Bisphosphonates for the Treatment of Calcinosis Cutis—A Retrospective Single-Center Study

(1) Background: Calcinosis cutis is a frequent symptom of autoimmune connective tissue diseases leading to pain, transcutaneous expulsion of calcified material and bacterial superinfection. There is a high need for new therapeutic options as no standardized treatment algorithm is established. While case reports indicate beneficial effects of bisphosphonates, standardized evaluation of treatment effects is missing. (2) Methods: In this retrospective analysis we evaluate the effects of intravenous pamidronate, a second-generation bisphosphonate, in seven patients with calcinosis cutis using consecutive clinical pictures, radiological examinations and patient’s subjective evaluation. (3) Results: 5/6 patients reported a reduction of pain, improvement of general condition and cessation of calcinosis progression. Regression of skin lesions was detectable in clinical pictures of 2/6 patients, while 1/6 patients had stable disease. Radiological examination revealed improvement or stable disease in 3/5 patients. Fever was the most common side effect. One out of seven patients developed osteonecrosis of the jaw. (4) Conclusions: Bisphosphonates appear to have beneficial effects in a subgroup of calcinosis cutis patients. While patient’s subjective evaluation was mainly positive, objective assessments showed improvement in approximately half of the cases. With regard to potential severe side effects, a careful risk-benefit evaluation is necessary before treatment initiation.

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Calcinosis cutis universalis in a patient with systemic lupus erythematosus: a case report

Lupus ◽

10.1177/0961203320942946 ◽

2020 ◽

Vol 29 (12) ◽

pp. 1630-1632

Author(s):

Mohammad Khudadah ◽

Ali Jawad ◽

Debasish Pyne

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Subcutaneous Tissue ◽

Connective Tissue Diseases ◽

Skin Lesions ◽

X Rays ◽

Systemic Lupus ◽

Calcinosis Cutis ◽

History Of ◽

Calcinosis Universalis

Calcinosis cutis is a deposition of calcium salts in the skin and subcutaneous tissue which can occur in connective tissue diseases such as scleroderma, dermatomyositis, myositis and overlap syndrome, but rarely in association with systemic lupus erythematosus (SLE). It is subdivided into a localized ‘circumscripta’ and diffuse ‘universalis’. The few reported cases of calcinosis in SLE were mainly of the circumscripta type. Calcinosis universalis is extremely rare and is usually associated with a history of chronic active SLE in female patients, with few proven effective treatments. We report a case of a young female patient with a long and complicated history of SLE. She presented with widespread pain and tenderness associated with multiple subcutaneous skin lesions. She was found to have evidence of calcinosis universalis on X-rays. Investigations did not reveal any associated conditions that could explain this diagnosis other than her prolonged history of SLE.

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Limited cutaneous systemic sclerosis: Total rehabilitation with fixed prosthesis on dental implants

Journal of Scleroderma and Related Disorders ◽

10.1177/23971983211004362 ◽

2021 ◽

pp. 239719832110043

Author(s):

Miguel Angel Garcés Villalá ◽

Carolina Zorrilla Albert

Keyword(s):

Systemic Sclerosis ◽

Bone Loss ◽

Dental Implants ◽

Social Life ◽

Connective Tissue Diseases ◽

The Body ◽

Calcinosis Cutis ◽

Complex Disorders ◽

Standardized Parameters

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. Gradual treatment with dental implants was diagnosed and planned to support a fixed total denture adapted to the ridge with self-cleaning characteristics. After implant insertion, panoramic radiographs with standardized parameters were taken to compare crestal bone levels at the time of prosthesis placement and with 10 years of follow-up. Conclusion: The average crestal bone loss of the 12 implants after the 10 years of follow-up was 1.26 mm for the maxilla and 1.17 mm for the mandible. The survival of the 12 support implants of two total fixed prostheses in a clinical/radiographic follow-up of 10 years was 100%. After 10 years of follow-up, the 12 implants inserted had a bone loss similar to that of healthy patients and no pathologies were registered, recovering function, aesthetics, and self-esteem. This therapy must be implemented before the interincisal distance decreases to 30 mm to allow intraoral surgical/prosthetic access. Implant-supported total fixed rehabilitation is a viable, predictable, and recommended therapy in patients with limited cutaneous systemic sclerosis.

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Evidence-based management of epistaxis in hereditary haemorrhagic telangiectasia

The Journal of Laryngology & Otology ◽

10.1017/s0022215115000365 ◽

2015 ◽

Vol 129 (5) ◽

pp. 410-415 ◽

Cited By ~ 8

Author(s):

I Syed ◽

V S Sunkaraneni

Keyword(s):

Assessment Tool ◽

Case Reports ◽

Treatment Algorithm ◽

Case Series ◽

Team Approach ◽

Hereditary Haemorrhagic Telangiectasia ◽

Questionnaire Studies ◽

Randomised Controlled ◽

Specific Management

AbstractBackground:There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis.Method:The Medline and Embase databases were interrogated on 15 November 2013 using the search items ‘hereditary haemorrhagic telangiectasia’ (title), ‘epistaxis’ (title) and ‘treatment’ (title and abstract), and limiting the search to articles published in English.Results:A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3in vitrostudies.Conclusion:There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.

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548 Heterotopic Ossification or Dystrophic Calcinosis Cutis in Burn Scars - A Case Series

Journal of Burn Care & Research ◽

10.1093/jbcr/irab032.198 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

pp. S122-S123

Author(s):

Sigrid A Blome-Eberwein ◽

Cassandra Pinataro

Keyword(s):

Heterotopic Ossification ◽

Calcium Metabolism ◽

Case Reports ◽

Case Series ◽

Preoperative Assessment ◽

X Rays ◽

Calcinosis Cutis ◽

Pathologic Evaluation ◽

Burn Survivors ◽

History Of

Abstract Introduction Heterotopic ossification of large joints is a commonly seen and described entity in Burn survivors. We have observed a rather uncommon phenomenon in a series of 4 Burn survivors who presented to our Burn center for scar treatment and open wounds many years after their injuries. A chronic, small, splinter-like subcutaneous irritation led to open wounds and infections in their burn scars. A similar condition is known as dystrophic calcinosis cutis in the dermatologic literature, but caused by calcium metabolism changes in otherwise healthy skin. Methods The demographics and history of illness was documented in all 4 patients. A literature review was performed. The calcified bone-like irritant was removed surgically in all patients, sometimes in more than one location. Pathologic evaluation was performed on the removed specimen. Strategies leading to healing of the wounds were evaluated. Results Four publications were found addressing this phenomenon in Burn scars, all case reports. All of our patients had undergone curettage of the lesions by a dermatologist in the past without resolution. All lesions were excised surgically and proved to extend subcutaneously far more extensively than the visible skin lesion. X rays proved not helpful in preoperative assessment of the extent. All wounds healed after the irritant was removed. Conclusions All lesions appeared to have formed along tendon or fascia remnants underneath the burn scars, exerting pressure on the overlying epithelium which eventually led to breakdown and necrosis. This finding prompted the term “heterotopic cutaneous ossification”. Chronic tension and inflammation were assumed in the etiology, rather than calcium metabolism abnormalities or systemic connective tissue disease, which were not present in these patients. Research is needed to establish the prevalence, cause, and prevention of this condition in Burn survivors.

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HOMOLOGOUS DISEASE IN THE ADULT RAT, A MODEL FOR AUTOIMMUNE DISEASE

Journal of Experimental Medicine ◽

10.1084/jem.118.4.635 ◽

1963 ◽

Vol 118 (4) ◽

pp. 635-648 ◽

Cited By ~ 75

Author(s):

Peter Stastny ◽

Vernie A. Stembridge ◽

Morris Ziff

Keyword(s):

Autoimmune Disease ◽

Lupus Erythematosus ◽

Connective Tissue Diseases ◽

Skin Lesions ◽

Cutaneous Lesions ◽

Adult Rats ◽

Hydropic Degeneration ◽

Skin Changes ◽

Adult Rat ◽

The One

The cutaneous lesions of adult rats with homologous disease are described, and evidence is presented to indicate that they have an immunologic basis. The skin changes included erythema, purpura, edema, and a variety of inflammatory lesions. In the more active lesions, dermal infiltration, hydropic degeneration, acanthosis, and atrophy of the epidermis with hyperkeratosis and follicular plugging were present. In some cases, ulceration and sloughing were also observed. More chronic lesions were characterized by atrophy of the epidermis and collagenization of the dermis with disappearance of the skin appendages. Rejection of autografts was observed simultaneously with acceptance of homografts. The histologic appearance of autografts undergoing rejection was similar to that of the spontaneous skin lesions, suggesting that the latter, too, had an immunologic basis. In favor of this, also, was the specificity of the dermatitis for the skin of the host, with sparing of neighboring homograft tissue. There was a histologic similarity between the spontaneous skin lesions of homologous disease and those of lupus erythematosus on the one hand, and scleroderma on the other, thus supporting the possibility that the cutaneous lesions of these connective tissue diseases of man may also have an immunologic basis. It was concluded that the adult rat with homologous disease may furnish a model for human autoimmune disease.

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Call for papers: Case Reports and Clinical Pictures

The Lancet Oncology ◽

10.1016/s1470-2045(05)70520-9 ◽

2006 ◽

Vol 7 (1) ◽

pp. 12

Author(s):

Lidia Siemaszkiewicz

Keyword(s):

Case Reports ◽

Clinical Pictures

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A Clinical Perspective of Low Carbohydrate Ketogenic Diets: A Narrative Review

Frontiers in Nutrition ◽

10.3389/fnut.2021.642628 ◽

2021 ◽

Vol 8 ◽

Author(s):

Samir Giuseppe Sukkar ◽

Maurizio Muscaritoli

Keyword(s):

Case Reports ◽

Scientific Evidence ◽

Experimental Models ◽

Narrative Review ◽

Caloric Content ◽

Beneficial Effects ◽

Cellular Processes ◽

Positive Effects ◽

Ketogenic Diets ◽

Morbid Conditions

Low carbohydrates diets (LCDs), which provide 20–120 g of carbohydrates per day, have long been used as therapeutic options in the treatment of severe obesity, type 2 diabetes mellitus and other morbid conditions, with good results in terms of weight loss and control of the main metabolic parameters, at least in the short and medium term. According to the caloric content and the macronutrient composition, we can classify LCDs in hypocaloric, normoproteic diets [such as the Very Low-Calorie Ketogenic Diet (VLCKD) or the protein-sparing modified fasting (PSMF)], hypocaloric, hyperproteic and hyperlipidic diets (e.g., Atkins, Paleo diets…) and normocaloric, normo-/hyperproteic diets (eucaloric KD), the latter mainly used in patients with brain tumors (gliomas) and refractory epilepsy. In addition to LCD diets, another interesting dietary approach which gained attention in the last few decades is fasting and its beneficial effects in terms of modulation of metabolic pathways, cellular processes and hormonal secretions. Due to the impossibility of using fasting regimens for long periods of time, several alternative strategies have been proposed that can mimic the effects, including calorie restriction, intermittent or alternating fasting, and the so-called fasting mimicking diets (FMDs). Recent preclinical studies have shown positive effects of FMDs in various experimental models of tumors, diabetes, Alzheimer Disease, and other morbid conditions, but to date, the scientific evidence in humans is limited to some opens studies and case reports. The purpose of our narrative review is to offer an overview of the characteristics of the main dietary regimens applied in the treatment of different clinical conditions as well as of the scientific evidence that justifies their use, focusing on low and zero-carb diets and on the different types of fasting.

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SWEET SYNDROME AND HASHIMOTO THYROIDITIS: A CASE REPORT AND REVIEW OF THE LITERATURE

AACE Clinical Case Reports ◽

10.4158/accr-2019-0579 ◽

2020 ◽

Vol 6 (4) ◽

pp. e179-e182

Author(s):

Jacob Goodwin ◽

Samuel Ives ◽

Hiba Hashmi

Keyword(s):

Thyroid Dysfunction ◽

Case Reports ◽

Hashimoto Thyroiditis ◽

Skin Lesions ◽

Thyroid Stimulating Hormone ◽

Disease Stage ◽

Thyroid Peroxidase ◽

Thyroid Function Tests ◽

Sweet Syndrome ◽

Pubmed Database

Objective: Sweet syndrome (SS) is characterized by an inflammatory rash that has been associated with a number of drugs and malignant, inflammatory, and infectious conditions. Rare accounts of Hashimoto thyroiditis (HT) presenting with SS exist in the literature. HT is usually identified after the onset of skin lesions and without signs of overt thyroid dysfunction, and the stage of thyroid disease stage at presentation is variable. Methods: A search of the PubMed database was performed using search criteria involving combinations of “Sweet syndrome” and “Hashimoto thyroiditis,” “autoimmune thyroiditis,” or “thyroiditis,” and the search was filtered for clinical case reports. Five case reports were identified to describe the coexistence of Sweet syndrome and Hashimoto thyroiditis, and full-text versions of these reports were obtained and reviewed. Of note, cases involving subacute or other types of thyroiditis were excluded. Results: A 57-year-old man presented with painful eruptions on his hands; he was initially treated with antibiotics for presumed cellulitis without relief. Skin biopsy later confirmed SS and subsequent workup identified underlying HT with an elevated thyroid-stimulating hormone of 19.24 mU/L (normal, 0.30 to 4.30 mU/L) and positive thyroid peroxidase (TPO) antibody at 236.4 IU/mL. Conclusion: Thyroid function tests should be universally evaluated in the workup of SS, and it may be appropriate to test for TPO antibodies even in the absence of objective thyroid dysfunction. Both SS and HT show immune diathesis, so further work should be undertaken to establish whether a common immunologic trigger exists.

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Knee myopericytoma-case report and literature review

MOJ Orthopedics & Rheumatology ◽

10.15406/mojor.2020.12.00534 ◽

2020 ◽

Vol 12 (6) ◽

pp. 127-128

Author(s):

Susana Rodrigues ◽

Catrine Ferreira ◽

Tiago Coelho ◽

Diogo Gaspar ◽

Jean Fallah ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Case Report ◽

Systemic Sclerosis ◽

Middle Age ◽

Subcutaneous Tissue ◽

Connective Tissue Diseases ◽

Systemic Lupus Erythematous ◽

Systemic Lupus ◽

Calcinosis Cutis ◽

Substantial Morbidity

Calcinosis cutis is a rare disorder characterized by of deposition of insoluble calcium salts in the skin and subcutaneous tissue. Five subtypes of calcinosis cutis are described: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis.1 Of these subtypes, dystrophic calcinosis (DC) is the most common, and it is the most frequently seen in association with underlying autoimmune connective tissue diseases.2 Dermatomyositis, systemic sclerosis and less commonly systemic lupus erythematous were described to be complicated by DC. However, DC associated with rheumatoid arthritis (RA) is extremely rare.2 The condition causes substantial morbidity and is associated with pain and limitation of movement when the process involves areas close to joints or when ulceration occurs.2 We report a middle age Sudanese woman with good controlled RA who developed dystrophic calcinosis cutis.

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Clinical profile of cutaneous manifestations of connective tissue diseases in North-East India

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20213347 ◽

2021 ◽

Vol 7 (5) ◽

pp. 702

Author(s):

Deepa Mala Subba ◽

Nandakishore Thokchom ◽

Linda Kongbam ◽

Erika Salam ◽

Deepa Yumnam

Keyword(s):

Connective Tissue ◽

Lupus Erythematosus ◽

Cutaneous Lupus Erythematosus ◽

Connective Tissue Diseases ◽

Skin Lesions ◽

Discoid Lupus Erythematosus ◽

Serological Tests ◽

Cross Sectional ◽

Cutaneous Manifestations ◽

North East

Background: Connective tissue diseases (CTDs) are a heterogeneous group of autoimmune disorders having overlapping clinical features. Skin is often involved and it may be the earliest sign of the disease. This study highlighted the various cutaneous manifestations of common CTDs.Methods: A hospital-based cross-sectional study was carried out for a period of two years in 83 patients with CTDs in dermatology OPD, RIMS, Imphal. Detailed history taking, examination and relevant serological tests were performed.Results: The mean age was 39.78±17.29 years with female to male ratio of 4.5:1. Majority of the patients had lupus erythematosus (LE) (N=45) followed by systemic sclerosis (SSc) (N=25), rheumatoid arthritis (RA) (N=6), mixed connective tissue disease (MCTD) (N=4) and morphea (N=3). The most common presentation was raised skin lesions (45.8%) followed by Raynaud’s phenomenon (36.1%), photosensitivity (27.7%), skin tightness (26.5%) and joint pain (19.3%). Among LE patients, chronic cutaneous lupus erythematosus (CCLE) was the commonest variant and localised discoid lupus erythematosus (DLE) (22.9%) was the commonest presentation followed by malar rash and annular subacute lupus erythematosus (SCLE). Skin induration, microstomia and sclerodactyly were seen in most patients of SSc. Antinuclear antibodies were positive in 89.1% of patients. Anti-dsDNA and anti-Sm antibodies were positive in 62.2% and 33.3% of LE patients, anti-Scl 70 antibody was positive in 68% of SSc patients.Conclusions: CTDs are rare but potentially life-threatening. Proper understanding of the spectrum of cutaneous manifestations of CTDs is therefore necessary for early diagnosis and efficient management.

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