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E-IJD CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 4  |  Page : 420
Extragenital lichen sclerosus et atrophicus


Department of Dermatology, Vivekananda Institute of Medical Sciences (VIMS), Ramakrishna Mission Seva Pratisthan, 99, Sarat Bose Road, Kolkata, West Bengal, India

Date of Web Publication10-Jul-2015

Correspondence Address:
Dr. Leelavathy Ganesan
Department of Dermatology, Vivekananda Institute of Medical Sciences (VIMS), Ramakrishna Mission Seva Pratisthan, 99, Sarat Bose Road, Kolkata, 700 026, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.160516

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   Abstract 

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis with anogenital and extragenital presentations. Extragenital lichen sclerosus is most common on the neck, shoulders and upper trunk. Linear lesions are uncommon in LSA. We report a case of linear extragenital LSA involving forehead and scalp, along with grouped white papules of LSA in the right side of the back in a postmenopausal woman. The patient showed atypical clinical presentation of LSA in face which clinically mimicked 'en coup de sabre' as seen in morphea, but other clinical features suggested the diagnosis of LSA and the histopathological findings confirmed it.


Keywords: En coup de sabre, extragenital, linear LSA


How to cite this article:
Ganesan L, Parmar H, Das JK, Gangopadhyay A. Extragenital lichen sclerosus et atrophicus. Indian J Dermatol 2015;60:420

How to cite this URL:
Ganesan L, Parmar H, Das JK, Gangopadhyay A. Extragenital lichen sclerosus et atrophicus. Indian J Dermatol [serial online] 2015 [cited 2021 Aug 5];60:420. Available from: https://www.e-ijd.org/text.asp?2015/60/4/420/160516

What was known?
Extragenital LSA is not uncommon, but linear LSA is quite rare. The relationship between LSA and morphea is controversial, and histopathological findings are crucial for the diagnosis. Close follow up of patients is needed as reports of transition of LSA to localized scleroderma and vice-versa on sequential biopsies have been reported.



   Introduction Top


Lichen sclerosus et atrophicus (LSA), described originally by Hallopeau in 1887, is a benign chronic inflammatory dermatosis of unclear pathogenesis and affecting both the epidermis and the dermis. [1] Typical findings are white opalescent papules that may cluster and progressively result in parchment-like skin. LSA occurs mostly in the anogenital area (83% to 98%), and sometimes extragenital sites (15% to 20%). [2],[3] It primarily affects the vulval, perineal and perianal skin of prepubertal, perimenopausal and postmenopausal women. However, extragenital LSA is not uncommon and it was found in 805 of 5207 cases reviewed by Meffert et al. [1] Extragenital LSA found to be relatively common on the neck, shoulder and upper trunk, is generally asymptomatic, but can occasionally be pruritic. Less common sites include the palms, soles, scalp and face. Extragenital LSA is commonly seen in association with plaque type morphea and some authors have suggested a common pathogenesis. [4]


   Case Report Top


A 54-year-old female presented with a single linear vertical hypopigmented plaque in the forehead, starting from the eyebrow level, and extended slowly up to the vertex for last 1 year. After 2 months or so she developed few tiny ivory white macules on the right side of her back. The patient was otherwise healthy, and there was no history of any autoimmune disorders. There was no family history of similar skin diseases. The patient had not undergone any treatment for her skin lesion before she presented to us.

Local cutaneous examination revealed a single linear vertically oriented grayish plaque extending from glabella to vertex, of size 12 cm × 1 cm [Figure 1]. The surface showed wrinkling along with prominent keratin plugs, particularly at its top end [Figure 2]. Induration, minimal scaling and scarring alopecia were present, and no bony depression was found. Examination of the back showed multiple grouped white macules, some of them were perifollicular [Figure 3]. Oral and genital mucosae were normal. Systemic examination failed to show any abnormality. Clinically, linear scleroderma ('en coup de sabre') and LSA were considered as differential diagnoses.
Figure 1: Linear vertical grayish plaque

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Figure 2: Plaque showing prominent keratin plugs with alopecia

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Figure 3: Grouped white macules in back

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Complete blood count, routine blood biochemistry, free T4 and TSH, antinuclear antibody (ANA) were within normal limits. X-ray skull, both anteroposterior and lateral views were normal. Histological examination of the biopsy taken from the lesion in forehead and back showed almost similar findings-follicular plugging, thinning of the epidermis, loss of the rete ridges, focal basal cell vacuolization, pigmentary incontinence, edema and hyalinization of the papillary dermis [Figure 4] and [Figure 5]. Based on the clinical and histological findings, this case was diagnosed as linear LSA involving face along with extragenital LSA on back. The patient was prescribed topical mometasone furoate 0.1% ointment once daily for 2 months and two intralesional injections of triamcinolone acetonide 10 mg/ml (started concurrently with the ointment) were given into multiple sites at 1 month interval 1 ml (10 mg) in each sitting. The lesion on face was completely flattened and hyperpigmented [Figure 6], and there was slight improvement of the lesions on back. A close follow up was planned.
Figure 4: Histopathology (H and E ¡¿40) shows thinned epidermis, focal basal membrane vacoulization, edema and hyalinization of papillary dermis

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Figure 5: Histopathology (H and E, ¡¿10) shows prominent follicular plugging

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Figure 6: Post treatment picture shows a flat and hyperpigmented lesion

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   Discussion Top


The first case report of linear LSA was described in 1995 by Izumi and Tajima. [5] Thereafter, a handful of cases of linear LSA have been reported, among which some developed in a pattern corresponding to the  Lines of Blaschko More Details. [6],[7] Ours is a case of linear LSA involving the face and the scalp along with typical LSA lesions in back, without any mucosal lesions. It resembled linear scleroderma ('en coup de sabre') quite closely as the middle of the face was involved, but the lesion was slightly to the left of the midline of forehead [Figure 1]. However, distinct follicular plugging, not seen in scleroderma, was noted on close inspection [Figure 2], and the histopathology was consistent with LSA.

The relationship between LSA and morphea is still controversial. Many authors have described coexistent LSA and morphea. [7],[8],[9] With sequential biopsies, several investigators have reported transition from LSA to morphea or vice versa. [4],[9] Nonetheless, other investigators believe there are enough clinical and histological differences between LSA and morphea to argue that they are distinct diseases and those coexistent lesions are coincidental. The therapeutic options for LSA are topical and intralesional corticosteroids, retinoids, phototherapy, [10] estrogen, vitamins, topical tacrolimus and surgical removal, but every option has shown variable and limited responses. In our case, partial improvement was noted with topical steroid ointment for 2 months and monthly intralesional steroid injections for 2 months.



 
   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.
Powell JJ, Wojnamwska F. Lichen sclerosus. Lancet 1999;353:1777-83.  Back to cited text no. 2
    
3.
Padmavathy L, Rao L, Dhana Lakshmi, Sylvester N, Ethirajan N. Lichen Sclerosus Atrophicus [LSA] in the Areolae: A Case Report. Case Rep Dermatol Med 2012;2012:825963.  Back to cited text no. 3
    
4.
Funaro D. Lichen sclerosus: A review and practical approach. Dermatol Ther 2004;17:28-37.  Back to cited text no. 4
    
5.
Izumi T, Tajima S. A case of linear type of lichen sclerosus et atrophicus? J Dermatol 1995;22:279-82.  Back to cited text no. 5
    
6.
Rameshwar Gutte et al. Extragenital unilateral lichen sclerosus et atrophicus in a child: A case report Egyptian Dermatology Online Journal 7 (1):10.  Back to cited text no. 6
    
7.
Bikash RK, Kanakalata D. Co-existence of Lichen Sclerosuset Atrophicus and Morphoea Along Lines of Blaschko. Indian J Dermatol 2014;59:77-9.  Back to cited text no. 7
    
8.
Tremaine R, Adam JE, Orizaga M. Morphea coexisting with lichen sclerosus et atrophicus. Int J Dermatol 1990;29:486-9.  Back to cited text no. 8
    
9.
Sawamura D, Yaguchi T, Hashimoto I, Nomura K, Konta R, Umeki K. Coexistence of generalized morphea with histological changes in lichen sclerosus et atrophicus and lichenplanus. J Dermatol 1998;25:409-11.  Back to cited text no. 9
    
10.
Kreuter A, Gambichler T, Avermaete A, Happe M, Bacharach-Buhles M, Hoffmann K, et al. Low-dose ultraviolet A1 phototherapy for extragenital lichen sclerosus: Results of a preliminary study. J Am Acad Dermatol 2002;46:251-5.  Back to cited text no. 10
    

What is new?
Occurrence of extragenital LSA as two forms (linear and grouped macules) in the same patient is extremely rare.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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    Abstract
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