Primary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study.

BACKGROUND: Primary cutaneous lymphomas (PCLs) refer to cutaneous lymphomas that primarily develop in the skin with no evidence of extracutaneous disease at the time of diagnosis. The epidemiological and clinical data of PCLs in Thailand are lacking. OBJECTIVES: To evaluate the frequency, demographic data, and clinical characteristics of different subtypes of PCLs in a tertiary care university hospital. METHODS: In total, 137 patients with PCLs diagnosed in our hospital in 2008-2017 were retrospectively reviewed. RESULTS: Of the 137 patients, 57 (41.6%) were male and 80 (58.4%) were female (M : F = 1 : 1.4). The median age at diagnosis was 40 years. Most patients (134, 97.8%) had cutaneous T-cell lymphomas (CTCLs). Three patients (2.2%) had cutaneous B-cell lymphomas (CBCLs). The most common subtype was mycosis fungoides (MF) (67.9%), followed by subcutaneous panniculitis-like T-cell lymphoma (SPTCL) (21.2%), primary cutaneous anaplastic large cell lymphoma (pcALCL) (3.6%), lymphomatoid papulosis (LyP) (1.5%), primary cutaneous gamma/delta T-cell lymphoma (pcGDTCL) (1.5%), Sézary syndrome (SS) (0.7%), extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) (0.7%), primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS) (0.7%), primary cutaneous diffuse large B-cell lymphoma, leg type (pcDLBCL-LT) (1.5%), and primary cutaneous follicle center lymphoma (pcFCL) (0.7%). Most patients with MF presented with early-stage disease (84.0%), with hypopigmented MF the most common variant (42.6%). CONCLUSIONS: Compared to earlier Caucasian and Asian studies, the present study revealed a higher proportion of CTCL patients with a younger age at onset and a female predominance. MF was the most common CTCL subtype, followed by SPTCL. More than 80% of MF patients were diagnosed at an early stage.

Pancreatic Panniculitis and Polyarthritis.

PURPOSE OF REVIEW: Polyarthritis can have numerous reasons and may thus constitute a challenge for differential diagnosis. One rare potential reason for sterile polyarthritis is underlying pancreatic disease with systemic hyperlipasemia, most often accompanied by painful skin lesions caused by a subcutaneous inflammatory process known as panniculitis. Systematic evidence on pancreatic panniculitis and polyarthritis is limited, particularly regarding its feature as facultative paraneoplasia with underlying intra- or even extra-pancreatic malignancy. Therefore, we will summarize the current knowledge about this orphan disease including epidemiological, pathophysiological, diagnostic, and treatment aspects in the present review. RECENT FINDINGS: Although direct evidence is lacking, it is highly probable that pancreatic polyarthritis and panniculitis are caused by peripheral lipolytic activity of lipase systemically circulating due to benign (e.g., acute or chronic pancreatitis) or malign (e.g., acinar cell carcinoma (ACC) or adenocarcinoma) pancreatic disease. In the latter case, pancreatic polyarthritis and panniculitis are associated with poor outcome. Pancreatic polyarthritis and panniculitis should always be included into diagnostic considerations, and once suspected, a thorough work-up to identify the underlying disease has to be performed.

Survival of Subcutaneous Panniculitis-Like T-Cell Lymphoma and Peripheral T-Cell Lymphoma Not Otherwise Specified: A Propensity-Matched Analysis of the Surveillance, Epidemiology, and End Results Database.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare entity with no previous population-based study. MATERIALS AND METHODS: We used the Surveillance, Epidemiology, and End Results 18 database to identify adult patients with SPTCL and peripheral T-cell lymphoma not otherwise specified (PTCL NOS) diagnosed between 1973 and 2011. The actuarial survival of SPTCL was compared with a propensity-matched cohort of PTCL NOS. Multivariate analysis was conducted using weighted Cox proportional hazard regression model. RESULTS: Patients with SPTCL (n = 118), compared with PTCL NOS (n = 3296), were more likely to be younger (median age of 47 vs. 62 years; P < .01), women (67% vs. 40%, P < .01), and diagnosed with stage I/II disease (46% vs. 36%; P = .01). The 5-year actuarial, relative, and cause-specific survival for SPTCL was 40%, 57%, and 64%, respectively. After propensity-matching, the 5-year overall survival (OS) of SPTCL was better than that of PTCL NOS (57% vs. 40%; P < .01). In a multivariate analysis, mortality was significantly lower among SPTCL versus PTCL NOS (hazard ratio, 0.54; 95% confidence interval, 0.39-0.75; P < .01). Among patients with SPTCL, advanced age (P < .01) and diagnosis before the year 2008 (P = .02) were predictors of worse OS. CONCLUSION: Our study provides characteristics and OS of a large cohort of SPTCL. Compared with PTCL NOS, SPTCL patients were more likely to be younger, female, and diagnosed at an early stage. The OS of SPTCL was better than PTCL NOS.

Panniculitis is a common unrecognized histopathological feature of cutaneous leishmaniasis.

BACKGROUND: Cutaneous leishmaniasis (CL) is a parasitic cutaneous infection caused by Leishmania parasite. The histopathology is usually granulomatous in nature. AIMS: The aim of the present study is to elucidate the histology of CL and evaluate the presence and the frequency of panniculitis among the affected patients. SETTINGS AND DESIGN: Case series interventional study. MATERIALS AND METHODS: Thirty-five patients with CL were diagnosed clinically between December-2012 and May-2013. Diagnostic confirmation established by smears, culture, and polymerase chain reaction (PCR). The histopathological assessment was carried out to study the general pathology and to look for the presence of panniculitis. STATISTICAL ANALYSIS USED: Simple statistics utilized via SPSS version 16.0 (SPSS, Inc., Chicago, USA). RESULTS: Eighteen women and 17 men with CL were enrolled in the present work with a mean duration of their disease was 3 months. The results of the diagnostic tests were as follow: The smear was positive in 21 (60%) of cases, Leishman-Donovan (LD) bodies were seen in 7 (20%) patients, culture was positive in 24 (68%), and PCR was positive in 32 (91.4%) patients. The epidermal changes included acanthosis, pseudoepitheliomatous hyperplasia, ulceration, focal spongiosis, and interface dermatitis while the dermal changes were dependent on the spectrum of the disease, so in the ulcerative lesions there was lymphohistiocytic infiltration with foci of plasma cells and sometimes aggregate of LD bodies, whereas in the dry lesions the pathology is mainly of epithelioid granuloma. Panniculitis was seen in 16 (46%) cases as a diffuse lymphohistiocytic infiltration of both the septum and lobules of the subcutaneous layer of the skin. CONCLUSION: Panniculitis is an important feature of CL that must be differentiated from other diseases that can simulate CL such as chronic skin infections, Discoid lupus erythematosus, and cutaneous lymphoma.

High prevalence of cardiovascular disease in South Asians: Central role for brown adipose tissue?

Cardiovascular disease (CVD) is the leading cause of death in modern society. Interestingly, the risk of developing CVD varies between different ethnic groups. A particularly high risk is faced by South Asians, representing over one-fifth of the world's population. Here, we review potential factors contributing to the increased cardiovascular risk in the South Asian population and discuss novel therapeutic strategies based on recent insights. In South Asians, classical ('metabolic') risk factors associated with CVD are highly prevalent and include central obesity, insulin resistance, type 2 diabetes, and dyslipidemia. A contributing factor that may underlie the development of this disadvantageous metabolic phenotype is the presence of a lower amount of brown adipose tissue (BAT) in South Asian subjects, resulting in lower energy expenditure and lower lipid oxidation and glucose uptake. As it has been established that the increased prevalence of classical risk factors in South Asians cannot fully explain their increased risk for CVD, other non-classical risk factors must underlie this residual risk. In South Asians, the prevalence of "inflammatory" risk factors including visceral adipose tissue inflammation, endothelial dysfunction, and HDL dysfunction are higher compared with Caucasians. We conclude that a potential novel therapy to lower CVD risk in the South Asian population is to enhance BAT volume or its activity in order to diminish classical risk factors. Furthermore, anti-inflammatory therapy may lower non-classical risk factors in this population and the combination of both strategies may be especially effective.

Menopause is a determinant of breast adipose inflammation.

Chronic inflammation is recognized as a risk factor for the development of several malignancies. Local white adipose tissue (WAT) inflammation, defined by the presence of dead or dying adipocytes encircled by macrophages that form crown-like structures (CLS), occurs in the breasts (CLS-B) of most overweight and obese women. Previously, we showed that the presence of CLS-B is associated with elevated tissue levels of proinflammatory mediators and aromatase, the rate-limiting enzyme for estrogen biosynthesis. The associated increased levels of aromatase in the breast provide a plausible mechanistic link between WAT inflammation and estrogen-dependent breast cancers. Thus, breast WAT inflammation could be relevant for explaining the high incidence of estrogen-dependent tumors with aging despite diminished circulating estrogen levels after menopause. To explore this possibility, we determined whether menopause in addition to body mass index (BMI) is associated with breast WAT inflammation among 237 prospectively enrolled women. The presence of CLS-B and its severity (CLS-B/cm(2)) as indicators of WAT inflammation correlated with menopausal status (P = 0.008 and P < 0.001) and BMI (P < 0.001 for both). In multivariable analyses adjusted for BMI, the postmenopausal state was independently associated with the presence (P = 0.03) and severity of breast WAT inflammation (P = 0.01). Mean adipocyte size increased in association with CLS-B (P < 0.001). Our findings demonstrate that breast WAT inflammation, which is associated with elevated aromatase levels, is increased in association with the postmenopausal state independent of BMI. Breast WAT inflammation, a process that can potentially be targeted, may help to explain the high incidence of estrogen-dependent tumors in postmenopausal women.

Panniculitis in patients treated with BRAF inhibitors: a case series.

Panniculitis is a rare complication of BRAF inhibitor therapy that is used to treat patients with BRAF-mutated metastatic melanoma. We present a clinicopathologic review of 9 patients who developed panniculitis while on BRAF inhibitor therapy. In 13% of patients on vemurafenib, 3% of patients on dabrafenib and 10% on combination of dabrafenib + trametinib, tender erythematous nodular lesions of panniculitis appeared on legs, arms and trunk. Histological evaluation of 8 biopsies from 7 patients showed predominantly neutrophilic infiltrate in 4, lymphocytic in 1, and mixed in 3. Lesions with neutrophilic infiltrate appeared in earlier stages of treatment than those with mixed or lymphocytic infiltrate. All biopsies showed lobular involvement and 5 also had a septal component. In addition, 1 biopsy had lichenoid inflammation in the epidermis and the other had evidence of vasculitis. Most patients responded to conservative medical management without the need to reduce or to stop BRAF inhibitor therapy. Panniculitis seems to be a rare class effect of BRAF inhibitors that is predominantly lobular and neutrophilic, although other patterns can be seen.

Locoregional multiple nodular panniculitis induced by Pseudomonas aeruginosa without septicemia: three cases and focus on predisposing factors.

IMPORTANCE: Pseudomonas aeruginosa-induced locoregional multiple nodular panniculitis without septicemia is an underreported condition, with only 3 cases reported to date. We report 3 new cases of P aeruginosa-induced multiple nodular panniculitis without septicemia and describe common features among all 6 cases, thus providing the first description, to our knowledge, of the natural history and potential predisposing factors for this entity. OBSERVATIONS: Median age of the 6 patients was 74 years (range, 54-84 years). Patients had inflammatory nodules on a lower limb (n = 6) that were unilateral (n = 6) and had no fever (n = 5). Blood cultures were negative (n = 5). Skin biopsy specimens revealed panniculitis (n = 5), with skin cultures positive for P aeruginosa (n = 6). Skin nodules resolved with systemic antibiotics (n = 5). The comorbidities recorded were type 1 or 2 diabetes mellitus (n = 5), overweight (n = 3), and combined locoregional anatomical changes in the lower limbs (n = 5). Local skin injury, which constituted the portal entry, was present in all cases, especially leg ulcers (n = 3). CONCLUSIONS AND RELEVANCE: We describe P aeruginosa-induced locoregional nodular panniculitis as a distinct entity. This should be investigated in elderly, diabetic, overweight patients with inflammatory nodules on a lower limb associated with locoregional anatomical changes and skin injury, with the optimal antibiotic regimen introduced as rapidly as possible.

How to make a specific diagnosis of panniculitis on clinical grounds alone: an integrated pathway of general criteria and specific findings.

A clinical approach to the vexing problem of diagnosis of panniculitis is traced in this paper, in order to obtain from the clinical findings, history and laboratory data of the patient useful, detailed and precise information, essential to address dermatologists to a specific clinical diagnosis of panniculitis. This approach is created in the same way as when a dermatologist faces any other dermatological disease, be it inflammatory or neoplastic. A common behavior in case of panniculitis is in fact just to take an adequate biopsy and wait for the pathologist report. This is indeed a limitation both for the dermatologist and above all for the pathologist, who is in tremendous need for detailed clinical information before signing his report. The most common types of panniculitides, taking into account their main clinical diagnostic criteria, will be considered. In particular, Erythema Nodosum, Panniculitides in Sarcoidosis, Pancreatic Panniculitis, Lupus Panniculitis, Erythema Induratum/Nodular Vasculitis and Weber-Christian Panniculitis/Rothman-Makai Pannicultis will be analyzed. Every chapter will consider general criteria (epidemiology, age and gender, distribution of the lesions, laboratory findings) and specific findings (characteristics of the lesions, i.e. redness, pain, tenderness, evolution, ulceration, sites of involvement) as well as comorbidities and systemic signs and symptoms. Detailed analysis of the general criteria integrated with the specific findings will allow the clinicians to reach a clinical diagnosis with a high degree of confidence.

Panniculitis in children.

This paper will give a comprehensive view of the most frequent panniculitides seen in childhood, with emphasis on the types exclusively found in infancy, and for all other types of panniculitides also found in adults. Aim of this paper is also to analyze the clinical differences between panniculitis in childhood and in adulthood, and to give reliable histopathologic criteria for a specific diagnosis. A review of the literature is here integrated by authors' personal contribution. Panniculitides in children is a heterogeneous group of diseases, as well as in adult life, characterized by inflammation of the subcutaneous fat. Only very few types of panniculitis are exclusively found in childhood, such as Sclerema neonatorum and subcutaneous fat necrosis of the newborn, while the vast majority of the other types may be found both in paediatric age and in adults. Furthermore, this paper will consider in detail panniculitis according to their frequency, such as Erythema nodosum, Lupus panniculitis, Cold panniculitis, panniculitis in Behçet disease, and poststeroid panniculitis. It will also describe rare forms of panniculitis, such as Eosinophilic panniculitis (a pathological entity debated by many authors), Subcutaneous panniculitis T-cell lymphoma, and the different forms of the so call "Lipophagic panniculitis", encompassing respectively the febrile relapsing panniculitis of Weber-Christian disease and the non-relapsing form of Rothmann-Makai disease. For each type of panniculitis considered concise information will be given about epidemiology, etiology, clinical findings, laboratory data, prognosis and therapy, while histopathologic findings will be described in detail.

Suprapatellar fat-pad mass effect: MRI findings and correlation with anterior knee pain.

OBJECTIVE: The purpose of this study was to evaluate the MRI characteristics of the suprapatellar fat-pad, determine the prevalence and pattern of suprapatellar fat-pad edema, and correlate the findings with the presence of anterior knee pain, patellofemoral malalignment, and patellofemoral joint osteoarthritis. MATERIALS AND METHODS: We retrospectively reviewed 879 consecutive knee MRI examinations of 843 patients for the presence of a suprapatellar fat-pad mass effect on the suprapatellar joint recess. The relative signal intensity and the maximum anteroposterior, craniocaudal, and oblique diameters of the suprapatellar fat-pad on sagittal fat-suppressed intermediate-weighted turbo spin-echo images were measured. Findings of anterior knee pain, patellofemoral malalignment, and patellofemoral joint osteoarthritis were also recorded. The Fisher exact, Mann-Whitney, and independent samples Student t tests and Spearman rank correlation coefficient were used for statistical analysis. RESULTS: The prevalence of suprapatellar fat-pad mass effect on the suprapatellar joint recess in our study population was 13.8%. The relative signal intensity (p < 0.0001) and maximum anteroposterior (p < 0.0001), craniocaudal (p = 0.0017), and oblique (p < 0.0001) diameters of the pad were significantly greater in patients with a mass effect. Significant correlation was found between the relative signal intensity and the maximum anteroposterior (ρ = 0.0986, p = 0.0053), craniocaudal (ρ = 0.0968, p = 0.0062), and oblique (ρ = 0.123, p = 0.0005) diameters. Mass effect was not significantly associated with anterior knee pain, patellofemoral malalignment, or patellofemoral joint osteoarthritis. Six patients with suprapatellar fat-pad edema had anterior knee pain. CONCLUSION: Suprapatellar fat-pad edema with a mass effect on the suprapatellar joint recess is a common finding at MRI examinations of the knee that is rarely associated with anterior knee pain.

Panniculitis attributable to Mycobacterium goodii in an immunocompetent dog in Georgia.

CASE DESCRIPTION: A 5-year-old 38.3-kg (84.5-lb) mixed-breed dog was examined because of acute onset of lethargy and anorexia. Four days later, a raised, firm, warm 15 × 10-cm lesion was detected in the right caudal paralumbar area. CLINICAL FINDINGS: Cephalexin treatment yielded a poor response. Formalin-fixed tissue and fluid samples from the cystic areas of the lesion were submitted for cytologic and histologic examinations, routine bacterial and mycobacterial culture, and genus identification and 16S partial sequencing via PCR assays. Cytologic examination revealed chronic pyogranulomatous inflammation. Histologic examination by use of routine, Giemsa, silver, acid-fast, and modified acid-fast stains revealed multifocal nodular granulomatous panniculitis without identifiable organisms. Mycobacteria were initially identified via PCR assay and mycobacterial culture within 3 days. Mycobaterium goodii was speciated by use of partial 16S RNA sequence analysis. TREATMENT AND OUTCOME: The lesion resolved after long-term treatment with a combination of rifampin and clarithromycin and insertion of a Penrose drain. There has been no recurrence of the condition. CLINICAL RELEVANCE: M goodii is an environmental rapidly growing mycobacterium and is a zoonotic pathogen. Infections have not been previously reported in domestic animals in North America, although there are rare reports of infection in humans associated with surgery, especially surgical implants. Domestic animals are a potential sentinel for this non-tuberculous mycobacterial infection in humans, although lack of speciation in infections of domestic animals likely underestimates the potential public health importance of this pathogenic organism. Current microbiological molecular methods allow for a rapid and inexpensive diagnosis.

Comparative analysis of subacute cutaneous lupus erythematosus and chronic cutaneous lupus erythematosus: clinical and immunological study of 270 patients.

Background Lupus erythematosus (LE) is a chronic, autoimmune disease resulting from an interaction of genetic, environmental and hormonal factors and characterized by a spectrum of clinical forms with variable evolution from a localized cutaneous form to a life-threatening systemic form. Objective To analyse and compare the prevalence and characteristics of the main clinical and immunological manifestations of subacute cutaneous LE (SCLE) and chronic CLE (CCLE). Methods A total of 270 patients with CLE (112 patients with SCLE and 158 patients with CCLE) were studied retrospectively. The clinical and serological characteristics of all the patients were collected in a chart review. Results The patients with SCLE had a higher prevalence of annular and papulosquamous lesions, Raynaud phenomenon, mucous membrane ulcers, malar rashes, photosensitivity, vasculitis and a lower frequency of discoid lesions and alopecia compared with patients with CCLE. Patients with SCLE had a higher prevalence of arthralgias (P < 0.001), xerophthalmia (P = 0.045), arthritis (P < 0.001), nephropathy (P = 0.003) and systemic LE (SLE) (P < 0.001) compared with patients with CCLE. Patients with SCLE also had a higher frequency of laboratory and serological abnormalities than patients with CCLE. Generalized discoid LE (DLE) was associated with a higher prevalence of photosensitivity (P < 0.001), panniculitis (P = 0.009) and SLE (P = 0.003) than localized DLE. In patients with SCLE and those with CCLE, photosensitivity, arthralgias, arthritis, nephropathy and xerophthalmia were associated with SLE. In patients with SCLE, significant correlations existed between clinical and immunological data. Conclusions In our series, differences in the expression of CCLE and SCLE were found with respect to the distribution and type of lesions, systemic features and immunological findings.

Childhood acquired lipodystrophy: a retrospective study.

OBJECTIVE: We sought to describe the clinical characteristics and complications of children with acquired lipodystrophy (LD). METHODS: We conducted a retrospective chart review at a tertiary, academic children's hospital of children clinically given a diagnosis of acquired LD between January 1997 and December 2004. RESULTS: During the study period, 23 patients were identified. Their mean age at diagnosis was 9.74 +/- 3.98 years. Of patients, 61% were girls. The length of the follow-up was 4.8 +/- 3.5 years from the time of LD diagnosis. Of patients, 30% had evidence of localized disease (7 of 23), 26% (6 of 23) had localized partial disease, and 44% (10 of 23) had generalized LD. The most common underlying diagnosis was dermatomyositis (78%), alone or in association with other autoimmune diseases (juvenile rheumatoid arthritis 17%). Panniculitis with autoimmunity was noted in 17% of the patients. More than half of the patients had at least one complication attributable to LD such as acanthosis nigricans (22%), hyperpigmentation (22%), hepatomegaly (13%), hypertension (13%), protuberant abdomen (9%), and hyperlipidemia (4%). The only predictor for development of complications was the age of diagnosis of LD, with younger age being associated with increased risk (7 vs 12 years, P = .04). LIMITATIONS: Limitations were inherent to the retrospective design. CONCLUSIONS: Childhood acquired LD is seen more frequently in the context of autoimmunity. Affected children should be monitored for the development of complications, particularly if given a diagnosis of LD at a younger age.