Abstract
Tumid lupus is an uncommon variant of lupus erythematosus. Patients present with subcutaneous lesions. Ophthalmic literature reports disease manifestation as orbital inflammation. Autoimmune serology is often negative. Without a high index of suspicion, the diagnosis is easily overlooked delaying treatment. Tumid lupus is not significantly discussed in the Otolaryngologic literature. Here we present a Case Report of a male who initially presented to Ophthalmology with unilateral orbital complaint of eyelid puffiness. Orbital biopsy and subsequent biopsy of his submental skin lesions ultimately led to this unexpected diagnosis. We discuss the method to diagnose tumid lupus including representative histopathologic findings.
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Introduction
Lupus erythematosus profundus (LEP), or tumid lupus, represents an uncommon variant of lupus erythematosus (LE) that may occur as an isolated skin condition or in association with systemic lupus erythematosus (SLE). We report a case where excisional biopsy of chronic submental subcutaneous masses coupled with unilateral right orbital symptoms led to an unexpected diagnosis of LEP. This case report was reviewed by the University of Illinois Institutional Review Board and deemed exempt research.
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Case report
A 57 year-old African-American male presented initially to Ophthalmology for an evaluation for cosmetic blepharoplasty due to right upper eyelid puffiness for 2 months. He denied symptoms of epiphora, worsening vision, pain, diplopia or pruritis. Ophthalmologic history was significant for refractive surgery 10 years prior to presentation. Examination showed right 20/30 visual acuity and 20/20 on the left, grade 2 + right upper eyelid edema, globe dystopia with downward displacement, full ocular range of motion, and normal anterior and posterior eye segment findings. Neck examination revealed bilateral submandibular intradermal lesions with mild overlying skin induration, both measuring 3.5 cm long.
Orbital CT scan ( Fig. 1 ) demonstrated diffuse right periorbital inflammatory fat stranding without focal mass. An anterior orbitotomy with biopsy through an eyelid crease was performed to evaluate for neoplasm. Histopathology showed perivascular chronic inflammation involving fibroadipose tissue, initially interpreted as orbital inflammatory syndrome ( Fig. 2 ). Autoimmune serology tests for angiotensin converting enzyme (ACE), rapid plasma reagin (RPR), tuberculosis quantiferon, anti-nuclear cytoplasmic antibody (ANCA), anti-nuclear antigen (ANA) and erythrocyte sedimentation rate (ESR) were negative. Immunoglobulin G levels were not elevated.
The patient was subsequently evaluated by Otolaryngology which resulted in excision of the submental subcutaneous lesions clinically felt to represent sebaceous cysts. Pathologic examination revealed stromal mucin deposition, lobular lymphocytic panniculitis with hyaline fat necrosis, accompanied by perivascular, periadnexal and perineural lymphoplasmacytic infiltrates ( Fig. 3 ). The preceding orbital biopsy was reexamined and similar histological findings were identified. Considered together lupus erythematosus profundus was ultimately diagnosed. The Rheumatology service in consultation found no evidence of systemic lupus erythematosus. After 13 months of Plaquenil therapy the patient has experienced improvement of the orbital symptoms.
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