Clinical Challenge: A Painful, Itchy Facial Rash

Tumid lupus, also known as lupus erythematosus tumidus (LET), is an uncommon autoimmune disorder caused by a multitude of factors such as genetics, the environment, and hormones.1 LET is more commonly seen in men aged 41 to 50 years. Dysregulation...

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Tumid lupus, also known as lupus erythematosus tumidus (LET), is an uncommon autoimmune disorder caused by a multitude of factors such as genetics, the environment, and hormones.¹ LET is more commonly seen in men aged 41 to 50 years. Dysregulation of the immune system has been postulated as contributing to the pathophysiology of LET.² Data show that a decrease in regulatory T cells results in lowered immune tolerance, especially in photosensitive conditions such as LET and subacute cutaneous lupus erythematosus. Environmental factors such as smoking are highly associated with LET.³

Clinically, LET presents as erythematous, indurated, nonscarring plaques on sun-exposed sites such as the face, upper back, neck, extensor aspects of arms, and shoulders.⁴ Histopathologic evaluation is required for diagnosis. Histology reveals well-demarcated perivascular and periadnexal lymphocytic infiltration and mucin deposition. The dermis appears edematous compared with the epidermal atrophy and basement membrane thickening seen in other types of cutaneous lupus erythematosus conditions. Direct immunofluorescence staining of skin lesions is negative for immunoglobulin or complement.

Photosensitivity is a main component of LET,¹ with phototesting establishing it as a photosensitivity subtype of cutaneous lupus erythematosus. More recently, LET has been classified as an intermittent subtype of chronic cutaneous lupus erythematosus.⁴ Induction of lesions with ultraviolet (UV) irradiation in the form of UV-A and UV-B rays has been discussed in vitro and in vivo studies.^4,5 No correlation between photosensitivity and anti-Sjögren-syndrome-related antigen A (SSA/AntiRo) antibodies in patients with LET is known. However, this correlation is seen in patients with subacute cutaneous lupus erythematosus. Approximately 80% of patients with LET do not test positive for antinuclear antibodies.⁶

The differential diagnosis includes polymorphous light eruption, reticular erythematous mucinosis, and Jessner lymphocytic infiltrate of the skin.¹ The latter is also a photosensitive disease but unlike LET presents with asymptomatic erythematous papules or nodules without scarring on the face or upper back.⁷ Polymorphous light eruption is differentiated from LET by a shorter symptomatic course; lesions resolve a few days after implementing sun protection measures.⁶ Reticular erythematous mucinosis presents as reticulated macular erythema or reticular-like plaques.⁸ 

Treatment of LET is based on limiting exposure to sunlight and symptomatic relief.⁴ Patients with LET are advised to avoid sun exposure, especially in the summer, and to avoid travel to regions closest to the equator where exposure is likely. Sunscreen should be applied 15 to 30 minutes before going outdoors. Approximately 50% of patients with LET have no recurrences after following sun protection measures. Studies have shown that topical corticosteroids and antimalarial medications can be used for symptomatic relief of pruritus and painful lesions in extensive disease. Chloroquine administered at a dosage of 3.5 to 4.0mg/kg of ideal body weight per day for 4 to 6 weeks has been shown to be effective. Hydroxychloroquine administered at a dosage of 6.0 to 6.5mg/kg of ideal body weight per day is an alternative to chloroquine. 

Skin punch biopsy of the forehead using a 4-mm punch tool was performed on the patient described in the case. Histopathology revealed tumid lupus. The patient was discharged after sutures were placed and proper dressing and care were discussed. She returned a few weeks later complaining of severe pruritus and pain, which was worse at night.  She experienced mild benefit from application of Vaseline®. The patient was prescribed oral prednisone 20 mg to be tapered over 5 days. She was advised to apply topical triamcinolone 0.1% ointment to the lesions daily for 2 weeks for flares, then only on the weekends. Side effects such as atrophy and hypopigmentation were discussed. The patient was advised to follow vigilant sun protection protocols using sunscreen with an SPF of 30 to 50 with reapplication every 2 hours while in the sun. The patient returned a third time reporting continued pruritus and pain of the lesions on her face. She was prescribed the same prednisone taper and halobetasol 0.05% topical ointment to be applied to the affected areas twice a day for 2 weeks, then as needed on the weekends.

Ashley Kwan, MMSPAS, PA-C, works as a breast cancer surgery physician assistant at Breastlink in Orange, California.

References
1. Foering K, Chang AY, Piette EW, Cucchiara A, Okawa J, Werth VP. Characterization of clinical photosensitivity in cutaneous lupus erythematosus. J Am Acad Dermatol. 2013;69(2):205-213.

2. Gambichler T, Pätzholz J, Schmitz L, Lahner N, Kreuter A. FOXP3+ and CD39+ regulatory T cells in subtypes of cutaneous lupus erythematosus. J Eur Acad Dermatol Venereol. 2015;29(10):1972-1977.

3. Böckle BC, Sepp NT. Smoking is highly associated with discoid lupus erythematosus and lupus erythematosus tumidus: analysis of 405 patients. Lupus. 2015;24(7):669-674.

4. Kuhn A, Bein D, Bonsmann G. The 100th anniversary of lupus erythematosus tumidus. Autoimmun Rev. 2009;8(6):441-448.

5. Kind P, Lehmann P, Plewig G. Phototesting in lupus erythematosus. J Invest Dermatol.1993;100(1):53S-57S.

6. Kuhn A, Richter-Hintz D, Oslislo C, Ruzicka T, Megahed M, Lehmann P. Lupus erythematosus tumidus–a neglected subset of cutaneous lupus erythematosus: report of 40 cases. Arch Dermatol. 2000;136(8):1033-1041.

7. Rémy-Leroux V, Léonard F, Lambert D, et al. Comparison of histopathologic–clinical characteristics of Jessner’s lymphocytic infiltration of the skin and lupus erythematosus tumidus: multicenter study of 46 cases. J Am Acad Dermatol. 2008;58(2):217-223.

8. Cinotti E, Merlo V, Kempf W, et al. Reticular erythematous mucinosis: histopathological and immunohistochemical features of 25 patients compared with 25 cases of lupus erythematosus tumidus. J Eur Acad Dermatology Venereol. 2015;29(4):689-697.