Unilateral, Blaschkolinear Lupus Erythematosus Tumidus

Dear Editor, Tumid lupus erythematosus (LE), a variant of chronic cutaneous lupus erythematosus (CCLE), was first documented by Dr Erich Hoffmann (1909).[1] Unlike other variants of CCLE, systemic involvement is absent, and antinuclear antibody (ANA) positivity is documented in 10% of the patients. Cases involving blaschkolinear spread are rare, with only a few cases reported so far. Here, we report a case of a young adult male who presented with unilateral blaschkoid tumid LE involving the face and scalp. A male in his 20s reported with erythematous plaques over the face and scalp since 2 years. Lesions initially appeared over the left cheek and over 4 months progressed to involve the ipsilateral upper lip, face and scalp in a blaschkoid pattern. There were complaints of mild lesional burning sensation upon exposure to sunlight. No systemic complaints could be elicited. On examination, there were multiple, well-defined, irregular, erythematous plaques over the left side of the face extending linearly from the upper lip to the scalp [Figure 1a]. Dermoscopic examination of the plaques revealed multiple well-focussed vessels of various morphologies (linear, linear curved, linear vessels with branches, dotted) over a pinkish-white background, few brown dots and lines [Figure 1c]. Laboratory investigations including ANA were done to exclude systemic involvement which were found to be normal. Skin punch biopsy was done from the plaque which revealed follicular plugs, focal vacoular damage and few apoptotic keratinocytes at the dermoepidermal junction. Dermis showed superficial and deep dermal lymphohistiocytic inflammatory infiltrate cells around vessels and appendages [Figure 2]. A final diagnosis of blaschkolinear LE tumidus was made after clinic–dermoscopic–pathological correlation [Table 1]. Patient was started on hydroxychloroquine 200 mg twice daily, topical tacrolimus and sunscreen after a baseline ophthalmological examination. Follow-up at 3 months showed a decrease in erythema and induration [Figure 1b]. Post-treatment dermoscopy showed decrease in erythema, vessels and increase in brown dots/globules, and brown lines indicating decrease in inflammation and underlying resolution with dermal melanophages [Figure 1d].Figure 1: (a) Multiple, erythematous plaques in a blaschkolinear spread extending over the left side of the face, involving mustache and scalp (pre-treatment). (b) Clinical image depicting a decrease in erythema seen over plaques 3 months post-treatment. (c) Dermoscopic examination showing linear, curved and dotted vessels on a pinkish-white background (DermLite DL 5, polarized, 10×) (pre-treatment). (d) Post-treatment (3 months) dermoscopic examination showing a decrease in erythema, vessels, and an increase in brown dots/globules and lines (DermLite DL5, polarized, 10×)Figure 2: (a) Histopathological examination showing moderately dense superficial and deep dermal lymphocytic infiltrate (H and E, 4×). (b) Focal interface change (H and E, 20×). (c) Perifollicular infiltrate (H and E, 20×). (d) Mucin deposition with perivascular lymphocytic infiltrate (H and E, 20×)Table 1: Dermoscopic–histopathological correlationClinical findings in tumid LE include erythematous, edematous non-scarring papules and plaques, predominantly involving the photo-exposed areas of face and upper trunk. Histopathological findings include perivascular and periadnexal infiltrate (lymphocytic with occasional neutrophils) involving papillary and reticular dermis. Interstitial mucin deposition is noted. Unlike other variants of LE, epidermal and dermoepidermal junctional changes are not typically observed.[2] However, hyperkeratosis, slight epidermal hyperplasia, smudging of the dermoepidermal junction has occasionally been observed.[3] Despite relapses, spontaneous resolution of lesions of tumid LE without any residual changes may be observed.[4] Broad differentials include Jessner’s lymphocytic infiltrate, linear morphea and sarcoidosis [Table 2]. Jessner’s lymphocytic infiltrate presents with erythematous papules and plaques involving the face, upper trunk and histopathological findings of mucin deposition[4] without any interface change. In the present case, there was absence of induration and subcutaneous atrophy on clinical examination, and no evidence of fibrosis or granulomatous infiltrate was observed histopathologically; ruling out morphea and sarcoidosis. Blaschkolinear involvement of tumid LE is rare, with only a few cases reported so far [Table 3]. This pattern of spread may be due to genetic/epigenetic modifications involving mosaic skin cells. An in-depth analysis of the interaction of these modifications with the immune system may provide further insights into pathogenesis and targeted therapeutic interventions.[5]Table 2: Dermoscopic findings in case-specific differential diagnosesTable 3: Literature review of blashckolinear tumid lupus erythematosus cases[ 1 ]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.