Multidermatomal Unilateral Primary Zosteriform Lichen Sclerosus: A Rare Clinical Presentation
Year: 2024
Type: article
Study design: Case Report
Abstract: Sir, Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory mucocutaneous dermatosis commonly affecting the anogenital area but can involve extragenital areas, especially thighs, trunk, and neck.[1] It is commonly associated with autoimmune diseases, hormonal disturbances, and trauma. Extragenital LSA evolves as ivory-white polygonal papules with follicular plugging on the surface of the plaques, healing with porcelain white atrophic scars.[2] Herein, we report a rare multidermatomal zosteriform presentation of extragenital LSA. A 54-year-old male presented with multiple asymptomatic, well-defined, non-tender, dull red to dusky brown plaques in a zosteriform distribution over the left side of T4, T8, and T12 dermatomes, with areas of atrophic scarring [Figure 1]. Some plaques along the T4 dermatome showed a rim of hypopigmentation. The lesion first appeared on the lower abdomen and slowly progressed to involve the remaining areas within one year. No prior history of trauma, surgical wounds, or herpes zoster was present. Dermoscopic examination revealed multiple comedo-like openings, white structureless areas, black dots, and telangiectasias [Figure 2]. Differential diagnoses of morphea, atrophoderma of Pasini and Pierini, and LSA were kept. Histopathology of the plaque over the T4 dermatome showed mild orthohyperkeratosis, epidermal atrophy with loss of rete ridges, focal basal cell vacuolization, and melanin incontinence. Dermis showed hypercollagenization and moderate perivascular and interstitial infiltrate comprising predominantly of lymphocytes. A diagnosis of LSA was made based on clinical-histopathological correlation. He was treated with methotrexate (10 mg) once weekly and topical corticosteroids.Figure 1: (a) - Shows multiple well-defined dull red to dusky brown plaques in a zosteriform distribution present over the T4, T8, and T12 dermatomes, with areas of ‘porcelain-white’ atrophic scarring. Inset shows the most active lesion in the T4 dermatome (red arrow) which shows a similar clinical picture with a rim of hypopigmentation. (b) - Dermoscopy of lesion showing multiple comedo-like openings (green arrow), white structureless areas (blue arrow), black dots (black arrow), and telangiectasias (yellow arrow)Figure 2: (a) - Histopathology (H and E, 40x) shows atrophic epidermis with mild inflammation in the dermis. (b) - Histopathology (H and E, 400x) shows mild orthohyperkeratosis, epidermal atrophy with loss of rete ridges, focal basal cell vacuolization, and melanin incontinence with dermis showing hypercollagenization and moderate perivascular and interstitial infiltrate comprising predominantly of lymphocytesLSA was described initially by Hallopeau in 1889 as a benign chronic idiopathic inflammatory dermatosis of both the dermis and epidermis.[3] It commonly affects the anogenital region and is 6–10 times more common in females than males. It presents as itchy ivory-white patches over the genitalia that can progress to scarring and have malignant potential in untreated patients.[4] On the contrary, extragenital LSA is non-carcinogenic and generally not associated with pruritus. It commonly presents as asymptomatic, ivory-white papules/plaques with multiple comedo-like openings corresponding to the appendageal ostia. Porcelain white atrophic scarring is the usual sequelae. It can rarely be present in a linear, blaschkoid, or zosteriform pattern. Blaschkoid pattern occurs due to genetic mosaicism, where an inactivated abnormal keratinocyte clone gets activated due to environmental factors and produces the clinical manifestation. Zosteriform pattern has been seen as a result of isotopic response (herpes zoster, granuloma annulare, pseudolymphoma, granulomatous folliculitis).[5,6] A case of primary bilateral zosteriform LSA has been described in the literature.[3] Our case is unique for its unilateral multi-dermatomal pattern of involvement, which is yet to be reported. Therefore, history was taken to rule out an isotopic response. Linear morphea presents as indurated plaques with waxy centers, and histopathology shows extensive dermal fibrosis and subcutaneous fibrous trabeculae without epidermal changes. Atrophoderma of pasini and pierini usually presents as brownish, depressed lesions in a bilaterally symmetrical pattern.[7] Histopathology shows unaffected epidermis with homogenization and clumping of collagen bundles in the dermis. In our case, epidermal atrophy, basal cell vacuolation, melanin incontinence, and inflammation limited to the upper dermis supported the diagnosis of LSA. Unilateral multi-dermatomal zosteriform presentation of LSA is unique and should be known to dermatologists for prompt diagnosis and early management, thus preventing long-term sequelae. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Indian Journal of Dermatology
Authors: Parul Bansal, Bikash R. Kar, Manoj K. Nayak, Kallolinee Samal, Nibedita Dixit
Institution: Institute of Medical Sciences and Sum Hospital
Language: English
Subfield: Dermatology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4405918291