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Orphanet J Rare Dis 2022 04 12;17(1):164. Epub 2022 Apr 12.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science& Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.

Objective: To investigate the clinical features of multicentric reticulohistiocytosis (MRH).

Methods: The clinical manifestations, laboratory examination results and histologic characteristics of eleven patients with MRH were collected and compared with those of 33 patients with rheumatoid arthritis.

Results: In total, 72. Read More

Front Immunol 2021 7;12:749669. Epub 2022 Jan 7.

Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China.

Multicentric reticulohistiocytosis (MRH) is a rare systemic disease of non-Langerhans cell histiocytosis. A number of studies in the literature have documented that it can coexist with malignancy or autoimmune disease, making it difficult to determine the most appropriate therapy. Here, we present a case study of MRH associated with posterior mediastinal adenosquamous carcinoma along with antinuclear antibody positivity and lupus anticoagulant positivity. Read More

Ann Clin Lab Sci 2021 Nov;51(6):894-895

Department of Pathology and Dermatology, Temple/St. Luke's School of Medicine, Bethlehem, PA, USA

Both reticulohistiocytoma and multicentric reticulohistiocytosis are defined by their shared histopathologic appearance of dermal proliferations of mononuclear histiocytes and multinucleated giant cells with eosinophilic "ground-glass" or "two-toned" cytoplasm. To assess the specificity of the histopathologic findings seen in reticulohistiocytoma and reticulohistiocytosis, this study retrospectively examined 109 random, unscreened excision specimens of ruptured cysts to determine at what frequency "ground-glass" or "two-toned" histiocytes and multinucleated giant cells are seen in reactive cutaneous infiltrates. Slightly more than half (56. Read More

Beijing Da Xue Xue Bao Yi Xue Ban 2021 Dec;53(6):1183-1187

Department of Traditional Chinese Medicine, Taian City Central Hospital, Tai'an 271000, Shandong, China.

A 65-year-old woman developed erythema, papules and nodules over the body. Some nodules of her auricles and hands like string beads. Besides, she suffered from symmetrical swelling and pain of multiple joints, morning stiffness with deformity of joints; She had elevated erythrocyte sedimentation rate and C reactive protein levels; Her rheumatoid factor and antinuclear antibody were positive; Joints destruction was found with X-ray imaging; Skin pathology showed Dermal infiltrate of abundant histiocytes, part of them with a ground-glass appearance; A CD68 immunohistochemical stain was positive and the cells were negative for S100, CD1a. Read More

Zhonghua Xue Ye Xue Za Zhi 2021 Oct;42(10):800-806

Department of Hematology, Affiliated Hospital of Xuzhou Medical University, Xuzhou 221002, China.

Factors influencing the prognosis of hemophagocytic lymphohistiocytosis (HLH) in adults were analyzed based on multicentric data. Clinical data of 124 adult patients with HLH diagnosed in eight medical centers in the Huaihai Lymphoma Working Group from March 2014 to July 2020 were collected. The optimal truncation value of continuous variables was obtained based on the Maxstat algorithm, X-Tile software, and restricted cubic spline. Read More

J Cutan Pathol 2022 Apr 12;49(4):388-392. Epub 2021 Dec 12.

Dermatology Department, Rutgers University, Robert Wood Johnson Medical Center, Somerset, New Jersey, USA.

Multicentric reticulohistiocytosis (MRH) is an insidious-onset, non-Langerhans-cell histiocytosis (NLCH) affecting the joints and skin. Early diagnosis is important to prevent destructive arthritis and disease-related complications. Reflectance confocal microscopy (RCM) is a technique that allows the visualization of the epidermis and superficial dermis noninvasively on a horizontal plane with quasi-histologic images of the skin. Read More

Eur J Med Res 2021 Oct 3;26(1):119. Epub 2021 Oct 3.

Department of Respiratory and Infectious Diseases, Beijing You An Hospital, Capital Medical University, Beijing Institute of Hepatology, Beijing, 100069, China.

Background: For a patient presenting with fever, multiple lymphadenopathy and splenomegaly, pathogen infection should be preferentially considered, followed by lymphoid malignancies. When traditional laboratory and pathological detection cannot find the pathogenic microorganism, metagenomic sequencing (MGS) which targets the person's genome for exceptional genetic disorders may detect a rare pathogen.

Case Presentation: Here, we introduced the diagnostic clue of a case of multicentric Castleman disease (MCD) with hemophagocytic syndrome which was elicited from the detection of human herpesvirus-8 in the blood of a HIV-1 infected person by MGS technology during pathogen inspection. Read More

J Clin Rheumatol 2022 03;28(2):e596-e604

Sección de Reumatología, Hospital José María Cullen, Santa Fe, Argentina.

Background: The tendency of IgG4-related disease (IgG4-RD) to form pseudotumors, as well as its multisystemic nature, makes it the perfect mimicker of many conditions. Moreover, some clinical, serological, radiological, or histological features of the disease might be shared with some mimickers.Recently, 4 clinical phenotypes have been identified, and patients grouped in each phenotype have distinctive demographic, clinical, and serological features and outcomes, and, as expected, for each phenotype, a set of differential diagnoses should be considered. Read More

Rev Chilena Infectol 2021 Jun;38(3):423-431

Departamento de Enfermedades Infecciosas e Inmunología Pediátrica, Escuela de Medicina, Pontificia Universidad Católica de Chile, Chile.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a severe syndrome, potentially lethal, with a pathological activation of the immune system and an extreme hyperinflammatory response. The etiology is classified in primary HLH (familiar or genetic) and secondary (infectious, oncological, and rheumatological diseases).

Aim: To analyze clinical and laboratory characteristics, treatment, and follow-up rates in pediatric patients with HLH. Read More

Australas J Dermatol 2021 Nov 16;62(4):527-529. Epub 2021 Aug 16.

Dermatology Clinic, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.

Rheumatology (Oxford) 2022 05;61(6):e163-e164

Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India.

J Yeungnam Med Sci 2022 Apr 5;39(2):153-160. Epub 2021 Jul 5.

Division of Rheumatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea.

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Read More

Pediatr Blood Cancer 2021 09 9;68(9):e29140. Epub 2021 Jun 9.

French Reference Center for Langerhans Cell Histiocytosis, Trousseau Hospital, Paris, France.

Background: The somatic BRAF mutation occurs in 38-64% of pediatric cases of Langerhans cell histiocytosis (LCH). Vemurafenib (VMF), a BRAF inhibitor, was approved for refractory BRAF mutated LCH. In adults, VMF causes frequent cutaneous adverse events (CAE) including skin tumors (squamous cell carcinomas, melanomas), but little is known in children. Read More

JAMA Dermatol 2021 06;157(6):735-737

Division of Rheumatology, Department of Medicine, Inflammation and Immunity Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

Joint Bone Spine 2021 10 20;88(5):105192. Epub 2021 Apr 20.

Department of Dermatology, APHP, Saint-Louis Hospital, Université de Paris, 1, avenue Claude Vellefaux, 75010 Paris, France. Electronic address:

Dermatol Online J 2021 Mar 15;27(3). Epub 2021 Mar 15.

Department of Dermatology, HCA Healthcare/USF Morsani College of Medicine Largo, FL.

Multicentric reticulohistiocytosis (MRH) is a rare type of non-Langerhans cell histiocytosis characterized by coral-toned papules with predilection for dorsal surfaces in addition to severe arthropathy. It sometimes proves difficult to differentiate these joint and skin findings clinically from certain rheumatologic diseases, primarily dermatomyositis. Herein, we present an 82-year-old woman who presented with the clinical findings described above and was subsequently diagnosed with MRH after biopsy and review of relevant clinical history. Read More

Skeletal Radiol 2021 Oct 31;50(10):1921-1940. Epub 2021 Mar 31.

Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, HA7 4LP, UK.

The non-Langerhans cell histiocytoses (N-LCH) represent a group of rare diseases with different clinical presentations and imaging features to classical LCH. While there is a long list of entities, only few present with musculoskeletal soft tissue and osseous manifestations alongside the more commonly reported systemic findings. Erdheim-Chester disease (ECD) is typically seen in adults as bilateral and symmetrical long bone osteosclerosis. Read More

JAAD Case Rep 2021 Mar 25;9:18-20. Epub 2020 Dec 25.

Dermatology and Venereology Department, Military Medical Academy, Sofia, Bulgaria.

BMJ Case Rep 2021 Jan 25;14(1). Epub 2021 Jan 25.

Pathology, Kangwon National University Hospital, Chuncheon, Kangwondo, Korea (the Republic of).

We report an extremely rare case of adult Langerhans cell histiocytosis (LCH) in a patient with papillary thyroid carcinoma (PTC) and Castleman disease (CD). A 46-year-old man visited our hospital with anaemia; systemic imaging showed an abdominal and a left thyroid mass. Biopsy confirmed CD for the abdominal mass and PTC for the thyroid mass. Read More

Sci Rep 2021 01 13;11(1):877. Epub 2021 Jan 13.

Univ Rennes, CNRS, IGDR (Institut de génétique et développement de Rennes) UMR6290, 35000, Rennes, France.

Circulating tumor DNA (ctDNA) has become an attractive biomarker in human oncology, and its use may be informative in canine cancer. Thus, we used droplet digital PCR or PCR for antigen receptor rearrangement, to explore tumor-specific point mutations, copy number alterations, and chromosomal rearrangements in the plasma of cancer-affected dogs. We detected ctDNA in 21/23 (91. Read More

J Clin Rheumatol 2022 Jan;28(1):e234-e239

From the Department of Dermatology, Fukushima Medical University, Fukushima, Japan.

Abstract: Multicentric reticulohistiocytosis (MRH) is a rare multisystem disorder, primarily affecting the skin and joints. As severe joint damage is a possible symptom, early diagnosis and therapeutic intervention are required. Cutaneous lesions present with characteristic features such as discrete reddish nodules, especially on acral locations. Read More

Clin Nucl Med 2021 May;46(5):e253-e255

From the Service de Médecine Nucléaire.

Abstract: We report the case of a 60-year-old woman who underwent 18F-FDG PET/CT to evaluate a metastatic breast carcinoma. Follow-up 18F-FDG PET/CT showed progressive disease with 18F-FDG increased in primary tumor, axillary lymph nodes, and pleural and bone diffuse metastases but also a concomitant uptake in multiples joints. The anatomopathological analysis from skin biopsy revealed a multicentric reticulohistiocytosis, considered paraneoplastic in the context. Read More

Indian Dermatol Online J 2020 Sep-Oct;11(5):849-851. Epub 2020 Sep 19.

Department of Dermatology, Maulana Azad Medical College, Bahadur Shah Zafar Marg, Delhi, India.

Medicina (Kaunas) 2020 Sep 8;56(9). Epub 2020 Sep 8.

Department of Internal Medicine, Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 16 Universității Street, 700115 Iași, Romania.

Multicentric reticulohistiocytosis (MRH) is a rare cause of destructive inflammatory arthritis involving both small, as well as larger joints. We report the case of a 40-year-old Caucasian female with a family history of neoplasia who was referred to our service witha two-month history of inflammatory joint pain. On examination, the patient had inflammatory arthritis, mainly involving the peripheral joints, sacroiliac joint pain, and numerous papulonodular mucocutaneous lesions, including periungual "coral beads". Read More

Clin Case Rep 2020 Aug 22;8(8):1560-1563. Epub 2020 Jun 22.

Department of Dermatology UCSF-Fresno Fresno CA USA.

Although MRH can mimic rheumatoid arthritis, its ability to rapidly progress to arthritis mutilans and association with malignancy in up to 25% of patients warrant prompt recognition and treatment along with age-appropriate malignancy work-up. Read More

Clin Exp Dermatol 2021 Apr 10;46(3):554-556. Epub 2020 Oct 10.

Academic Rheumatology, Clinical Sciences Building, City Hospital Nottingham, Nottingham, UK.

Eur J Case Rep Intern Med 2020 25;7(8):001568. Epub 2020 May 25.

FTC, Faculdade de Tecnologia e Ciências, Salvador, Bahia, Brazil.

Objectives: We describe a case of systemic sclerosis (SS) with acro-osteolysis associated with cutaneous mucinosis, usually characterized by mucin deposition in the skin. The main differential diagnosis was multicentric reticulohistiocytosis due to the presentation of papulonodular skin lesions.

Materials And Methods: A physical examination, imaging studies and laboratory tests were performed. Read More

Intern Med 2020 09 9;59(18):2337-2338. Epub 2020 Jun 9.

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

Blood Rev 2021 01 15;45:100707. Epub 2020 May 15.

Division of Hematology, University of British Columbia, Canada.

A subset of patients with severe COVID-19 develop profound inflammation and multi-organ dysfunction consistent with a "Cytokine Storm Syndrome" (CSS). In this review we compare the clinical features, diagnosis, and pathogenesis of COVID-CSS with other hematological CSS, namely secondary hemophagocytic lymphohistiocytosis (sHLH), idiopathic multicentric Castleman disease (iMCD), and CAR-T cell therapy associated Cytokine Release Syndrome (CRS). Novel therapeutics targeting cytokines or inhibiting cell signaling pathways have now become the mainstay of treatment in these CSS. Read More

Med Clin (Barc) 2021 03 12;156(6):310-311. Epub 2020 Mar 12.

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