A 32-year-old man is referred for evaluation of an erythematous rash on his upper back that has been present for approximately 2 months. At age 25, he was diagnosed with systemic lupus erythematosus (SLE), a disease that also affects his mother. Treatment with hydroxychloroquine 200mg/d was restarted approximately 1 month ago. Examination reveals annular, indurated erythematous plaques, as well as faint malar erythema.
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Tumid lupus, also referred to as lupus erythematosus tumidus (LET), is a rare subtype of cutaneous lupus erythematosus. Lupus erythematosus (LE) is an autoimmune disorder with a presentation that ranges from a localized cutaneous form to a life-threatening systemic form.1 Of the 3 subtypes of cutaneous forms of LE, tumid lupus is a subset of chronic cutaneous lupus erythematosus, with several lesion characteristics that differentiate it from other common forms of cutaneous lupus. The clinical presentation of tumid lupus is characterized by single or multiple nonscarring, erythematous, succulent, urticarial plaques.1 Tumid lupus manifests without epidermal involvement; there is no atrophy, ulceration, follicular plugging, scarring, or dyspigmentation.2 As tumid lupus is one of the most photosensitive types of cutaneous lupus, it typically presents on sun-exposed areas such as the face, upper trunk, arms, and shoulders.3 A lesion may resolve spontaneously within a few weeks after development. Recurrence during the summer months is not uncommon.1 Unlike other common forms of cutaneous lupus, tumid lupus heals without scarring or pigmentary changes.4 Tumid lupus also has a weak association with progression to SLE.2
This case documents the uncommon association of SLE with tumid lupus. If a diagnosis of tumid lupus is suspected, biopsy should be performed from an active erythematous plaque.2 When a diagnosis of tumid lupus is confirmed, the patient should be thoroughly evaluated for systemic disease, regardless of the weak association with SLE.2 First-line therapy for localized tumid lupus is photoprotection and corticosteroids. If these therapies do not afford adequate control, treatment with antimalarial medications should be initiated.
- 1. Kuhn A, Richter-Hintz D, Oslislo C, Ruzicka T, Megahed M, Lehmann P. Lupus erythematosus tumidus: a neglected subset of cutaneous Lupus erythematous: report of 40 cases. Arch Dermatol. 2000;136(8):1033-1041.
- 2. Saleh D, Crane JS. Tumid lupus erythematosus. StatPearls. https://www.ncbi.nlm.nih.gov/books/NBK482515/. Accessed November 15, 2018.
- 3. Schur PH, Massarotti EM, eds. Lupus Erythematosus: Clinical Evaluation and Treatment. 1st ed. New York, NY: Springer Science and Business Media. 2012:68-77.
- 4. Alexiades-Armenakas MR, Baldassano M, Bince B, et al. Tumid lupus erythematous: criteria for classification with immunohistochemical analysis. Arthritis Rheum. 2003;49(4):494-500.