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

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

Semin Diagn Pathol 2019 Jan 31. 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

Arthritis Rheumatol 2019 Jan 28. Epub 2019 Jan 28.

University of Washington, 1959 NE Pacific St, BB561, Seattle, WA, 98195.

A 47 year-old man presented with cutaneous papules and hoarseness. Laryngoscopy showed supraglottic masses; histopathology revealed fibrohistiocytic proliferation with lipid-laden macrophages and Touton giant cells. Immunohistochemistry was positive for CD68 and negative for S100. 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 Sep;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

Mol Med Rep 2016 Jul 12;14(1):209-17. Epub 2016 May 12.

Department of Rheumatology and Immunology, Xiangya Hospital, Central South University, Changsha, Hunan 410008, P.R. China.

Multicentric reticulohistiocytosis (MRH) is a rare and debilitating systemic disorder characterized by cutaneous nodules and destructive polyarthritis. Due to its unknown etiology, the treatment of MRH varies with different rates of success, which causes treatment options to be rather independent and empirical. In the present study, a case of a 48‑year‑old woman with a 12‑month history of polyarthralgia and skin nodules was reported. Read More

Springerplus 2016 25;5:180. Epub 2016 Feb 25.

Department of Histopathology, AFIP (Armed Forces Institute of Pathology) , Rawalpindi, Pakistan.

Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. We present a case report of this disease and an extensive review of the literature. We reviewed the data between 1991 and 2014 and extracted 52 individual cases. Read More

Indian J Dermatol 2016 Jan-Feb;61(1):121

Department of Dermatology, Mahatma Gandhi Mission Hospital, Navi Mumbai, Maharashtra, India.

Multicentric reticulohistiocytosis is a rare non-Langerhans cell histiocytosis characterized in its full form by severe destructive arthritis, cutaneous nodules, and systemic manifestations. Cutaneous lesions may precede, accompany, or more commonly develop later than other features in this disease. We describe a case of multiple cutaneous reticulohistiocytoma without any systemic associations after thorough investigations. Read More

Am J Clin Pathol 2016 Feb 23;145(2):211-21. Epub 2016 Jan 23.

From the Departments of Pathology and.

Objectives: To describe the radiologic and clinicopathologic features of extranodal Rosai-Dorfman disease (RDD) in our patient population.

Methods: Via a data mining engine, we evaluated 13 cases of extranodal RDD in 10 patients treated at our institution from 2000 to 2014.

Results: There was a marked female predominance (90%) in our series. Read More

Dermatology 2016 8;232(2):171-6. Epub 2016 Jan 8.

Departamento de Neurologx00ED;a, Clx00ED;nica Las Condes, Santiago, Chile.

A young male presented with panhypopituitarism (including diabetes insipidus) and temporal lobe epilepsy. A histology specimen of cutaneous papules was diagnostic of non-Langerhans histiocytosis. The diagnosis of xanthoma granulomata was considered based on the clinical and brain MRI findings. Read More

Respirol Case Rep 2015 Dec 15;3(4):125-7. Epub 2015 Sep 15.

Department of Respiratory Medicine Iwata City Hospital Iwata Japan.

Organizing pneumonia (OP) is a clinicopathological entity that occurs idiopathically or in association with several conditions such as connective tissue diseases. Multicentric reticulohistiocytosis (MRH) is a systemic disease characterized by polyarthritis and mucocutaneous lesions, but lung involvement is uncommon. We report a patient with MRH associated with OP. Read More

Int J Hematol 2016 Apr 21;103(4):469-72. Epub 2015 Dec 21.

Section of Clinical Immunology, Department of Internal Medicine, Erasmus University MC, University Medical Center, Room D-419, PO Box 2040, 3000 CA, Rotterdam, The Netherlands.

Human herpes virus 8 (HHV-8)-associated secondary hemophagocytic lymphohistiocytosis is a rare but critical immuno-hematological entity in immunocompetent patients. Establishing a diagnosis is challenging as is the monitoring of disease activity and therapeutic effects. We report a case of a HHV-8-associated hemophagocytic lymphohistiocytosis in a HIV-negative adult patient with multicentric Castleman's disease. Read More

Scand J Rheumatol 2016 14;45(3):247-9. Epub 2015 Dec 14.

a Department of Rheumatology , Hiroshima City Hiroshima Citizens Hospital , Hiroshima , Japan.

Clin Orthop Surg 2015 Dec 13;7(4):509-14. Epub 2015 Nov 13.

Department of Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Multicentric reticulohistiocytosis (MRH) is a rare systemic disease, which commonly manifests as muco-cutaneous papulonodules and inflammatory erosive polyarthropathy. In this research, we report the clinical manifestations and management of a rare case of MRH with destructive arthropathy of bilateral hip joints and arthritis mutilans presenting with characteristic deformities. Disabling hip arthropathy that occurs secondary to MRH can be successfully managed with bilateral total hip arthroplasty (THA). Read More

Clin Nucl Med 2016 Apr;41(4):333-5

From the Departments of *Nuclear Medicine, and †Dermatology, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.

Multicentric reticulohistiocytosis is a rare systemic inflammatory disease of unknown etiology characterized by the infiltration of histiocytes and multinucleated giant cells into multiple systems. The definitive diagnosis depends on biopsy of the affected tissues. Here, we report the F-FDG PET/CT findings of a 62-year-old man with multicentric reticulohistiocytosis. Read More

Dermatol Clin 2015 Jul;33(3):465-72; discussion 473

Department of Dermatology, UCSF, 1701 Divisadero Street, San Francisco, CA 94115, USA. Electronic address:

Adult xanthogranuloma presents most commonly as an orange-tan firm solitary nodule with no systemic manifestations. Recently, some cases have been reported in conjunction with lymphoproliferative disorders. Adult reticulohistiocytosis classically presents as red to yellow-red dermal nodules. Read More

Transplantation 2016 Jan;100(1):134-40

1 Institute of Pathology, University Hospital (CHUV), Lausanne, Switzerland. 2 Institute of Microbiology, University Hospital (CHUV) and University of Lausanne, Lausanne, Switzerland. 3 Infectious Diseases Service, University Hospital (CHUV) and University of Lausanne, Lausanne, Switzerland. 4 Transplantation Center, University Hospital (CHUV) and University of Lausanne, Lausanne, Switzerland.

Kaposi sarcoma is the most common human herpesvirus 8 (HHV-8)-related disease described after solid organ transplantation. Multicentric Castleman disease and hemophagocytic syndrome are other potential HHV-8-induced entities but are less frequently reported. We describe the case of a liver transplant recipient who presented with an acute febrile illness 1 year after transplantation with a rapidly fatal outcome. Read More

Acta Derm Venereol 2016 Jan;96(1):124-5

Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan.

Int J Clin Exp Pathol 2015 1;8(3):3304-7. Epub 2015 Mar 1.

Hubei Huanggang Central Hospital Huanggang 438000, Hubei Province, China.

We report a rare case of multicentric reticulohistiocytosis (MRH) associated with liver carcinoma. A 36-year-old man who had been diagnosed as having liver carcinoma for 2 years presented with a 2-month history of multiple papulonodules on the face, ears, neck, and upper chest, accompanied by progressive polyarthralgia of the hands, wrists, elbows and knee joints without fever or chills. Skin histology revealed well defined dermal infiltrate consisting of multinucleated giant cells and macrophages having abundant eosinophilic finely granular cytoplasm with ground glass appearance. Read More

Curr Rheumatol Rep 2015 Jun;17(6):511

BIOMETRA Department, University of Milan, Milan, Italy.

The literature reports approximately 300 cases of multicentric reticulohistiocytosis (MRH) worldwide, mostly women of Caucasian origin. MRH manifests as a symmetric erosive polyarthritis mimicking rheumatoid arthritis with the subsequent appearance of typical papulonodular skin lesions. The disease may rapidly progress towards mutilans arthritis in the majority of cases, but it generally remits spontaneously in 10 years. Read More