Cutaneous Involvement Research Articles

Neuro-leishmaniasis with cauda equina syndrome and cranial nerve palsy: a rare manifestation of recurrent atypical visceral leishmaniasis

BackgroundVisceral leishmaniasis (VL) is a neglected tropical disease primarily affecting Brazil, East Africa, and India, with India accounting for 18% of the global burden. While VL typically presents with systemic symptoms like fever, weight loss, and splenomegaly, it can occasionally manifest atypically, posing significant diagnostic challenges. Neurological presentations of VL are extremely rare, making them difficult to suspect and diagnose. Cases where VL predominantly presents with neurological symptoms are particularly novel, underscoring the need for heightened awareness of such atypical manifestations in endemic regions.Clinical caseA 38-year-old man with history of recurrent atypical VL presented with diffuse lower back pain, progressive tingling, numbness, weakness in the lower extremities, and double vision for one month. Clinical and radiological evaluations suggested cauda equina syndrome and cranial nerve palsy, accompanied by generalized lymphadenopathy, subcutaneous nodules, and skin papules. The differential diagnosis initially included disseminated tuberculosis, histoplasmosis, and lymphoma. Cerebrospinal fluid (CSF) analysis revealed an inflammatory syndrome. Histopathology of lymph node and bone marrow revealed Leishmania amastigotes and subcutaneous nodule and skin biopsy revealed inflammatory cells with granulomas. Furthermore, the qPCR test on DNA from a subcutaneous nodule, lymph node, and CSF was positive for Leishmania kinetoplast DNA. The species was further confirmed as Leishmania donovani through ITS-based PCR amplification and sequencing. Finally, a diagnosis of relapse of VL with lymph node, cutaneous, and neurological involvement, including abducens nerve palsy and cauda equina syndrome, was established. He was treated with combination of liposomal amphotericin B and miltefosine, along with intrathecal hyaluronidase, resulting in significant improvement.ConclusionUnlike previously reported cases with both systemic and neurological symptoms, our patient predominantly presented with neurological manifestations, making this a unique and novel presentation of VL. This case highlights diagnostic challenges and management of atypical VL, emphasizing neurological involvement and successful therapeutic strategies.

Scalp Lesion as the First Manifestation of Pancreatic Adenocarcinoma, A Very Rare Case.

Pancreatic adenocarcinoma is one of the most aggressive tumors. Its diagnosis is usually made in locally advanced or metastatic disease and survival is less than one year. The most frequent sites of metastatic involvement are the liver, peritoneum and lungs. Other organs such as the bones or the brain may be affected to a lesser extent. Cutaneous involvement of pancreatic adenocarcinoma is extremely rare with less than 150 cases reported in the literature since 1960. Most cases with cutaneous involvement involve the periumbilical region, in a lesion known as "Sister Mary Joseph's Node". Scalp metastases are very rare and their diagnosis suggests advanced disease and the prognosis will be dismal. It is very important to perform a complete physical examination and a global anamnesis to guide the request for diagnostic tests. Once the diagnosis of pancreatic adenocarcinoma has been made, a global assessment will be necessary, involving different medical specialists, nurses, psychologists and social workers among others. In many cases, supportive care is the mainstay of treatment.

Sarcoidosis Treatment Patterns in the United States: 2016-2022

There are limited FDA-approved medications and real-world data on sarcoidosis treatment in the U.S. and concordance of practice patterns with guideline recommendations have not been well characterized. What are the practice patterns and factors associated with treatment for patients with sarcoidosis in the year following diagnosis? We conducted a retrospective analysis of patients with sarcoidosis from 2016 - 2022 using a multicenter, all-payer, claims database (TriNetX). We ascertained treatments with corticosteroids and/or non-steroidal immunosuppressive medications (methotrexate, mycophenolate, leflunomide, hydroxychloroquine, cyclophosphamide, infliximab, adalimumab, azathioprine, rituximab, and janus kinase inhibitors) within one year of diagnosis. We summarized treatment rates, sequence of prescribed medications by mean rank, and used multivariable logistic regression analyses to identify factors associated with treatment. Out of 13,330 patients with sarcoidosis meeting inclusion, 5,671 (42.5%) received treatment within a year of diagnosis. Of those treated, 60% received steroids alone, 13% received non-steroidal immunosuppressives alone, and 27% received both. Further, 25% of treated patients received a non-steroidal immunosuppressive as their first medication. Corticosteroids had the lowest mean rank order, indicating they were, on average, the first medication initiated. Among those with pulmonary or cutaneous involvement, the second medication initiated on average was hydroxychloroquine, while in those with cardiac or neurologic involvement it was adalimumab and mycophenolate, respectively. Factors associated with higher odds of treatment were Black race, organ involvement at baseline (pulmonary, cardiac, and neurologic), and comorbid diagnoses (fatigue, hypercalcemia, and ILD). Within the first year of diagnosis, 43% of patients with sarcoidosis were started on treatment. Non-steroidal immunosuppressives were used in 40% of treated patients. While factors associated with treatment initiation aligned with guideline recommendations, practice patterns of treatment were variable, particularly in choice and sequence of non-steroidal immunosuppressive therapy, underscoring the need for future trials and comparative effectiveness studies.

An update on EBV-related cutaneous lymphoproliferative disorders: a systematic review.

Epstein Barr virus (EBV) positive B lymphoproliferative disorders (LPD) with cutaneous involvement include a series of rare entities that go from indolent processes to aggressive lymphomas. Since the latest WHO-EORTC classification of cutaneous lymphomas in 2018, significant changes have been included in the new classifications of hematological malignancies. B-cell EBV+ LPD mainly affect immunocompromised patients while T-cell EBV+ LPD are more prevalent in specific geographic regions such as Asia, Central America, and South America. This systematic review summarizes the main clinical, histological, immunophenotypic and molecular characteristics of B- and T-cell EBV+ LPD that may compromise the skin at diagnosis. B-cell EBV+ LPD include primary cutaneous lymphomas such as EBV-MCU, as well as cutaneous systemic lymphomas that may present such as lymphomatoid granulomatosis (LG), EBV+ diffuse large B cell lymphoma, NOS, plasmablastic lymphoma (PBL), and extracavitary primary effusion lymphoma (EC-PEL). EBV+, EBV-positive polymorphic B cell LPD, or post-transplant lymphoproliferative disorders (PTLD). Regarding T-cell EBV+ LPD, most of these entities are categorized within T/NK-cell lymphoproliferative processes and lymphomas of childhood, including extranodal T/NK lymphoma, and even more exceptional forms such as EBV-positive T-cell centrofollicular lymphoma and intravascular T/NK-cell lymphoma. Diagnosis is based on integrating the clinical, histological, immunohistochemical, and genetic criteria discussed throughout this article. Management is multidisciplinary, posing a challenge for dermatologists and hematologists involved in the management of these patients. Having scientific evidence available in this field is of paramount importance because overtreatment must be carefully avoided.

Left atrial volumetric-mechanical coupling index as a marker of subclinical primary heart involvement in systemic sclerosis

Abstract Background Primary heart involvement in systemic sclerosis (SSc) is a common yet often clinically silent marker of poor prognosis. Up to 80% of patients have histopathological evidence of heart involvement, highlighting the need for better screening and diagnostic methods. N-terminal pro-brain natriuretic peptide (NTproBNP), a well-established cardiac biomarker, is often increased in patients with SSc, but with a not fully understood mechanism. Meanwhile, left atrial volumetric-mechanical coupling index (LACI), a new echocardiographic parameter, has been shown to predict cardiovascular outcomes in heart failure patients and the general population, but it has not been studied in SSc. Purpose To identify new echocardiographic parameters, particularly related to left atrial volume and function, as markers of increased NTproBNP in SSc patients without overt heart involvement. Methods Consecutive SSc patients without overt heart disease referred for routine cardiac screening underwent 2D and 3D transthoracic echocardiography, including atrial and ventricular strain analysis. LACI was calculated by both 2D and 3D echocardiography as the ratio between the left atrial volume index and the tissue-Doppler-imaging septal a’ velocity. Patients with significant structural and functional changes suggestive of systolic or diastolic dysfunction or pulmonary hypertension, and those with impaired global left ventricular strain were excluded. Logistic regression, Pearson correlation coefficients, and area under the receiver-operating characteristic curve (AUC) are used to analyze the association and predictive power of LACI for elevated NTproBNP. Results A total of 36 SSc patients (55±10 years old; 34 (94%) women; mean disease duration 14±11 years) were included. Sixteen (44.4%) had diffuse cutaneous involvement (dcSSc). Seventeen (47%) patients had increased NTproBNP levels (i.e. >125 pg/mL). Among all tested variables, 3D LACI had the best correlation with NTproBNP values (r = 0.63, p<0.001). A 3D LACI of >2 associated with NTproBNP of >125 pg/mL (odds ratio 7.33 [95%CI 1.38-38.8]; p=0.01) and predicted these increased values with a sensitivity of 76.9% and a specificity of 81.2% (AUC 0.78). NTproBNP was also correlated with 2D LACI (r=0.55, p<0.001), peak left atrial longitudinal strain (r=-0.42, p=0.01), and left atrial contraction strain (r=0.48, p=0.005). Compared to patients with limited cutaneous involvement, those with dcSSc had higher NTproBNP values (250±139.6 vs 107.2±110.9 pg/mL, p=0.001), higher 3D LACI values (2.6±0.9 vs 1.9±0.5, p=0.02), with similar left atrial and ventricular strain parameters. Conclusion Left atrial volumetric-mechanical coupling index assessed by 3D echocardiography is significantly associated with and predictive of elevated NTproBNP levels in SSc patients without overt heart disease and may be a potentially useful non-invasive marker for screening for early subclinical heart involvement in SSc patients.

A cross‐sectional study on quality of life in adult indolent systemic mastocytosis and its association with cutaneous involvement

AbstractBackgroundSystemic mastocytosis (SM) is characterized by abnormal mast cell accumulation in various tissues, including the skin, and encompasses several clinical variants, ranging from less aggressive to more severe forms. ISM, the most common variant, often presents with cutaneous manifestations, causing significant impairment in QoL due to mediator‐release symptoms and stigma associated with typical cutaneous lesions.ObjectivesThe study aimed to assess QoL impairment in ISM patients and its correlation with demographic and clinical data.MethodsA cohort of 52 adult ISM patients was evaluated using the Dermatology Life Quality Index (DLQI) and the mastocytosis QoL questionnaire (MC‐QoL). Questionnaire scores were then analyzed in relation to the clinical and demographic characteristics of the patients.ResultsOver half of the patients experienced mild or higher levels of impairment in QoL, with female patients showing greater impairment than males. Patients with recent symptom onset reported higher MC‐QoL scores, possibly due to psychological factors and lack of disease knowledge. Cutaneous symptoms significantly impacted QoL, and the visibility of lesions affected DLQI scores more than MC‐QoL scores. A moderately strong correlation was observed between DLQI and MC‐QoL scores, particularly in the Skin and Social Life domains. The study highlights gender differences in QoL impairment, suggesting the need for further investigation into potential social or cultural factors.ConclusionsWhile the study provides valuable insights into QoL impairment in ISM patients, its monocentric nature and small sample size are notable limitations. Further research is warranted to better understand the impact of SM on patients' well‐being and to identify effective strategies for managing symptoms and improving overall QoL in this population.

Real-World Evaluation of the Effectiveness and Safety of Dupilumab in Bullous Pemphigoid: An Ambispective Multicenter Case Series.

Bullous pemphigoid affects elderly individuals with multiple comorbidities, making conventional treatments unsuitable. Evaluate the effectiveness and safety of dupilumab in the treatment of bullous pemphigoid. A multicenter ambispective cohort study was conducted in 34 hospitals. Patients with bullous pemphigoid treated with Dupilumab were included. Most of patients (97.1%) received an initial 600 mg dose followed by 300 mg every two weeks. The primary outcome was the proportion of patients achieving complete remission within 4 weeks, defined as Investigator Global Assessment score of 0 or 1. Complete remission at weeks 16, 24, and 52, adverse events, reductions in peak pruritus numerical rating scale, and systemic glucocorticoid use were also assessed. The study included 103 patients with a median age of 77.3 years, 58.0% male. Complete remission was achieved by 53.4% within 4 weeks and 95.7% by week 52. Peak pruritus scale reduced by 70.0% by week 4 and was completely controlled by week 24. Thirteen patients presented adverse events, most of which were mild. Systemic glucocorticoid use reduced by 82.1% by week 52. Shorter disease duration and exclusive cutaneous involvement predicted better response at 16 weeks. No differences in response rates to dupilumab were observed between drug-associated bullous pemphigoid and idiopathic cases. No significant difference in response rates was observed between patients treated with dupilumab in monotherapy and those receiving dupilumab with concomitant treatments. Dupilumab is effective, rapid, and safe in managing bullous pemphigoid, reducing the need for corticosteroids and other treatments. Early initiation and exclusive skin involvement predict better outcomes.

Multisystem ALK-Positive Histiocytosis With DCTN1::ALKFusion in an Adult, Responsive to Alectinib: Case Report and Literature Review.

Anaplastic lymphoma kinase (ALK)-positive histiocytosis has emerged as a clinically relevant diagnosis featuring a wide span of clinical presentations, which are unified by the presence of ALK-positive histiocytes on histopathology and molecular drivers involving the ALK kinase gene. This report presents an adult case of multisystem ALK-positive histiocytosis with xanthogranuloma-like features on histopathology that was responsive to ALK inhibition, and includes a review of ALK-positive histiocytoses with cutaneous involvement reported in the literature. A 56-year-old male developed a widespread eruption of red-brown papules on the face, trunk, and upper extremities. Histopathological evaluation revealed a well-circumscribed, nodular dermal infiltrate of epithelioid histiocytes with Touton giant cells, rare bizarre multinucleated cells, and focal emperipolesis. The lesional cells were positive for CD68 and ALK1 immunohistochemical stains, and negative for CD1a. Next-generation sequencing identified a DCTN1::ALK fusion. On imaging, he was found to have bone, lung, soft tissue, and salivary gland involvement. ALK inhibition was initiated with alectinib, resulting in rapid improvement of cutaneous lesions and eventual complete resolution of abnormal imaging findings, which was sustained at 24 months of follow-up. This case adds to the spectrum of ALK-positive histiocytoses and further demonstrates the positive response with targeted therapy.

An Unusual Cause of Erythroderma.

Indeterminate dendritic cell tumors are exceedingly uncommon and have been described mainly in adults with cutaneous involvement mainly in the form of nodules only. To the authors’ knowledge, this is the first case of an erythrodermic presentation of underlying IDCT. This condition is known to involve the skin in existing reports and is important for a dermatologist to suspect in order to facilitate the correct diagnosis and treatment.

Coping style and psychopathological morbidity in patients with advanced melanoma: differences between cancer therapy, metastatic location and staging.

The incidence of melanoma has been steadily increasing in recent years and remains one of the most frequent cancers in adolescents and young adults. The literature shows how a high percentage of melanoma patients present with high anxiety and depression scores and a correlation between worsening quality of life and impaired body image has been shown. The present study aimed to investigate the relationship between cutaneous and visceral metastasis in a group of 73 patients with advanced melanoma in relation to body image, anxiety, depression, and coping style. Hospital Anxiety and Depression Scale, Body Image Scale and Mini-Mac questionnaires were administered to a sample of 73 subjects with advanced melanoma (stage III and IV); Analyses were performed with SPSS version 28.01 and a P value <0.05 was considered significant. Emerging results indicated greater body image impairment in patients with skin metastasis, no significant difference in anxiety and depression by staging. Patients receiving target therapy showed a reduction in anxiety and depression compared with patients receiving immunotherapy. In line with the literature, cutaneous metastatic involvement negatively affected body image, a prevalence rate of anxiety and depression of 28,8% and 19.2%, respectively, emerged in the sample, while 17.8% reported significant body image impairment, suggesting how melanoma impacts in patient's quality of life.

Clinicopathological characteristics of extranodal Rosai-Dorfman disease: A retrospective case series of 25 patients

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis, classically affecting the lymph nodes. Even rarer extranodal disease is diagnostically challenging due to overlapping histologic features with other entities and lack of a universally agreed set of biomarkers. Cyclin D1 immunohistochemistry (IHC) may serve as a useful adjunct in diagnosing extranodal RDD. We present a retrospective case series of patients diagnosed with extranodal RDD between January 2013 and December 2023. IHC staining for cyclin D1 was performed on archived tissue samples. Baseline IHC results for biomarkers supporting the RDD diagnosis were recorded along with patient demographic characteristics, clinical features, and disease outcomes. A total of 25 patients with extranodal RDD were included: 21 women (84 %) and 4 men (16 %). The mean age at diagnosis was 42.6 years. Cutaneous and deep tissue involvement was seen in 5 (20 %) and 20 (80 %) patients, respectively. 11 patients (44 %) had disease localized to the trunk and extremities, and 13 had disease in the head and neck region (52 %), of which 5 occurred in the nose and paranasal tissues. Available follow-up data showed most patients fully recovered (n = 11; 78.6 %). However, 1 patient had disease recurrence, 1 developed blindness, and 1 developed deafness. Cyclin D1 IHC was positive in all samples (100 %), consistent with previous studies. The clinicopathologic findings in this study highlight the spectrum of potential disease sites, possible morbid outcomes related to disease site, and the diagnostic utility of cyclin D1 IHC.

Hydroxychloroquine monotherapy in sarcoidosis: Indications, efficacy, and side effects.

In sarcoidosis which is not threatening vital internal organs such as heart, central nervous system or lungs immunomodulatory treatment, such as hydroxychloroquine can be considered. Despite its common use, limited data are available regarding effectiveness and side effects as monotherapy in sarcoidosis. Recommendations on its usage are based on expert opinion as literature is scarce. This retrospective study examines real-world data about the indications, effectivity and side effects of hydroxychloroquine monotherapy in sarcoidosis patients with no damage to the vital internal organs. Successful treatment was defined as continuation after 24 weeks without step up therapy or worsening of symptoms. Sixty patients were eligible for the study. Starting dose was 400mg/day, lowered to 200mg/day after 3 months in most patients. The predominant treatment indications were musculoskeletal 45 (75%) and cutaneous involvement 13 (22%). Thirty-three patients (55%) continued treatment after 24 weeks. Twenty-four patients (40%) mentioned side effects, mainly gastrointestinal, leading to treatment discontinuation in eleven patients (18%). No severe side effects were seen. Continuation after 24 weeks was significantly higher in patients with cutaneous involvement compared to other indications 85% vs 47% respectively (p =0.02). Treatment with hydroxychloroquine monotherapy was satisfactory in 55% of patients, especially for cutaneous involvement. However, it poses considerable non-severe side-effects.

Solitary erythematous nodule on the breast

In this clinicopathological case, we describe the case of a female in her sixties with relapsed IgG kappa plasma cell myeloma, commonly known as multiple myeloma (MM), on no active therapy who presented to dermatology with a solitary erythematous nodule on her right breast, biopsied as cutaneous involvement by MM. We include clinical, dermoscopic, and histopathologic images of the patient’s striking lesion. We then provide a brief review of the literature on cutaneous infiltration by MM along with discussion of the differential of plasma cell neoplasms of the skin, which can appear similar on H&amp;E, but are differentiated by immunohistochemical staining.

Predicting the risk of cardiovascular and cerebrovascular event in systemic lupus erythematosus: a Chinese SLE treatment and research group study XXVI

ObjectivePatients with systemic lupus erythematosus (SLE) have an increased risk of cardiovascular and cerebrovascular events (CCEs). Furthermore, CCE was a significant factor contributing to mortality in patients with SLE. However,...

The spectrum of idiopathic inflammatory myopathies: a Tunisian cohort

Background Idiopathic inflammatory myopathies (IIM) encompass a heterogenous group of auto-immune diseases. The identification of myositis specific antibodies (MSA) and their associations with distinct phenotypes has improved the categorization of these conditions. Objective The aim of this study was to describe and report the clinical and immunological characteristics of IIM among Tunisian patients. Method A retrospective study conducted in the internal medicine department at the Rabta University Hospital Center over 22 years, including adult patients with IIM according to the American college of rheumatology/European league against rheumatism (ACR/EULAR) classification criteria and Connors’ criteria for anti-synthetase syndrome (ASS). Inclusion body myositis and myositis associated with other conditions were excluded. Demographic, clinical, and immunological characteristics were analyzed and compared. Results Ninety-seven patients were included (Male/female ratio= 0.36, mean age = 48.4 +- 13.8 years). Muscular involvement was present in 88% of patients, affecting locomotor muscles (88%), gastrointestinal (43%), laryngeal (10%), cardiac (8%), and respiratory (1%) systems. Muscle weakness was primarily noted in the pelvic girdle (81%), scapular region (74%), axial muscles (20%), and distal muscles (5%). Myolysis was observed in 77% of patients, and histological evidence of myositis in 73%. Diffuse interstitial pneumonia (DIP) was present in 45% of patients, cutaneous involvement in 85%, and articular involvement in 48%. MSAs were detected in 52% of patients. Analysis revealed significantly higher frequencies of amyopathic forms, DIP, palmar hyperkeratosis, and articular involvement in the ASS group. The DM group exhibited higher frequencies of gastrointestinal signs, Gottron’s papules, heliotrope rash, photosensitive rashes, ulcerations, and skin necrosis. The NAM group had higher frequencies of gastrointestinal signs, myolysis, and lower frequencies of DIP and cutaneous involvement. Conclusion Our findings corroborate previously established clinico-immunological associations reported in the literature underscoring the need for a combined clinico-serological approach in classifying IIM.

Systemic Mycoses by Novel Onygenalean Fungal Pathogens Emergomyces spp and Blastomyces percursus in Rwanda.

We report 2 cases of infection by fungi unprecedented in Rwanda. One patient with emergomycosis presented with disseminated disease and prominent cutaneous involvement and one patient with African blastomycosis had cutaneous and osseous disease. These cases illustrate the clinicopathologic and molecular traits of novel dimorphic onygenalean species in Rwanda.

A Study on Clinical and Immunological pattern of Systemic Lupus Erythematosus patients in a Tertiary care Hospital – A Retrospective Study

Introduction Systemic Lupus Erythematosus (SLE) is a Chronic Multisystem Autoimmune disorder that exhibits a wide spectrum of clinical and immunological abnormalities ranging from localized cutaneous involvement to life-threatening systemic involvement. The heterogeneity of SLE manifestations is due to an interplay of genetic, environmental, and hormonal influences[1]. Onset occurs commonly in women of reproductive age, in the third and fourth decades of life, with a Male: Female ratio of about 1:10. The clinical course of the disease is unpredictable with periods of remission and flares.

Hereditary C1q Deficiency is Associated with Type 1 Interferon-Pathway Activation and a High Risk of Central Nervous System Inflammation

Hereditary C1q deficiency (C1QDef) is a rare monogenic disorder leading to defective complement pathway activation and systemic lupus erythematosus (SLE)-like manifestations. The link between impairment of the complement cascade and autoimmunity remains incompletely understood. Here, we assessed type 1 interferon pathway activation in patients with C1QDef. Twelve patients with genetically confirmed C1QDef were recruited through an international collaboration. Clinical, biological and radiological data were collected retrospectively. The expression of a standardized panel of interferon stimulated genes (ISGs) in peripheral blood was measured, and the level of interferon alpha (IFNα) protein in cerebrospinal fluid (CSF) determined using SIMOA technology. Central nervous system (encompassing basal ganglia calcification, encephalitis, vasculitis, chronic pachymeningitis), mucocutaneous and renal involvement were present, respectively, in 10, 11 and 2 of 12 patients, and severe infections recorded in 2/12 patients. Elevated ISG expression was observed in all patients tested (n = 10/10), and serum and CSF IFNα elevated in 2/2 patients. Three patients were treated with Janus-kinase inhibitors (JAKi), with variable outcome; one displaying an apparently favourable response in respect of cutaneous and neurological features, and two others experiencing persistent disease despite JAKi therapy. To our knowledge, we report the largest original series of genetically confirmed C1QDef yet described. Additionally, we present a review of all previously described genetically confirmed cases of C1QDef. Overall, individuals with C1QDef demonstrate many characteristics of recognized monogenic interferonopathies: particularly, cutaneous involvement (malar rash, acral vasculitic/papular rash, chilblains), SLE-like disease, basal ganglia calcification, increased expression of ISGs in peripheral blood, and elevated levels of CSF IFNα.

Treatment outcomes between cyclosporin and chemotherapy in adult subcutaneous panniculitis-like T-cell lymphoma: a report from nation-wide Thai lymphoma study group registry.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of T-cell lymphomas with a characteristic feature of subcutaneous nodules associated with hemophagocytic lymphohistiocytosis (HLH). Treatment options for SPTCL are mainly chemotherapy (CMT) or immunosuppressive agents with selection currently dependent on physician decisions. Outcomes between the 2 treatment remedies have not yet been comprehensively compared. This study aimed to compare complete remission (CR) rates between SPTCL patients receiving cyclosporin (CSA)-based regimen (CSA +/- steroid) and CMT. The 5-year overall survival (OS) and 5-year progression free survival (PFS) were also analyzed. Clinical data from patients with SPTCL were drawn from the Thai Lymphoma Study Group registry who were newly diagnosed between 2007 and 2023. A total of 93 patients were selected with 45 cases having received CSA-based regimen and 48 cases having received CMT. There were more patients with limited stage at skin in the CSA group (63.8% vs. 36.2%, p = 0.003), while more patients with hepato- and/or splenomegaly were found in the CMT group (56.2% vs. 24.5%; p = 0.002). Germline HAVCR2 mutations were detected in 26/33 (78.8%) cases. The CR rate was significantly higher in patients treated with CSA (87% vs. 58.3%; OR = 6.5 [95%CI, 2.7-15.3]; p = 0.002). At a median follow-up of 87.8 months (range 0-185), the 5-year OS (98% vs. 87%, p = 0.19) and PFS (72.4% vs. 69.2%, p = 0.19) showed a trend favoring patients treated with CSA. Based on our study, CSA-based regimens are the preferred first-line treatment remedy for newly diagnosed SPTCL, especially in patients with limited cutaneous involvement.

Cutaneous T cell Lymphoma in Skin of Color: A Review.

People of color (POC) affected by skin cancer suffer disproportionately from worse morbidity and mortality. Although skin cancers occur most frequently in White individuals overall, cutaneous T-cell lymphoma (CTCL) is an exception. CTCL is a rare skin cancer comprising several subtypes of non-Hodgkin lymphoma; each contains a unique clinical profile that varies with race. Our aim is to review and compile the differences in epidemiology, clinical presentation, treatments, and outcomes of the CTCL subtypes in Black, Asian or Pacific-Islander (API), and Hispanic patients. The current literature supports that there are nuances in the course of CTCL that differ with race. Across multiple studies, racial differences in incidence patterns have been reported, with the highest rates among Black patients. Cutaneous manifestation of CTCL are highly variable in POC, and the predilection for clinical CTCL variants often differs with race, as well as severity of cutaneous involvement (BSA). Response to and type of treatment also differs among POC, and may be partially attributable to the varying CTCL subtypes experienced by certain races. Prognostic factors tend to vary with race, although Black patients consistently experience poor outcomes, while API patients may have a more favorable prognosis. Currently, there is no definitive conclusion to account for differences observed in CTCL skin of color patients, however biologic and socioeconomic factors have been proposed as potential drivers. As POC comprise an increasing portion of our population, adequate physician awareness and knowledge of racial nuances in CTCL are necessary to begin addressing these disparities.