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QJM 2022 Jun 22. Epub 2022 Jun 22.

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

Clin Rev Allergy Immunol 2022 Jun 18. Epub 2022 Jun 18.

Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, piazzale Spedali Civili 1, Brescia, 25123, Italy.

Disease-specific autoantibodies are considered the most important biomarkers for systemic sclerosis (SSc), due to their ability to stratify patients with different severity and prognosis. Anti-nuclear antibodies (ANA), occurring in subjects with isolated Raynuad's phenomenon, are considered the strongest independent predictors of definite SSc and digital microvascular damage, as observed by nailfold videocapillaroscopy. ANA are present in more than 90% of SSc, but ANA negativity does not exclude SSc diagnosis: a little rate of SSc ANA negative exists and shows a distinct subtype of disease, with less vasculopathy, but more frequent lower gastrointestinal involvement and severe disease course. Read More

Semin Arthritis Rheum 2022 05 18;55:152027. Epub 2022 May 18.

Dept. Internal Medicine, div. Vascular Medicine, University of Groningen, University Medical Center Groningen, the Netherlands. Electronic address:

Objectives: Calcinosis cutis affects 20-40% of patients with systemic sclerosis (SSc). When calcinosis cutis becomes clinically apparent, it is irreversible in most cases. Detection of active calcification formation might allow early disease-modifying interventions. Read More

Endocrinol Diabetes Metab Case Rep 2022 Jun 1;2022. Epub 2022 Jun 1.

Department of Diabetology and Endocrinology, Mohammed VI Hospital.

Summary: Cushing's disease or pituitary adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome is considered a rare condition. It is caused by hypersecretion of the ACTH by a pituitary adenoma that ultimately induces endogenous hypercortisolism by stimulating the adrenal glands. It is responsible for significant morbidity and mortality. Read More

Ren Fail 2022 Dec;44(1):914-922

Institute of Nephrology, Zhong Da Hospital, Southeast University, School of Medicine, Nanjing, China.

Background: Sodium thiosulfate (STS) can be used to treat patients diagnosed with calciphylaxis, which is a rare life-threatening syndrome. However, our patients treated with the recommended STS regimen presented with serious adverse events, resulting in treatment withdrawal. Then an optimized STS regimen was used to increase the tolerance of patients to STS and improve treatment continuation. Read More

ARP Rheumatol 2022 Jan-Mar;1(1):21-29

Serviço de Reumatologia, Hospital Garcia de Orta; Unidade de Investigação em Reumatologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa.

Background: Systemic sclerosis (SSc) is a rare connective tissue disorder with heterogeneous manifestations and outcomes. Besides differences in disease characteristics among distinct ethnic groups and geographical regions, several questions regarding the impact of the disease and the effectiveness of treatments remain unanswered. To address these questions, the Rheumatic Diseases Portuguese Register (Reuma. Read More

Front Pharmacol 2022 9;13:906212. Epub 2022 May 9.

National Innovation and Attracting Talents "111" Base, Key Laboratory of Biorheological Science and Technology, Ministry of Education, College of Bioengineering, Chongqing University, Chongqing, China.

Keloids are fibroproliferative skin disorder caused by abnormal healing of injured or irritated skin and are characterized by excessive extracellular matrix (ECM) synthesis and deposition, which results in excessive collagen disorders and calcinosis, increasing the remodeling and stiffness of keloid matrix. The pathogenesis of keloid is very complex, and may include changes in cell function, genetics, inflammation, and other factors. In this review, we aim to discuss the role of biomechanical factors in keloid formation. Read More

BMJ Case Rep 2022 May 18;15(5). Epub 2022 May 18.

Systemic Autoimmune and Rares Diseases Unit. Internal Medicine Department, Hospital Virgen del Rocio, Seville, Spain.

Calcinosis cutis (CC) is the umbrella term for calcium salt deposition on skin and subcutaneous tissue. We present a unique case of CC associated with anti-Mi2-positive dermatomyositis, having a distinctive distribution of subcutaneous calcifications appearing as a 'lumbar belt'. Treatment of CC remains challenging for clinicians due to a lack of high-quality evidence. Read More

J Rheumatol 2022 May 15. Epub 2022 May 15.

Hadiya Elahmar MD, Dermatologist at U-turn Dermatology Clinic, Kuwait; Dermatology and Venerology, Ain Shams University, Cairo, Egypt. Brian M Feldman MD MSc, Pediatrics, Medicine, Institute of Health Policy Management and Evaluation, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada. Sindhu R Johnson MD PhD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Institute of Health Policy Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. Funding: Dr. Johnson is supported by a Canadian Institutes of Health Research New Investigator Award, Gurmej Kaur Dhanda Scleroderma Research Award, and the Oscar and Elanor Markovitz Scleroderma Research Fund. Dr. Feldman holds the Ho Family Chair in Autoimmune Diseases. Corresponding Author. Dr. Sindhu Johnson, Division of Rheumatology, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, Ontario, Canada, M5T 2S8. Phone 1-416-603-6417 Fax.1-416-603-4348. Email:

Calcinosis (hydroxyapatite and calcium phosphate crystal deposition) within the extracellular matrix of the dermis and subcutaneous tissue is a frequent manifestation of adult and pediatric systemic autoimmune rheumatic diseases, specifically systemic sclerosis, dermatomyositis, mixed connective tissue disease and systemic lupus erythematosus. In this article, we review classification of calcinosis, highlight mechanisms that may contribute to the pathogenesis of calcinosis and summarize the evidence evaluating non-pharmacologic and pharmacologic interventions for the treatment of calcinosis. Read More

Ceylon Med J 2021 06;66(2):100-101

District General Hospital, Nuwaraeliya, Sri Lanka,

Skinmed 2022 30;20(2):145-148. Epub 2022 Apr 30.

Department of Dermatology, Ed. Herriot Hospital, Lyon, France;

A French (Caucasian) woman with a history of nonobstructive hypertrophic cardiopathy, type 1 diabetes mellitus, cataract, and ante-hypophysary insufficiency had undergone multiple magnetic resonance imaging (MRI) studies. She had developed end-stage renal disease (ESRD) and had undergone hemodialysis for 10 years before receiving a kidney-pancreas allotransplantation at the age of 48 years. She received antithymocyte globulins as induction immunosuppression and steroids (5 mg/d), mycophenolate mofetil (2 g/d), and tacrolimus (5 mg/d) as maintenance immunosuppression. Read More

Nephrol Ther 2022 Jun 2;18(3):180-188. Epub 2022 May 2.

Service d'explorations fonctionnelles rénales, AURA Nord Saint-Ouen, Saint-Ouen, université Paris V, hôpital Necker, René Descartes, Paris, France.

Uremic calciphylaxis is a rare disease that affects patients with chronic end-stage renal disease. It is a pathology of the microvessels of the dermis and hypodermis which are calcified and whose thrombosis leads to skin necrosis. Calciphylaxis lesions can be distal and axial. Read More

Dermatol Online J 2021 Dec 15;27(12). Epub 2021 Dec 15.

Department of Laboratory Medicine and Pathology, University of Washington School of Medicine, Seattle, Washington, USA.

Cutaneous dystrophic calcification as a late change of radiation therapy is a rarely reported finding. Initially, it was almost exclusively described as occurring on the chest wall in breast cancer patients but has since been described in several other malignancies. We describe the first reported case of radiotherapy-induced calcinosis cutis occurring at the site of a previous liposarcoma. Read More

Int J Biol Macromol 2022 Jun 27;209(Pt B):1695-1702. Epub 2022 Apr 27.

Key Laboratory of Leather Chemistry and Engineering (Sichuan University), Ministry of Education, Chengdu 610065, China; The Research Center of Biomedicine Engineering of Sichuan University, Chengdu 610065, China.

Biological patch is a kind of tissue substitute material derived from natural polymer materials for the repair of human soft tissue defects. The serious calcification of biological patch after implantation is one of the reasons for the decline and failure of patch. In previous studies, we synthesized a new biomaterial crosslinker epoxidized chitosan quaternary ammonium salt (EHTCC) and used it for the crosslinking of porcine acellular dermal matrix (pADM). Read More

Z Rheumatol 2022 Apr 29. Epub 2022 Apr 29.

Universitätsklinik für Rheumatologie und Immunologie, Inselspital Bern, 3010, Bern, Schweiz.

Dermatomyositis (DM) is an inflammatory multisystem disease of unknown etiology, which can already occur in children but first onset can also be in older adulthood. Myalgia and muscle weakness can occur later in the course of the disease or even be completely absent in some forms. Classical signs on the skin include heliotrope rash, facial erythema, Gottron's papules and nailfold capillary abnormalities. Read More

Indian J Nucl Med 2022 Jan-Mar;37(1):71-73. Epub 2022 Mar 25.

Department of Nuclear Medicine, All India Institute of Medical Sciences, Bhubaneswar, India.

Calcinosis of soft tissue is a rare but known complication of dermatomyositis (DM), mostly associated with juvenile DM and rare in adult DM. Bone scan with Tc-99m Methylene diphosphonate is useful in disease mapping and has high sensitivity to know the extent of calcinosis. However, there is scanty literature available on the utility of bone scan in treatment response evaluation in DM. Read More

Vet Rec 2022 Apr 23:e1630. Epub 2022 Apr 23.

Department of Small Animal Medicine and Surgery, Veterinary School, Complutense University of Madrid, Madrid, Spain.

Background: Twice daily low trilostane doses have proven to be effective to manage canine Cushing's syndrome. However, survival and prognostic factors in dogs treated with this protocol have not been evaluated. The aim of the study was to evaluate survival and prognostic factors, including systolic blood pressure (SBP) at diagnosis, in dogs with pituitary-dependent hypercortisolism (PDH) treated with low trilostane doses. Read More

Cureus 2022 Mar 17;14(3):e23261. Epub 2022 Mar 17.

Pathology and Neuropathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

Calcinosis cutis (CC) is characterized by calcium deposition in the subcutaneous tissues. Subepidermal calcified nodule (SCN) is a variant of idiopathic calcinosis most commonly seen in the head and neck region of children and adolescents as a single, small, painless, yellow-white papule. A 13-year-old boy with a medical history of neurofibromatosis type 1 (NF1) presented with a firm 0. Read More

Pediatr Rheumatol Online J 2022 Apr 20;20(1):30. Epub 2022 Apr 20.

Seattle Children's Hospital and Research Center, 4800 Sand Point Way NE, PO Box 5371, Seattle, WA, 98105, USA.

Background: We present two cases of Nodular Regenerative Hyperplasia (NRH) associated with Juvenile Dermatomyositis (JDM).

Case Presentation: Case 1: A nine-year-old Caucasian male with refractory JDM and anti-NXP2 autoantibodies was diagnosed at age two. Over seven years, he developed arthritis, dysphagia, dysphonia, severe calcinosis, and colitis. Read More

Pediatr Rheumatol Online J 2022 Apr 11;20(1):24. Epub 2022 Apr 11.

Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.

Background: Singleton-Merten syndrome 1 (SGMRT1) is a rare type I interferonopathy caused by heterozygous mutations in the IFIH1 gene. IFIH1 encodes the pattern recognition receptor MDA5 which senses viral dsRNA and activates antiviral type I interferon (IFN) signaling. In SGMRT1, IFIH1 mutations confer a gain-of-function which causes overactivation of type I interferon (IFN) signaling leading to autoinflammation. Read More

J Ultrasound 2022 Apr 9. Epub 2022 Apr 9.

Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Mas Casanovas 90, 08041, Barcelona, Spain.

Calcinosis cutis (CC) is characterized by deposit of calcium salts in the skin and subcutaneous tissue; its clinical presentation consists of indurated painful nodules, which can ulcerate and become superinfected. CC treatment remains a challenge, yet successful treatment with intralesional (IL) sodium thiosulfate (STS) has been reported in several CC subtypes. Herein we are reporting on a case series of 5 patients with CC successfully treated with IL-STS. Read More

BMJ Case Rep 2022 Apr 6;15(4). Epub 2022 Apr 6.

Dermatology, St Vincent's Hospital Melbourne Pty Ltd, Fitzroy, Victoria, Australia.

Dystrophic calcinosis cutis can be associated with severe pain, decreased mobility, increased risk of infection and significantly decreased quality of life. We report a case of recalcitrant calcinosis cutis on the background of eosinophilic fasciitis, which achieved rapid reduction in calcium deposits following a novel injecting protocol of intralesional sodium thiosulfate. Read More

J Scleroderma Relat Disord 2021 Oct 1;6(3):299-305. Epub 2021 Apr 1.

Universidad Nacional de Córdoba, Córdoba, Argentina.

Introduction: Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is part of the group of connective tissue diseases, these rare autoimmune systemic pathologies cause thickening and hardening of tissues in different parts of the body and can lead to complex disorders. Oral manifestations of systemic sclerosis may include limited ability to open the mouth, xerostomia, periodontal disease, enlarged periodontal ligament, and bone resorption of the jaw.

Case Description: A 54-year-old Caucasian patient presented with oral pain, swallowing, phonation and chewing difficulties associated with dental instability, hygiene/handling difficulties and her main problem with microstomia, which prevented her from removing the skeletal prosthesis for 4 years, depriving her of social life. Read More

J Scleroderma Relat Disord 2022 Feb 28;7(1):7-23. Epub 2021 Oct 28.

Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA, USA.

Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It is a manifestation of several autoimmune connective tissue diseases, most frequently with systemic sclerosis and juvenile dermatomyositis, followed by adult dermatomyositis. Autoimmune connective tissue disease-associated calcinosis is of the dystrophic subtype, which occurs at sites of damaged tissue in the setting of normal serum calcium and phosphate levels. Read More

J Cutan Pathol 2022 Mar 31. Epub 2022 Mar 31.

Dermatology Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria La Princesa (IIS-IP), Madrid, Spain.

Background: Attempts have been made to establish discriminative criteria between classic calciphylaxis (CPX) and those cases in which cutaneous vascular calcification (CVC) represents an incidental finding (epiphenomenon).

Methods: Retrospective, observational cohort study of patients with CVC to distinguish clinicopathological features between CVC as classic CPX (CVC in cutaneous lesions with erythematous-violaceous plaques with or without ulceration) or as an epiphenomenon (CVC in cutaneous lesions with known diagnosis). Different clinicopathological parameters and the presence of perieccrine calcification and pseudoxanthoma elasticum (PXE)-like changes were evaluated. Read More

AANA J 2022 Apr;90(2):100-104

is a professor in the Department of Anesthesiology at the University of Kentucky Albert B. Chandler Medical Center, Lexington, Kentucky.

Calcinosis universalis is a rare subtype of connective tissue diseases known as calcinosis cutis. The disease is described as diffuse calcium salt deposits in subcutaneous, fibrous muscle structures, and tendons. Calcinosis cutis typically occurs secondary to a tissue damaging disease such as dermatomyositis, systemic lupus erythematosus, and Sjogren syndrome, presenting before age 20 and predominantly affecting women. Read More

J Am Acad Dermatol 2022 Mar 22. Epub 2022 Mar 22.

Department of Dermatology, Massachusetts General Hospital of Harvard Medical School, Boston, Massachusetts. Electronic address:

Biochem Biophys Res Commun 2022 05 10;604:83-87. Epub 2022 Mar 10.

Department of Rheumatology, Kawasaki Medical School, Kurashiki, Okayama, 701-0192, Japan.

Background: Vascular calcification is characterized by mineral deposition in the vasculature, which is triggered by chronic systemic inflammation, including psoriasis. Psoriasis is an IL-17A-mediated inflammatory skin disease that is associated with exacerbated vascular calcification and high cardiovascular mortality. Although previous studies have shown that IL-17A induces vascular dysfunction in murine psoriasis models, it has not been clarified whether IL-17A induces vascular calcification. Read More

Pan Afr Med J 2021 22;40:258. Epub 2021 Dec 22.

Department of Clinical Immunology, Centro Hospitalar Universitário do Porto, Porto, Portugal.

Calcinosis cutis is a rare and potentially disabling condition characterized by calcium deposition in soft tissues. When associated with autoimmune connective tissue diseases, calcinosis cutis is classified as Dystrophic Calcinosis Cutis (DCC), being its occurrence in systemic lupus erythematosus (SLE) patients fairly uncommon. We report a case of DCC in a 49 years old woman with eleven years evolution SLE that presented with a two years history of multiple painful skin lesions, some of them ulcerated and exhibiting a chalky white-yellow floor, in both hands, forearms, thighs, buttocks, abdomen and left breast. Read More

J Med Case Rep 2022 Mar 1;16(1):86. Epub 2022 Mar 1.

King Fahad Medical City, Riyadh, Saudi Arabia.

Background: Calcinosis cutis is a rare condition, characterized by an accumulation of calcium salts in the skin and subcutaneous tissue. There are several types of this condition, including dystrophic, metastatic, idiopathic, calciphylaxis, and iatrogenic calcinosis cutis. The type related to our case is iatrogenic calcinosis cutis, and one its possible causes is calcium intravenous infusion. Read More