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Intern Med 2020 Jun 9. Epub 2020 Jun 9.

Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan.

Med Clin (Barc) 2020 Mar 12. Epub 2020 Mar 12.

Servicio de Dermatología, Hospital Royo Villanova, Zaragoza, España.

Oncogene 2020 Apr 12;39(15):3218-3225. Epub 2020 Feb 12.

Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, NY, 10065, USA.

TAFRO syndrome, a clinical subtype of idiopathic multicentric Castleman disease (iMCD), consists of a constellation of symptoms/signs including thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, and organomegaly. The etiology of iMCD-TAFRO and the basis for cytokine hypersecretion commonly seen in iMCD-TAFRO patients has not been elucidated. Here, we identified a somatic MEK2 mutation and a germline RUNX1 mutation in two patients with iMCD-TAFRO, respectively. Read More

J Eur Acad Dermatol Venereol 2020 Jan 19. Epub 2020 Jan 19.

Unit of Dermatology, IRCCS Ca' Granda - Maggiore Policlinico Hospital Foundation, University of Milan, Milan, Italy.

Reticulohistiocytoses (RH) are rare and clinically heterogeneous histiocytic disorders of dermatological interest. Three clinical entities with superimposable histopathological features are currently considered, namely solitary reticulohistiocytoma, diffuse/generalized reticulohistiocytosis and multicentric reticulohistiocytosis. Although in the last decade, RH studies have only minimally progressed, histiocytosis research has advanced considerably: the prognostic and therapeutic importance of the clinical subclassification of histiocytosis patients as well as of the detection of genetic alterations in the genes of the ERK pathway has been highlighted. Read More

Radiographics 2020 Jan-Feb;40(1):223-240

From the Division of Nuclear Medicine, Department of Radiology (M.H., R.M.), and Division of Rheumatic Diseases (H.K., H.Y.), National Center for Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo 162-8655, Japan.

Rheumatic diseases are various painful conditions that affect joints, bones, cartilage, tendons, ligaments, and muscles. Arthritis is a typical condition of rheumatic disease. Although rheumatoid arthritis is a representative rheumatic disease, various diseases other than rheumatoid arthritis can also affect joints, and differential diagnosis of rheumatic diseases is often difficult owing to the similar clinical manifestations. Read More

Postepy Dermatol Alergol 2019 Oct 12;36(5):646-647. Epub 2019 Nov 12.

Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland.

Int J Dermatol 2020 Jun 28;59(6):e203-e204. Epub 2019 Nov 28.

Division of Rheumatology, Allergy & Clinical Immunology, School of Medicine, University of California, Davis, CA, USA.

Rheumatology (Oxford) 2019 Nov 19. Epub 2019 Nov 19.

Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN.

Objectives: Multicentric reticulohistiocytosis (MRH), a rare histiocytic disease that can mimic other rheumatic conditions, may be associated with cancer and other autoimmune disorders. To better understand the disorder and its other associations, we aimed to evaluate clinical correlates and outcomes of all patients with MRH seen at Mayo Clinic, Rochester between 1980 and 2017.

Methods: A retrospective medical record review was conducted to identify all patients with MRH between 1 January 1980 and 30 April 2017. Read More

Cureus 2019 Aug 24;11(8):e5476. Epub 2019 Aug 24.

Pathology, All India Institute of Medical Sciences, Bhubaneswar, IND.

Multicentric reticulohistiocytosis (MRH) is a rare multisystem macrophage disorder of unknown etiology characterized by papulonodular skin and mucosal lesions, rapidly progressive erosive symmetric polyarthritis, and inflammation of internal organs. Most often, it is misdiagnosed as rheumatoid arthritis (RA). Here, we report the case of a 60-year-old woman found to have features of both MRH and RA with positive rheumatoid factor and high titer of anti-cyclic citrullinated peptide antibody in serum. Read More

JAAD Case Rep 2019 Aug 5;5(8):720-722. Epub 2019 Aug 5.

Department of Dermatology, University of California Irvine, Irvine, California.

J Hand Surg Am 2020 May 15;45(5):457.e1-457.e5. Epub 2019 Jul 15.

Department of Orthopaedic Surgery, McLaren Regional Medical Center, Flint, MI.

Multicentric reticulohistiocytosis is a rare non-Langerhans cell histiocytic rheumatologic disorder characterized by multiple skin lesions, painful hand nodules, and destructive polyarthritis that results in arthritis mutilans in 20% to 30% of patients. In the current literature, there have been approximately 300 cases reported and the pathogenesis remains unclear. Currently, there is no diagnostic serologic test and no consensus on management. Read More

Haematologica 2020 31;105(2):e61-e64. Epub 2020 Jan 31.

Department of Rehabilitation, Nagoya University Hospital, Nagoya, Japan

J Dtsch Dermatol Ges 2019 Jul 30;17(7):746-748. Epub 2019 May 30.

Department of Dermatology, Venereology and Allergology, University Medical Center Ruprecht-Karls-University Heidelberg, Germany.

Acta Derm Venereol 2019 Jul;99(9):844-845

Department of Dermatology, University of Padova, IT-35128 Padova, Italy.

Pediatr Blood Cancer 2019 07 15;66(7):e27759. Epub 2019 Apr 15.

Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

Cutis 2019 Jan;103(1):E20-E22

Department of Dermatology, Yale School of Medicine, New Haven, Connecticut, USA.

Semin Diagn Pathol 2019 Jul 31;36(4):211-228. Epub 2019 Jan 31.

PRW Laboratories, Charlottesville, VA, United State.

A variety of cutaneous abnormalities can be seen in patients with malignant diseases, some of which are infectious, with others representing direct involvement of the skin by the underlying disorder. Yet another group of lesions can be regarded as associated markers of the malignant process, and, as such, are termed "paraneoplastic." This review considers the latter collection of conditions, grouping them by the generic type of malignancy that is usually linked to the paraneoplasia. Read More

Dermatol Case Rep 2019 8;4(1). Epub 2019 Apr 8.

Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, Texas, USA.

A patient with Multifocal Reticulohistiocytosis (MRH) of skin and joints failed treatment with etanercept, methotrexate, hydroxychloroquine, prednisone, bisphosphonates and hydroxyzine. Long term treatment with thalidomide led to marked improvement in joint and cutaneous manifestations. Read More

Hautarzt 2018 Oct;69(Suppl 1):56-58

Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland.

Medicine (Baltimore) 2018 09;97(38):e12617

BMC Res Notes 2018 Sep 4;11(1):647. Epub 2018 Sep 4.

Rheumatology Department, VUMC Amsterdam, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands.

Background: Multicentric reticulohistiocytosis is a rare form of non-langerhans cell histiocytosis presenting with skin changes and erosive arthritis. Infiltration of histiocytes and multinucleated giant cells are typical histological findings and confirm the diagnosis.

Case Presentation: This case report describes a newly diagnosed case of multicentric reticulohistiocytosis in a healthy 26-year-old female originally from the Philippines. Read More

Medicine (Baltimore) 2018 Aug;97(33):e11449

Department of Rheumatology.

Introduction: Multicentric reticulohistiocytosis (MRH) is a rare histiocytic disorder that involves the skin, joints, and visceral organs.

Case Presentation: We report a 67-year-old woman with MRH who presented with a 2-years history of polyarthralgia and skin nodules. Her symptoms were an inflammatory polyarthropathy with punched-out lesions of the distal interphalangeal (DIP) joints of both hands. Read More

EFORT Open Rev 2018 Jun 23;3(6):381-390. Epub 2018 Jun 23.

Second Department of Internal Medicine, Hematology Unit, National and Kapodistrian University of Athens, School of Medicine, Greece.

Non-Langerhans cell histiocytosis (N-LCH) summarizes a group of rare diseases with different clinical presentations, pathogenesis and morphology. These include primary cutaneous N-LCH, cutaneous N-LCH with systemic involvement, and primary extracutaneous systemic forms with occasional cutaneous involvement.The juvenile (JXG) and non-juvenile xanthogranuloma (N-JXG) family of histiocytoses are N-LCH: the JXG family consisting of the JXG (cutaneous), xanthoma disseminatum (cutaneous and systemic) and Erdheim-Chester disease (ECD; systemic); and the N-JXG family consisting of the solitary reticulohistiocytoma (cutaneous), multicentric reticulohistiocytosis (cutaneous and systemic) and Rosai-Dorfman disease (RDD; systemic). Read More

G Ital Dermatol Venereol 2018 Jun;153(3):447-449

Unit of Dermatology, Department of Surgical, Microsurgical and Medical Sciences, University of Sassari, Sassari, Italy.

J Dtsch Dermatol Ges 2018 Jun 26;16(6):781-783. Epub 2018 Apr 26.

Department of Dermatology, University of California Irvine, Irvine, California, USA.

Blood Adv 2018 03;2(5):481-491

Department of Pathology, Stanford University, Stanford, CA.

Castleman disease (CD) is a rare lymphoproliferative disorder subclassified as unicentric CD (UCD) or multicentric CD (MCD) based on clinical features and the distribution of enlarged lymph nodes with characteristic histopathology. MCD can be further subtyped based on human herpes virus 8 (HHV8) infection into HHV8-associated MCD, HHV8/idiopathic MCD (iMCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin change (POEMS)-associated MCD. In a subset of cases of UCD, an associated follicular dendritic cell sarcoma (FDCS) may be seen. Read More

Ugeskr Laeger 2018 01;180(1)

A 59-year-old woman developed a rash and severe arthralgia, which primarily affected her fingers. She displayed digital arthritis and nodules on the hands, chest, face, and oral cavity. Blood samples were normal. Read More

J Dermatol 2018 Jun 26;45(6):e138-e139. Epub 2017 Dec 26.

Department of Gastroenterology, Buddhist Tzu-Chi General Hospital and Tzu-Chi University, Hualien, Taiwan.

J Clin Rheumatol 2018 Aug;24(5):285-287

Division of Rheumatology Mayo Clinic Jacksonville, FL. Division of Rheumatology Mayo Clinic Jacksonville, FL Department of Dermatology Mayo Clinic Jacksonville, FL.

Semin Diagn Pathol 2018 Jan 2;35(1):44-53. Epub 2017 Dec 2.

Pathology Service, Hospital Universitario Marques de Valdecilla, Santander, Spain.

This chapter describes the main features of two different diseases, Castleman Disease (CD) and Rosai-Dorfman Disease (RDD). Castleman disease (CD) is a clinical and histopathologically heterogeneous lymphoproliferative disorder that encompasses at least three distinct entities with some common overlapping morphological features: Hyaline Vascular CD (HVCD), Unicentric Plasma Cell CD and Multicentric CD. The most important feature of HVCD is the presence of abnormal germinal centers with hyaline-vascular transformation, sometimes showing multiple germinal centers within a single reactive lymphoid follicle, this outlining HVCD as a disorder of follicular dendritic cells. Read More

J Clin Rheumatol 2018 Jan;24(1):45-49

Division of Immunology University of Iowa Hospitals and Clinics Iowa City, IA Department of Dermatology Indiana University Indianapolis, IN Department of Dermatology and Pathology University of Iowa Hospitals and Clinics Iowa City, IA Griffith University School of Medicine and Gold Coast University Hospital Southport Queensland, Australia Medical Research Corporal Michael J. Crescenz Veterans Affairs Medical Center (Philadelphia) and Department of Dermatology Perelman School of Medicine at the University of Pennsylvania Philadelphia, PA.

Rheumatology (Oxford) 2017 Aug 11. Epub 2017 Aug 11.

Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Intern Med 2017 1;56(15):2063-2066. Epub 2017 Aug 1.

Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Japan.

A 68-year-old man presented with polyarthritis, proximal muscle weakness, and erythema of the face, arms, neck, and anterior chest that resembled the V-neck sign. Initially, dermatomyositis (DM) was considered because of the erythema, polyarthritis, and muscle weakness. He also had mediastinal and hilar lymphadenopathy on contrast-enhanced computed tomography. Read More

Rheumatology (Oxford) 2017 Oct;56(10):1706

Department of Internal Medicine, Maricopa Medical Center.

Cureus 2017 May 3;9(5):e1220. Epub 2017 May 3.

Rheumatology, Orlando VA Medical Center.

Multicentric reticulohistiocytosis (MRH) is an idiopathic multisystemic inflammatory disease characterized by symmetric erosive polyarthritis and typical papulonodular skin lesions. MRH can be associated with autoimmune diseases, malignancy, mycobacterial infections, and hyperlipidemia, and it is important to consider appropriate screening in this population. There is no specific diagnostic laboratory test for MRH. Read More

Int J Dermatol 2017 08 29;56(8):e173-e175. Epub 2017 May 29.

Department of Dermatology, Jichi Medical University, Shimotsuke, Japan.

J Eur Acad Dermatol Venereol 2017 Oct 30;31(10):e442-e443. Epub 2017 May 30.

Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Kliniczna 1a Street, 80-402, Gdańsk, Poland.

Australas J Dermatol 2017 Nov 4;58(4):e249-e252. Epub 2017 Apr 4.

Department of Dermatology, University General Hospital of Heraklion, Heraklion, Crete, Greece.

A 63-year-old Caucasian man presented with a 4-month history of disseminated asymptomatic reddish-brown papulonodular lesions. A skin biopsy showed dermal infiltration with CD68 histiocytes, predominantly with eosinophilic cytoplasm, some with a ground-glass cytoplasm, and a small number of giant cells. The diagnosis of multiple cutaneous reticulohistiocytosis was made. Read More

Clin Nucl Med 2017 Jul;42(7):567-568

From the Departments of *Nuclear Medicine, †Dermatology, and ‡Urology, National Center for Global Health and Medicine, Tokyo, Japan.

We report the case of a 75-year-old woman who underwent F-FDG PET/CT to evaluate the recurrence of urothelial carcinoma. PET/CT showed F-FDG-avid muscles and lymph nodes, mimicking recurrence. However, F-FDG uptake was also seen in multiple joints and subcutaneous tissue, representing an uncommon finding for recurrence. Read More

Best Pract Res Clin Rheumatol 2016 04 1;30(2):250-260. Epub 2016 Oct 1.

Department of Internal Medicine, Division of Rheumatology, Istanbul Faculty of Medicine, Istanbul, Turkey. Electronic address:

Multicentric reticulohistiocytosis (MRH) is a rare, multisystemic non-Langerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. There are no specific laboratory findings for MRH. Diagnosis is based on clinical findings and skin or synovial biopsy results. Read More

Rev Med Interne 2017 May 22;38(5):291-299. Epub 2016 Nov 22.

Service de médecine interne-rhumatologie, groupe hospitalier Diaconesses-Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.

Introduction: Acid sphingomyelinase deficiency (ASMD) is an autosomal recessive disease with a clinical spectrum ranging from a neurovisceral infantile form (Niemann-Pick disease type A) to a chronic visceral form also encountered in adults (Niemann-Pick disease type B, NP-B).

Methods: Retrospective multicentric analysis of French adult patients with ASMD over the period 1985-March 2015. Clinical, biological, and imaging data were analyzed. Read More

J Stroke Cerebrovasc Dis 2017 Jan 11;26(1):e22-e24. Epub 2016 Nov 11.

Department of Neurology, University of California, Los Angeles, California.

Multicentric reticulohistiocytosis (MRH) is a rare inflammatory disorder that presents with diverse systemic infiltrative manifestations. We report the first case of biopsy-proven MRH that presented with acute ischemic stroke in an embolic pattern. We discuss the clinical presentation, imaging findings, and diagnostic approach in our case. Read More

Am J Gastroenterol 2016 Sep;111(9):1227

Medical Faculty, University of Tuzla, Tuzla, Bosnia and Herzegovina.

J Pediatr 2016 Oct 1;177:328-328.e1. Epub 2016 Aug 1.

Department of Dermatology Section of Dermatopathology Medical College of Wisconsin Milwaukee, Wisconsin.

Clin Breast Cancer 2016 12 25;16(6):e203-e207. Epub 2016 Jun 25.

Neag Comprehensive Cancer Center, UConn Health, Farmington, CT.

Hematol Oncol 2017 Dec 3;35(4):828-834. Epub 2016 Jun 3.

Hematology, Spedali Civili, Brescia, Italy.

Adult onset hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which can develop as a complication of many disorders. Early diagnosis is essential in order to avoid a fatal outcome. To confirm the diagnosis of acquired HLH made in a single institution series of adult patients with HLH-04 criteria, we applied the HScore and evaluated prognostic factors associated with clinical outcome. Read More

J Clin Rheumatol 2016 Jun;22(4):220-1

From the *Servei de Reumatologia and †Servei de Dermatologia; and Hospital Moisès Broggi (CSI), Sant Joan Despí, Barcelona, Catalonia, Spain.

Pediatrics 2016 06;137(6)

Pediatrics, and.

Multicentric reticulohistiocytosis (MRH) is a rare histiocytic proliferative disorder of uncertain etiology, characterized by mucocutaneous papulonodular lesions and progressive, symmetric erosive arthritis. MRH can coexist with various autoimmune disorders, tuberculosis, and malignancy. It usually occurs in the elderly and is very rare in children. Read More

Semin Oncol 2016 06 23;43(3):341-6. Epub 2016 Feb 23.

University of Louisville Division of Dermatology, Louisville, KY. Electronic address:

The skin is often the herald of an underlying systemic illness, and gastrointestinal malignancies can present in numerous ways in the skin. Paraneoplastic phenomenon, such as acanthosis nigricans and tripe palm, may be the first indicator of a gastrointestinal malignancy. In addition, gastrointestinal cancers can metastasize to the skin, as described in the well-known Sister Mary Joseph's nodule. Read More

Semin Oncol 2016 06 23;43(3):331-4. Epub 2016 Feb 23.

Levine Cancer Institute, Carolinas HealthCare System, Charlotte, NC. Electronic address:

Breast cancer may present with cutaneous symptoms. The skin manifestations of breast cancer are varied. Some of the more common clinical presentations of metastatic cutaneous lesions from breast cancer will be described. Read More