Lupus erythematosus tumidus

Alternate names

Intermittent cutaneous lupus; Tumid lupus erythematosus

Definition

Tumid erythematosus lupus is considered a rare type of chronic cutaneous lupus erythematosus. Cutaneous lupus erythematosus (CLE) can be divided into acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus.

Epidemiology

It is more frequent in the African American population. The epidemiology of tumid lupus specifically has not been well studied.[1].

Cause

• No distinct cause for TLE has been identified.[2].

• However, triggering factors such as ultraviolet (UV) radiation have been implicated in worsening lesions of TLE.
• Its association with autoimmune disease has been controversial; if an autoimmune disease is suspected, an autoimmune workup may be initiated.

Signs and symptoms

• Tumid erythematosus lupus is characterized by smooth, non-scarring, pink- to violet-colored pimples (papules) on the skin without any other apparent skin changes, such as scarring.
• Patients with tumid lupus erythematosus usually do not have other symptoms of systemic lupus erythematosus or other types of cutaneous lupus erythematosus.
• The papules appear on sun-exposed areas of the face, upper back, V area of the neck, trunk, and arms, and more rarely on thighs and legs.
• They usually affect equally both sides of the body, but may affect only one side.
• Normally, the papules clear without leaving scars.

Diagnosis

• A lesional biopsy should be taken from an active, erythematous plaque. [3].
• A punch biopsy (4 mm is recommended on the trunk or 3 mm on cosmetically sensitive areas such as the face) should be performed to include the full thickness of the dermis.
• If the histopathologic findings definitively support a diagnosis of tumid lupus, the patient should be evaluated for systemic disease, despite the weak association with SLE. T
• he evaluation for systemic lupus includes a full history and review of systems, physical examination with special attention taken to lymphadenopathy or arthritis, and laboratory tests (ANA with anti-dsDNA and anti-Sm profiles), urinalysis, complete blood count (CBC) with differential, chemistries, ESR, CRP, complement levels (C3, C4), and antiphospholipid antibodies.
• For a more extensive autoimmune workup, autoantibodies such as SS-A/Ro and SS-B/La, anti-U1RNP, and anti-histone antibody can be drawn.
• A lesional biopsy can be sent for direct immunofluorescence (DIF).
• Phototesting can be beneficial for diagnosing TLE; reproduction of lesions after UVA/UVB irradiation may support a diagnosis of tumid lupus.

Treatment

The treatment is very effective in most cases, and may include sun protection, anti-malarials drugs, local corticosteroids, topical tacrolimus and light therapy.

NIH genetic and rare disease info

• NIH info on Lupus erythematosus tumidus

Lupus erythematosus tumidus is a rare disease.

Lupus erythematosus tumidus Resources
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Saleh D, Crane JS. Tumid Lupus Erythematosus. [Updated 2020 Aug 16]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482515/