Thursday, March 23, 2023

Study warns of diagnostic delay in rare type of lupus that causes irreversible atrophy

Lupus erythematosus panniculitis is characterized by erythematous, often painful, subcutaneous nodules or plaques.

Systemic lupus erythematosus (SLE) is a chronic disease caused by autoimmunity that affects the body’s organs, especially the skin, joints, blood, kidneys, and central nervous system. Photo: Shutterstock.

Lupus erythematosus panniculitis (LEP) is a rare type of cutaneous lupus erythematosus characterized by erythematous, often painful, subcutaneous nodules or plaques that are tender to the touch in fatty areas of the body.

Deep lipoatrophy occurs frequently, which can lead to severe deformity. Although LEP is often isolated, may coexist with discoid lupus erythematosus (DLE) (referred to as lupus profundus) or systemic lupus erythematosus (SLE). Diagnosis is often delayed, increasing the risk of sequelae such as atrophy and calcinosis.

The existing literature on LEP is limited to small case series, which limits knowing the clinical features of the condition for early diagnosis and effective treatment. For this reason, one study presented an analysis of case series of 61 patients with confirmed LEP and their initial presentation, treatment, and characteristics associated with association with systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE). did.

The group identified all LEP cases diagnosed on the basis of biopsy and/or expert opinion from January 2000 to October 2019. Reviewing 5,717 cutaneous lupus erythematosus medical records, we identified 61 LEP cases, and 54 of 61 (88%) were confirmed by biopsy.

test result

Of the 61 patients, 52 (85%) were female. Lupus erythematosus panniculitis affected 2 or more sites at presentation in 36 patients (59%) for a total of 105 distinct lesions.

Lesions were located in the proximal upper extremity (27.6%), face (17.1%), proximal lower extremity (14.3%) and back (10.5%).

Most presented with active erythematous nodules/palpable plaque and atrophy or change in contour.

Overall, the median time from initial clinical presentation of LEP symptoms to diagnosis was 19.2 months. For those with a history of SLE or SDL at the time of LEP diagnosis, the median time to diagnosis was 29.8 months, while it was 8.8 months for those with a history of SLE and/or SDL.

Of the 61 patients, 21% had coexisting SLE, 28% had coexisting SLE, and 13% had both. Of those with SLE, 17 were diagnosed with SLE before the onset of LEP, 3 developed SLE after LEP, and 1 were diagnosed simultaneously.

Overall, disease control required an average of 2.5 systemic treatments, and 30 of 58 patients (52%) required at least 1 immunosuppressant drug. The most commonly used therapies were hydroxychloroquine and methotrexate.

Patients without SCI or coexisting SCI required 2.1 systemic treatments, whereas those with SCI or co-existing SCI required 2.8 systemic treatments for LEP control.

In this large group of LEP patients, LEP occurred mostly in middle-aged women and affected their proximal extremities, face and back.

The medical literature indicates that lupus erythematosus panniculitis is rare and difficult to diagnose, especially without SLE or DLE at presentation.

Therefore, the researchers indicated that the findings of this study demonstrate delayed diagnosis and refractory disease, thus underscoring the need for earlier diagnosis and treatment to prevent pain and irreversible atrophy.

However, they highlight that limitations of this study include its retrospective design and limited sample size, although to our knowledge, it is one of the largest LEP clusters described so far.

Access the study here.

Nation World News Desk
Nation World News Desk
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