Lupus Erythematosus Profundus: A Case Series and Review of Literature

Thapa, Deeptara Pathak; Kubba, Raj; Kubba, Ali

doi:10.35248/2684-1630.18.3.133

Cited by 3 publications

(1 citation statement)

References 22 publications

(37 reference statements)

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“…Lupus profundus (LP), also known as Lupus panniculitis, is a rare form of chronic cutaneous lupus erythematosus that can occur alone as erythematous nodules or plaques, or in conjunction with discoid lupus erythematosus (DLE) in up to 10% of cases 1 or systemic lupus erythematosus (SLE) in 1–3 percent of cases. 2…”

Section: Discussionmentioning

confidence: 99%

Intravenous immunoglobulin in the management of refractory lupus profundus

Namas

Abdulla

Elarabi

2022

Lupus

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Lupus profundus, often known as lupus panniculitis, is a rare form of persistent cutaneous lupus erythematosus. It usually manifests as painful plaques or nodules that can ulcerate and cause atrophy and scarring. It may respond to topical treatments and antimalarials, although treatment might be difficult at times, necessitating immunosuppressive medications. A 36-year-old woman from the United Arab Emirates presented with multiple painful disfiguring nodules involving the face and shoulders. The disfiguring skin nodules were resistant to systemic glucocorticoids, hydroxychloroquine, azathioprine, mycophenolate mofetil, and cyclosporine, but they significantly improved with monthly intravenous immunoglobulin over a 6-month period.

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Section: Discussionmentioning

confidence: 99%

Intravenous immunoglobulin in the management of refractory lupus profundus

Namas

Abdulla

Elarabi

2022

Lupus

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Lupus Erythematosus Profundus with Multiple Overlying Cutaneous Ulcerations: A Rare Case

Sutedja,

Widjaya,

Dharmadji

et al. 2023

CCID

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Lupus erythematosus profundus (LEP) is a rare subset of chronic cutaneous lupus erythematosus (CCLE), with a reported incidence of 1–3% in all LE cases. The most common cutaneous clinical presentation includes indurated plaques or subcutaneous nodules with an overlying normal skin. The clinical findings range from skin redness to features of CCLE, such as scaling, follicular plugging, and atrophy. Ulceration is rare and occurs in 28% of all LEP cases. We present a case report of LEP with multiple cutaneous ulcers on the right cheek and scalp accompanied by cicatricial alopecia. No other systemic manifestations were noted. Histopathological examination revealed periadipocyte, perivascular, and perivascular infiltration of lymphocytes, eosinophils, and plasma cells, supporting the diagnosis of LEP. The topical treatments given to the patient were sunscreen, 2% mupirocin cream, and wound dressing with dialkyl carbamoyl chloride (DACC). The patient was also treated systemically with oral corticosteroids and hydroxychloroquine. Clinical improvements were observed in the 3rd month of follow-up, and ulcer healing resulted in atrophic scars and fading erythematous macules. LEP is seldom associated with systemic or discoid lupus erythematosus. This occurs twice as frequently as a distinct entity does. Diagnosis accuracy plays an important role in determining the appropriate wound care, topical, and systemic treatments for LEP patients with multiple overlying cutaneous ulcerations.

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Lupus Panniculitis in Association with Anti-Phospholipid Antibody Syndrome on a Background of Systemic Lupus Erythematosus: A Case Report and Literature Review

2020

EMJ Allergy Immunol

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Lupus panniculitis occurs in 1–3% of the patients diagnosed with systemic lupus erythematosus (SLE) and 10% of the patients diagnosed with discoid lupus erythematosus (DLE). It is a disorder of autoimmune origin, manifesting as deep erythematous plaques and nodules involving the trunk, breasts, buttocks, face, and proximal extremities. It does not commonly ulcerate. This report highlights the case of a 22-year-old Asian female with a history of coeliac disease and significant family history of antiphospholipid antibody syndrome (APS) who presented with fever, malaise, weight loss, and subcutaneous non-tender nodules over the forearm, back, bilateral thighs, and feet. Laboratory investigations revealed positive antinuclear antibodies, anti-Ro/SSA antibody, and lupus anticoagulant, resulting in a diagnosis of APS. Biopsies of lesions were consistent with findings of lupus panniculitis. Every case of SLE and DLE with discrete skin lesions should be reviewed for any distinct entity such as lupus panniculitis, as it may be associated with greater risk of flares and systemic involvement. The purpose of this case report is to emphasise that early diagnosis and prompt treatment is crucial to improving the prognosis of such patients.

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Lupus Erythematosus Profundus: A Case Series and Review of Literature

Cited by 3 publications

References 22 publications

Intravenous immunoglobulin in the management of refractory lupus profundus

Intravenous immunoglobulin in the management of refractory lupus profundus

Lupus Erythematosus Profundus with Multiple Overlying Cutaneous Ulcerations: A Rare Case

Lupus Panniculitis in Association with Anti-Phospholipid Antibody Syndrome on a Background of Systemic Lupus Erythematosus: A Case Report and Literature Review

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