Lupus Tumidus Research Articles

Cutaneous lupus subtypes

A broad spectrum of dermatologic manifestations, known as cutaneous lupus erythematosus (CLE), may or may not be linked to the development of systemic disease. There are numerous subtypes of cutaneous lupus, such as acute cutaneous lupus (ACLE), subacute cutaneous lupus (SCLE), and chronic cutaneous lupus (CCLE). CCLE encompasses lupus tumidus, chilblain cutaneous lupus, LE profundus (LEP), and discoid lupus erythematosus (DLE). In order to diagnose these diseases, it is necessary to accurately classify the subtype. This is achieved through a combination of physical examination, laboratory studies, histology, antibody serology, and occasionally direct immunofluorescence, while also excluding systemic disease. The treatment of cutaneous lupus involves the provision of appropriate topical and systemic agents, as well as patient education regarding solar protection. In cases where the disease is pervasive, scarring, or treatment-refractory, systemic agents are recommended. In this chapter, we address the classification and diagnosis of the diverse subtypes of CLE and offer a comprehensive update on therapeutic management.

Large-scale mutational analysis identifies UNC93B1 variants that drive TLR-mediated autoimmunity in mice and humans.

Nucleic acid-sensing Toll-like receptors (TLR) 3, 7/8, and 9 are key innate immune sensors whose activities must be tightly regulated to prevent systemic autoimmune or autoinflammatory disease or virus-associated immunopathology. Here, we report a systematic scanning-alanine mutagenesis screen of all cytosolic and luminal residues of the TLR chaperone protein UNC93B1, which identified both negative and positive regulatory regions affecting TLR3, TLR7, and TLR9 responses. We subsequently identified two families harboring heterozygous coding mutations in UNC93B1, UNC93B1+/T93I and UNC93B1+/R336C, both in key negative regulatory regions identified in our screen. These patients presented with cutaneous tumid lupus and juvenile idiopathic arthritis plus neuroinflammatory disease, respectively. Disruption of UNC93B1-mediated regulation by these mutations led to enhanced TLR7/8 responses, and both variants resulted in systemic autoimmune or inflammatory disease when introduced into mice via genome editing. Altogether, our results implicate the UNC93B1-TLR7/8 axis in human monogenic autoimmune diseases and provide a functional resource to assess the impact of yet-to-be-reported UNC93B1 mutations.

Tumid Lupus Erythematosus (TLE): A Review of a Rare Variant of Chronic Cutaneous Lupus Erythematosus (cCLE) with Emphasis on Differential Diagnosis.

Tumid lupus erythematosus (TLE) has been the subject of heated debate regarding its correct nosographic classification. The definition of TLE has changed over time, varying according to the different studies performed. In this review, we address the initial definition of TLE, the changes that have taken place in the understanding of TLE, and its placement within the classification of cutaneous lupus erythematosus (CLE), with a focus on clinical, histopathological, immunophenotypical, and differential diagnosis aspects.

Tumid Lupus Case Report

ABSTRACT Cutaneous lupus erythematosus (CLE) can present with a wide variety of dermatologic manifestations and is broken down into acute, subacute, and chronic subtypes. Tumid lupus, a variant of CLE, is rarely associated with systemic lupus erythematosus. Lesions of CLE and tumid lupus can mimic multiple other dermatologic conditions. We present a case of a patient with tumid lupus who was initially treated for erythema migrans and systemic symptoms suspected to represent Lyme disease. This article is intended for nurse practitioners, nurses, and other clinicians practicing in dermatology, primary care, urgent care, and emergency medicine settings in the care of patients presenting with annular skin lesions.

An unusual presentation of lupus tumidus mimicking cutaneous lymphoma

No abstract available

Hydroxychloroquine-induced generalized myopathy in a patient with lupus tumidus: a case report.

A subtype of cutaneous lupus erythematosus known as lupus erythematosus tumidus (LET) is characterized by sun-exposed areas that typically display urticaria-like papules and plaques. For LET, systemic therapy with antimalarials - particularly hydroxychloroquine (HCQ) - is the first line of treatment. Even though the safety profile of these medications appears to be high, there have been very few reports of side effects in the literature, including hemolytic anemia, retinal toxicity, maculopapular rash, gastrointestinal disturbance, and blue-gray discoloration of the skin or mucous membranes. Here, we report a unique instance of a 46-year-old LET smoker who, following HCQ treatment, developed a generalized myopathy.

IL-1β and IL-17 in cutaneous lupus erythematous skin biopsies: could immunohistochemicals indicate a tendency towards systemic involvement?

BackgroundOnly a fraction of patients with cutaneous lupus erythematosus (CLE) will eventually progress toward systemic disease (SLE). ObjectiveTo find inflammatory biomarkers which could predict the progression of cutaneous lupus erythematosus (CLE) into systemic lupus erythematosus (SLE) using immunohistochemical (IHC) assays. MethodsImmunohistochemical markers for cytotoxic, inflammatory, and anti-inflammatory responses and morphometric methods were applied to routine paraffin sections of skin biopsies, taken from lesions of 59 patients with discoid lupus, subacute lupus, and lupus tumidus. For the diagnosis of SLE, patients were classified by both the American College of Rheumatology (ACR-82) and the Systemic Lupus International Collaborating Clinics (SLICC-12) systems. ResultsSkin samples from CLE/SLE+patients presented higher expression of IL-1β (ARC-82: p=0.024; SLICC-12: p=0.0143) and a significantly higher number of cells marked with granzyme B and perforin (ARC: p=0.0097; SLICC-12: p=0.0148). Biopsies from CLE/SLE- individuals had higher expression of IL-17 (ARC-82: p=0.0003; SLICC-12: p=0.0351) and presented a positive correlation between the density of granzyme A+and FoxP3+ cells (ARC-82: p=0.0257; SLICC-12: p=0.0285) and CD8+ cells (ARC-82: p=0.0075; SLICC-12: p=0.0102), as well as between granulysin-positive and CD8+ cells (ARC-82: p=0.0024; SLICC-12: p=0.0116). Study limitationsPatients were evaluated at a specific point in their evolution and according to the presence or not of systemic disease. The authors cannot predict how many more, from each group, would have evolved towards SLE in the following years. ConclusionsIn this cohort, immunohistochemical findings suggested that patients with a tendency to systemic disease will show strong reactivity for IL-1β, while those with purely cutaneous involvement will tend to express IL-17 more intensely.

Autologous Fat Transfer in Lupus Panniculitis Facial Lipoatrophy.

Lupus panniculitis (LP) often presents with tender nodules and intermittent ulcers that then heal with scarring and lipoatrophy. The current mainstay of treatment is medical treatment. Research regarding the treatment of lipoatrophy from LP with autologous fat grafting is limited. We would like to share our experience in this rare case, which was treated with autologous fat transfer. A 48-year-old female presented with erythematous plaque, tender nodules, and ulcers following by a depression of the lesion at the left temporal area. The patient also had indurated erythematous plaque at her left cheek. Both lesions were aggravated by sunlight exposure. After several investigations, she was diagnosed as LP with secondary lipoatrophy and tumid lupus erythematosus at her left temporal and left cheek, respectively. She received antimalarial drug and topical steroids. The patient underwent two sessions of autologous fat transfer. She was satisfied with the volume and contour improvement in the scar following the injection of 8 and 3.7 mL of fat. Furthermore, the patient reported the remission of tender nodules and ulcers since the first fat graft injection. In conclusion, the autologous fat transfer is a simple and effective treatment for lipoatrophy and scar secondary to LP with promising results.

Histology of Skin Alterations in Lupus Erythematosus

Abstract Lupus erythematosus is an autoimmune connective tissue disorder showing a broad spectrum of clinical manifestations. The aim of this study was to assess the correlation of skin histology and different types of lupus erythematosus. Materials and methods: Fifty-one skin specimens were assessed from 39 female and 12 male patients with acute, subcutaneous and chronic lupus erythematosus, diagnosed and treated in the Department of Dermatology and Venereology, Alexandrovska University Hospital for a 4-year period. Results: Follicular hyperkeratosis, epidermal atrophy, vacuolar degeneration and interface dermatitis were the most frequently observed lesions in chronic cutaneous lupus erythematosus while diffuse hyperkeratosis, epidermal atrophy and indistinct interface dermatitis in the dermis were predominant in subacute cutaneous lupus erythematosus. Lupus tumidus, a rare intermittent variant of cutaneous lupus erythematosus, showed almost no epidermal involvement and mucin deposition in the dermis. However, in one of our lupus tumidus patients the disease progressed to a systemic form with histological changes of acute cutaneous lupus erythematosus including atrophy, dermal-epidermal smoothing and lymphocytic infiltration in the dermis. Of note, a few patients showed histological changes of urticarial vasculitis-like and rheumatic-like patterns. Conclusion: The correlation of clinical course, histopathological findings and immunological tests are of vital importance for the correct diagnosis and follow up of patients with lupus erythematodes, thus preventing complications and improving their quality of life.

Uncommon non-infectious annular dermatoses

Several cutaneous diseases can present with annular lesions, making a distinction by physical appearance alone challenging. They can be distinguished into infectious and non-infectious, and common and uncommon annular dermatoses. Common non-infectious diseases include granuloma annulare, urticaria, and subacute lupus erythematosus. In addition, there are rare non-infectious non-neoplastic annular dermatoses whose nosographic attribution is established, including annually recurring erythema annulare centrifugum (EAC) and annular erythema in Sjögren syndrome and others whose nosographic positioning is still debated. They are neutrophilic figurate erythema, palpable migratory arciform erythema, eosinophilic annular erythema, and annular lichenoid dermatitis of youth. Their etiopathogenesis is largely unknown, although immune-mediated mechanisms are likely involved. It is difficult to establish if they are variants of reaction patterns or separate clinic-pathological entities. In fact, EAC and annually recurring EAC may represent different aspects of the same disease. Palpable migratory arciform erythema is hardly distinguishable from EAC deep type, Jessner-Kanof disease, and lupus tumidus. Neutrophilic figurate erythema and eosinophilic figurate erythema are clinically very similar and differing only in the relative proportion of eosinophils and neutrophils.

Diagnosis of Lyme Borreliosis With a Novel, Seminested Real-Time Polymerase Chain Reaction Targeting the 5S-23S Intergenic Spacer Region: Clinical Features, Histopathology, and Immunophenotype in 44 Patients.

Lyme borreliosis (LB) is the most common tick-borne infection in Europe and North America. Polymerase chain reaction (PCR) is an important tool to confirm the diagnosis, but not always successful, especially when organisms are sparse. We developed a novel, seminested real-time PCR assay [target: 5S-23S intergenic spacer region (IGS)] and compared it with 3 well-established conventional PCR assays (IGS/OspA/real-time IGS) on 596 formalin-fixed, paraffin-embedded routine skin biopsies. The seminested real-time assay identified 46 cases of borreliosis while 25, 27, and 38 were identified by the 3 other assays, respectively (P 0.01, P 0.02, and P 0.42; significance P < 0.05). Clinicopathologic and immunophenotypic analysis of PCR-positive cases revealed 38 erythema migrans (EM), 6 Borrelia lymphocytomas, and 2 acrodermatitis chronica atrophicans (ACA). In the 44 PCR-confirmed cases, plasma cells were present in only a third of EM cases. By contrast, CD123-positive plasmacytoid dendritic cells were common (74%) and therefore are unlikely to be helpful in the differential diagnosis between EM and tumid lupus erythematosus. A loss of CD34 in a third of all LB specimens limits its diagnostic value in the differential diagnosis with morphea. Interstitial macrophages were common in cutaneous LB (42/43) forming interstitial granulomas in a third of all cases, and 3/38 EM, 3/6 Borrelia lymphocytomas, and 1/2 ACA were only identified by the new seminested real-time assay, suggesting that it is especially helpful in confirming the diagnosis of Borrelia lymphocytoma.

British Association of Dermatologists guidelines for the management of people with cutaneous lupus erythematosus 2021.

The overall objective of the guideline is to provide up-to-date, evidence-based recommendations for the management of cutaneous lupus erythematosus (CLE) in the presence or absence of systemic lupus erythematosus (SLE) in adults, young people and children. The document aims to: offer an appraisal of all relevant literature up to December 2020, focusing on any key developments address important, practical clinical questions relating to the primary guideline objective. provide guideline recommendations and if appropriate research recommendations.

A rare case of tumid lupus erythematosis in a patient with fabrys disease – case report

A rare case of tumid lupus erythematosis in a patient with fabrys disease – case report

Cutaneous lupus tumidus: An unusual unilateral presentation

Lupus tumidus is a rare subtype of chronic cutaneous lupus erythematosus characterized by erythema and bright urticarial erythematous and violaceous lesions on sun-exposed areas that heal without leaving scars. Lupus tumidus follows a benign and intermittent clinical course and is rarely associated with systemic lupus erythematosus. Treatment involves photoprotection, topical corticosteroids, and antimalarials. We report the case of a 42-year-old patient with an atypical unilateral form of lupus tumidus successfully treated with the administration of hydroxychloroquine in combination with photoprotection and tacrolimus.

A Rare Case of Tumid Lupus Erythematosus with Unilateral Linear Distribution in A Young Child

Tumid lupus erythematosus (TLE) as a rare variant of cutaneous lupus erythematosus (CLE) is characterized by edematous, urticarial-like annular papules and plaques. TLE has similar histopathologic findings to CLE such as periadnexal lymphocytic infiltration and interstitial mucin deposition. Although TLE develops on sun-exposed areas at any age, it is rarely distributed along the Blaschko lines and develops in infancy and childhood. Unlike CLE, skin lesion of TLE heals without leaving scarring or dyspigmentation. Here, we report a rare case of unilateral linear TLE in a 4-year-old girl, which was improved by intralesional corticosteroid injection and oral antimalarial drug with leaving postinflammatory hyperpigmentation.

Tumid Lupus Erythematosus: A Rare and Distinctive Variant of Cutaneous Lupus Erythematosus Masquerading as Urticarial Vasculitis

Tumid lupus erythematosus (TLE) is a rare form of chronic cutaneous lupus that has triggered much debate regarding its clinical and histopathological features. It has been classically defined as annular erythematous, succulent, plaques involving the face and trunk that typically are devoid of any papulosquamous features such as scale and follicular plugging. These lesions are a clinical mimicker of other urticarial lesions such as urticarial vasculitis and lymphocytic infiltrate of Jessner. We report a case of TLE presenting in a 49-year-old Caucasian female whose initial clinical presentation was concerning for urticarial vasculitis due to presence of urticarial-like lesions present for approximately three months. Laboratory studies and histopathological correlations confirmed the diagnosis of TLE and the patient was successfully treated with topical corticosteroids.

A rare case of tumid lupus erythematosus with unilateral linear distribution in a young child

Tumid lupus erythematosus (TLE) as a rare variant of cutaneous lupus erythematosus (CLE) is characterized by edematous, urticarial-like annular papules and plaques. TLE has similar histopathologic findings to CLE such as periadnexal lymphocytic infiltration and interstitial mucin deposition. Although TLE develops on sun-exposed areas at any age, it is rarely distributed along the Blaschko lines and develops in infancy and childhood. Unlike CLE, skin lesion of TLE heals without leaving scarring or dyspigmentation. Here, we report a rare case of unilateral linear TLE in a 4-year-old girl, which was improved by intralesional corticosteroid injection and oral antimalarial drug with leaving postinflammatory hyperpigmentation.

Lupus tumidus: A series of eight cases from a third-level hospital in Mexico City

Lupus tumidus: A series of eight cases from a third-level hospital in Mexico City

The Histopathological Spectrum of Pseudolymphomatous Infiltrates in Cutaneous Lupus Erythematosus.

The occurrence of pseudolymphomatous infiltrates in cutaneous lupus erythematosus (cLE) is described mainly in lupus panniculitis and lupus tumidus/lymphocytic infiltration of the skin (Jessner-Kanof). We collected 15 cases of pseudolymphomatous cLE other than lupus panniculitis and lupus tumidus (M:F = 4:11; age range: 23-79 years; mean age: 50.9 years; median age: 57 years). Of the 15 cases, 9 (60%) were characterized by dense nodular infiltrates. Three cases (20%) showed an angiocentric pattern with cytological atypia of lymphoid cells; 2 cases (13.3%) showed a band-like infiltrate mimicking mycosis fungoides, and 1 case had mixed features of the band-like and angiocentric patterns. Clues to the histopathological diagnosis of cLE were presence of interface dermatitis, clusters of plasmacytoid dendritic cells, and dermal mucin deposition. Our study shows that the spectrum of pseudolymphomatous presentations of cLE is broader than previously described, including band-like cases that may be misconstrued as mycosis fungoides, and angiocentric cases that may be misinterpreted as an aggressive lymphoma. Recognition of such cases is possible only on careful clinicopathologic correlation and requires a high level of histopathological suspicion to allow a correct diagnosis and the proper management of the patients.

Cutaneous lupus erythematosus: clinico-pathologic correlation.

Cutaneous lupus erythematosus (CLE) is a chronic-relapsing disease. It is defined as a LE localized to the skin without any significant systemic symptoms. Its annual incidence is of 4 cases per 100,000 persons with a prevalence of 73 cases per 100,000 persons. The etiology is unknown but it is considered as a prototype of autoimmune disease in which genetic factors (HLA), environmental factors (photo exposure and cigarette smoking) and pharmacological agents play an important role. The most accepted classification includes three clinical variants: acute (ACLE), subacute (SCLE) and chronic (CCLE). A fourth variety is the intermittent form (ICLE) also called "lupus tumidus" (LET) which is considered by some authors a distinct form from CCLE. The skin lesions are subdivided into LE specific and LE non-specific. The latter have a considerable importance as a symptom of evolution of the disease towards a systemic form of lupus (SLE). The histopathology of CLE is characterized by an interface dermatitis with vacuolization of the basal layer, a predominantly lymphocytic, perivascular and periadnexal infiltrate, epidermal and follicular hyperkeratosis, deposit of positive PAS material at the dermo-epidermal junction leading to atrophic-cicatricial evolution. Depending on the clinical variants, these microscopic features are more or less evident and are associated with peculiarities such as deposits of mucin (SCLE and LET), involvement of the panniculus in LE panniculitis, disappearance of the adnexa (cicatricial alopecia). The relationship between SLE/CLE is still under study: the progression of CLE in SLE is reported in a variable percentage of cases ranging from 12 to 18%. CLE therapy is aimed at preventing recurrences and scarring outcomes. Photoprotection with clothing, chemical and physical sunscreens active on UVA and UVB radiations is very important. Topical therapy is based on the use of steroids and calcineurin inhibitors, while the systemic therapy includes hydroxychloroquine as the first drug of choice.