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This study aimed to investigate the relationship between centrifugal lipodystrophy (CLD) and lupus erythematosus panniculitis (LEP), and the efficacy and safety of hydroxychloroquine (HCQ) for treating CLD in children. A total of 29 cases clinically diagnosed as CLD (n = 24) and CLD/LEP overlap (n = 5) were enrolled and all were confirmed by skin biopsies of CLD and LEP. The clinicopathological findings, clinical outcomes, and prognosis with the treatment of HCQ between CLD and LEP were compared. All 29 cases (male: female = 1:1.6; median age at onset: 3 years) had cutaneous lesions of centrifugally expanding lipoatrophy, of which five cases overlapped with LEP lesions presented as erythematous indurated plaque (n = 2), subcutaneous nodules (n = 2) and alopecia along Blaschko's lines (n = 1). Antinuclear antibodies were found in six (25.0%) CLD and two (40.0%) overlapped patients (p = 0.597). Histopathologically, of the 24 cases of CLD, 14 (58.5%) exhibited subcutis loss or mild lobular inflammation. Ten (41.7%) cases displayed lobular panniculitis with moderate to dense lymphohistiocytic infiltrate and plasma cells, similar to the five cases of overlap. Small clusters of CD123 positive plasmacytoid dendritic cells were found in 62.5% (5/8) of CLD and 66.7% (2/3) of overlap cases (p 0.99). HCQ (5 mg/kg/d) treatment showed improvement in 91.3% (21/23) of CLD and all overlap cases, including four cases unresponsive to previous oral glucocorticosteroid treatment. Our findings suggested that CLD and LEP represent a spectrum within the same disease. HCQ (5 mg/kg/d) was effective and safe for treating CLD (age 1.5 years), and early treatment and a regular long-term follow-up are essential.

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. Gene expression unsupervised analysis of the samples differentiated SPTCL from LEP samples. Most overlapping cases were clustered with LEP cases. Differentially expressed genes were observed when comparing SPTCL with LEP cases; and overlapping with LEP cases. Gene set enrichment analysis recognized gene sets defining each group. In conclusion, SPTCL and LEP have distinctive molecular profiles and the molecular background of overlapping cases more closely resembles LEP.

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.

Lupus mastitis is a rare complication of systemic or discoid lupus erythematosus with an uncommon initial presentation when limited to the breast. In this article, we report a 42-year-old woman who presented with constant pain and tenderness in her breasts. Ultrasound imaging of the left breast revealed a 14-mm oval mass, suspicious for malignancy; a needle core biopsy was performed. Sections showed necrosis of the fat lobules with associated mixed nodular lymphoplasmacytic aggregates. Karyorrhectic debris, fibrinoid necrosis of small vessels, and microcalcifications were all present while the background breast parenchyma was unremarkable. The diagnosis of lupus mastitis was rendered. Subsequent serology showed negative dsDNA but positive antinuclear antibodies, C4, and anti -Sjogren';s-syndrome-related antigen A antibodies. Clinical features of active systemic disease were not identified at the patient's follow-up dermatology appointment 1 month after the biopsy, and she elected management for her nodules with steroids. To the best of our knowledge, only 40 other cases of lupus mastitis have been reported in the English literature, of which 25 presented as a mass and only in 6 lupus mastitis of the breast was the initial presentation. In conclusion, we bring much needed awareness to lupus mastitis as the first presentation of disease.

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. The lesions were resistant to multiple conventional therapies and remarkably responded to tocilizumab.

BACKGROUND The aim of this study was to identify feature autophagy-related genes (ARGs) in systemic lupus erythematosus (SLE), evaluate their diagnostic value, and further explore DNA methylation and expression levels in the pathogenesis of SLE. MATERIAL AND METHODS WGCNA was used to construct network and selected hub genes based on gene expression dataset GSE81622. ARGS were overlapped with hub genes, and feature ARGs were identified. A diagnostic model was established by these feature ARGs using LASSO. GSE96879 was used to analyze the methylation levels of feature ARGs. The expression and methylation levels of feature ARGs were verified using RT-PCR and methylation-specific PCR. RESULTS We found that 55 hub genes were highly connected to the red module of WGCNA, and ARGs were extracted from the Human Autophagy Database and the GO_AUTOPHAGY gene set. Overlapping of 55 hub gene with ARGs resulted in 18 feature ARGs. S100A8, MyD88, and NCR3 from the 18 feature ARGs showed higher good diagnostic value for SLE. Five differentially methylated positions locating to S100A8, MyD88, and NCR3 genes were identified from GSE96879. After validation tests, RT-PCR showed that gene expressions of MyD88 and S100A8 were increased in the PBMCs samples of SLE patients compared with healthy controls, whereas NCR3 was the opposite. MSP found that cg24898863 (S100A8) was hypomethylated, while cg27490128 (NCR3) was hypermethylated in the SLE group, and S100A8 and NCR3 methylation were positively correlated with their expressions. CONCLUSIONS Our present study identified the potential roles of feature ARGs in SLE diagnosis, and shows correlation among DNA methylation and gene expressions of these feature ARGs in SLE.

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.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. She has had repeated admissions due to soft tissue infection and persistent pleural effusions. She experienced eruption of numerous, nonpruritic, erythematous maculopapular rashes on her body. Initial laboratory workup showed persistent cytopenia (anemia and leukopenia),10x elevated ferritin and LDH, and elevated transaminases and procalcitonin. Other work-ups, such as bone marrow studies, pleural fluid flow cytometry (comprehensive lymphoma panel), CT-PET, and connective tissue disease (LE and antiphospholipid syndrome) panels, were unremarkable. SPTL with LE profundus overlap was diagnosed upon review of the nodule biopsy report. The patient was treated with prednisone and cyclosporine with a good response, as seen on follow-up.SPTL can arise in patients with LE or vice versa. The illness itself may also present initially as benign panniculitis, eczema, dermatitis, psoriasis, cellulitis, or other skin and soft tissue infections. The culprit behind this disease is a HAVCR2 (Hepatitis A Virus Cellular Receptor 2) mutation, which occurs in 60% of SPTL cases and is more typically seen in Asian and Eastern patients. Its presence is related to HLH-refractory and severe disease course. For patients with indolent course, the suggested first-line remedies encompass immunosuppressive agents together with prednisone, cyclophosphamide, or methotrexate; extreme conditions with aggressive infiltrative lymphocytes may also require a combination of chemotherapy regimens. In patients resistant to chemotherapy, cyclosporine A alone or combined with steroids causes desirable effects, including in HPS patients.SPTL is a diagnostic challenge. A high index of suspicion and correlation of clinical, imaging, and histopathologic reports will increase the probability of detecting this disorder, consequently lessening the probability of the delay in treatment. Indolent courses have desirable overall survival, except for patients presenting with HLH/HPS upon diagnosis.

The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.

BACKGROUND Panniculitides are a heterogeneous group of inflammatory dermatoses involving the subcutaneous fatty tissue. Histologically, they are classified into septal and lobular panniculitis, according to the predominant localization of the inflammatory infiltrate. Neutrophils are frequently found in panniculitis, mainly at the early stages. Here, we investigated whether neutrophils contribute to various types of cutaneous panniculitis by releasing neutrophil extracellular traps (NETs). MATERIALS AND METHODS Formalin-fixed paraffin-embedded skin biopsies from 25 patients with panniculitis were included in the study. Our cohort was divided into n = 10 erythema nodosum (septal panniculitis) and n = 15 lobular panniculitis, including n = 7 lupus panniculitis, n = 1 pancreatic panniculitis, n = 1 Weber-Christian disease, n = 1 deep fungal infection, n = 2 lipodermatosclerosis, and three cases did not have an identified etiology. The presence of neutrophils and NETs was assessed by double immunofluorescence using antibodies against elastase, a neutrophilic marker, and citrullinated histone 3, a marker of NETs. RESULTS The mean percentages (±SEM) of elastase-positive neutrophils showing NETs were 44% ± 3% in erythema nodosum and 43% ± 7% in lobular panniculitis. The difference was not statistically significant and reflects the implication of NETs not only in severe scarring lobular panniculitis but also in benign non-scarring self-remitting reactive inflammation such as erythema nodosum. In tissues, NETs were located in the interlobular septa in erythema nodosum and in the inflamed fat lobules in lobular panniculitis. CONCLUSIONS NETs are massively present in septal and lobular subtypes of panniculitides, suggesting their involvement in tissue damage.

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).We compared the clinical presentations, histopathology, immunophenotypical features and T-cell receptor (TCR) gene rearrangement findings of 9 cases of LEP and SPTL.For clinical features, most patients of LEP occurred on head and face without systemic symptoms. LEP patients responded well to hydroxycholorquine treatment with good prognosis. Most patients of SPTL tended to lower extremities involvement and accompanied with systemic symptoms, the patients with disseminated lesions or hemophagocytic syndrome(HPS) showed poorer prognosis. For histopathology, LEP patients showed dense inflammatory infiltrate in the dermis consisting predominantly of lymphocytes with less numbers of plasma cells. However, the dermis was spared in SPTL, and rimming of adipocytes and erythrophagocytosis was observed in SPTL. Lymphocytes of LEP expressing CD4Base on different clinical and pathological features, it is easy to distinguish LEP from SPTL. However, a minority of lesions in LEP localize at subcutaneous tissue, which may turn to immunophenotypical and TCR gene rearrangement test for diagnosis.

The distinction between subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and lupus erythematosus (LE) panniculitis is remarkably challenging. Rimming by lymphocytes with an elevated Ki-67 cell proliferation index has been forwarded as a potential diagnostic finding in biopsies of SPTCL but has not been rigorously compared with biopsies from patients with LE panniculitis. Nineteen and 17 examples of SPTCL and LE panniculitis, respectively, were evaluated for periadipocytic rimming by lymphocytes expressing Ki-67, CD8, and βF1 and for attributes associated with LE, including clusters of CD123-positive cells. The identification of periadiopocytic rimming using Ki-67, CD8, and βF1 held sensitivity of 79%, 100%, and 89.5% and specificity of 100%, 52.9%, and 88.2%, respectively (P < 0.01). CD123-positive cells were in both disorders. LE-like histopathology was commonly encountered in SPTCL. In conclusion, an elevated Ki-67 cell proliferation index with rimming is useful for distinguishing SPTCL from LE panniculitis. Notably, many features of LE panniculitis can also be encountered in SPTCL.

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cutaneous T cell malignancy of cytotoxic T cell origin. It is frequently associated with autoimmune diseases. It is known to preferentially involve subcutaneous adipose tissue and histologically resembles lupus panniculitis. The aetiology and risk factors of SPTCL are unclear and there are limited studies available since this entity was initially described in 2001. There are even fewer case reports describing the association between SPTCL and chronic lymphocytic leukemia (CLL). In this article, we present a case of SPTCL arising during treatment for CLL. We conducted an extensive review of literature to delve into the possible risk factors for SPTCL development in association with CLL, including pre-existing haematological malignancies, autoimmune conditions, immunomodulation and immunosuppressive chemotherapy.

Lupus mastitis is an uncommon manifestation of systemic lupus erythematosus (SLE) that affects the subcutaneous fat in the breast, much like lupus panniculitis, but additionally involves the mammary gland. We report on two women for whom lupus mastitis was the initial manifestation of SLE and provide a literature review of 34 additional cases reported in the Anglo-Saxon and French literature since 1971, making this the largest review to date. Lupus mastitis (LM) can manifest clinically as subcutaneous masses that may be painful, or may present cutaneous involvement such as thickening and discolouration. The radiologic manifestations of LM are broad and include calcifications, masses and asymmetries. Most often, excluding malignancy requires percutaneous biopsy, with histologic findings that are virtually pathognomonic for SLE. Thus, surgery is avoided and medical management can begin, antimalarial drugs and corticosteroids in most cases.

Trabalho Final do Mestrado Integrado em Medicina apresentado à Faculdade de Medicina O Lupus eritematoso (LE) é uma doença autoimune que, atualmente, continua adespoletar muitas dúvidas e em relação ao qual ainda muito há por descobrir. Neste artigode revisão o objetivo fundamental foi perceber a importância das manifestações cutâneasnesta patologia. Manifestações cutâneas que podem aparecer isoladamente e sem qualquerenvolvimento sistémico ou que, por outro lado, podem surgir aquando de uma doençasistémica de base. Perceber a relação entre o aparecimento destas manifestações e apatologia de base, neste caso o LE, foi o maior foco deste artigo. Entender a variedade demanifestações cutâneas que surgem no decorrer desta patologia e as suas caraterísticasbem como o os meios complementares de diagnósticos, manifestações clínicas e diferençasentre as mesmas esteve na base deste estudo.O LE Sistémico (LES), LE Cutâneo (LEC) e os subtipos de LEC (LEC agudo, LECsubagudo e LEC discóide) foram abordados e distinguidos para uma melhor percepção dasdiferenças entre eles. Foram distinguidos não só nas manifestações clínicas como na suaincidência na população e também na maior ou menor tendência em fazerem-seacompanhar de manifestações sistémicas.Foram também dadas a conhecer algumas das medidas preventivas para evitar oucontrolar eventuais exacerbações do LE e também algum do tratamento habitualmenteutilizado, mas que continua ainda debaixo de um estudo e investigação intensa devido àsdiferentes reações e eficácias demonstradas nos diversos doentes atingidos por estapatologia e estudos realizados. Lupus erythematosus (LE) is an autoimmune disease that still continues to risemany doubts and much remains to be discovered. This review article’s main objective is tounderstand the importance of cutaneous manifestations in this pathology. These cutaneousmanifestations may occur isolated and without systemic involvement or, on the other hand,may arise in the course of a basic systemic disease. The relationship between the onset ofthese manifestations and the underlying pathology, in this case LE, was the main focus.Understanding the variety of cutaneous manifestations that arise during this pathology andits characteristics as well as the complementary means of diagnosis, clinical manifestationsand differences between them was the basis of this study.Systemic LE (LE), Cutaneous LE (LEC) and ECC subtypes (acute ECG, subacuteECC and discoid ECC) were addressed and distinguished for a better perception of thedifferences between them. They were distinguished, not only in the clinical manifestations, aswell as in their incidence in the population and also in the greater or less tendency to beaccompanied by systemic manifestations.Some preventive measures have also been reported to prevent or control possibleexacerbations of LE and also some of the usual treatment which is still under intense studyand investigation due to the different reactions and efficacies demonstrated in the severalpatients affected by this pathology and studies carried out .

Cutaneous manifestations are second most common presenting feature of lupus. Discoid lupus erythematosus is the most common variant amongst all; lupus panniculitis being described in only 2-5% of cases. Most cases of cutaneous lupus are associated with autoantibodies and either precede or follow the systemic manifestations of lupus. There is no proven treatment for cutaneous manifestations of lupus including lupus panniculitis. Available non-randomized studies show efficacy of hydroxychoroquine in most cases, whereas methotrexate and other immunosuppressant are used in relapsing cases. We describe a case of lupus panniculitis presenting as isolated manifestation of lupus with negative autoantibody titers which responded well to methotrexate. We observed that lesions went into drug free remission in 1 year and did not recur on 1 year follow-up. There was no residual skin atrophy or scarring. Drug free remission in isolated cases of lupus panniculitis variant could be possible with timely intervention in the absence of autoantibodies. Keywords: lupus; Methotrexate; Nepal; panniculitis.

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. Lesions tend to resolve spontaneously and may follow a chronic course of remission and exacerbation that persists for months to years. The imaging features of facial LP are extremely scarce in the literature. We present a case of facial lupus panniculitis and describe the associated characteristic ultrasound, CT, and MR imaging findings along with histopathologic correlation.

AIMS Histopathological overlap between lupus erythematosus and certain types of cutaneous T cell lymphoma (CTCL) is well documented. CD123+ plasmacytoid dendritic cells (PDCs) are typically increased in lupus erythematosus, but have not been well studied in CTCL. We aimed to compare CD123 immunostaining and histopathological features in these conditions. METHODS AND RESULTS Skin biopsies of cutaneous lupus erythematosus (CLE, n = 18), lupus erythematosus panniculitis (LEP, n = 17), mycosis fungoides (MF, n = 25) and subcutaneous panniculitis-like T cell lymphoma (SPTCL, n = 9) were retrospectively reviewed and immunostained with CD123. Percentage, distribution and clustering of CD123+ cells were compared between CLE and MF and between LEP and SPTCL using χ2 and two-tailed t-tests. A higher percentage of CD123+ cells was observed in CLE than MF (P < 0.01), more frequently comprising ≥20% of the entire infiltrate (P < 0.01) and forming clusters (P < 0.01). Similarly, LEP showed a higher percentage of CD123+ cells than SPTCL (P = 0.01), more frequently comprising ≥20% of the infiltrate (P = 0.04) and forming clusters (P = 0.01). Basal vacuolar change or dyskeratosis was observed in all CLE cases and in 48% cases of MF cases (P = 0.05). Plasma cells were readily identified in 76% cases of LEP but in none of the SPTCL cases (P = 0.01). Adipocyte rimming by lymphocytes, hyaline fat necrosis and fibrinoid/grungy necrosis did not significantly differ between LEP and SPTCL. Dermal mucin also failed to distinguish between groups. CONCLUSIONS CD123 immunostaining is helpful in differentiating CLE from MF and LEP from SPTCL, but should be interpreted in conjunction with clinicopathological features and other ancillary studies to ensure accurate diagnosis.

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.

Background A facial depression deformity secondary to lupus erythematosus panniculitis results from fat necrosis, which seriously alters the patients' appearance and thus affects their psychological health. Few studies have discussed the repair of depression deformities. The authors repaired depression deformities due to atrophic lesions by using autologous fat grafting to obtain volumetric restoration of the facial skin, and the authors report our outcomes. Methods In this series, 30 depression deformities in 18 patients were repaired. The authors suctioned fat grafts from the abdomen and centrifuged them at 500×g for 2 minutes. After discarding the upper oil and bottom liquid, the middle fat was injected into the depressed areas (ie, the cheek, temple, and zygoma) by using multiplane and multitunnel injections. The patients, plastic surgeons on staff, and laypersons evaluated the cosmetic outcome of each patient. Results No infection, subcutaneous nodules or cysts, cutaneous necrosis, blood vessel embolism, or other complications were found in any patient. Five patients had 1 injection, 5 had 2 injections, and 8 had 3 injections. All depression deformities improved. Regarding the cosmetic outcome, 33.3% of patients, 27.8% of laypersons, and 38.9% of doctors were satisfied with the results; 44.4% of patients, 55.6% of laypersons, and 50.0% of doctors were mostly satisfied. Conclusion Autologous fat grafting can be a good choice for repairing facial depression deformities in patients with lupus erythematosus panniculitis, although 1 or more subsequent injections may be required to maintain the improved outcome.

Some cases of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and lupus erythematosus panniculitis (LEP) demonstrate clinical and histopathologic overlap, raising the possibility that they represent opposite ends of a disease spectrum. SPTCL, however, is typically associated with greater morbidity and risk for hemophagocytic lymphohistiocytosis (HLH); therefore, diagnostic distinction is clinically important. We present the histopathologic, immunophenotypic, and molecular findings with long-term clinical follow-up of 13 patients with SPTCL (median, 64 mo follow-up) and 7 with LEP (median, 50 mo follow-up) in our multidisciplinary cutaneous oncology clinic. Six SPTCL patients developed HLH, including 2 under the age of 21 years. In the SPTCL group, 2 of 13 patients died of disease. In contrast, we had no mortality or development of HLH in our LEP cohort. We demonstrate that a limited panel (Ki-67, CD3, CD4, and CD8 immunostains) reveals foci of "Ki-67 hotspots" enriched in cytotoxic atypical CD8+ T cells in SPTCL. Ki-67 hotspots were not identified in LEP, thus aiding the distinction of SPTCL from LEP. Lymphocyte atypia combined with adipocyte rimming of CD8+ T cells within Ki-67 hotspots was also highly specific for the diagnosis of SPTCL. Hyaline lipomembranous change, B-cell aggregates, plasmacytoid dendritic cell clusters, and plasma cell aggregates favored the diagnosis of LEP but were identified in some cases of SPTCL including patients with HLH. We confirm that SPTCL and LEP can show significant histologic overlap, suggest a role for high-throughput sequencing in confirming neoplastic clones, and introduce the concept of SPTCL "Ki-67 hotspots" in evolving disease.

Background Published case series describe lenalidomide as an effective treatment of refractory cutaneous lupus erythematosus (CLE). Objectives The present study aimed to further characterize lenalidomide use in the treatment of CLE. Methods A retrospective review of patients treated with lenalidomide for CLE from January 1, 2000, to December 17, 2014, was conducted. Results Eight of the nine patients (89%) were women. Their median age at initiation of lenalidomide was 62 years (range: 41–86 years). Subtypes of CLE included discoid lupus erythematosus (DLE) (n = 6), lupus panniculitis (n = 2), and subacute CLE (n = 1). Before the initiation of lenalidomide, all patients had been previously treated unsuccessfully or were intolerant to at least one antimalarial and one immunosuppressive agent. With lenalidomide, five patients achieved a complete response (CR), two a partial response, and two had no response (lupus panniculitis). Time to initial response (dose range: 2.5–10.0 mg/d) varied from 2 weeks to 3 months; the median time to CR in five patients was 3 months (range: 3–6 months). The median duration of lenalidomide therapy was 12 months (range: 2–67 months). The median duration of follow-up was 48 months (range: 20–103 months). Adverse effects included mild leukopenia; one patient had deep vein thrombosis of unclear etiology during a hospitalization. No patients developed or showed progression of systemic LE while receiving lenalidomide. Conclusions Lenalidomide was effective for the treatment of CLE (particularly DLE) but not for the treatment of lupus panniculitis in this series.

Tumid lupus is an uncommon variant of lupus erythematosus. Patients present with subcutaneous lesions. Ophthalmic literature reports disease manifestation as orbital inflammation. Autoimmune serology is often negative. Without a high index of suspicion, the diagnosis is easily overlooked delaying treatment. Tumid lupus is not significantly discussed in the Otolaryngologic literature. Here we present a Case Report of a male who initially presented to Ophthalmology with unilateral orbital complaint of eyelid puffiness. Orbital biopsy and subsequent biopsy of his submental skin lesions ultimately led to this unexpected diagnosis. We discuss the method to diagnose tumid lupus including representative histopathologic findings.

Importance Childhood-onset lupus erythematosus panniculitis (LEP) is a rare and chronic disfiguring disease. A paucity of literature exists on the clinical manifestations of this disease and how best to treat it. Objectives To describe the clinical features of childhood-onset LEP and report on the use of rituximab in treatment-refractory childhood-onset LEP. Design, Setting, and Participants A retrospective, observational case series study was conducted of 4 patients with childhood-onset LEP who presented to a single-center, tertiary care clinic with pediatric dermatology and pediatric rheumatology clinics between July 1, 2014, and July 1, 2018, and were treated with rituximab. A literature review was conducted of the clinical features and treatment of childhood-onset LEP. Exposure Rituximab therapy for childhood-onset LEP. Main Outcomes and Measures Reduction in the number and size of erythematous and tender subcutaneous nodules (both visually and by palpation), reduction of facial atrophy (documented with serial photography), and tolerability of rituximab at 2 to 22 months after initiation of therapy. Results Four patients (3 male; mean [SD] age at treatment, 15 [5.9] years) with refractory childhood-onset LEP were successfully treated with rituximab. All patients had a rapid and sustained response to therapy with rituximab. One patient (25%) had minor infusion reactions; otherwise, treatment was well tolerated. Conclusions and Relevance This case series suggests that rituximab may hold promise as a treatment for refractory, childhood-onset LEP. Larger, prospective studies are needed to validate these findings; however, given the rarity of disease, large studies may be difficult to conduct.

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 = 26.4 [10-53] years, female: male ratio = 1:1, mean disease duration = 86.89 [8-336] weeks). The most commonly affected areas of scalp included parietal (70%), frontal (45%), temporal (40%), occipital (30%), and vertex (10%), along 70% of Blaschko's lines with morphologic lesions linear, annular, arch-shaped, and ulcer. Besides, ANA (60%) was in particular noted. Hydroxychloroquine, oral prednisolone, intralesional corticosteroid, and methotrexate were the most common treatments, with complete response after an average period of 8.08 (2-12) weeks. Systemic lupus erythematosus (SLE) was developed in four cases (20%) during follow-up, with high recurrence of 35%. We reported distinctive and rare cases of LPS.The continuing follow-up was highly recommended to avoid probable recurrence and SLE development.

Background and objective Lupus panniculitis (LP) is a rare variant of chronic cutaneous lupus erythematosus, which diagnosis requires clinicopathological correlation, especially in those patients without any other manifestation of lupus erythematosus (LE). According to the phase when the biopsy is performed, histological findings can be non-specific. Few series have been published to date. Hence, we report our own experience in the diagnosis and management of this disease. Materials and methods We conducted a retrospective descriptive clinicopathological study of 12 patients diagnosed in our centre. Results All the patients had painful and recurrent plaques and/or nodules, with a predilection for proximal extremities, face and scalp. Histopathologic examination showed mostly lobular panniculitis and lymphoplasmacytic infiltrate. For the diagnosis, we also considered the coexistence of other clinical manifestations of LE as well as the expression of CD123 by immunohistochemistry. In 3 patients, LP was the first manifestation of LE. Conclusions The diagnosis of LP can be difficult. The presence of other clinical and/or histological manifestations of LE along with immunohistochemistry techniques could help in the differential diagnosis with other panniculitis.

Rationale: Lupus erythematosus panniculitis (LEP) is a rare subset of lupus erythematosus. The incidence of LEP in systemic lupus erythematosus (SLE) ranges from 2% to 5%. In the previous literature, most LEP patients were women aged from 20 to 60 years, while pediatric cases were rare, all of whom appeared on their own without SLE.A rare LEP in a 10-year-old female child with severe SLE is presented. Patient concerns: A 10-year-old girl was admitted to our hospital for marasmus and fatigue without other typical manifestations of SLE well before the appearance of skin lesions. The only proof to support the SLE is that we observed a weakly positive antinuclear antibody (ANA) in serum at the onset. Diagnoses: A 10-year-old girl diagnosed to the Division of Nephrology, Department of Pediatrics, the Second Xiangya Hospital, Central South University, for LEP with severe SLE. Interventions: The patient was administered with high-dose corticosteroids and cyclophosphamide. Outcome: The patient died of severe lung involvement despite the use of high-dose corticosteroids and cyclophosphamide. Lessons: This report highlights an unusual manifestation of LEP associated with SLE in a child. It also suggests that pediatricians should be aware of occult onset of SLE, such as unclear marasmus and fatigue found in this case. Repeat tests of antinuclear antibody and anti-double strand DNA antibody (anti-dsDNA) as well as renal biopsy in a timely manner will be effective to achieve early recognition and immediate treatment for saving lives.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare condition that falls underneath the umbrella of primary cutaneous T-cell lymphomas (CTCLs). SPTCL can be very difficult to diagnose as it may mimic other subtypes of CTCL, such as γ/δ T-cell lymphoma (TCL), or other forms of panniculitis. Confirmation of diagnosis often requires immunohistochemical analysis and is essential for proper prognosis and therapeutic management. Herein, we present a case of SPTCL that mimicked lupus panniculitis and was successfully treated with prednisone taper and methotrexate.

This is a case description of a single male patient found to have T-cell-mediated inflammation and lymphoproliferation of the orbit. Chronic T-cell-mediated inflammatory disease can pose a diagnostic challenge particularly in its differentiation from a neoplastic process. The histopathology in this case demonstrated features of both lupus erythematosus panniculitis and features of orbital T-cell lymphoma. While both are rare, lupus erythematosus panniculitis of the orbit is even more exceptional; this patient's indolent, chronic relapsing course distinguished itself from the typical aggression of orbital T-cell lymphoma. We believe this rare case may actually represent an example of a newly described disease spectrum that incorporates lupus erythematosus panniculitis as well as subcutaneous panniculitis-like T-cell lymphoma.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a malignant primary cutaneous T-cell lymphoma that shares significant clinical, histopathologic and immunophenotypic overlap with lupus erythematosus panniculitis (LEP).We performed immunohistochemistry for the MYC oncoprotein on 23 cases of SPTCL (1 CD8 negative) and 12 cases of LEP to evaluate if there are quantitative or qualitative differences in protein expression of this marker in these entities.In SPTCL cases, the percentage of all cells that were c-Myc positive ranged from 0.8% to 16%, with a mean of 5.0% and a median of 4.4%. In contrast, in the LEP cases, the percentage of c-Myc-positive cells in the cases ranged from 0.34% to 3.7%, averaged 1.4% and the median was 0.8%. The difference between the means of these 2 diagnostic categories was statistically significant. Fluorescence in situ hybridization performed on 4 cases of SPTCL with a relatively high level of MYC immunohistochemical staining, however, failed to demonstrate evidence of MYC rearrangement or amplification.Our work demonstrates that MYC expression levels differ between these 2 histologic mimics and suggests that this important oncoprotein may play a role in the pathogenesis of SPTCL.

Rationale: Lupus panniculitis (LP) is a unique variant of cutaneous lupus erythematosus. Clinical manifestations are typically mild and include erythema, nodules, and small ulcers. In certain cases, diagnosing LP may be challenging. Skin overlying the typical subcutaneous inflammation may appear normal, and bacterial superinfections of the skin sometimes mask the underlying LP. It has been suggested that a computed tomography (CT) scan may help to identify obscure LP lesions. Here, we report a case of a 54-year-old woman with an unusually severe form of LP, in which the full disease extent was only revealed by a fluorodeoxyglucose positron emission tomography (FDG-PET)/CT scan. Patient concerns/Diagnoses/Interventions/Outcomes: Our patient initially presented with a bacterial infection of the skin. After initial improvement with antibiotic treatment, new erythematous lesions and sterile subcutaneous pus collections developed. An FDG-PET/CT scan revealed extensive subcutaneous inflammation at sites that had appeared normal during physical examination and on CT scan. As the subcutaneous lesions showed a remarkably linear pattern on FDG-PET/CT scan, the patient was suspected of having LP. After confirmation of this diagnosis by a deep-skin biopsy, our patient was treated with systemic glucocorticoids. Eventually, our patient succumbed to complications of LP and its treatment. Lessons: Our case demonstrates that clinical manifestations of LP are not always mild and that timely diagnosis is needed. Furthermore, we show that obscure LP lesions are more readily identified on an FDG-PET/CT scan than CT scan.

A woman aged 45 years presented for evaluation of skin lesions. She reported an 8–9-year history of occasionally tender, waxing-and-waning skin nodules refractory to dapsone, prednisone and methotrexate. Examination revealed multiple indurated subcutaneous nodules distributed on the upper extremities, with scattered patches of lipoatrophy in areas of nodule regression (figure 1). Her medical history was unremarkable; CBC and CMP were within normal limits, with no history of radiotherapy or evidence of internal organ involvement. She had a positive ANA titre (1:160, speckled), but negative anti-dsDNA, anti-Smith, anti-Ro and anti-La antibodies. Figure 1 Multiple erythematous subcutaneous nodules distributed over the patient's right arm. Differential diagnosis included erythema nodosum (EN), erythema induratum of Bazin (EIB), lupus profundus (LP) and cutaneous lymphoma. Initial wedge biopsy in 2008 disclosed a predominantly lobular panniculitic process with some septal involvement (figure 2A). Broad zones of necrosis were present (figure 2B). The infiltrate consisted of a pleomorphic population of lymphocytes with occasional larger atypical lymphocytes (figure 2C). There were foci of adipocyte rimming by the atypical lymphocytes (figure 2C). Immunophenotyping revealed predominance of CD3+ T cells …

A young woman presented with panniculitis, fever and blood cytopenias, later going on to develop hemophagocytic lymphohistiocytosis. Further evaluation revealed the presence of subcutaneous panniculitis like T-cell lymphoma (SPTCL). With this case we present an approach to panniculitis, a commonly encountered skin manifestation with a wide range of differentials. We discuss the close similarity between lupus erythematous panniculitis (LEP) and lymphoma.

Lupus erythematosus panniculitis (LEP) has been reported to be the first manifestation of systemic lupus erythematosus (SLE)1. Face and upper limbs are most commonly involved; however, extensive LEP in the buttock region or breasts is relatively rare, but also reported2,3. Differential diagnosis such as subcutaneous panniculitis-like T cell lymphoma …