Tumid lupus erythematosus_0212 Derm Clin1
A woman aged 28 years presented with asymptomatic lesions on her neck. Similar lesions noted on her arms the year before had healed without scarring. The woman admitted to sun sensitivity but denied fevers, chills, myalgias or arthralgias.
Examination revealed six to seven variably sized, nonscaly, pink papules and plaques on the left side of the neck. Biopsy demonstrated an interface dermatitis with a lymphocytic perivascular and periadnexal infiltrate. Special stains showed an increase in dermal mucin with negative immunofluorescent staining. Laboratory data demonstrated a low titer anti-nuclear antibody (1:80) with a negative anti-Smith antibody. What’s your diagnosis?
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The clinical, laboratory, and histologic findings of this case are diagnostic for tumid lupus erythematosus (TLE). Lupus erythematosus (LE) encompasses a wide range of autoimmune diseases, for which the exact pathogenic mechanisms are unknown. The spectrum of LE ranges from various cutaneous disorders without visceral organ involvement to a multiorgan systemic disease with or without cutaneous changes. For most individuals, TLE is a purely cutaneous disease.
TLE was first reported in 1909.1 Since then, various distinct subtypes of chronic cutaneous lupus erythematosus (CLE) have been described and characterized; these include chronic cutaneous (discoid) LE (CCLE), subacute cutaneous LE (SCLE), lupus panniculitis, and nonbullous neutrophilic dermatosis of LE, as well as TLE.
Furthermore, TLE also has clinical or histologic features that resemble a number of such non-LE cutaneous diseases as polymorphous light eruption (PMLE), pseudolymphoma, reticular erythematous mucinosis (REM), and Jessner lymphocytic infiltrate of the skin (JLI).2 Without complete histologic and immunologic data, it is highly likely that TLE could be confused with any of these other conditions. Fortunately, the diagnosis of TLE is easier to make with modern technology; serologic, immunohistochemical and histopathologic features have been have been identified, defining TLE as a distinct entity.3
TLE typically develops as innocuous papules, plaques and/or nodules; although most patients are asymptomatic, a minority may note mild pruritus. Lesions are often annular in shape and range from mild pink to a more violaceous color. Unlike most subtypes of cutaneous lupus, the lesional surface of TLE is characteristically smooth, lacking scale, follicular plugging, scarring, telangiectasia or atrophy.4-6
Sun-exposed areas including the face, upper back, chest and upper extremities are most commonly affected; rarely are lesions found on the lower extremities or inner aspect of the arms. Consistent with a photoactivated process, there is a higher incidence of TLE in the summer months.5 When multiple lesions are present, they tend to develop in a bilaterally symmetric distribution similar to other subtypes of cutaneous lupus. Although TLE can affect individuals of any age, the mean age of onset is the third or fourth decade of life;5 there appears to be no gender predominance.
A skin biopsy using special stains is necessary to confirm the diagnosis of TLE. Histologically, one finds a moderate-to-dense, superficial and deep lymphocytic infiltrate affecting the perivascular and periadnexal areas.3-5 An increased amount of interstitial dermal mucin can be demonstrated with such stains as colloidal iron.6 The mucin deposition of TLE resembles that of REM but is distinct from DLE and JLI. Whereas REM classically has a greater amount of mucin and fewer inflammatory cells than does TLE, both DLE and JLI have minimal mucin deposition.2 Inflammation of the dermoepidermal junction is a frequent finding in most subtypes of CLE, but this interface dermatitis is less consistently observed in TLE, comparatively.2,4 When observed, involvement is typically focal, demonstrating vacuolar alteration with necrotic keratinocytes.4
Pseudolymphoma and PMLE can be differentiated from TLE through immunohistologic staining for lymphocyte markers. The infiltrate in TLE is composed predominantly of T-lymphocytes that are CD3-positive and CD4-positive; the typical CD4-to-CD8 ratio is 3:1.2,4 Pseudolymphoma has more variable B and T cells, and PMLE has a predominance of CD8-positive cells.2
Serologic studies are nondiagnostic, since most individuals with true TLE have negative test results for lupus auto-antibodies (i.e., antinuclear antibody [ANA], anti-Ro, anti-La, anti-dsDNA, and anti-Sm).4-6 However, some may have a low-positive ANA titer. Cases in which patients have high auto-antibody titers most likely represent patients with an overlap of TLE and systemic lupus erythematosus (SLE).2,4 Because of this rare association, it is critical that all patients with TLE—or any of the subtypes of cutaneous lupus—be evaluated for SLE.2
The clinical course of TLE is usually benign with a favorable prognosis: Complete resolution of skin lesions occurs in the majority of cases, sometimes spontaneously without treatment. All patients need to be told of the photosensitive nature of the condition and educated appropriately about sun protection and sun avoidance. Broad-spectrum sunscreens should be recommended. Both UVA and visible light can pass through glass, so protection is needed in sunlit rooms and cars. Inorganic sunblocks and sun-protective clothing may provide more effective types of sun protection.
For mild and limited disease, a topical corticosteroid may be effective monotherapy. Steroid class is dictated by anatomic location. For example, a class 1 steroid may be used short-term on extremities, neck and torso; a class 5 steroid would be more appropriate on the face.
Consider antimalarial medications, preferably hydroxychloroquine (HCQ) (Plaquenil, Quineprox), for people who have more widespread lesions or cannot tolerate or are unresponsive to topical steroids.4,5 Pretesting and laboratory monitoring are necessary with HCQ therapy. Periodic ophthalmologic examinations are also essential because of the potential risk (though low) of retinal toxicity.5 Once in remission, taper treatment if possible; recurrences are not uncommon and typically respond to the same regimen.
This patient was treated with clobetasol cream. HCQ 200 mg twice daily was added because of her concern for cutaneous atrophy from use of the topical steroid. The woman was advised to use an adequate form of birth control; HCQ is a pregnancy-prescribing category C medication.
Ashley Brown is a fourth-year student at Medical College of Virginia Hospitals, Virginia Commonwealth University, in Richmond, where Julia R. Nunley, MD, is professor of dermatology. Neither author has any relationship to disclose relating to the content of this article.
1. Hoffman E, Demonstrationen A. “Isolierter lupus erythematodes tumidus der gesichtshaut.” Derm Zeitschr. 1909;16:159-60.
2. Stead J, Headley C, Ioffreda M, et al. “Coexistence of tumid lupus erythematosus with systemic lupus erythematosus and discoid lupus erythematosus: a report of two cases of tumid lupus.” J Clin Rheumatol. 2008;14:338-341.
3. Kuhn A, Sonntag M, Ruzicka T et al. “Histopathologic finding in lupus erythematosus tumidus: review of 80 patients.” J Am Acad Dermatol 2003; 48:901-908.
4. Alexiades-Armenakas MR, Baldassano M, Bince B et al. “Tumid lupus erythematosus: Criteria for classification with immunohistochemical analysis.” Arthritis Rheum. 2003;49: 494-500.
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