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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Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous-violaceous lesions that leave no scars after regression. Histopathology reveals perivascular and periannexal lymphohistiocytic infiltrates in the papillary and reticular dermis and interstitial mucin deposition. Treatment is based on photoprotection, topical corticosteroids and antimalarials. We report two cases of lupus tumidus, which deserve attention for their low frequency in the literature, in addition to their relevance as a differential diagnosis among dermatologic disorders.
Lupus erythematosus is a multisystem, heterogeneous, autoimmune disease characterized by the production of autoantibodies against cellular constituents.
The most affected organ is the skin, which may be involved in isolation or accompanied by systemic manifestations. Genetic, environmental, socio-cultural and demographic differences may contribute to differing incidences as well as the clinical expression of the disease.
Cutaneous manifestations of lupus can be classified into specific subtypes — which include chronic cutaneous lupus erythematosus CCLElhpus cutaneous lupus erythematosus SCLE and acute cutaneous lupus erythematosus ACLE — and nonspecific skin lesions — such as panniculitis, vasculitis and tumid lesions.
Lupus tumidus was first described by Gougerot and Bournier in Histopathologically, the epidermis is generally spared or presents discrete focal vacuolar degeneration of the basal membrane, associated with a perivascular lymphohistiocytic lesion infiltrated in the papillary and reticular dermis and interstitial mucin deposition.
A year-old female patient presented with a complaint of humidus on the thorax for 4 months. We performed an incisional biopsy and stained the sample with hematoxylin-eosin, colloidal iron and Ziehl-Neelsen.
Pathologic study revealed a moderate predominantly perivascular lymphocytic infiltrate not affecting the blood vessels in the papillary dermis and superficial and deep reticular dermis Figure 3.
Collagen fibers were separated by mucin accumulation, which was confirmed by colloidal iron staining Figure 4. Epidermis showed atrophy of the spinous layer and numerical and volumetric reduction of interpapillary ridges. Moderate perivascular infiltrate in the dermis.
Clinical and histopathological findings confirmed our hypothesis of lupus tumidus. The lipus responded well to treatment and we observed no new lesions.
She also mentioned the appearance of a small erythematous-infiltrated plaque about two centimeters in diameter at the left frontoparietal region fifteen days before the medical appointment. Biopsies of the skin on the left frontal region and of the left nasolabial sulcus suggested the diagnosis of lupus tumidus.
Anti-nuclear antibody, anti-Ro antibody, complete blood count and complement tests were all within the normal range. Treatment consisted of oral prednisone, chloroquine, and topic hydrocortisone lotion.
Chronic cutaneous lupus erythematosus CCLE has polymorphous presentations that may occasionally mimic other clinical conditions, causing diagnostic difficulties.
Lupus tumidus is a rare subtype of CCLE, and its diagnosis can be confirmed by the correlation between clinical and histopathological manifestations. The diagnosis of lupus tumidus is usually delayed, as it can be confused with other dermatoses due to the absence of systemic manifestations. The clinical criteria is met with the presence of erythematosus, thick plates with a smooth and edematous surface on sun-exposed areas that leave no scar after regression.
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 tumius differs in several aspects from the other variants of CCLE, such as the absence of scars when involuted, epidermal atrophy and follicular plugging and adherent hyperkeratotic scaling, all of which are present with discoid lupus. Unlike subacute cutaneous lupus erythematosus SCLElupus tumidus presents no residual hypopigmentation. Histopathology reveals no follicular hyperkeratosis, epidermal atrophy, vacuolar degeneration or basal tumidis thickening, usually identified at SCLE and discoid lupus.
The disease fills the diagnostic criteria for lupus tumidus proposed by Kuhn, which justifies the controversy in the medical literature that question whether Jessner is a lupus tumidus variant or if it is an autonomous entity.
Lupus Erythematosus Tumidus: A Unique Disease Entity
Reticular erythematous mucinosis REM — a primitive form of mucinosis that worsens with sun exposure and presents satisfactory response to treatment with antimalarial drugs – should also be listed in the differential diagnosis.
Despite the histopathologic similarities to lupus tumidus, the lymphocytic infiltration is usually less dense and mucin accumulates mainly at the papillary dermis in REM.
The main difference between the diseases is the clinical manifestation. We suggest systemic antimalarial drugs as the treatment of choice for lupus tumidus. The treatment can also include topical corticosteroids, systemic corticosteroids and high sun tymidus factor levels 30or higher.
Due to the rarity of cases reported, we emphasize the importance of detailed clinical examination supplemented by histopathological study since isolated examination tumidux lead to underdiagnosing the disease. National Center for Biotechnology InformationU.
Lupus tumidus: a report of two cases
Journal List An Bras Dermatol v. Author information Article notes Copyright and License information Disclaimer. Bianca Pinheiro Bousquet Muylaert, R. Received Jul 17; Accepted Oct 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. Abstract Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous-violaceous lesions that leave no scars after regression.
Antimalarials, Autoimmune diseases, Lupus erythematosus, cutaneous. Open in a separate window. Erythematous infiltrated lesion tumkdus the thorax with central clearing.
Footnotes Conflict of Interest: Colagenoses e kupus Dermatologia.
Gougerot H, Bournier R. Histopathologic findings in lupus erythematosus tumidus: Review of 80 patients.
Lupus Erythematosus Tumidus: A Unique Disease Entity
J Am Acad Dermatol. Support Center Support Center.