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Table of Contents 
CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 1  |  Page : 77-79
Co-existence of Lichen sclerosus et atrophicus and morphoea along Lines of Blaschko


1 Department of Dermatology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India
2 Department of Pathology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India

Date of Web Publication23-Dec-2013

Correspondence Address:
Bikash Ranjan Kar
Department of Dermatology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar - 751 003, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.123507

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   Abstract 

Lichen sclerosus (LS) is an inflammatory dermatitis of unknown etiology that mostly affects the genital region in both the sexes. In active cases the histopathologic changes differentiate between LS and morphoea though in chronic cases it is very difficult to diagnose with certainty. Coexistence of both the conditions in a single patient indicates that these lesions represent a spectrum of similar pathologic process. Coexistence of both the conditions along Blaschko's lines is so far not described in literature. We report an Indian patient with both LS and morphoea occurring along Blaschko's lines.


Keywords: Lichen sclerosus et atrophicus, lines of Blaschko, morphoea


How to cite this article:
Kar BR, Dash K. Co-existence of Lichen sclerosus et atrophicus and morphoea along Lines of Blaschko. Indian J Dermatol 2014;59:77-9

How to cite this URL:
Kar BR, Dash K. Co-existence of Lichen sclerosus et atrophicus and morphoea along Lines of Blaschko. Indian J Dermatol [serial online] 2014 [cited 2019 Dec 8];59:77-9. Available from: http://www.e-ijd.org/text.asp?2014/59/1/77/123507

What was known?
1. Purely extragenital LS contributes to 20% of the total cases.
2. Morphoea may coexist with LS.
3. LS lesions may follow lines of Blaschko.



   Introduction Top


Lichen sclerosus (LS) is an inflammatory dermatitis of unknown etiology affecting primarily vulvar, perianal and perineal skin of prepubertal, perimenopausal and postmenopausal women. Extra genital LS [1] is most common on the face, neck, shoulders, and upper portion of the trunk. Libow and Coots [2] first described extragenital LS developing in a pattern corresponding to Blaschko's lines in 1998. We report a case of extra genital LS et atrophicus along with morphoea presenting in a blaschkoid pattern in an Indian patient.


   Case Report Top


A 40-year-old female presented with slightly pruritic eruption that initially started over the left shoulder 2 years back and then gradually enlarged to involve the left arm, left side of her abdomen, and the left lower limb. The lesions reached the present size 1 year back and remained stable since then. An examination revealed discrete and confluent, porcelain white, sharply circumscribed, slightly atrophic, papuloplaques over the left arm and left leg and left side of abdomen with a sharp demarcation at the midline [Figure 1] and [Figure 2]. Several areas showed cigarette paper wrinkling suggestive of atrophy of epidermis. The genital, perianal, and the mucosal areas were normal. The skin over the posterolateral aspect of abdomen on the left side and over the lower part of thigh was slightly hyperpigmented and indurated. Over this affected area the epidermal changes were missing and the overlying skin was smooth.
Figure 1: Hypo and hyperpigmented macules over the trunk with a sharp demarcation at the midline near the umbilicus

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Figure 2: Lichen sclerosus lesions on the left lower limb

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Routine hemogram and urine examination were within normal limits. Biopsy done from the area with porcelain-white discoloration of epidermis showed follicular plugging overlying a thinned out epidermis devoid of rete ridges. Vacuolar alteration of basal cell layer was present. Moderate papillary dermal edema along with focal homogenization of the collagen was present. Periadnexal lymphohistiocytic infiltrate was evident in the dermis. The findings were consistent with LS [Figure 3]. Histopathology done from the smooth, hyperpigmented, and indurated lesion on the left flank showed a relatively normal epidermis without follicular plugging. The basal cell layer was intact without vacuolation. The dermis appeared homogenous and little oedematous [Figure 4]. There was patchy periappendageal inflammation and subcutaneous fat layer showed patchy septal inflammation with lymphocytic infiltrates. The histopathology was consistent with morphoea.
Figure 3: Follicular plugging overlying a thinned out epidermis devoid of rete ridges with vacuolar alteration of basal cell layer. Moderate papillary dermal edema along with focal homogenization and periadnexal lymphohistiocytic infiltrate was evident in the dermis (H and E, original magnification ×40)

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Figure 4: Thinned out epidermis with collagenization of dermis along with patchy dermal inflammatory infiltrate (H and E, original magnification ×40)

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The patient was started on twice daily topical Tacrolimus 0.1% ointment. The lesions were less obvious after 1 month of starting the therapy though there was no change in the cutaneous atrophy component.


   Discussion Top


LS predominantly affects the genitals in females though the female preponderance could be due to a reporting bias. [1] Balanitis xerotica obliterans is believed to be the male counterpart of the same disease involving the glans penis and/or the prepuce. Meffert et al., [1] found that purely extragenital LS accounted for approximately 20% of the total cases. The neck, shoulders, and upper portion of the trunk were the most frequent sites of involvement. The first case of LS following Blaschko's lines, described by Libow and Coots [2] had a zosteriform involvement of the trunk. Our index case differs from their case in having involvement of the left half of the body with a definite demarcation at the midline near the umbilicus. In the index case we also had coexistence of LS along with morphoea. The coexistence of morphea and LS in the same patient suggests that these lesions represent a spectrum which may reflect similar etiologic events or closely related pathologic processes in these two diseases. [3]

Isolated reports of LS involving the scalp, palms, soles, or even widespread exist but LS following Blaschko's lines are rare. The  Lines of Blaschko More Details were first described in 1901 and represent a pattern that is observed in many skin disorders. [4] These lines, which are invisible under normal conditions, indicate the normal embryonic movements of the skin that occur during embryogenesis. Disorders that occur along the lines of Blaschko are believed to result from two different clones of cells that evolve early in embryogenesis.  Lyonization More Details or random inactivations in X-linked disorders, postzygotic somatic mutations in somatic conditions, and gametic half-chromatid mutations have been proposed as mechanisms. [5] To our knowledge this is the first case of LS with morphoea in an Indian patient corresponding to the lines of Blaschko.

 
   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
[PUBMED]    
2.Libow LF, Coots NV. Lichen sclerosus following lines of Blaschko. J Am Acad Dermatol 1998;38 (5 Pt 2):831-3.  Back to cited text no. 2
    
3.Uitto J, Santa Cruz DJ, Bauer EA, Eisen AZ. Morphea and lichen sclerosus et atrophicus: Clinical and histopathologic studies in patients with combined features. J Am Acad Dermatol 1980;3:271-9.  Back to cited text no. 3
[PUBMED]    
4.Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol 1994;31 (2 Pt 1):157-90.  Back to cited text no. 4
    
5.Meyer WJ 3 rd , Migeon BR, Migeon CJ. Locus on human X chromosome for dihydroxytestosterone receptor and androgen insensitivity. Proc Nat Acad Sci U S A 1975;72:1469-72.  Back to cited text no. 5
    


What is new?
Extragenital LS with morphoea occurring in the same patient along the lines of Blaschko.


    Figures

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

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    Abstract
   Introduction
   Case Report
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    References
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