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CORRESPONDENCE |
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| Year : 2022 | Volume
: 67
| Issue : 1 | Page : 76-77 |
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| Linear DLE in photoprotected area |
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Surajit Gorai1, Piyali Banerjee2, Kinnor Das3, Enam Murshid Khan4, Subhas Chandra Nag5
1 Department of Dermatology, Apollo Multispeciality Hospital, Kolkata, West Bengal, India
2 Department of Dermatology, St. Barnabas Hospital, Ranchi, Jharkhand, India
3 Department of Dermatology, Venereology and Leprosy, Silchar Medical College and Hospital, Silchar, Assam, India
4 Department of Pathology, Apollo Multispeciality Hospital, Kolkata, West Bengal, India
5 Department of Dermatology, Santiniketan Medical College, Bolpur Birbhum, West Bengal, India
| Date of Web Publication | 19-Apr-2022 |
Correspondence Address:
Piyali Banerjee
Department of Dermatology, St. Barnabas Hospital, Ranchi, Jharkhand
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_219_21
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How to cite this article:
Gorai S, Banerjee P, Das K, Khan EM, Nag SC. Linear DLE in photoprotected area. Indian J Dermatol 2022;67:76-7 |
Sir,
Lupus erythematosus (LE) is a multisystem inflammatory autoimmune disease with numerous clinical presentations that range from the localized form of the disease that is restricted to the skin to the systemic form. Cutaneous LE has assorted variants. The most common types are acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE). The most common subset of CCLE is discoid LE (DLE). Other infrequent forms of CCLE include hypertrophic LE, tumid LE, LE panniculitis, oral DLE, DLE involving the palms and soles.[1],[2],[3] One of the rare variants of DLE is known as linear DLE where the lesions are present in a linear fashion along Blaschko's lines. It occurs for the most part in children and relatively young populations with a similar incidence in both genders, however, photosensitivity is less frequent in such cases. These lesions appear as linear mostly unilateral erythematous plaques and predominate in the face, nevertheless the neck, trunk, and extremities may be affected as well.[4],[5]
Here, we present a case of postmenopausal 48 years female who presented with linear scaly, erythematous plaques over the left breast, both inguinal regions for 2 years following Blaschko's lines [Figure 1] and [Figure 2]. Along with it, similar lesions were also present in the hands and other parts of the trunk. There was no history of joint pains or any systemic complaints. There was no history of photosensitivity, smoking, or consumption of alcohol.
On examination, multiple linear well-defined plaques with induration, atrophy, and scarring were seen. They were covered with a few small scales. On examination, temperature, blood pressure, heart rate, cardiovascular system, and respiratory system were normal. On investigation, her anti-nuclear antibody (ANA) titer, hepatitis B surface antigen, anti-hepatitis C virus, hemogram, urine microscopy, liver, and renal functions were within normal limits. A punch biopsy was done for histopathological examination. The histopathology of skin biopsy showed focal epidermal atrophy with hyperkeratosis, hydropic degeneration of basal cell layer, thickening of the basement membrane zone, and dilated vessels. Throughout, dermis lymphocytic inflammatory infiltrates were surrounding the adnexa and the blood vessels. There was mucin deposition in the reticular dermis. The hair follicles showed keratotic plugging and significant damage to the basal cells of follicular epithelium accompanied by infiltration of lymphocytes and histiocytes extending to the lower dermis [Figure 3]a and [Figure 3]b. Dermoscopy and immunofluorescence study were not done as the histopathological examination (HPE) was diagnostic. The patient was prescribed methotrexate (15 mg/week) along with mometasone furoate 0.1% w/w cream for 3 months. With this treatment, she responded satisfactorily and showed significant improvement. In the next 3 months, there was decreased infiltration and erythema. | Figure 3: (a) HPE image of the affected tissue (PAS-stained). (b) HPE image of the affected tissue (PAS-stained)
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Genetic mosaicism and/or epigenetic modification of the keratinocytes clubbed with some abnormality in the immune system along the lines of Blaschko might be the etiology of the development of this specific variant of chronic lupus erythematosus. The keratinocytes which are present along the lines of Blaschko may be triggered by various forms of injury. Irradiation or various forms of ultraviolet rays may express different antigens which stimulate the origin of cutaneous LE. Keratinocyte apoptosis through various pathways (like p53, tumor necrosis factor (TNF), and Fas/Fas L) has been found to be an important event in starting CLE as well. Nevertheless, different schools of thought conjectured whether the genetically variant keratinocytes are truly in short of proteins crucial for the regulation of apoptosis or they are immunologically distinct with anomalous major histocompatibility complex (MHC) expression or there are some relaxing anomalous cytokines.[6],[7]
Our case is unusual as our patient was postmenopausal. Furthermore, the lesions were present in sun-protected areas. In this case, besides genetic mosaicism, the hormonal changes, and local pressure (the continuous wearing of tight petticoat and innerwear) might be the triggering factor for the development of linear DLE. So, it is our responsibility to bring this uncommon variant of a routinely observed case into the public domain.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Udompanich S, Chanprapaph K, Suchonwanit P. Hair and scalp changes in cutaneous and systemic lupus erythematosus. Am J Clin Dermatol 2018;19:679-94.  |
| 2. | Dammacco R, Procaccio P, Racanelli V, Vacca A, Dammacco F. Ocular involvement in systemic lupus erythematosus: The experience of two tertiary referral centers. Ocul Immunol Inflamm 2018;26:1154-65. |
| 3. | Abadías-Granado I, Felipo-Berlanga F, Ara-Martín M, Sánchez-Bernal J. Coexistence of Tumid Lupus Erythematosus and Discoid Lupus Erythematosus. 2019. |
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| 5. | Röckmann H, Feller G, Schadendorf D, Goerdt S. Subacute cutaneous lupus erythematosus on the lines of Blaschko. Eur J Dermatol 2006;16:302-6. |
| 6. | Jin H, Zhang G, Zhou Y, Chang C, Lu Q. Old lines tell new tales: Blaschko linear lupus erythematosis. Autoimmun Rev 2016;15:291-306. |
| 7. | Seitz C, Bröcker E, Trautmann A. Linear variant of chronic cutaneous lupus erythematosus: A clue for the pathogenesis of chronic cutaneous lupus erythematosus? Lupus 2008;17:1136-9. |
[Figure 1], [Figure 2], [Figure 3] |
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