Dr. Y.S. Marfatia, Prof.& Head,

Dept. Of Skin & V.D, Baroda Medical College,

S.S.G.H.,Hospital, Raopura, Vadodara.

Dr.Sonia Jain,

A,14, Dhanvantri Nagar,

M.G.I.M.S, Sewagram, Wardha, Maharashtra.

ABSTRACT

Lichen sclerosus et atrophicus is a chronic inflammatory dermatosis that results in white plaques and epidermal atrophy. The condition has both genital and extragenital presentations. Here we describe the case of a twelve year old girl who presented to us with white plaques over her genitals and no manifestation of extra genital disease.

Key words: Lichen sclerosus et atrophicus, lichen albus, white spot disease.

INTRODUCTION

Lichen Sclerosus is a chronic inflammatory dermatosis that most commonly affects the anogenital region and leads to intractable pruritus and soreness. The condition is more common in females¹ as in the present case also the patient is a young girl who presented with complaints of white plaques with itching over the genitals since her prepubertal years. The condition involves the risk of malignant transformation more so over the genital lesions but the precise incidence has not been defined. Pathophysiologically, the condition is associated with the presence of autoantibodies to glycoprotein extracellular matrix protein 1 (ECM1)². Several risk factors have also been proposed including autoimmune diseases, infections and genetic predisposition³. There is evidence of its association with thyroid disease⁴.

CASE SUMMARY

Here we report the case of a twelve year old young girl who presented to us with depigmented patches over the genitals which had an insidious onset and were gradually progressive over a period of one year. She had moderate itching over the site and she had seen many doctors for her complaints but had no relief. On dermatological examination, labia majora showed atrophy and along with depigmentation of the labia minora. The depigmented patches extended from the fourchette to the vestibule and she had no oral or cutaneous lesions elsewhere. There was no history of sexual abuse or any high risk behaviour and none of the family members had a similar clinical picture. She had no urinary or bowel complaints. We performed a labial biopsy from the depigmented patch after taking a written informed consent and the histopathological findings were consistent with Lichen sclerosus et atrophicus showing homogenization of the collagen and inflammatory infiltrate in the dermis (Fig.1) .

LEGENDS

Fig. 1 Histology showing homogenization of the collagen and inflammatory infiltrate in the dermis .

DISCUSSION

Lichen sclerosus (LS) is also known as lichen sclerosus et atrophicus (LSA), balanitis xerotica obliterans (BXO) in men, Csillag’s disease, White Spot Disease, Lichen albus and krauosis vulvae. Lichen sclerosus et atrophicus was first described in 1887 by Dr. Hallopeau.

A case series of 42 children (all females) suffering form LSA has been reported by Shirley A Warrington and Camille de San Lazaro where they found a high incidence of sexual abuse associated with a higher incidence of LSA in pediatric population. Besides, they also described the association of LSA with infection, autoimmunity and trauma.

There has been a case on the records wherein SK Virdi and AS Kanwar reported the co-existence of localised cutaneous morphea with LSA and submucosal fibrosis in a middle aged man in his late thirties⁵. Another citation of a similar association between the aforesaid conditions has been mentioned by P Prasad, L Padmavathy and et al wherein they reported a the case of young male in his mid twenties who presented with generalized morphea and LSA along with osteolytic bone changes⁶. However, our patient showed no signs of morphea or any other systemic disease.

REFERENCES

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2. 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. Sep 2004; 29(5):499-504.
3. Yesudian, PD; Sugunendran, H; Bates, CM; OMahony."Lichen sclerosus". Int J STD AIDS 2005; 16 (7): 465–473.
4. Birenbaum, DL; Young, RC (2007). "High prevalence of thyroid disease in patients with lichen sclerosus". J Reprod Med. 2007; 52 (1): 28–30.
5. Virdi SK, Kanwar AS. Generalized morphea, lichen sclerosis et atrophicus associated with oral submucosal fibrosis in an adult male. Indian J Dermatol Venereol Leprol 2009;75:56-9
6. Prasad P, Padmavathy L, Sethurajan S, Kumar P. Generalised morphoea with lichen sclerosus et atrophicus and unusual bone changes. Indian J Dermatol Venereol Leprol. 1995; 61:113-5.