Lupus erythematosus tumidus with discoid lupus erythematosus-induced alopecia of the scalp
Published Web Locationhttps://doi.org/10.5070/D30z4556qj
Lupus erythematosus tumidus with discoid lupus erythematosus-induced alopecia of the scalpDepartment of Dermatology, New York University, New York, New York
Stephanie Lehrhoff MD, Julia Tzu MD, Rishi Patel MD, Miguel Sanchez MD, Andrew Franks Jr MD
Dermatology Online Journal 17 (10): 24
Lupus erythematosus tumidus (LET) is an uncommon variant of cutaneous lupus erythematosus. Lupus erythematodes tumidus is characterized by smooth, erythematous plaques on sun-exposed areas without surface changes, such as follicular plugs, atrophy, or scale. Histopathologic features include a lymphocytic, perivascular and periadnexal infiltrate with abundant interstitial, superficial, and deep dermal mucin without appreciable epidermal and vacuolar changes. Typically, LET is responsive to treatment with systemic antimalarials. We present a unique case of alopecia associated with LET, which was partially responsive to hydroxychloroquine. We also note that the plaque of LET is adjacent to the plaque of discoid lupus erythematosus.
A 29-year-old African American man presented to the Dermatology Clinic at Bellevue Hospital Center in August, 2010, with a two-year history of discreet areas of hair loss at the right frontal hairline and mid frontal scalp. The areas of hair loss had enlarged during the previous three months but were asymptomatic. He had used minoxidil 5 percent topical solution twice daily for three weeks and a topical antifungal cream without benefit. He denied other medical problems or associated symptoms, which included joint pains, fatigue, or oral ulcers, but reported a history of sun sensitivity and sunburns.
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A horizontally symmetrical receding hairline with a normal vertex was present. Present on the superior mid aspect of the forehead slightly to the right side was a 3 x 4 cm, smooth-surfaced, annular plaque with an indurated pink inferior border. Two firm, infiltrated, round, slightly-elevated, brown-red, 0.5 to 1.1 cm papules and one 2.3 x 1.6 cm, oval plaque became confluent to form the superior border that infiltrated the frontal hairline. A 1 x 1 cm patch of the medial right frontal hairline included within it discreet, hyperpigmented, indurated papules, which on dermatoscopic examination showed follicular plugs. More laterally, there was a 2 x 1 cm, hairless plaque adjacent to the hairline. A 0.8 x 0.8 cm, indurated, hairless macule with a pigmented center and pink borders was present at the mid-frontal scalp.
The white-cell count was 3.4 x 109/L. Basic metabolic and hepatic panels were normal. An antinuclear antibody test was positive with a titer of 1:320 and a speckled pattern. SSA/Ro antibody was positive 4.7/AI. SSB/La antibody was negative. A glucose 6-phosphate dehydrogenase level and a reticulocyte count were normal.
There is a superficial and deep, dense, perivascular and periadnexal infiltrate that is comprised predominantly of lymphocytes and scattered plasma cells. A colloidal-iron stain shows increased deposits of connective-tissue mucin.
The term lupus erythematodes tumidus (LET) was coined in 1909 to describe two patients, who presented with round, erythematous, elevated tumorous lesions on the face without surface changes . Many years later five more cases were described with similar clinical features . Lupus erythematodes tumidus is considered to be a variant of chronic cutaneous lupus erythematosus (CCLE) although some authors have argued that the lesions are intermittent and resolve spontaneously . Differentiating the clinical and histopathologic features of LET from other cutaneous diseases, such as subacute cutaneous lupus erythematosus (SCLE), pseudolymphoma, polymorphous light eruption, reticular erythematous mucinosis, and Jessner lymphocytic infiltrate, can be a challenge. However, utilizing the algorithm suggested by Alexaides-Armenakas, et al, the CD4/CD8 lymphocytic ratio greatly aids in its correct diagnosis .
Like other forms of cutaneous LE, the pathogenesis of LET remains largely unknown. Interferon alpha and its major cellular producers, plasmacytoid dendritic cells are implicated in the immunopathogenesis of lupus erythematosus . Tumid lupus lesions have plasmacytoid dendritic cells that are localized to perivascular and periadnexal regions, which correspond to the inflammatory infiltrate characteristic of LET. Similarly, interferon alpha positivity was demonstrated in the same perivascular and periadnexal regions. In contrast, normal skin demonstrated plasmacytoid dendritic cells in the epidermis and superficial dermis but not within the deep dermis .
Lupus erythematodes tumidus or colloquially tumid lupus is uncommon but may be under recognized. The exact incidence is unknown. No clear gender predominance is observed [3, 7, 8]. The mean age of onset is similar to that of discoid lupus erythematosus (DLE) and SCLE. In a cohort of 40 patients with LET, the mean age of onset was 36.4 years . Children rarely develop this form of lupus erythematosus . Most patients with LET have been Caucasian, possibly as a result of selection bias. To our knowledge only four other cases of tumid lupus in African American patients have been reported [4, 10, 11].
Lupus erythematodes tumidus is characterized by the presence of one or more, indurated, firm, sharply-demarcated, smooth-surfaced, erythematous papules, plaques, or nodules of various sizes, which are devoid of surface changes, which include follicular plugs or telangiectases . The lesions develop predominantly on sun-exposed areas and have been described as succulent and urticaria-like. The lesions of LET may coalesce to produce annular, arcuate, or gyrate configurations and can swell at the periphery and flatten in the center .
Non-scarring causes of hair loss that are associated with lupus erythematosus include diffuse hair shedding during acute exacerbations, telogen effluvium, alopecia areata, and alopecia secondary to medications, such as antimalarials, systemic glucocorticoids, and azathioprine [13, 14]. Scarring alopecia is invariably associated with DLE. Notably, our patient’s patch of alopecia located at the frontal hairline has features of both LET and DLE in distinct areas.
In addition to the clinical morphology of the lesions, other criteria for LET include characteristic histopathologic findings and rapid and effective systemic treatment with antimalarials in most patients . Lupus erythematodes tumidus is widely accepted as a photosensitive form of CCLE. Like most patients with LET, the connection to lesion onset and sun exposure in our patient was not readily apparent although he did report extreme photosensitivity.
Characteristic histopathologic features of LET include a fairly well-circumscribed, lymphocytic, dermal infiltrate in a perivascular and periadnexal pattern, abundant interstitial mucin both superficially and deep, and superficial papillary dermal edema. Distinguishing LET from other forms of cutaneous LE is a lack of appreciable epidermal change, specifically vacuolar change and thickening of the basement membrane although focal vacuolar change and minimal thickening of basement membrane have been reported in lesions clinically consistent with LET . The direct immunofluorescence (DIF) test is variable; some studies report that all DIF tests are negative, and others have demonstrated deposits of IgG and IgM along the dermoepidermal junction [4, 7, 8]. The histopathologic features in this case demonstrate the characteristic perivascular and periadnexal lymphocytic infiltrate with superficial and deep interstitial mucin, without appreciable epidermal changes and only focal vacuolar changes.
In addition to characteristic skin findings, histopathologic appearance, and photosensitivity, LET is highly responsive to treatment with systemic antimalarials, specifically hydroxychloroquine 5 to 6 mg/kg/day and chloroquine 4 mg/kg/day . Rare cases of antimalarial failure have responded to methotrexate and systemic glucocorticoids . Our patient has had complete regrowth of the 8-mm, round patch at the mid frontal scalp and partial regrowth of a portion of the right frontal hairline during administration of hydroxychloroquine at a dose of 200 mg twice daily. The most medial aspect of the right frontal hairline alopecic plaque, which corresponds to the follicular plugs observed on dermatoscopic examinations, remains hairless.
Our patient is one of only a few reported African-American patients with LET. Classically, LET is not associated with other forms of CCLE. A 34 year-old woman with existing DLE of the lip was found to have pink, flesh-colored papules of her chest, which were histopathologically consistent with LET. The chest papules improved after an increase in the dosage of hydroxychloroquine . Perhaps, our patient represents another case of concurrent LET and DLE.
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