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Cancer Biol Ther 2019 Apr 19:1-7. Epub 2019 Apr 19.

a Department of Pathology , Radboud Institute for Molecular Life Sciences, Radboud university medical center , Nijmegen , The Netherlands.

Acinar cell carcinoma (ACC) is a rare pancreatic neoplasm with dismal prognosis. Insights into the molecular basis of ACC can pave the way for the application of more effective, personalized therapies and detection of patients with hereditary predisposition. Molecular analysis revealed a germline BRCA2 (and CHEK2) mutation in a patient with a rare pancreatic ACC with extensive intraductal growth. Read More

Oxf Med Case Reports 2019 Mar 15;2019(3):omz023. Epub 2019 Apr 15.

Department of General Medicine, Woodlands Health Campus, 2 Yishun Central 2, Singapore.

Mesenteric panniculitis is the inflammation of intestinal mesentery. It may be associated with malignancy. We present a case of mesenteric panniculitis that manifested as unresolved fever which subsequently was found to be due to poorly differentiated metastatic carcinoma. Read More

J Clin Rheumatol 2019 Apr 12. Epub 2019 Apr 12.

Dermatology, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, the Netherlands.

Clin Case Rep 2019 Apr 11;7(4):773-775. Epub 2019 Mar 11.

Department of Dermatology Peter MacCallum Cancer Centre Melbourne Victoria Australia.

Pembrolizumab is an immune checkpoint inhibitor with antitumor activity in other organ malignancies. We present this case -demonstrating multiple inflammatory adverse events associated with Pembrolizumab (in a single patient), in order to increase awareness and facilitate earlier identification of the wide-ranging cutaneous side effects associated with immunotherapy. Read More

J Dtsch Dermatol Ges 2019 Apr 17. Epub 2019 Apr 17.

Department of Dermatology, University Hospital Magdeburg, Magdeburg, Germany.

J Cutan Pathol 2019 Apr 8. Epub 2019 Apr 8.

Department of Dermatology, University of Utah, Salt Lake City, Utah.

Secondary cutaneous involvement by mantle cell lymphoma (MCL), an uncommon aggressive B-cell malignancy, predominantly involves the dermis, with few reports of pannicular involvement. Lymphocytic infiltration of subcutaneous tissue is associated with inflammatory panniculitides and certain T-cell lymphomas, primarily subcutaneous panniculitis-like T-Cell lymphoma (SPTCL), which is characterized by rimming of adipocytes by tumor cells. This article is protected by copyright. Read More

Br J Dermatol 2019 Apr 1. Epub 2019 Apr 1.

Department of Dermatology, University of Minnesota, Minneapolis, MN.

Primary cutaneous gamma/delta T-cell lymphoma (pcGDTCL) is a rare, aggressive T-cell lymphoma that presents with ulcerated nodules, extranodal dissemination, constitutional symptoms, and often, hemophagocytic lymphohistiocytosis (HLH). pcGDTCL is so named given the gamma/delta phenotype of the T-cell receptor (TCR). This article is protected by copyright. Read More

Pathol Res Pract 2019 Mar 27. Epub 2019 Mar 27.

Department of Pathology, Duke University School of Medicine, Durham, NC 27710, United States. Electronic address:

Sickle cell disease (SCD) is a hereditary blood disorder that often has multiple comorbidities. Patients occasionally develop malignant neoplasms, but the risk of lymphoma in SCD is currently unknown. Here, we report a unique case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 25-year-old male patient with SCD. Read More

PLoS One 2019 2;14(4):e0214884. Epub 2019 Apr 2.

Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, Medical Oncology, University of Brescia at ASST-Spedali Civili, Brescia, Italy.

Panniculitis and vitiligo-like lesions have been recently identified as rare cutaneous side effects of the combination of BRAF and MEK inhibitors, a standard of care in metastatic and locally advanced BRAF V600 mutated melanoma. An immune-mediated mechanism has been advocated in the pathogenesis of these skin lesions. Herein we retrospectively reviewed our institutional experience with the aim to explore the association between the occurrence of panniculitis and vitiligo-like lesions during combination therapy with dabrafenib (D) and trametinib (T) and outcome of advanced melanoma patients. Read More

Clin Exp Dermatol 2019 Apr 1. Epub 2019 Apr 1.

Department of Dermatology, Cardiff and Vale University Health Board, Cardiff, UK.

Clin Diabetes Endocrinol 2019 14;5. Epub 2019 Mar 14.

1Division of Metabolism Endocrinology and Diabetes, Department of Internal Medicine, University of Michigan and Brehm Center for Diabetes, 1000 Wall Street, Room 5313, Ann Arbor, MI 48105 USA.

Background: Metreleptin, a recombinant methionyl -human -leptin, was approved to treat patients with generalized lipodystrophy (GL) in February 2014. However, leptin therapy has been associated with the development of lymphoma. We present a unique case of a patient with prior history of T cell lymphoma in remission, who was diagnosed with Acquired Generalized Lipodystrophy (AGL) during the following year after a clinical remission of her lymphoma without receiving leptin therapy. Read More

Am J Dermatopathol 2019 Mar 15. Epub 2019 Mar 15.

Department of Dermatopathology, California Skin Institute, San Jose, CA.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma preferentially localized in the subcutaneous adipose tissue and composed of cytotoxic T cells with an α/β immunophenotype. The neoplastic T cells can be variably admixed with other inflammatory cells, including histiocytes, which can rarely form noncaseating granulomas. We present a case of SPTCL in which granulomas are the predominant feature, composing 75%-80% of the inflammatory infiltrate. Read More

Dermatol Ther 2019 Mar 18:e12878. Epub 2019 Mar 18.

Medical Oncology, The Second Affiliated Hospital of Dalian Medical University, Liaoning Sheng, China.

Vet Dermatol 2019 Mar 18. Epub 2019 Mar 18.

Institute of Veterinary Bacteriology, Vetsuisse Faculty, University of Zurich, Winterthurerstrasse 270, 8057, Zurich, Switzerland.

Background: Cutaneous disseminated mycobacteriosis is rare in dogs. To the best of the authors' knowledge, the slowly growing mycobacterial species Mycobacterium nebraskense has not been described before in this species.

Objective: Description of clinical features, laboratory analyses and treatment regimen of this unusual case. Read More

Curr Drug Saf 2019 Mar 3. Epub 2019 Mar 3.

GCS Medical College, Ahmedabad. India.

Calciphylaxis is a complex dermatological lesion of micro vascular calcification that is typically presented as panniculitis with gangrenous painful lesions having uremic and non-uremic causes. We present a case of a 48-year male with a history of paroxysmal atrial fibrillation and hypertension taking amlodipine 5 mg and warfarin 5 mg daily for the last 26 month. The patient had a 6-month history of painful swelling followed by necrotic skin ulcer over the right leg. Read More

Dermatol Surg 2019 Feb 15. Epub 2019 Feb 15.

Department of Medicine Dermatology Service, Memorial Sloan Kettering Cancer Center, New York, New York.

EMBO Mol Med 2019 Mar;11(3)

Hebrew University Hadassah Medical School, Jerusalem, Israel

The deubiquitinase OTULIN removes methionine-1 (M1)-linked polyubiquitin signals conjugated by the linear ubiquitin chain assembly complex (LUBAC) and is critical for preventing TNF-driven inflammation in OTULIN-related autoinflammatory syndrome (ORAS). Five ORAS patients have been reported, but how dysregulated M1-linked polyubiquitin signalling causes their symptoms is unclear. Here, we report a new case of ORAS in which an OTULIN-Gly281Arg mutation leads to reduced activity and stability and in cells. Read More

Blood Adv 2019 Feb;3(4):588-595

Department of Pathology and Tumor Biology, Kyoto University, Kyoto, Japan.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of peripheral T-cell lymphoma affecting younger patients and associated with hemophagocytic lymphohistiocytosis. To clarify the molecular pathogenesis of SPTCL, we analyzed paired tumor and germline DNAs from 13 patients by whole-exome sequencing. All cases were Asians and were phenotypically sporadic with no family history of SPTCL. Read More

J Clin Endocrinol Metab 2019 Feb 19. Epub 2019 Feb 19.

Department of Endocrinology and Metabolism, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (USP), São Paulo, Brazil.

Context: Lipodystrophy syndromes are rare disorders characterized by the selective loss of adipose tissue. We aimed to report the first case of acquired generalized lipodystrophy possibly associated with nivolumab.

Case Description: A woman was referred to our Endocrinology Department for uncontrolled diabetes mellitus. Read More

Minerva Gastroenterol Dietol 2019 Feb 12. Epub 2019 Feb 12.

Department of Radiology, San Paolo Hospital, Milan, Italy.

Eur J Case Rep Intern Med 2018 24;5(4):000788. Epub 2018 Apr 24.

Department of Internal Medicine A, Coimbra Hospital and University Center, Coimbra, Portugal.

The association between mesenteric panniculitis and Sjögren's syndrome, although rare, is starting to be recognized. Usually, mesenteric panniculitis is symptomatic, presenting with either general or gastrointestinal symptoms. Sjögren's syndrome is an autoimmune disease that typically affects secretory glands, but may have serious systemic involvement. Read More

Pediatr Dermatol 2019 Mar 12;36(2):258-259. Epub 2019 Feb 12.

Paediatric Haematology Unit, Mubarak Al-Kabeer Hospital, Kuwait.

Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations. Read More

J Pediatr Hematol Oncol 2019 Feb 5. Epub 2019 Feb 5.

Clinical Haematology and Bone Marrow Transplantation, Christian Medical College, Ludhiana, Punjab.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct subtype of peripheral T-cell lymphoma associated with aggressive clinical behavior. Since its original description, it has continued to be a rare disease, and <200 cases have been reported in literature. We report an 11-year-old boy who presented with SPTCL and hemophagocytic lymphohistiocytosis (HLH) and responded to high-dose multiagent chemotherapy. Read More

An Bras Dermatol 2019 Jan-Feb;94(1):96-98

Department of Infectious Diseases, Hospital Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Histoplasmosis usually presents primarily as lung infection. Occasionally, mainly in immunocompromised hosts, it can spread and cause systemic manifestations. Skin lesions have been reported in 10 to 15 percent of cases of disseminated histoplasmosis, and panniculitis has been described as an unusual form of presentation in affected patients. Read More

Chronic Obstr Pulm Dis 2018 Sep 19;5(4):267-276. Epub 2018 Sep 19.

Department of Clinical Biochemistry and Pharmacology, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder which most commonly manifests as pulmonary emphysema. Accordingly, alpha-1 antitrypsin (AAT) augmentation therapy aims to reduce the progression of emphysema, as achieved by life-long weekly slow-drip infusions of plasma-derived affinity-purified human AAT. However, not all AATD patients will receive this therapy, due to either lack of medical coverage or low patient compliance. Read More

Ter Arkh 2018 May;90(5):55-60

V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.

Aim: The study of the course and outcomes of panniculitis (PN) in modern rheumatology practice.

Materials And Methods: We observed 209 patients (pts) (f-185, m-24 in age 17 to 80 years) with the input diagnosis "Erythema nodosum? Undifferentiated panniculitis?" and duration of illness in from 1 week to 25 years, observed in V.A. Read More

Radiol Case Rep 2019 Apr 17;14(4):430-435. Epub 2019 Jan 17.

Department of Pathology, Mount Sinai Downtown Union Square. 10 Union Square East, NY 10003, New York, USA.

To date, mammographic involvement in mixed connective tissue disease has not been described in the literature to the authors' knowledge. In addition, only 2 case reports have described panniculitis in mixed connective tissue disease. We present a patient with mixed connective tissue disease with coarse sheet-like calcifications on mammography bilaterally, which increased over time corresponding to rise in levels of anti-dsDNA. Read More

BMC Infect Dis 2019 Jan 29;19(1):97. Epub 2019 Jan 29.

Department of Medicine, University of Calgary, Alberta, Canada.

Background: Erythema induratum of Bazin (EIB) - nodular vasculitis associated with Mycobacterium tuberculosis (TB) - and Tuberculosis-Associated Ocular Inflammation (TB-AOI) represent uncommon manifestations of TB. There is limited data and a lack of diagnostic and treatment standards for these conditions.

Methods: Eleven-year retrospective review of EIB and TB-AOI cases managed in a provincial TB program with prospective phone-based follow-up of anti-tubercular therapy (ATT) recipients. Read More

Korean J Gastroenterol 2019 Jan;73(1):50-55

Division of Gastroenterology, Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.

Sclerosing mesenteritis is a rare benign disease with a prevalence of 0.16-3.4% and is characterized by chronic nonspecific inflammation and extensive fibrosis in the adipose tissue of the mesentery although the exact pathogenesis is still elusive. Read More

Dermatol Online J 2018 Sep 15;24(9). Epub 2018 Sep 15.

Department of Dermatology, Oregon Health & Science University, Portland, Oregon.

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. Read More

Adv Rheumatol 2019 Jan 18;59(1). Epub 2019 Jan 18.

Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR, Brazil.

Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis.

Methods: Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0. Read More

Australas J Dermatol 2019 Jan 17. Epub 2019 Jan 17.

Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Clin Case Rep 2019 Jan 22;7(1):128-130. Epub 2018 Nov 22.

Division of Dermatology, Faculty of Medicine Phramongkutklao Hospital Bangkok Thailand.

Panniculitis is rarely seen in polymyositis. To the best of our knowledge, we report the first case of panniculitis which precedes the antisynthetase syndrome. Read More

Clin Exp Gastroenterol 2019 27;12:1-8. Epub 2018 Dec 27.

Department of Radiology, Jordan University of Science and Technology, King Abdullah University Hospital, Irbid, Jordan,

Purpose: The aim was to compare computed tomography (CT) findings between patients with mesenteric panniculitis (MP) with and without known malignancy.

Patients And Methods: We retrospectively analyzed 116 consecutive patients who were diagnosed with MP on the basis of CT findings and categorized them according to the absence (Group 1: 73 patients) or presence (Group 2: 43 patients) of malignancy. Patient age and sex, diameter, size, mass effect, location, and fat density of the MP mass, presence of a pseudocapsule and/or halo sign, and lymph node status were compared between the two groups. Read More

Dermatol Ther 2019 Mar 5;32(2):e12821. Epub 2019 Feb 5.

Department of Clinical-Surgical, Diagnostic, and Pediatric Science, Institute of Dermatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

We report the case of a female patient who developed a firm, wooden-like, nonpitting edema of the left lower leg after a dermo-hypodermitis. The clinical picture was accompanied by intense pain, strongly impacting the patient's quality of life. A soft-tissue ultrasound demonstrated several millimetric hyperechoic linear lesions whose histopathological examination was conclusive for panniculitis ossificans. Read More

G Ital Dermatol Venereol 2019 Jan 9. Epub 2019 Jan 9.

Dermatological Unit, Department of Clinical and Molecular Sciences, Polytehnic Marche University, Ancona, Italy.

Dermatol Res Pract 2018 29;2018:5276528. Epub 2018 Nov 29.

Department of Physiotherapy, Federal University of São Carlos, São Carlos, Brazil.

Cryolipolysis is the noninvasive treatment of localized fat through cold-induced panniculitis. The purpose of the present study was to evaluate the safety and efficacy of contrast cryolipolysis for subcutaneous-fat reduction. Contrast cryolipolysis mixes the principles of conventional cryolipolysis and periods of heating in accordance with the contrast lipocryolysis process. Read More

J Dermatol 2019 Jan 10. Epub 2019 Jan 10.

Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Am J Dermatopathol 2018 Dec 26. Epub 2018 Dec 26.

Department of Pathology, Florida Hospital Orlando, Orlando, FL.

RAS-associated autoimmune leukoproliferative disease (RALD) is a recently described noninfectious and nonmalignant clinical syndrome characterized by autoimmune disorders, massive splenomegaly, modest lymphadenopathy, and monocytosis. On the molecular level, RALD is defined by somatic mutations of either NRAS or KRAS gene in a subset of hematopoietic cells. To date, there is a dearth of well-documented histopathologic description of cutaneous involvement by RALD in the literature. Read More

Histopathology 2018 Dec 31. Epub 2018 Dec 31.

Department of Pathology, University of Michigan, Ann Arbor, MI, USA.

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. Read More

Cancer Treat Res 2019;176:195-224

Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Australia.

There are a number of rare T-cell lymphoma subtypes that may be encountered in clinical practice. In recent years, improved immunohistochemical techniques and molecular tumor profiling have permitted refinement of some of the diagnostic categories in this group, as well as the recognition of distinct conditions not previously well elucidated. In this chapter, we cover the diagnostic and clinical features of some of the more common of these conditions, including subcutaneous panniculitis-like T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma, enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, CD4-positive small/medium T-cell lymphoproliferative disorder, and acral CD8-positive T-cell lymphoma. Read More

Vnitr Lek Fall 2018;64(10):961-965

Weber-Christian disease is a rare disease from the group of chronic fibrosing conditions characterized by inflamma-tion of the adipose tissue - panniculitis and fibrosing with frequent systemic manifestations. Etiopathogenesis of the disease is not fully known, participation of autoimmune mechanisms is anticipated. Here, we report a case of a patient with this rare disease, diagnosed after a long and demanding diagnostic process, including repeated lapa-rotomies. Read More

Am J Case Rep 2018 Dec 27;19:1530-1535. Epub 2018 Dec 27.

Cancer Vaccine Center, Kurume University, Kurume, Fukuoka, Japan.

BACKGROUND Personalized peptide vaccine therapy is regarded as a well-tolerated, safe and effective immunotherapy for patients with advanced cancers. Herein we report an exceptional case of a patient with advanced pancreatic cancer who developed delayed lobular panniculitis at sites corresponding to vaccine injections. CASE REPORT A 64-year-old Japanese female visited our clinic due to thirst and polydipsia; she was diagnosed as having type 2 diabetes. Read More

Reumatismo 2018 Dec 20;70(4):268-269. Epub 2018 Dec 20.

Clinica Reumatologica, Policlinico San Martino, Genova.

Hashimoto's encephalopathy (HE) is an autoimmune form of encephalopathy, associated with autoimmune thyroiditis. Its prevalence is estimated to be 2:100,000. HE is characterized by behavioral changes, mental confusion, dysarthria, ataxia, psychosis, paranoia, convulsions, hallucinations, headache and hyperthermia. Read More

World J Clin Cases 2018 Dec;6(15):1036-1041

Department of Pancreatic Surgery, West China Hospital/West China School of Medicine, Sichuan University, Chengdu 610041, Sichuan Province, China.

Solid pseudopapillary tumor of the pancreas (SPTP), also known as solid and papillary epithelial neoplasm of the pancreas, is a rare pancreatic exocrine tumor that is difficult to diagnose before surgery. Pancreatic panniculitis is a rare type that occurs in less than 3% of all patients with pancreatic diseases. We here report a 19-year-old woman who presented with persistent left upper quadrant pain without obvious cause for 1 d. Read More

Int J Surg Case Rep 2018 3;53:265-268. Epub 2018 Nov 3.

Indraprastha Apollo Hospital Delhi, India.

Mesenteric panniculitis (MP), also known as sclerosing mesenteritis (SM), retractile mesenteritis or mesenteric lipodystrophy, is a rarely diagnosed inflammatory condition of unknown etiology that involves the mesenteric adipose tissue. We are presenting a case of sclerosing mesenteritis which presented us with features suggestive of malignant lesion and turned out to be benign one. An elderly gentleman of 68 years male presented to us with history of nonprogressive pain abdomen since 2 months associated with low grade fever and on and off constipation. Read More

Front Pediatr 2018 3;6:377. Epub 2018 Dec 3.

Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Autoinflammatory diseases (AID) are diseases of the innate immune system, characterized by recurrent episodes of localized or systemic inflammation. Vasculitis may accompany AID. The causes of the association of vasculitis with monogenic AID are still debated. Read More

Isr Med Assoc J 2018 Dec;20(12):770-772

Department of Internal Medicine F, Soroka University Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.

Background: Erythema nodosum (EN) is the most common type of panniculitis, commonly secondary to infectious diseases.

Objectives: To elucidate the causative factors and the clinical presentation of patients with EN (2004-2014) and to compare their data to those reported in a previous study.

Methods: A retrospective study was conducted of all patients diagnosed with EN who were hospitalized at Soroka University Medical Center (2004-2014). Read More

Int J Hematol 2019 Feb 13;109(2):187-196. Epub 2018 Dec 13.

Division of Hematology, Department of Medicine, Taipei Veterans General Hospital, No. 201, Sec 2, Shih-Pai Road, Taipei, 112, Taiwan.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare form of cytotoxic T-cell lymphoma. It is believed that SPTL in patients without hemophagocytic syndrome (HPS) follows an indolent course; in contrast, SPTL in patients with HPS has been associated with unfavorable survival. To provide more clinical data on SPTL in Asian populations and to identify optimal therapeutic strategies for SPTL, we assessed the clinicopathological features and long-term follow-up data of 10 Taiwanese SPTL patients diagnosed at a single center. Read More