|Year : 2020 | Volume
| Issue : 4 | Page : 225-227
A rare case of tumid lupus erythematosus with unilateral linear distribution in a young child
Gwang Hoon Kim, Jong Heon Jeong, Jong Soo Hong, Seung Ho Lee, Ai-Young Lee
Department of Dermatology, Dongguk University Ilsan Hospital, Goyang-si, Gyeonggi-do 410-773, Republic of Korea
|Date of Submission||21-Dec-2019|
|Date of Decision||25-Mar-2020|
|Date of Acceptance||02-Apr-2020|
|Date of Web Publication||16-Dec-2020|
Dr. Ai-Young Lee
Department of Dermatology, Dongguk University Ilsan Hospital, 814 Siksa-dong, Ilsandong-gu, Goyang-si, Gyenggi-do, 410-773
Republic of Korea
Source of Support: None, Conflict of Interest: None
Tumid lupus erythematosus (TLE) as a rare variant of cutaneous lupus erythematosus (CLE) is characterized by edematous, urticarial-like annular papules and plaques. TLE has similar histopathologic findings to CLE such as periadnexal lymphocytic infiltration and interstitial mucin deposition. Although TLE develops on sun-exposed areas at any age, it is rarely distributed along the Blaschko lines and develops in infancy and childhood. Unlike CLE, skin lesion of TLE heals without leaving scarring or dyspigmentation. Here, we report a rare case of unilateral linear TLE in a 4-year-old girl, which was improved by intralesional corticosteroid injection and oral antimalarial drug with leaving postinflammatory hyperpigmentation.
Keywords: Tumid lupus erythematosus, unilateral linear distribution, young child
|How to cite this article:|
Kim GH, Jeong JH, Hong JS, Lee SH, Lee AY. A rare case of tumid lupus erythematosus with unilateral linear distribution in a young child. Dermatol Sin 2020;38:225-7
|How to cite this URL:|
Kim GH, Jeong JH, Hong JS, Lee SH, Lee AY. A rare case of tumid lupus erythematosus with unilateral linear distribution in a young child. Dermatol Sin [serial online] 2020 [cited 2021 May 7];38:225-7. Available from: https://www.dermsinica.org/text.asp?2020/38/4/225/303688
| Introduction|| |
The tumid lupus erythematosus (TLE) is a subtype of chronic cutaneous lupus erythematosus (CLE). It was first reported by Gougerot and Burnier. The word “tumid” derives from tumidus, which means “swollen” in Latin. As the name suggests, the clinical manifestation of TLE can be characterized by edematous, wheal-like erythema. Like CLE, TLE has a photosensitive nature. Its skin lesions tend to develop symmetrically at sun-exposed sites. These lesions showing linear distribution are unusual. Only three cases have been reported so far. TLE affects both genders nearly equally. Even though it can occur at any age, its occurrence in childhood is extremely rare. Treatment of TLE varies by the extent of the disease. TLE lesions tend to heal without leaving scarring or pigmentary alteration. Here, we present a rare case of unilateral linear TLE developed in a 4-year-old girl. Her TLE showed an improvement, although she had postinflammatory hyperpigmentation.
| Case Report|| |
A 4-year-old girl presented with asymptomatic wheal-like erythematous to brownish plaques arranged linearly on the right side of her upper back, upper chest, and the same side of the forearm and hand for 1 year [Figure 1]a and [Figure 1]b. Before visiting our clinic, she had been treated with topical tacrolimus without improvement. Her parents denied sun exposure of truncal lesions because she never took off her clothes when she was outside. Medical history was unremarkable. Laboratory findings showed positive results for antinuclear antibody (1:320), but negative results for anti-double-stranded DNA and anti-Scl-70 (anti-topoisomerase I). Histopathologic findings of biopsied skin specimens from forearm revealed perivascular and periadnexal lymphoplasmacytic infiltration and widening of spaces between dermal collagen bundles without significant changes in the epidermis [Figure 2]a and [Figure 2]b. Alcian blue stains revealed interstitial mucin deposition on the dermis [Figure 2]c. Direct immunofluorescence showed linear deposition of immunoglobulin M on the basement membrane. Based on these clinical and histopathologic findings, the case was diagnosed as TLE. Although the lesions were treated with intralesional triamcinolone injection (2.5 mg/ml) every 2 weeks and a topical steroid for 5 months, the improvement was not remarkable. In the meantime, new lesions appeared. Due to insufficient effect of those treatments, we added oral hydroxychloroquine (50 mg daily) after baseline ophthalmologic examination. After 4 months of treatment, lesions gradually improved without developing new lesions. However, she had brownish hyperpigmentation [Figure 1]c.
|Figure 1: (a) Erythematous to brownish edematous plaques were linearly arranged along the inner forearm. (b) Erythematous plaque on the same side of the upper back. (c) Lesions improved with brown discoloration after administration of oral hydroxychloroquine|
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|Figure 2: (a) There was no significant pathologic change in epidermis (H and E, ×40). (b) Lymphoplasmacytic infiltration around the skin appendage (H and E, ×200). (c) Alcian blue stain showed mucin deposition between dermal collagen bundles (×100)|
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| Discussion|| |
TLE is diagnosed based on clinical and histopathologic findings to differentiate it from other variants of light eruption (LE), polymorphous LE, Jessner's lymphocytic infiltration of the skin, reticular erythematous mucinosis, and pseudolymphoma,, although its exact diagnostic criteria have not been established yet. TLE is a subtype of CLE. TLE and CLE share some characteristic histopathologic findings, including perivascular and periadnexal infiltration of lymphocytes and plasma cells with interstitial mucin deposition, as shown in our case [Figure 2]a, [Figure 2]b, [Figure 2]c. Lack of involvement of the epidermis is a usual finding of TLE, as shown in our case [Figure 2]a, although there might be minor epidermal changes such as vacuolar degeneration in the basal layer or epidermal atrophy in some cases. Therefore, our case could be compatible with TLE.
Clinical findings of smooth-surfaced, erythematous edematous papules and plaques in our case [Figure 1]a and [Figure 1]b were in accord with TLE, supporting that diagnosis. However, there were unique findings in our case. Ultraviolet light is a well-known triggering factor of TLE, and most of the skin lesions develop on sun-exposed areas. However, the skin lesions in our case developed on nonexposed areas such as the inner wrist, forearm and arm, anterior chest, and back [Figure 1]a and [Figure 1]b. In addition, the lesions were distributed linearly along the lines of Blaschko [Figure 1]a and [Figure 1]b. Blaschkolinear distribution is a very rare clinical presentation of TLE. Only three cases have been reported in the English written literature.,, These cases along with our case are summarized in [Table 1]. Including our case, two patients had skin lesions on the arm and the same side of the back. Although the other two patients had lesions on their foreheads, they denied sun exposure. These findings suggest a potential role of somatic mosaicism in its pathogenesis. Differing from other three cases, only our case developed in childhood. TLE usually occurs in the fourth decade. Childhood TLE in itself is rare. Five patients have been reported so far.,
|Table 1: Characteristic findings of linear tumid lupus erythematosus: Reported cases versus this case|
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TLE has been treated with topical corticosteroids or intralesional corticosteroid injection, although these treatments were insufficient to control skin lesions in our patient. The outcome of topical therapy showed various success rates, ranging from 12% to 80%., When TLE is refractory to topical therapies, systemic antimalarial agents for 4–6 weeks may show a good response. Treatment with hydroxychloroquine in our case also brought significant flattening of lesion without developing new lesions. However, complete resolution by hydroxychloroquine for 4 months was not examined [Figure 1]c. Lower doses (approximately 2 mg/kg/day) than recommendation (up to 6 mg/kg/day) might be responsible for the less effective outcome. TLE usually spontaneously disappears after several years, although it may last for 30 years. TLE is known to heal without scarring, atrophy, or dyspigmentation. However, our patient had hyperpigmentation after her lesions improved. Considering that there are reports about TLE with hypopigmentation in black patients and hyperpigmentation in a Korean patient,, TLE may show pigmentary alteration depending on their skin color.
Our case showed Blaschkolinear distribution on nonexposed area, onset in early childhood, and improvement leaving postinflammatory hyperpigmentation as rare findings in TLE.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]