Unraveling Centrofacial Swelling: A Clinicopathological Perspective of Tumid Lupus Erythematosus

Dear Editor, Centrofacial swelling has diverse etiologies ranging from infections to neoplasms. The infectious causes include cutaneous leishmaniasis and invasive fungal infections; the inflammatory cause is Wegener’s granulomatosis; and the neoplastic causes include subcutaneous panniculitis-like T cell lymphoma, Natural Killer (NK)/T cell lymphoma, and mucoepidermoid carcinoma. Herein, we report an unusual presentation of tumid lupus erythematosus (LE) manifesting as centrofacial swelling. A 28-year-old female presented with an asymptomatic, soft-to-firm, indurated swelling for 1 month. The patient gave a history of traveling to a region near the Gangetic Plain one week prior to the onset of swelling. There were no symptoms of nasal obstruction, epistaxis, blurring of vision, ocular pain, ulceration, or serosanguinous discharge. The patient could not recollect any history of an insect bite. She denied a history of photosensitivity, oral ulcers, or constitutional symptoms, such as fever, weight loss, or loss of appetite. Examination revealed an ill-defined, brown hyperpigmented to dusky erythematous, indurated plaque on the dorsum of the nose extending over the medial aspect of the cheek, measuring 6 × 3 cm, with periorbital edema [Figure 1a]. There was no evidence of intranasal and intraoral mass; the visual field and visual acuity were normal. The rest of the mucosal, lymph node, and systemic examination were normal.Figure 1: (a) Ill-defined dusky erythematous indurated swelling on the dorsa of the nose extending till mid-cheek with periorbital edema. (b) Histopathological image showing follicular plugging, basement membrane thickening, and upper and deep dermal moderately dense infiltrates composed of lymphohistiocytic cells and plasma cells [Hematoxylin and eosin, 50×] (c) Periodic acid-Schiff depicting basement membrane thickening [PAS, 100×] (d) Alcian blue highlighting dense dermal mucin deposits [AB, 400×]Histopathology from the indurated plaque revealed follicular plugging, atrophic epidermis, basement membrane thickening, upper and deep dermal moderately dense infiltrates of lymphohistiocytic cells, and plasma cells with dense deposition of mucin in the lower dermis [Figure 1b]. Periodic acid-Schiff and alcian blue stains highlighted basement membrane thickening and increased mucin deposition in the deep dermis [Figure 1c and 1d]. Tissue biopsy for potassium hydroxide staining and fungal culture yielded negative results. Crush smear and Giemsa staining of tissue for Leishmania Donovan bodies, and serum for rapid antigen detection (rk39) were negative. Antinuclear antibodies and extractable nuclear antigen panel were negative. Complete hemogram, liver and renal function, and urine for protein were normal. A final diagnosis of tumid LE was considered. The patient was started on prednisolone 40 mg and hydroxychloroquine 200 mg twice daily, with complete resolution of the lesion within 2 weeks of follow-up [Supplementary Figure 1]. Tumid LE is a chronic relapsing and remitting variant of LE presenting with characteristic elevated, erythematous, edematous, and non-scaly urticated plaques predominantly distributed on photoexposed regions. Histopathology demonstrates superficial and deep periadnexal and perivascular lymphocytic infiltrates, with dense mucinous deposits, and no significant epidermal alterations. Treatment includes topical tacrolimus, topical and oral corticosteroids, and hydroxychloroquine.[1] Other infectious etiologies of centrofacial plaque include mucorales or entomophthorales and localized cutaneous leishmaniasis.[2,3] Noninfectious etiology includes extranodal NK/T-cell lymphoma (nasal-type) and Jessner’s lymphocytic infiltrate.[4,5] The differentiating features between these entities are summarized in Table 1.Table 1: Differentiating features between centrofacial swellings[ 2-5 ]Our case additionally highlights an unusual presentation of upper eyelid edema possibly due to interstitial mucin deposition, which is rarely described in the literature in LE. It is frequently misdiagnosed as the heliotrope rash of dermatomyositis; however, the absence of dusky erythema and myositis helps distinguish it from dermatomyositis. This case emphasizes that tumid LE may rarely present as centrofacial swelling with eyelid edema and could be considered as a differential diagnosis in such a presentation. Authors’ contributions We confirm that all authors have read and approved the manuscript for submission. All authors meet authorship criteria, and the manuscript represents honest work. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her identifiable images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Use of artificial intelligence (AI) The preparation of this manuscript was carried out entirely by the authors without the use of artificial intelligence technologies.Supplementary Figure 1: Periorbital edema and plaque induration resolved completely, with only residual hyperpigmentation on the nose post-therapy