lupus profundus

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T cell dyscrasias that demonstrate a proclivity for the subcutaneous fat include atypical lymphocytic lobular panniculitis, lupus profundus, and primary subcutaneous T cell lymphoma, including subcutaneous panniculitis-like T cell lymphoma (SPTCL). We encountered two patients who developed fever and indurated abdominal erythema at their peginterferon alfa-2a injection sites. Biopsies showed an atypical CD8 positive, granzyme positive, CD5 negative, MXA negative lymphocytic lobular panniculitis, diagnostic of SPTCL...

Lupus erythematous profoundus (LEP) is an uncommon manifestation of chronic lupus erythematous (CLE) involving inflammation of the subcutaneous fat and deep dermis. It is rarely seen in the otolaryngological practice. We describe a case of a 33-year-old female who presented with bilateral acute onset cheek swelling, which led to the unexpected diagnosis of LEP. We describe the diagnostic pitfalls that may potentially bias the surgeon towards the management of such patients.

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...

There are T-cell lymphomas, which exhibit unique homing properties of the neoplastic lymphocytes for the subcutaneous fat. Primary cutaneous gamma delta T-cell lymphomas of the fat (PGD-TCL) typically have an aggressive clinical course; the more indolent variant is subcutaneous panniculitis like T-cell lymphoma (SPTCL), comprising T-cells of the alpha beta subset. These t wo forms of subcutaneous T-cell neoplasms exhibit many overlapping histologic features with lupus profundus (LP); it has been previously suggested by one of the authors that lupus profundus is a form of a panniculitic T-cell dyscrasia...

Some T cell lymphomas exhibit unique homing properties of the neoplastic lymphocytes for the subcutaneous fat. There are two primary forms of subcutaneous panniculitic lymphomas of T cell origin. The first falls under the designation of primary cutaneous gamma delta T cell lymphomas of the fat (PGD-TCL). The neoplastic cells are double negative for CD8+ gamma delta T cells and often have an aggressive clinical course. The other form of panniculitis, T cell lymphoma, falls under the designation of subcutaneous panniculitis-like T cell lymphoma (SPTCL)...

A 53-year-old man presented with refractory panniculitis on the left upper arm that had persisted for 10 months. The patient was diagnosed with lupus profundus, wherein oral glucocorticoid therapy was initiated. Four months prior, ulceration was observed in the same area. Dapson was administered instead, scarring the ulcer but enlarging the panniculitis. Five weeks earlier, he developed a fever, productive cough, and dyspnoea. Three weeks earlier, a skin rash was observed on the forehead, left auricle posterior to the neck, and extensor aspect of the left elbow...

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Sclerodermic or scleroderma-like lupus erythematosus panniculitis (SLEP) shares both clinical and histopathological features between lupus panniculitis and localized scleroderma. It is exceedingly rare. We herein report a case of SLEP manifested with a solitary, firm-to-hard, erythematous plaque in an Asian woman. This patient responded well to intralesional corticosteroid and antimalarials. We have reviewed the pathogenesis of fibrosis in patients with chronic cutaneous lupus erythematosus as well as documented cases of SLEP in the literature...

OBJECTIVE: Lupus erythematosus (LE) is a complicated disease with highly heterogeneous clinical manifestations. Previous studies have rarely included all subgroups of patients with lupus and have overlooked the importance of the cutaneous manifestations thereof. We aimed to compare the demographic and clinical differences among patients with different subtypes of lupus. METHODS: This is the first real-world study with a relatively large sample size that simultaneously includes patients with isolated cutaneous lupus erythematosus (iCLE) and SLE...

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INTRODUCTION: Lupus erythematous panniculitis (LEP) is a rare type of chronic cutaneous lupus erythematous. Clinical characteristics are tender, subcutaneous nodules, erythematous plaques. Disfigurement of face and body might develop which affects the patient's quality of life. LEP can be the first sign of systemic lupus erythematous (SLE). OBJECTIVE: Our aim was to review the clinicopathological characteristics and the course of LEP through our own patients. METHODS: We retrospectively analyzed the clinical records of 17 LEP patients at Semmelweis University's Department of Dermatology, Venerology and Dermatooncology between 2000 and 2022...

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INTRODUCTION: Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is an extraordinary form of peripheral T-cell lymphoma, accounting for much less than 1% of all non-Hodgkin lymphoma. It usually happens amongst women with a median age of onset of 36 years. It can arise with autoimmune disease ~20%. SPTL may also coexist with different autoimmune diseases (e.g., lupus erythematosus [LE]) despite normal workup. CASE: A 44-year-old woman presented with a small, 2×2 cm mass on her right inner thigh that was erythematous, non-movable, and tender...

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INTRODUCTION: Chronic cutaneous lupus erythematosus (CCLE) comprises three major clinical variants: discoid lupus erythematosus (DLE), chilblain lupus erythematosus (CHLE), and lupus erythematosus profundus, also referred to as lupus erythematosus panniculitis (LEP). The aim of the current study was to systematically describe the dermoscopic features of CCLE in Polish patients with Fitzpatrick skin phototypes I-III. METHODS: The videodermoscopic images from patients with various clinical variants of CCLE (DLE, CHLE and LEP) were reviewed...

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...

Erythema induratum of Bazin (EIB) is a predominantly lobular panniculitis with or without vasculitis due to Mycobacterium tuberculosis (MTB) infection. Atrophic scars may remain after EIB was healed. Rare cases of EIB showing sharp skin depression may mimic deep morphea and lupus profundus. A rare case of EIB was reported in a 35-year-old female with slightly tender erythematous-violaceous nodules and hyperpigmented depressed scars on both lower legs, resulting in asymmetrical lower leg shape. Histopathological examination showed lobular panniculitis without vasculitis and granulomatous inflammation consisted of caseous necrosis, epithelioid cells, and multinucleated giant cells...

Cutaneous lupus erythematosus (CLE) is an inflammatory, autoimmune disease encompassing a broad spectrum of subtypes including acute, subacute, chronic and intermittent CLE. Among these, chronic CLE can be further classified into several subclasses of lupus erythematosus (LE) such as discoid LE, verrucous LE, LE profundus, chilblain LE and Blaschko linear LE. To provide all dermatologists and rheumatologists with a practical guideline for the diagnosis, treatment and long-term management of CLE, this evidence- and consensus-based guideline was developed following the checklist established by the international Reporting Items for Practice Guidelines in Healthcare (RIGHT) Working Group and was registered at the International Practice Guideline Registry Platform...

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic cutaneous lymphoma. Differential diagnosis with lupus erythematosus panniculitis (LEP) can be challenging and overlapping cases have been described. In this study, we investigate whether gene expression profiling may or not identify markers that can be used to improve our understanding of the disease and to make a precise differential diagnosis. SPTCL, LEP, and overlapping cases were analyzed using a customized NanoString platform including 208 genes related to T-cell differentiation, stromal signatures, oncogenes, and tumor suppressor genes...