Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous- violaceous. Tumid lupus erythematosus (TLE), also known as lupus erythematosus tumidus, is a highly photosensitive form of cutaneous lupus erythematosus (cutaneous. MalaCards based summary: Lupus Erythematosus Tumidus, also known as intermittent cutaneous lupus, is related to lupus erythematosus and discoid lupus .

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UVB irradiation causes keratinocyte necrosisimmune system activation and antibody formation. Histological signs are perivascular and periadnexal lymphocytic infiltrate, interstitial mucin deposition and, in some cases, diffuse lymphocytes, or absence of epidermal involvement or any change in the dermoepidermal junction.

Lupus Erythematosus Tumidus: A Unique Disease Entity

Schmitt, et al, studied the characteristics of 44 patients with CLE, 24 of whom were diagnosed with LET, and found that LET had significantly decreased damage scores and increased mucin deposition, absent interface dermatitis, and alteration of hair follicles on histology when compared to other variants tumidue CLE. Features of neonatal cutaneous LE may include:.

She was also being treated for biopsy proven class V lupus nephritis with mycophenolate mofetil mg twice a day, tacrolimus 2mg twice a day, and hydroxychloroquine sulfate mg daily 4. Development of characteristic skin lesions are seen 1 week after UV-A irradiation for 3 consecutive days. Because of the rapid and effective improvement of the skin lesions after treatment with antimalarials, systemic corticosteroids or immunosuppressants were temporarily necessary in only 2 patients.

In a few cases, there is a tendency of the skin lesions to coalesce in the periphery, producing a gyrate configuration, and tuimdus, annular lesions develop tumixus the cheeks and upper extremities of some patients, imitating the annular type of SCLE.

Create a personal account to register for email alerts with links to tuidus full-text articles. Clinical and immunologic studies in reticular erythematous mucinosis and Jessner’s lymphocytic infiltration of the skin. Multiple prednisone tapers may lead to adrenal insufficiency and other excess corticosteroid effects. Cutaneous manifestations of lupus can be classified into specific subtypes — which include chronic cutaneous lupus erythematosus CCLEsubacute cutaneous lupus erythematosus SCLE and acute cutaneous lupus erythematosus ACLE — and nonspecific skin lesions — such as panniculitis, vasculitis and tumid lesions.

Active and burned-out disease can lead to social isolation and depression. The main differential diagnoses are polymorphous light eruption, Jessner’s lymphocytic infiltration of the skin, reticular erythematous mucinosis, and pseudolymphoma. These patients were placed on the same antimalarial therapy and again achieved remission within 12 weeks. None of the 40 patients had a history of drug-induced LET in our study after up to 15 years of follow-up.

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In 3 patients, the dose had to be increased to 6—6. However, in our opinion, there is no doubt about LET being a separate entity that has been neglected in the literature since first being described in She complained of mild pruritis and tenderness.

Consent for publication was obtained by our patient. tumdus

In contrast, Kind and Goerz 15 have reported a rare but possible systemic involvement in patients with LET, but no specific manifestations have been documented. Succulent, edematous plaques on the cheek left and forehead right of 2 patients.

Vasculitis and antiphospholipid syndrome involving internal organs can be serious. This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. It is more prevalent in patients with skin of colour, who are at greater risk of postinflammatory hyperpigmentation and hypertrophic scarring.

Follicular plugging and adherent hyperkeratotic scaling, which are also features of DLE, have not been seen in any of the patients. Interestingly, the mainstay of treatment for LET, in the limited case reports and series that exist, is with antimalarial drugs, which our patient had already been taking for SLE.

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. More than drugs have been associated with the onset of subacute cutaneous LE.

Positive results of provocative phototesting of lupus erythematosus tumidus. Diagnostic features on biopsy are more likely to be found in LE-specific skin lesions than in LE-nonspecific cutaneous LE. In the following years, 3 additional patients with similar skin lesions were described by the same authors 7 – 9 ; however, the next case reports of LET were not mentioned until in the German and the French literature.

Copyright American Medical Association.

Because of the latency period in development of positive phototest reactions, it may be difficult for patients to link sun exposure with their skin lesions. Forty white patients with erythematous, succulent, nonscarring, elevated plaques of variable extent over the face, upper part of the back and chest, shoulders, and extensor aspects of the arms were included in our study.


This, however, places our patient at risk for adverse drug reactions such as retinopathy or agranulocytosis with hydroxychloroquine therapy. Privacy Policy Terms of Use. All patients receiving antimalarials for several months or years underwent laboratory studies, including complete blood cell counts and renal and liver function tests, before the onset of therapy and every 4 to 6 weeks thereafter, as well as ophthalmologic examinations eg, fundoscopy, perimetry, color vision, and electro-oculography every 6 months.

Patients, materials, and methods. Our data emphasize the importance of defining LET as a separate entity and demonstrate that this disease has been neglected in the literature since first described in What are the specific features of cutaneous lupus erythematosus?

Circulating autoantibodies and immune complexes are due to loss of normal immune tolerance and are pathogenic. Articles which use infobox templates with no data rows Infobox medical condition new All stub articles.

J Am Acad Dermatol. The youngest patient was aged 9 months at initial diagnosis and had already had recurrent skin lesions for 10 years. LE is classified as autoimmuneas it is associated with pathogenic antibodies directed against components of cell nuclei in various tissues. Jessner’s lymphocytic infiltration of the skin. Lupus erythematosus LE is an autoimmune connective tissue disorder that can affect one or several organs. The perivascular and periadnexal infiltrate is composed of lymphocytes and, in some cases, scattered neutrophils are seen.

Lupus erythematosus tumidus is a separate subtype of cutaneous lupus erythematosus.

Cutaneous lupus erythematosus

Lupus erythematosus tumidus—a neglected subset of cutaneous Lupus erythematosus: Histology Histological features include perivascular and periadnexal lymphocytic infiltration and interstitial mucin deposition.

The treatment can also include topical corticosteroids, systemic corticosteroids and high sun protection factor levels 30or higher.

The tukidus of treatment for cutaneous LE is to prevent flares, improve appearance and to prevent scarring. Test areas were evaluated until specific lesions appeared for up to 4 weeks after the last irradiation.