Cutaneous Variant Research Articles

A rare cutaneous manifestation of systemic sclerosis

A 43-year-old male with a history of intravenous drug use and alcohol consumption presented to the emergency department with three-month history of failure to thrive. The patient exhibited a constellation of constitutional symptoms including cough, weight loss, fatigue, decreased appetite, nausea and vomiting. The skin examination revealed multiple subcutaneous hyperpigmented, indurated plaques and nodules on the trunk and arms. Laboratory evaluation revealed abnormal autoimmune tests, anaemia, elevated inflammatory markers and radiological evidence of interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). An excisional biopsy from a skin lesion demonstrated dermal sclerosis consistent with scleroderma. The patient was diagnosed with diffuse systemic scleroderma with cutaneous findings consistent with nodular or keloidal scleroderma variant. This case highlights a rare cutaneous variant of systemic scleroderma called nodular or keloidal scleroderma.

Concordance and Prognostic Relevance of Different Definitions of Systemic Sclerosis Interstitial Lung Disease Progression.

Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a common complication that has varied progression rate and prognosis. Different progression definitions are available: include minimal clinically important worsening of forced vital capacity (FVC MCIW), EUSTAR (EUropean Scleroderma Trials and Research group) progression, OMERACT (Outcome Measures in Rheumatology Clinical Trials) progression, and Erice ILD working group progression. Pulmonary function and symptoms changes may act as specific confounding factors applying these definitions in SSc. To assess the concordance and prognostic value of four different definitions in SSc-ILD patients overall and specific clinical groups. Progression status in consecutive SSc-ILD patients was assessed over 24 months, 60-month disease-related mortality risk was compared between progressors and non-progressors using the four definitions. Among 245 patients, 26 SSc-related deaths were reported. Mortality was linked to progression for FVC MCIW (HR 2.27, 95% CI 1.03-4.97), OMERACT (HR 2.90, 95% CI 1.28-6.57), and Erice definitions (HR 11.02, 95% CI 2.38-51.08). The association between progression and mortality was poor in patients with disease duration ≥3 years, mild functional impairment, and pulmonary artery systolic pressure (PASP)≥40 mmHg. Erice criteria appeared superior in patients with duration ≥3 years, limited cutaneous variant, and PASP<40 mmHg. OMERACT criteria performed better in diffuse cutaneous variant patients with severe functional impairment. The four evaluated definitions of progression in SSc-ILD are not interchangeable, resulting in up to a third of cases being classified differently based on the adopted criteria, and presenting different prognostic values, particularly within specific clinical groups.

Sézary Syndrome in West Sweden: Exploring Epidemiology, Clinical Features, and Treatment Patterns in a Registry-Based Retrospective Analysis.

Sézary syndrome (SS) is a rare primary cutaneous T-cell lymphoma variant. Despite various treatment options, it remains incurable, with a poor prognosis. There is an urgent need for additional descriptive research to enhance our understanding and treatment of SS. The aim of this retrospective register-based study was to outline patients' demographic characteristics; investigate the clinical, histopathological, and molecular findings; and assess treatment effectiveness with a focus on time to next treatment (TTNT) and disease progression. Data on 17 patients with SS were obtained from the primary cutaneous lymphoma register in West Sweden between 2012 and 2024. The results revealed that not all patients exhibited the classical triad of symptoms at diagnosis, emphasizing the need for personalized diagnostic approaches. The median survival was only 2.1 years, which reflects the aggressive nature of SS. The longest median TTNT was observed in triple therapy involving retinoids, interferon alpha, and extracorporeal photopheresis (ECP). There was no significant difference in TTNT between various lines of treatment. Early initiation of ECP treatment did not result in improved outcomes. This study highlights the importance of combination therapy for improved outcomes and underscores the need for future studies to identify optimal treatment approaches.

Unusual presentation of extranodal Rosai-Dorfman disease: A scalp lump

Rosai-Dorfman disease (RDD) is a benign and idiopathic proliferative histiocytic disorder that typically manifests with extensive cervical lymphadenopathy, fever, polyclonal gammopathy, and leukocytosis with neutrophilia. However, the purely cutaneous variant of RDD is exceptionally uncommon, occurring in only 3% of all RDD cases. In this abstract, we present a rare case of RDD exhibiting a purely cutaneous form. The patient, a 40-year-old male, presented with a scalp lump. Diagnostic investigations, including histopathological examination, confirmed the diagnosis of RDD. No lymphadenopathy or systemic involvement was detected, highlighting the uniqueness of the cutaneous presentation. The rarity of pure cutaneous RDD underscores the significance of reporting such cases to enhance our understanding of the diverse clinical manifestations of this condition. Awareness of this atypical presentation is crucial for accurate diagnosis and appropriate management, as the treatment approach for purely cutaneous RDD may differ from the more common systemic forms. Through this case report, we aim to contribute to the existing medical literature on RDD, emphasizing the need for careful evaluation of skin lesions to include RDD in the differential diagnosis, even in the absence of lymphadenopathy or systemic symptoms. Further research and documentation of similar cases will undoubt­edly advance our knowledge of this intriguing disease and aid clinicians in providing optimal patient care.

A case of cutaneous variant of intravascular large B-cell lymphoma in which dermoscopy revealed telangiectasias associated with erythematous induration.

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal, diffuse, large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumen of small blood vessels, with no lymphadenopathy or masses. Herein, we report a cutaneous variant of IVLBCL that is rare in Asia. A healthy 73-year-old Japanese woman presented to our hospital with painful erythematous indurations and telangiectasia of the lower extremities, which was confirmed on dermoscopy. Physical examination revealed no systemic involvement, and laboratory parameters were within normal ranges. No abnormal fluorodeoxyglucose (FDG) uptake was detected on 18FDG positron emission tomography/computed tomography. Histopathological examination revealed proliferation and dilatation of blood vessels in the subcutis layer, occluded by CD20-positive atypical lymphoid cells. Thus, the patient was diagnosed with a cutaneous variant of IVLBCL without systemic symptoms. In conclusion, it is important to confirm telangiectasia using dermoscopy and perform skin biopsies in patients presenting with sudden-onset erythematous induration.

Intravascular Large B-Cell Lymphoma Detected by Cutaneous Biopsy with Pancytopenia and Negative Bone Marrow Biopsy

Intravascular large B-cell lymphoma is a B-cell lineage neoplasm, which is typically recognized as a high-grade neoplasm with poor outcomes. Though rare, the cutaneous variant can present with multiple lesions of the skin and negative systemic staging. We present a case of intravascular large B-cell lymphoma first identified on cutaneous biopsy, with pancytopenia, in addition to negative bone marrow biopsy and serum protein electrophoresis. The patient is a 71-year-old male who presented with upper body pruritis and pancytopenia, as well as weight loss and fatigue. A full body skin exam incidentally revealed a well-demarcated, firm, bound down atrophic lichenified plaque on the right anterior thigh. A punch biopsy revealed large, atypical lymphoid cells exclusively localized within the lumina of variably sized blood vessels, immunoreactive for CD20, BCL6, MUM1, and CD5, but negative for CD3, CD10, cyclin D1, and TdT. A diagnosis of intravascular large B-cell lymphoma was made, and he was referred to oncology for further treatment. Full body skin exam is a mainstay of dermatologic practice, and it is essential to pursue biopsy with uncharacteristic lesions, regardless of the presenting concern.

The development of adult T cell leukemia/lymphoma in renal transplant recipients: report of two cases with literature review

BackgroundsTherefore, reports on the risk of HTLV-1-related diseases in organ transplantation have increased in recent years, and the management of HTLV in renal transplantation remains a challenge.Patients and methodsWe retrospectively analyzed four HTLV-1-positive recipients or donors among 89 renal transplantation cases from 2006 to 2021.ResultsAmong the four HTLV-1-positive recipients, two patients developed adult T cell leukemia/lymphoma (ATL) derived from recipients at approximately 3 years (1016 days and 1195 days) after renal transplantation. Case 1 developed lymphoma-type ATL (an extranodal primary cutaneous variant), including skin and pulmonary lesions. The patient achieved CR with FK tapering and CHOP therapy following cord blood stem cell transplantation. However, the patient died 101 days after ATL development because of a severe fungal infection. Case 2 developed acute-type ATL with an unusual phenotype of CD4+8+30+. The patient was treated with FK tapering and palliative therapy because of poor PS. Notably, in case 1, histopathological findings showed high numbers of PD-1-positive TIL cells in ATL, suggesting exhausted T cells and a correlation with the early onset of ATL. Furthermore, in Case 2, histopathological findings revealed CD 30 expression in ATL cells, suggesting the importance of CD 30 in ATL development. Importantly, case 2 showed typical driver mutations, including CCR4 truncation mutations of the C-terminal, TBL1XR1 mutation, and TP53 mutation in the splice site. Notably, our present study and our previous study on renal transplantation strongly indicated that two out of two and one out of 59 “recipient” positive cases developed ATL, respectively. Furthermore, our previous nationwide study 4 out of 10 “donor” positive cases developed HAM. These findings showed that ATL may be derived from HTLV-I+ recipient cells and HAM may be derived from HTLV-1+ donor cells, although the precise mechanism remains unknown.ConclusionsThus, early onset and rapid progression of ATL with poor outcomes should be considered in HTLV-1 endemic areas. Furthermore, immunological or genetic mechanisms may be related to the development of ATL after renal transplantation. We believe that the mechanism of onset of ATL after transplantation may be important when considering the immune environment of ATL itself.

Coexpression of natural killer cell antigens by T-cell large granular lymphocytes in hydroa vacciniforme lymphoproliferative disorder and the involvement of Vδ1 + epithelial-type γδT cells

Hydroa vacciniforme lymphoproliferative disorder (HV-LPD) is a cutaneous variant of chronic active Epstein–Barr virus disease. We examined the coexpression of T- and natural killer (NK)-cell antigens in five patients with classic HV (cHV) and five with systemic HV (sHV). T-cell receptor (TCR) repertoire analysis was performed with high‑throughput sequencing. All five cHV patients had increased γδT cells (> 5%), whereas five sHV patients showed γδT- and αβT-cell dominance in two patients each, and a mixture of abnormal γδT and αβT cells in one. Circulating CD3 + T cells expressed CD16/CD56 at 7.8–42.3% and 1.1–9.7% in sHV and cHV, respectively. The percentage of CD16/CD56 + T cells was higher in the large granular lymphocyte or atypical T-cell fractions in sHV, but no TCR Vα24 invariant chain characteristic of NKT cells was detected. Considerable numbers of CD3 + cells expressing CD56 were observed in sHV skin infiltrates. Of the circulating γδT cells tested, TCR Vδ1 + cells characteristic of the epithelial type of γδT cells were dominant in two sHV cases. Thus, atypical αβT and γδT cells in HV-LPD can express NK-cell antigens, such as CD16 and CD56, and Vδ1 + epithelial-type γδT cells are a major cell type in some HV-LPD cases.

Direct Immunofluorescence in Cutaneous and Systemic Lupus Erythematosus: A Literature Review

Cutaneous manifestations of lupus erythematosus (LE) have a variety of clinical phenotypes and require proper investigation for diagnosis. Knowledge of the direct immunofluorescence (DIF) technique has improved and played an important role in the diagnosis of cutaneous LE. This review explores and summarizes reported DIF findings of each cutaneous LE variant. Historically, DIF findings of cutaneous LE have revealed deposits of multiple immunoreactants at the dermo-epidermal junction with either linear or granular patterns. Immunoglobulin M is the most common immunoreactant and DIF findings of cutaneous LE variants overlap. Therefore, diagnosis of cutaneous LE requires a combination of monitoring patient history, physical examinations and laboratory studies. This review helps interpret and better understand the application of DIF studies in cutaneous LE.

Intravascular Large B-Cell Lymphoma: Two Cases Observed at a Single Institution

Intravascular large B-cell Lymphoma (IVLBCL) is a rare subtype of extranodal non-Hodgkin’s lymphoma that is challenging to diagnose and has a poor prognosis. Here, we describe two patients newly diagnosed with IVLBCL at our institution: an African man with hemophagocytic-syndrome-associated IVLBCL and an Italian woman with a cutaneous variant of IVLBCL. They presented with very different clinical manifestations. Both cases were diagnosed in a timely manner, successfully treated, and achieved long-lasting remissions.

Lichenoid Sarcoidosis Simulating Pseudofolliculitis Barbae

Lichenoid sarcoidosis is an extremely rare cutaneous variant of sarcoidosis, occurring in approximately 1-2% of all cutaneous presentations [1]. It is classically described in Asian children and usually affects the eyes and joints, sparing respiratory involvement [2] .However, case reports in adults and the elderly are frequent.

Effective treatment of canine chronic cutaneous lupus erythematosus variants with oclacitinib: Seven cases.

The treatment of canine chronic cutaneous lupus erythematosus (CCLE) variants generally requires immunosuppression, which often results in potentially severe adverse effects. Janus kinase inhibitors, like oclacitinib, might be a valuable treatment option due to their rapid inhibition of the action of interferons known to be relevant in the pathogenesis of CCLE. To report the efficacy and safety of oral oclacitinib for the treatment of canine CCLE variants. Seven dogs were diagnosed with CCLE based on clinical signs and compatible histopathological findings. Oclacitinib was administered at the induction dosage of 0.45 mg/kg twice daily to 1.8mg/kg once daily. The response to treatment was graded as 'good' when there was ≥50% lesion reduction, or as 'complete remission' if all active lesions had resolved. Complete blood counts were performed at variable intervals. A complete remission of all lesions was obtained in the dog with exfoliative cutaneous lupus erythematosus, both dogs with mucocutaneous lupus erythematosus and three of four dogs with facial discoid lupus erythematosus (FDLE); a good response was seen in the remaining dog with FDLE. The first visible improvement of signs was seen within 2-to-3 weeks, while the time to complete remission was around 2 months. Clinical adverse effects were not seen, and haematological parameters remained within the reference range. Oclacitinib may be considered an effective treatment option for different variants of canine CCLE.

P06: Oral mucosal melanoma: not the same as the cutaneous variant

P06: Oral mucosal melanoma: not the same as the cutaneous variant

A CASE OF DISSEMINATED INTRAVASCULAR LYMPHOMA PRESENTING AS SEPSIS WITH MULTIORGAN FAILURE

TOPIC: Critical Care TYPE: Fellow Case Reports INTRODUCTION: Intravascular lymphoma (IVL) is a rare subtype of lymphoma proliferating in the lumen causing small vessel occlusion. Antemortem diagnosis is difficult. The majority of cases are diagnosed from autopsy. We report a patient with IVL who presented with sepsis of unknown source complicated by multiorgan failure. CASE PRESENTATION: A 58-year-old male with hypertension and chronic obstructive lung disease presented with fever for 3 weeks and was found to be hypotensive. Laboratory investigation was remarkable for platelet count of 84,000 per microliter, serum creatinine 3 mg/dl, aspartate aminotransferase of 50 units per liter (U/L), total bilirubin of 5 mg/dl, ferritin of 1265 microgram per liter, lactate dehydrogenase of 2051 U/L. Blood, respiratory and urine cultures were negative. CT abdomen and pelvis did not reveal a septisis source. Transthoracic echocardiogram showed a preserve ejection fraction without tamponade. Hemodynamic data from pulmonary artery catheter showed wedge pressure of 14, and mixed venous oxygen saturation of 72. He was diagnosed with septic shock with unknown source and multiorgan failure. He was treated with broad spectrum antimicrobials including Meropenem, Vancomycin and Micafungin. He was started on continuous renal replacement therapy. His clinical course continued to deteriorate. The family decided to withdraw life support. He expired on hospital day 11. An autopsy revealed intravascular large B-cell lymphoma. DISCUSSION: IVL is an extremely rare extranodal lymphoma, predominantly affecting elderly patients. The incidence remains unknown due to its rarity. The clinical presentation is variable, ranging from asymptomatic, constitutional B symptoms to multiorgan failure caused by vessel occlusion. IVL can be diagnosed by the presence of large lymphoma cells within small-to-medium blood vessels. Antemortem diagnosis of IVL is difficult due to lack of pathognomonic features or tumor markers. Symptoms could mimic other more common diseases since the clinical manifestations are heterogeneous which could lead to a delayed or missed diagnosis. IVL is aggressive and usually disseminated at the time of diagnosis. Treatment is systemic chemotherapy. CONCLUSIONS: While IVL is an extremely rare subtype of lymphoma, it should be considered in the differential diagnosis of unexplained multiorgan failure with no other clear cause. The diagnosis of IVL requires a high index of suspicion and should be suspected in patients suggestive relevant clinical findings which may lead to early treatment and better outcomes. REFERENCE #1: Bouzani M et al., "Disseminated intravascular b-cell lymphoma: clinicopathological features and outcome of three cases treated with anthracycline-based immunochemotherapy," The Oncologist, vol. 11, no. 8, pp. 923–928, 2006. REFERENCE #2: Ferreri AJ, Campo E, Seymour JF; International Extranodal Lymphoma Study Group (IELSG). Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'. Br J Haematol. 2004 Oct;127(2):173-83. REFERENCE #3: Murase T, Yamaguchi M, Suzuki R et al. Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood. 2007 Jan 15;109(2):478-85. DISCLOSURES: No relevant relationships by Maythawee Bintvihok, source=Web Response No relevant relationships by Parneet Dhaliwal, source=Web Response No relevant relationships by Joseph Guileyardo, source=Web Response No relevant relationships by Adam Hayek, source=Web Response No relevant relationships by Adan (Adam) Mora, source=Web Response

An international, multicenter, retrospective study on the positive impact of cutaneous involvement on the clinical outcome of adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma (ATLL) is a largely incurable disease. Cutaneous involvement is common and could be first symptom of the disease. We analyzed 169 patients with ATLL of whom 63 had cutaneous involvement. Cutaneous involvement was found in 48, 27, 17, and 60% of acute, lymphomatous, chronic and smoldering ATLL cases, respectively. Eight cases had primary cutaneous tumoral variant. Erythroderma (24%) and plaques (22%) were the most frequent skin lesions. The presence of cutaneous involvement was associated with better overall survival compared to non-cutaneous involvement (aHR 0.55 [95% CI: 0.37–0.82], p < 0.01; 1-year OS 53 vs. 27%, respectively, p = 0.012). Combination zidovudine and interferon-alpha (AZT-IFN) yielded high response rates (overall response, OR = 100%, n = 8; complete response 62.5%) compared to chemotherapy (OR = 33.3%, n = 12/36). In conclusion, cutaneous involvement was associated with better survival in Latin American patients with ATLL. AZT-IFN demonstrated encouraging responses in ATLL patients with cutaneous involvement.

Results of NC-6300 (nanoparticle epirubicin) in an expansion cohort of patients with angiosarcoma.

11543 Background: NC-6300 is a polymeric micelle exhibiting increased tumor accumulation compared to small-molecule epirubicin through enhanced pharmacokinetics and controlled release within the tumor through a pH-sensitive linker conjugated to epirubicin. In a phase 1b trial, which accrued twenty-nine patients with various types of sarcoma as well as solid tumors, observed dose-limiting toxicities included thrombocytopenia, stomatitis, lung infection, and febrile neutropenia. The maximum tolerated dose and the recommended phase 2 dose were determined to be 185 mg/m2 and 150 mg/m2, respectively. The objective response rate (ORR) in soft tissue sarcoma subset (n = 17) was 18% with both angiosarcoma patients achieving partial response. To further evaluate the anti-tumor activity of NC-6300 in angiosarcoma, we conducted an expansion cohort of 10 additional angiosarcoma patients. Methods: Eligible patients, at least age 18 years old, with histologically confirmed angiosarcoma, including cutaneous and non-cutaneous variants, not amenable to curative treatment with surgery or radiotherapy were included. No more than two lines of prior systemic therapy were allowed. NC-6300 was administered at the dose of 150 mg/m2 intravenously on Day 1 of a 21-day cycle. Treatment was continued until disease progression or unacceptable toxicity. Disease assessment was performed every 6 weeks using RECIST v1.1. The primary endpoint was median progression-free survival (mPFS). Results: Ten patients (cutaneous: 2 pts; non-cutaneous: 8 pts) were enrolled and deemed evaluable. Median line of prior systemic treatment in the advanced disease setting was 1.0 and seven patients (70%) received prior anthracycline therapy. Objective response rate (ORR) was 30% (cutaneous: 1 pt, non-cutaneous: 2 pts) and mPFS was 5.4 months (95% CI: 1.2-NA). Across all angiosarcoma patients included in the phase 1 portion and expansion cohort (phase 1b portion: 2 pts, expansion cohort: 10 pts), ORR and mPFS was 42% and 7.3 months (95%CI: 3.3-NA), respectively. All patients enrolled in the expansion cohort experienced grade 3/4 AEs and no treatment related death was observed. Most frequent grade 3/4 AEs were neutropenia without fever (80%), thrombocytopenia (40%), anemia (20%) and leukopenia (20%). AEs led to NC-6300 dose reduction and medication withdrawal were seen in 70% and 10% of patients, respectively. Conclusions: Promising anti-tumor activity was observed in this cohort of patients with cutaneous and non-cutaneous angiosarcoma. The safety profile of this expansion cohort was consistent with previous clinical study results of NC-6300. Our study results warrant further development of NC-6300 for angoisarcoma. Clinical trial information: NCT03168061.

POS0424 DETERMINING CIRCULATING ENDOTHELIAL CELLS USING CELLSEARCH SYSTEM IN SYSTEMIC SCLEROSIS PATIENTS

Background:Endothelial damage and fibroproliferative vasculopathy of small vessels are pathological hallmarks of Systemic Sclerosis (SSc). Detection and analysis of circulating endothelial cells (CECs) detached from affected blood vessels may be an informative tool to study vascular dysfunction and could be considered a novel biomarker of scleroderma vasculopathy. Our group first showed the presence of CECs in SSc by fluorescence-activated cell sorting (FACS), demonstrating that a raised counts of active CECs may represent direct evidence of active vascular disease in SSc. Despite these interesting data, issues related to difficulties in CEC counting through FACS analysis, due their very low concentration in peripheral blood, prevented further investigations in this field. Recently, a specific kit for the detection of CECs has been developed through the CellSearch System (CS), a semi-automated device for the standardized analysis of rare cells, such as CECs, in peripheral blood.Objectives:To assess the counts of CECs determined by the CS in SSc patients and to evaluate their clinical implication and potential as vascular biomarker in SSc.Methods:10mL of blood samples were collected from 29 subjects (19 SSc patients and 10 healthy donors - HDs) and stored in tubes containing a specific preservative, to allow the analysis of 4mL of blood within 72 hours, according to manufacturer instructions. Out of 19 SSc patients, 18 were female, 10 had the limited form and 9 the diffuse cutaneous variant of SSc. CS uses a proprietary kit containing a ferrofluid-based reagent, that target CD146 to magnetically capture CECs, and the immunofluorescent reagents to stain the CECs, defined as CD146+, CD105-PE+, DAPI+ and CD45-APC-. Clinical, laboratoristic and demographic data were also collected.Results:The mean number of CECs in patients with SSc was significantly higher in comparison to HDs (554/4mL vs. 53.5/4 mL, p=0.0042). When analyzed according to disease subset, both lcSSc and dcSSc showed significantly increased levels of CECs in comparison with HDs (p=0.003 and p=0.005, respectively). No statistical difference was observed in the mean number of CECs in patients with lcSSc compared to those with dcSSc. Regarding vascular involvement, the CECs counts strictly correlated with the presence of digital ulcers (DUs) (p=0.0001) showing a median of 863cells/4mL for the SSc patients with DUs versus a median of 276.2/4mL for the SSc patients without DUs. No statistical correlation was found between CECs and serological autoantibody pattern, skin parameters, or joint and muscle involvement. Patients with active disease, according to the EUSTAR Activity Index, showed a higher CECs value than those with inactive disease (p=0.0012).Conclusion:The amount of CECs detectable in peripheral blood has been recently proposed as a marker of endothelial damage in different vascular diseases, including SSc. However, currently no standardized method is available to determine CEC counts, which makes reported data on CECs reliable and suitable. The CS system is a commercially available semi-automated system that enables standardized determination of CECs. Thus, we examined clinical utility of CECs count by this system in SSc patients. Our results confirm that baseline CEC counts, evaluated by a new standardized method, may represent direct evidence of endothelial damage in SSc and could be a promising tool for monitoring active disease and evaluating therapeutic responses to vascular and immunosuppressive treatments.

POS0289 CANCER RISK IN IMMUNOSUPPRESSED SCLERODERMA PATIENTS: A PROPENSITY SCORE MATCHING ANALYSIS

Background:An increased incidence of cancer in patients with systemic sclerosis (SSc) is well-established1. Current knowledge about the onco-transforming power of immunosuppressive treatments in both non-rheumatological and rheumatological pathologies, suggests an increased incidence of hematological and solid neoplasms2. Evidences on the possible role of the immunosuppressants in the onset of cancer during SSc are lacking.Objectives:To evaluate the incidence of malignancies in SSc patients exposed to immunosuppressive therapy.Methods:The incidence of neoplasia in a cohort of 600 patients with SSc was evaluated retrospectively. Patients diagnosed with malignancy within 36 months from SSc onset were excluded from the analysis since suspected for paraneoplastic form. Patients exposed to methotrexate, cyclophosphamide, azathioprine and mycophenolate were confronted with a group comparable for age at onset, sex, disease variant, autoantibodies and exposure to other disease-specific therapies, using propensity score matching analysis. The considered follow-up was between the clinical onset and the diagnosis of cancer or the last available visit or the twenty-fifth year of illness.Results:The analysis was carried out on 526 patients observed for an average period of 12.1 ± 6.0 years (males 9.5%, age at onset 49.0 ± 15.3 years); 24.9% had diffuse cutaneous variant of the disease and 39.0% and 34.8% were respectively positive for anti-centromere and anti-Scl70 antibodies. During the follow-up, 19.0% of patients were exposed to cyclophosphamide, 15.3% to azathioprine, 14.4% to methotrexate and 11.6% to mycophenolate mofetil. Sixty-five cancer diagnoses were made after the 36th month from onset (incidence 1.02: 100 patients/year), comprising 16 mammary cancers, 12 cancers of the gastro-intestinal tract, 11 cancers of the head-neck area, 10 cancers of the lungs, 9 cases of skin-cancer, 5 haematological malignancies and 1 brain tumour. The incidence of cancers did not differ in relation to treatment with cyclophosphamide (X2 = 0.001, p = 0.961), azathioprine (X2 = 2.141, p = 0.143), mycophenolate mofetil (X2 = 0.001, p = 0.993) or methotrexate (X2 = 0.920, p = 0.337) (Figure 1).Conclusion:Our data are consistent with an increased incidence of neoplasms in the course of SSc, with a rate that appears almost doubled compared to the general Italian population with similar sex and age distribution (0.55: 100 patients/year; Italian Association of Tumor Registries data3). In our SSc cohort this risk is independent of exposure to immunosuppressive drugs commonly used for the treatment of scleroderma disease.

A cross-sectional observational study of clinicodermatoscopic features in cutaneous lichen planus in Indian skin

Background: Lichen planus (LP) is a papulosquamous skin disorder characterized by violaceous polygonal papules and plaques associated with itching. Dermoscopy in LP shows variable forms of whitish structures that correlate with Wickham striae (WS), vascular structures, and pigmentary changes that aid in its diagnosis. Aim: To study the correlation between clinical and dermatoscopic features in cutaneous variants of LP in Indian skin. Method: Patients with LP presenting over a period of 1 year were included. Dermoscopy of the lesions in polarized mode was done using video dermatoscope − Dino-Lite Premier AM4113ZT model. Both clinical and dermoscopy findings were photographed, recorded, and studied. Result: Fifty-six percent patients (39/70) had classical LP (CLP), 14% patients (10/70) had hypertrophic LP which were the most common clinical variants. Reticulate pattern of WS was the most common pattern observed in 40% (28/70) cases and a new rosette pattern of WS was observed in 13% (9/70) cases. Nonvascular findings such as WS, comedo-like openings, and grey blue dots showed statistically significant association with CLP. Among the pigmentary findings, pepper-like pigmentation was seen in 64% (25/39) CLP and reticulate pattern of pigmentation in 85% (6/7) cases of LP pigmentosus. Perifollicular pigmentation showed statistical significance in 100% cases of lichen planopilaris. Conclusion: In view of consistent dermoscopic features observed in LP, it aids as a valuable noninvasive diagnostic tool, many a times obviating the need for skin biopsy. Limitations: Small sample size and the exclusion of lesions of LP over the nails, genitals, and oral mucosa.

Tumor Lysis Syndrome: Introduction of a Cutaneous Variant and a New Classification System.

Tumor lysis syndrome, an oncological emergency, is characterized by laboratory parameters such as hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia, as well as renal injury with an elevated creatinine. Tumor lysis syndrome is seen in patients with aggressive malignancies and high tumor burden. More frequently, it occurs in individuals with hematologic malignancies such as high-grade lymphomas (such as Burkitt lymphoma) and leukemia (such as acute lymphocytic leukemia). It also, albeit less commonly, can be seen in patients with widespread solid tumors that are rapidly proliferating and are markedly sensitivity to antineoplastic therapy. Tumor lysis syndrome is usually preceded by cancer-directed therapy; however, the syndrome can present spontaneously prior to the individual receiving malignancy-directed treatment. We reported a man with metastatic salivary duct carcinoma who had cutaneous metastases that presented as carcinoma hemorrhagiectoides. Microscopic examination demonstrated that the metastatic tumor cells had infiltrated and replaced the entire dermis. After the patient received his first dose of antineoplastic therapy, he had an excellent response and the cutaneous metastases developed into ulcers; we hypothesize that most of the dermis, which had been replaced by tumor cells, disappeared as a result of the therapeutic response, and the overlying epidermis became necrotic and shed, leaving an ulcer. His dramatic response to treatment prompted us to propose a new classification of tumor lysis syndrome, which should include the systemic form of the condition as well as the new variant: cutaneous tumor lysis syndrome. We anticipate that, with improvement in targeted therapies, there may be an increase in therapy-associated cutaneous tumor lysis syndrome.