lupus profundus

Lupus erythematosus profundus (LEP) is a rare form of chronic cutaneous lupus erythematosus. We report on a case of a 56-year-old Caucasian woman who presented with a single, persistent, painful rash on the left hip and lateral aspect of the left upper thigh, which had been present for 2.5 years. The patient had a history of previous injury to this area before the rash started. Clinical findings showed an inflamed, hyperpigmented, and indurated plaque with a linear skin invagination and no associated systemic symptoms...

Lupus erythematosus panniculitis, also known as lupus profundus, is a variant in the clinicopathological spectrum of lupus erythematosus (LE) affecting about 2%-3% of LE patients. A linear configuration of LE panniculitis has been reported rarely with rare reports describing the coexistence of different forms of cutaneous LE and localized morphea. In this study, the authors present a 9-year-old girl with linear arrangement of subcutaneous nodules on her left forearm. Microscopic findings from 2 biopsies included lymphocytes at the dermoepidermal junction with mild interface dermatitis, a dense lymphocytic infiltrate that was concentrated around adnexae and subcutaneous fat in concert with thickened collagen bundles and mild widening of fibrous septae surrounding fat lobules...

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Alopecia areata (AA) over scalp is known to present in various shapes and extents of hair loss. Typically it presents as circumscribed patches of alopecia with underlying skin remaining normal. We describe a rare variant of AA presenting in linear band-like form. Only four cases of linear alopecia have been reported in medical literature till today, all four being diagnosed as lupus erythematosus profundus.

INTRODUCTION: Spontaneous flexor tendon rupture is usually caused by trauma, systemic diseases, or carpal bone and joint disorders. Here we report a case of spontaneous flexor tendon rupture occurring in a systemic lupus erythematosus (SLE) patient following nonunion of the hamate hook after an insufficiency fracture, and which was also associated with tendon degeneration caused by SLE. CASE PRESENTATION: A 57-year-old woman was diagnosed with SLE 22 years ago and being treated with oral prednisolone...

Lupus mastitis is an uncommon presentation of lupus erythematosus profundus (LE profundus), characterized by inflammation of the subcutaneous fat. LE profundus occurring as initial manifestation of LE is rare. We report a case where the patient presented with lupus mastitis and years later, she developed disseminated discoid LE (DLE) and polyarteritis nodosa (PAN). PAN and DLE are connective tissue diseases with different etiologies, clinical, immunological, and histopathological features. One connective tissue disease evolving into another is rare, and the reasons remain unexplained...

The diagnosis of panniculitis is a relatively rare occurrence for many practising pathologists. The smaller subset of lymphocyte-predominant panniculitis is further complicated by the diagnostic consideration of T cell lymphoma involving the subcutaneous tissue, mimicking inflammatory causes of panniculitis. Accurate classification of the panniculitis is crucial to direct clinical management as treatment options may vary from non-medical therapy to immunosuppressive agents to aggressive chemotherapy. Many diseases show significant overlap in clinical and histological features, making the process of determining a specific diagnosis very challenging...

A lot of diseases, including lupus profundus, morphea, lipodystrophy, and Parry-Romberg syndrome, may manifest progressive hemifacial atrophy. These diseases usually progress slowly and rapid progression of atrophy is extremely rare. We report a case of elderly-onset rapid progression of hemifacial atrophy only in three weeks. Our case did not meet variable differential diagnoses. We discuss the clinical character of the patient against the past of literature and suppose it may be a new clinical entity.

INTRODUCTION: Lupus erythematosus is a multisystemic disease that is characterized by autoantibody production and immune complex deposition in such tissues as the mucosa, joints, the central nervous system, and skin. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Chronic cutaneous lupus erythematosus comprises discoid lupus erythematosus (DLE) and lupus profundus (LP). AIM: To analyze the expression of proapoptotic molecules in patients with lupus erythematosus discoid and lupus profundus...

Ocular complications associated with cutaneous lupus erythematosus (CLE) are less studied compared with those ones associated with systemic lupus erythematosus (SLE). The main ocular sites involved in patients affected by discoid lupus erythematosus (DLE) are eyelids followed by orbit and periorbit, the least being cornea. The most common complications are blepharitis usually affecting the lower lid and associated with some type of lid lesion such as plaque or erythematosus patches and madarosis. Few cases with LE profundus (LEP) and ocular complications are reported, but they are associated with orbital inflammatory syndrome and severe complications...

Facial lipoatrophy refers to the loss of subcutaneous fat tissue presenting by flattening or indentation of convex contour of the face. Facial lipoatrophy is a feature of the normal ageing process. It may be also a manifestation of chronic diseases, most frequently it affects HIV-infected individuals treated with highly active antiretroviral therapy (HAART) and may constitute a complication of connective tissue diseases, like lupus erythematosus profundus or morphea. Early recognition and treatment of the active stage of connective tissue diseases is of essential significance in prevention of subsequent scarring and atrophy lesions...

Lupus erythematosus profundus (LEP), which is a variant of chronic cutaneous lupus erythematosus (CLE), is seen in approximately 2∼3% of CLE patients, and only 10% to 20% of LEP patients present with systemic LE (SLE). LEP shows subcutaneous nodules with or without discoid LE (DLE). Linear LEP, a very rare variant of LEP, was first reported in 1991 in Japanese and in 1998 in English. Since LEP sometimes leaves skin depressions or scars as a result of atrophy of adipose tissue, early and adequate treatments are necessary...

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is characterized by panniculitic infiltrates that may be difficult to distinguish from inflammatory disorders, particularly lupus erythematosus profundus (LEP). We report on 11 patients (M:F=5:6; median age: 49 y; range: 20 to 75 y) presenting with lobular panniculitic infiltrates showing histopathologic features of both SPTCL and LEP in different parts of the same biopsy specimen. The areas showing aspects of SPTCL revealed dense infiltrates of small and medium-sized, atypical α/β T-cytotoxic lymphocytes with focal rimming of the adipocytes and high proliferation...

No abstract text is available yet for this article.

Lupus erythematosus profundus is a rare inflammatory disorder of subcutaneous fat in patients with lupus ery-thematosus. Previous reports suggested that plasmacytoid dendritic cells, which expressed CD123 and CD303 antigens, play a central proinflammatory role in the patho-genesis of lupus erythematosus. To find the factors that determine the response to treatment, we analysed 23 skin specimens from the patients with lupus erythematosus profundus. The patients with considerable lymphocytic inflammation with high percentages of CD123+ cells in dermis and subcutaneous fat significantly responded to the systemic corticosteroid therapies...

Cutaneous lupus erythematosus (CLE) encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several sub-types, including acute CLE (ACLE), sub-acute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain cutaneous lupus and lupus tumidus. The diagnosis of these diseases requires proper classification of the sub-type, through a combination of physical examination, laboratory studies, histology, antibody serology and occasionally direct immunofluorescence, while ensuring to exclude systemic disease...

Lupus erythematosus (LE) can cause various cutaneous lesions including panniculitis (LE profundus), but salivary gland involvement has been extremely rare in patients with LE. Herein, we report the first documented case of systemic LE with prominent mucoid degeneration and lymphoplasmacytic infiltration in the parotid gland. A 38-year-old Japanese male with histories of autoimmune hemolytic anemia and systemic LE presented with a swelling of the bilateral cervical region. A physical examination revealed a swelling of the bilateral parotid gland and erythema of the right cheek...

Lupus erythematosus panniculitis (LEP) or lupus profundus (LP) is a clinical variant of lupus eryhematosus that involves the deep dermis and the subcutaneous fat and is associated with tender subcutaneous nodules or plaques, with occasional ulceration, atrophy, and scarring. The management of this entity can be difficult because of the lack of response to conventional treatments, such as systemic steroids and antimalarials. The two patients of this study presented LP refractory to several therapies that demonstrated a remarkable improve to the infusion of the anti-CD20 monoclonal antibody, rituximab at a dosage of 375 mg/m²/week...

No abstract text is available yet for this article.

Lupus profundus is a rare manifestation of cutaneous lupus erythematosus, seen in 1% to 3% of patients. It most commonly presents in association with classic chronic cutaneous lesions of discoid lupus erythematosus; however, such lesions, as well as a clinical history of lupus erythematosus, may be lacking. The differential diagnosis for lymphocytic lobular panniculitides is broad; however, the consideration of subcutaneous panniculitis-like T-cell lymphoma is most critical. Recently, there have been several reports emphasizing the overlapping histomorphologic features between lupus profundus and subcutaneous panniculitis-like T-cell lymphoma...