lupus profundus

BACKGROUND: Age at disease onset may modulate systemic lupus erythematosus (SLE), but its relation to cutaneous/extracutaneous manifestation remains understudied. OBJECTIVE: To compare the cutaneous, systemic features, laboratory characteristics, and disease severity between late- and adult-onset SLE patients. METHODS: Analyses of the cutaneous, systemic involvement, laboratory investigations, SLE disease activity index 2000 (SLEDAI-2K), and disease damage were performed to compare between groups...

Herein, the different skin manifestations in patients with lupus erythematosus are reviewed, and their diagnostic, pathogenic and prognostic relevance are discussed, as well as their impact on therapeutic choices. The so-called specific lesions of LE result from an autoimmune pathomechanism and they allow diagnosis of LE by simple clinicopathological correlation since the findings are characteristic. They include the classic acute, subacute and chronic variants, characterised microscopically by interface dermatitis; the dermal variants of lupus, such as tumid lupus, displaying dermal perivascular lymphocytic infiltrate with mucin deposition under the microscope, and lupus profundus, in which lymphocytic lobular panniculitis progressing to hyaline fibrosis is found...

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous T-cell lymphoma. There may be a significant histologic overlap with traumatic panniculitis and lupus profundus. We describe a 54-year-old woman who had received a diagnosis of SPTCL based upon a left parietal scalp biopsy 5 years earlier. This diagnosis was supported by immunohistochemistry (IHC) demonstrating a CD8+ predominant lymphocyte population in the subcutis. T-cell gene rearrangement studies were not performed at that time...

Introduction Discoid lupus erythematosus (DLE) is the most common form of cutaneous lupus erythematosus. It is a chronic, scar-forming, photosensitive autoimmune dermatosis presenting with erythematous and scaly lesions. Predisposed areas include sun-exposed areas like the nose, forehead, and cheeks, as well as the upper body and extremities. The histological findings are typical, with interface dermatitis. Immunoglobulin M (IgM) and immunoglobulin G (IgG) are the most common deposits in the dermoepidermal junction of the involved skin...

This case series characterizes the initial presentation and treatment of lupus erythematosus panniculitis/profundus and its association with systemic lupus erythematosus and discoid lupus erythematosus.

Lupus erythematosus profundus (LEP) is an unusual form of cutaneous lupus erythematosus (CLE) characterized by multiple subcutaneous induration and associated with considerable physical and psychological morbidity. The heterogeneity of CLE makes it difficult to understand its underlying pathogenesis and represents a therapeutic challenge. Recently, new insight into the pathogenesis of CLE has implicated various cytokines, opening doors to targeted biologic agents. We report a case of a 23-year-old female who presented with refractory LEP ulcers as an initial presentation of systemic lupus erythematosus...

Primary Cutaneous Gamma-Delta (γδ) T-Cell Lymphoma (PCGDTCL) is a rare primary cutaneous lymphoma of aggressive nature. Only a few cases with an initially indolent course over years have been published. PCGDTCL can mimic diseases with benign behavior in their clinical and histopathological presentation, such as lupus erythematosus profundus, but also other lymphomas, for example subcutaneous panniculitis-like T-cell lymphoma. In our patient, the results of histopathological, immunofluorescence microscopy, and clinical examinations of early lesions first led to the diagnosis of lupus erythematosus profundus...

OBJECTIVE: To explore the similarities and differences in clinical pathological features and gene rearrangement of lupus erythematosus profundus(LEP) and subcutaneous panniculitis-like T-cell lymphoma(SPTL). METHODS: We compared the clinical presentations, histopathology, immunophenotypical features and T-cell receptor (TCR) gene rearrangement findings of 9 cases of LEP and SPTL. RESULTS: For clinical features, most patients of LEP occurred on head and face without systemic symptoms...

Dermatofibroma (DF) is a benign skin tumor that is well-known among dermatologists. We herein present a rare case of atrophic dermatofibroma presenting linear skin dimpling. The patient was a 25-year-old woman with a history of wild-type recessive dystrophic epidermolysis bullosa who had noticed linear concavity on her right lateral back 1 year before her initial presentation. Anetoderma, atrophic scar, localized morphea, or lupus erythematosus profundus were clinically suspected; however, a biopsy specimen from the dimpling lesion showed the fibrous and histiocytic tumor in the deep dermis...

Dapsone (4, 4'-diaminodiphenylsulfone, DDS) is a potent anti-inflammatory and antibacterial compound which has been used in the treatment of leprosy, vasculitis and dermatitis herpetiformis, lupus erythematosus profundus and even as an antimalarial in combination with proguanil. This study investigated the effect of the administration of dapsone on the reproductive activities of male rats using in vivo and in vitro techniques. In the in vivo study, dapsone was administered orally to male Wistar rats for 5 days or 6 weeks after which their body weight, relative reproductive organ weights, sperm parameters and reproductive hormones were determined while testicular and epididymal histology were also assessed...

Lupus panniculitis (LP), also referred to as lupus erythematosus profundus (LEP), is a chronic recurrent inflammation condition of the subcutaneous fat. It occurs in 1 to 3% of patients with systemic lupus erythematosus (SLE) and in 10% of patients with discoid lupus erythematosus (DLE), but can also occur as an entity of its own. Patients with lupus panniculitis usually present with persistent, often tender and painful skin lesions, or subcutaneous nodules, that range from 1 to 5 cm in diameter. The overlying skin may appear erythematous; lesions may become ulcerated, and heal with atrophy, skin depression, dimpling and scaring...

Pruritus is an important symptom frequently accompanying various inflammatory skin conditions. Some recent data have indicated that it may also be associated with autoimmune connective tissue diseases, including systemic sclerosis and dermatomyositis; however, studies on the prevalence and clinical characteristics of pruritus in CLE are limited. We have performed a multinational, prospective, cross-sectional study in order to assess the prevalence and intensity of pruritus in adult patients suffering from various subtypes of CLE...

Lupus panniculitis of the scalp (LPS) is a rare and distinctive clinical feature of lupus erythematosus panniculitis (LEP) with linear alopecia along Blaschko's lines. In this study, we investigated clinical features and treatments of LPS by literature review of articles in the English language from PubMed and SCOPUS databases up to April 2018. The following key words, "lupus panniculitis, "lupus erythematosus panniculitis", "lupus profundus", "head", and "scalp", were used. Twenty cases of LPS were identified (mean age = 26...

Dear Editor, Lupus panniculitis or lupus profundus is a rare inflammatory complication found in patients with systemic lupus erythematosus (SLE), or discoid lupus erythematosus (DLE) (1). When the breast is involved, the term lupus mastitis (LM) is used. This disease involving the breast is rare, and the lesions may precede, coincide with, or occur later than the onset of other lupus lesions. Tissue biopsy is required to confirm the suspected diagnoses of LM. We report a case of a patient with lupus mastitis due to the important differential diagnosis...

Lupus erythematosus (LE) is termed as an autoimmune chronic condition which involves a spectrum of symptoms. It is a part of the connective tissue diseases. Its cutaneous form is termed as cutaneous lupus erythematosus (CLE). Prevalence of CLE is about 70 cases per 100,000 persons. The least common variety of CLE is lupus profundus (LP)-only 5% of cases. Lupus profundus, although rare, must be kept in the differential diagnoses of ulcerated lesions. It may present as a localized entity or in association with systemic lupus erythematosus (SLE) or it may lead to SLE later in life...

While the majority of panniculitides are benign in nature, there are rare instances when panniculitis presents as the initial sign of a complex disease state. We describe a case of panniculitis initially diagnosed as lupus profundus that was challenged when features of haemophagocytic lymphohistiocytosis became apparent. We illustrate how some key clinical features and newer investigations can help differentiate between benign and malignant panniculitis.

Cutaneous and systemic lupus erythematosus (SLE) commonly involves the hair and scalp. Alopecia can result from direct activity of disease on the scalp or from the state of physical stress in the form of telogen effluvium. Discoid lupus erythematosus and lupus panniculitis/profundus are known to cause scarring alopecia, while accumulation of recent studies has shown that non-scarring alopecia in SLE may have different subtypes, comprising lupus erythematosus-specific and lupus erythematosus-nonspecific changes on histology...

Lupus erythematosus profundus (LEP) is a variant of lupus erythematosus, involving the deep dermis and subcutaneous fat. LEP is characterized by the presence of lymphoid follicles (LF) and germinal centers (GC). However, it remains unknown whether these lymphoid structures correspond to the lymphoid tissues such as cutaneous tertiary lymphoid organs (TLO). Previously, we identified dynamically orchestrated cellular elements in murine contact dermatitis that resembled lymphoid structures, which we termed inducible skin-associated lymphoid tissues (iSALT)...

No abstract text is available yet for this article.

Lupus alopecia is usually difficult to treat. We report a case of a 40-year-old woman with Sjögren's syndrome and atopic dermatitis who presented with discoid lupus erythematosus on the forearms and lupus erythematosus profundus with alopecia involving the scalp. A biopsy specimen taken from the discoid lupus erythematosus lesion on the forearm further exhibited a xanthomatous reaction, which however was not detected in another specimen from the lupus erythematosus profundus on the scalp. Treatment with oral hydroxychloroquine showed dramatic effects and complete hair regrowth was obtained 3 months later...