital sites are trunk (upper), sites of pressure (underneath bra strip and belts), flexor aspect of the wrist, around the neck, periumbilical area, buttocks, and thighs. Less common sites include palms, soles, scalp, face infraorbital, and scrotal regions [1].

Our case showed upper limb involvement over the arm and the forearm below elbow unilaterally, which is unique. Subclinical trauma caused by friction with the collar could be responsible for the lesions on the shoulder as a result of isomorphic phenomenon of Koebner. As opposed to case of genital lesions, no increased risk for malignant transformation has been found in extragenital LSA [8].

Location of lesions preferentially on the left side of the body in most of the reported cases has been attributed to stronger cell-mediated immune hypersensitivity on the left side of the body compared with the right in healthy young individuals, and it is speculated that the cellular immune responsiveness might influence the confinement of the Blaschko-linear lichen sclerosus to the left side of the body [9]. Diwan et al. [10] reported LSA on the right side of the body along the  Lines of Blaschko More Details, as seen in our case in a localized area, where the cause is not known.

In 1892, Darier reported the histological changes characterized by marked orthohyperkeratosis, keratotic plugging of the hair follicles and dermal appendages, atrophy of 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. The blood and lymphatic vessels are dilated, and there may be areas of hemorrhage. The lower dermis is characterized by the presence of dense lymphocytic infiltrate in the mid dermis [8].

Extragenital LSA mimics guttate morphea and atrophic lichen planus. In morphea, thickened collagen bundles or atrophic eccrine glands are seen. Morphea can be differentiated from LSA by the presence of inflammation in the subcutis often as lymphoid follicle, extensive fibrosis, presence of elastic fibers, and absence of follicular plugging, basal cell degeneration, and prominent edema in papillar dermis. Atrophic lichen planus shows flat atrophic epidermis with basal cell degeneration, max joseph space, colloid bodies in the lower epidermis, pigment continence, and the presence of melanophages without homogenization of collagen. In our case, superficial dermis showing pronounced edema and collagenization ruled out other two conditions.

Therapy of extragenital LSA is indicated only for extensive, bullous, and hemorrhagic lesions, symptomatic and cosmetically disfiguring lesions. First-line treatment of LSA includes topical superpotent corticosteroid ointment, whereas the second-line therapy includes topical calcineurin inhibitors. Other therapies reported to be beneficial include topical testosterone, estrogen, potassium para amino benzoate, calcipotriol, intralesional steroids, systemic corticosteroids, systemic retinoids, methotrexate, cyclosporine, sulphasalzine, penicillamine, narrow band ultraviolet B, photodynamic therapy, and surgery [3],[11].


  Conclusion Top


Our case was unique with isolated extragenital lesion in a male on the right side of body unilaterally with positive history of vitiligo in mother. Extragenital LSA can affect men with atrophic papules or plaques but without prominent dilated pilosebaceous over upper limb in isolation that need to be diagnosed at the earliest for better prognosis and to halt the disease progression.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Vasudevan B, Sagar A, Bahal A, Mohanty AP. Extra genital lichen sclerosus with aetiological link to borrelia. Armed Forces Med J India 2011;67:370–373.  Back to cited text no. 1
    
2.
Choi SW, Yang JE, Park HJ, Kim CW. A case of extragenital lichen sclerosus following Blaschko’s lines. J Am Acad Dermatol 2000;43:903–904.  Back to cited text no. 2
    
3.
Ganesan L, Parmar H, Das JK, Gangopadhyay A. Extragenital lichen sclerosus et atrophicus. Indian J Dermatol 2015;60:420.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.
Pai GS, Kamath KN, Kuruvila M. Disorders of connective tissue. In: Valia RG, Valia AR, editors. IADVL textbook of dermatology. 3rd ed. Mumbai, Maharashtra: Bhalani Publishing House; 2008. pp. 1160–1163.  Back to cited text no. 4
    
5.
Iraji F, Schofield O. A comparative study of the predisposing factors and natural history of lichen sclerosus et atrophicus (LSA) in children and adults. Indian J Dermatol 2003;48:36–38.  Back to cited text no. 5
  Medknow Journal  
6.
Chan I, Oyama N, Neill SM, Wojnarowska F, Black MM, McGrath JA. Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus. Clin Exp Dermatol 2004;29:499–504.  Back to cited text no. 6
    
7.
Powell JJ, Wojanrowska F. Lichen sclerosus. Lancet 1999;353:1777–1783.  Back to cited text no. 7
    
8.
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. 8
[PUBMED]  Medknow Journal  
9.
Gutte R, Khopkar U. Extragenital unilateral lichen sclerosus et atrophicus in a child: a case report. Egypt Dermatol Online J 2011;7:10.  Back to cited text no. 9
    
10.
Diwan NG, Nair PA. Extragenital lichen sclerosus et atrophicus along the lines of Blaschko. Indian Dermatol Online J 2015;6:342–344.  Back to cited text no. 10
[PUBMED]  Medknow Journal  
11.
Colbert RL, Chiang MP, Carlin CS, Fleming M. Progressive extragenital lichen sclerosus successfully treated with narrowband UV-B phototherapy. Arch Dermatol 2007;143:19–20.  Back to cited text no. 11
    


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