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Calcinosis cutis is a rare disorder characterized by the deposition of calcium in the skin and subcutaneous tissue. Unilateral idiopathic calcinosis cutis has only rarely been reported in the literature. Here, we report the case of a 7-year-old healthy girl who presented with multiple asymptomatic hard nodules on the right side of her body. Histopathological, radiological, and extensive blood investigations confirmed the diagnosis of unilateral idiopathic calcinosis cutis.

Calcinosis cutis is a rare disease characterized by the deposition of insoluble calcium phosphate crystals in the epidermis. Etiologically it is divided into 4 types: dystrophic, metastatic, iatrogenic and idiopathic. The most common type is the dystrophic type and the rarest idiopathic type. The pathophysiology of calcinosis cutis is not clearly known. Its definitive diagnosis is made histopathologically by showing calcium accumulation in the skin and clinically excluding other types. A pediatric patient was admitted to our clinic with a left auricular mass, and histopathological findings were diagnosed as idiopathic calcinosis cutis. Our case is presented in the light of literature because it is seen in auricula, an atypical localization of idiopathic calcinosis cutis, which is a rare type.

Leg ulcers are not a disease themselves, they are a symptom of a disease. To manage them properly, finding the correct diagnosis of the disease is mandatory. Our case is a model to underline that leg ulcers are a significant burden for the patient, because leg ulcer got ahead of the patient’s other serious complaints such as Raynaud’s phenomenon or sclerodactyly. Furthermore, our patient is also a model, and an attentive clinical evaluation is inevitable to find the correct diagnosis. We present our case aiming to underline the significance of full dermatologic examination in each ulceration to discover underlying disease.

Calcinosis cutis is a rare presentation and not many cases have been reported especially of idiopathic type. We are reporting a case of idiopathic calcinosis cutis of lower limbs in a 33-year old female who presented to our clinic for multiple painless swellings over her lower limbs for the past six months, without any history of trauma or infection. We have decided to observe her condition on regular follow-up and conservative management.

Dermatomyositis is distinctive among the family of idiopathic inflammatory myopathies as patients display a characteristic set of skin manifestations. The cutaneous findings, which can be subdivided into active lesions or those resulting from scarring, occur in particular anatomic distributions. In this chapter, the skin manifestations of dermatomyositis are discussed including facial erythema, “heliotrope” rash, “V sign,” “shawl sign,” “Gottron’s papules,” “Gottron’s sign,” “holster sign,” “mechanic’s hands,” “ragged” cuticles with nailfold changes, and calcinosis cutis. This chapter will also highlight the prevalence of these findings within the various myositis subtypes. An understanding of the protean cutaneous features of dermatomyositis is essential to accurately diagnose and treat this autoimmune disease.

Calcinosis cutis is a rare metabolic disorder characterized by cutaneous and subcutaneous deposition of insoluble calcium salts. Ocular adnexal involvement is uncommon but mostly seen in males and in the upper eyelid. Solitary lesions are more common than multiple. The treatment of choice is excision biopsy. Histopathological examination provides a definitive diagnosis. Directed systemic investigations help to rule out underlying pathologies and aids further management. The present case reports the punctal and peripunctal involvement in a case of ocular adnexal calcinosis cutis.

Aim: The aim of this study was to evaluate retrospectively the patients who were referred to the urology clinic with genital skin lesions and evaluated by dermatology. Materaials and Methods: Forty-four male patients over 18 years of age who were applied to urology clinic in the last 4 months with a skin lesion in the genital area and who were evaluated by the department of dermatology were included. Patients who applied to the urology clinic with a skin lesion in the genital area were first evaluated by a urologist. These patients were then directed to the dermatology polyclinic and evaluated by a dermatologist. Results: A total of 44 male patients between 19 and 60 years of age were included in the study. The mean age of the patients was 36.97 ± 12.17. In the inflammatory diseases category, contact dermatitis was found in 11 (25%) patients, lichen simplex chronicus in 2 (4.5%) patients, fix drug eruption in 2 (4.5%) patients, psoriasis in 2 (4.5% , 3) lichen planus in 1 (%2,3) patient, and lichen sclerosus in 1 (2,3%) patient had. In the infectious diseases category, 17 (38.6%) patients had condyloma acuminata, 3 (6.8%) patients had herpes simplex infection and 1 (2.3%) patient had molluscum contagiosum. In the other diseases category, angiokeratom in 2 (4.5%) patients, vitiligo in 1 (2,3%) patient and calcinosis cutis in 1 (2,3%) patient were present. Conclusion: A multidisciplinary approach is needed in the diagnosis and treatment of diseases in the genital region.

Presence of the characteristic heliotrope rash on eyelids, violaceus rash in sun exposed areas and the Gottron papules on knuckles, is necessary for the diagnosis of juvenile dermatomyositis Symptomatic lung diseases and association with underlying malignancy are more common in adult dermatomyositis but not juvenile dermatomyositis Calcinosis cutis and cutaneous ulcerations are seen up to 30% cases of juvenile dermatomyositis Intravenous immunoglobulin, cyclophosphamide, and mycophenolate mofetil are first-line immunosuppressive therapy in juvenile dermatomyositis Intensive physiotherapy and strict photoprotection are essential ellements in treatment of dermatomyositis

A 6-year-old girl presented with a history of blistering and scarring in trauma-prone areas. On examination, calcium deposits were seen on bilateral palms and soles within her non-healing wounds. Clinical, genetic and radiological evaluation confirmed the diagnosis of autosomal recessive dystrophic epidermolysis bullosa with dystrophic calcification. The patient was started on topical 10% sodium thiosulfate for her calcinosis cutis. Identification and management of dystrophic calcification are important as it impairs wound healing.

Background Pheochromocytoma (PCC) has poor prognosis and adrenalectomy is hard to be performed, in case of caudal vena cava invasion. The long-term administration of phenoxybenzamine in PCC has not been reported in dogs. Case presentation A 14-year-old castrated male Poodle dog presented with an abdominal mass. On physical examination, hypertension, increased lens opacity, calcinosis cutis, generalized alopecia, and systolic murmur were observed. Serum chemistry and urinalysis profiles revealed hyperglycemia, hypercholesterolemia, elevated liver enzymes, and glucosuria. Abdominal ultrasonography showed a right adrenal mass with invasion of the caudal vena cava, which was cytologically diagnosed as suspected PCC. An adrenal mass (width × height × length, 28 × 26 × 48 mm3) was found on computed tomography and diagnosed as PCC with increased plasma metanephrines and normetanephrines. An adrenocorticotropin hormone stimulation test showed elevated adrenal hormones (androstenedione, estradiol, progesterone, and 17-OH progesterone) with normal cortisol, compatible with atypical Cushing’s syndrome. The dog was managed with trilostane, phenoxybenzamine, and insulin therapy. Glycosylated hemoglobin and fructosamine levels gradually decreased, and hypertension resolved. In the 10-month follow-up period, the liver enzymes levels gradually decreased, and the clinical signs of the dog were well-controlled without deterioration. Conclusions This case report describes long-term medical management without adrenalectomy of PCC complicated with atypical Cushing’s syndrome and DM.

A 10-year-old, neutered female, Australian Shepherd was referred for acute respiratory distress and a history of chronic exogenous steroid administration. On thoracic radiographs, a severe increase in mineral opacity characterized as a generalized unstructured interstitial pulmonary pattern, diffuse calcinosis cutis, and moderate hepatomegaly were noted. Cor pulmonale was identified on echocardiography. The patient developed a pneumothorax following sampling and had a cardiac arrest. Postmortem histopathology of the lungs revealed pulmonary interstitial mineralization and alveolar microlithiasis. This report supports including generalized pulmonary mineralization due to chronic exogenous steroid administration as a differential diagnosis for dogs with these clinical and imaging findings.

Mujer de 54 años, ama de casa, hipertensa, con antecedente de cáncer de mama derecha. Luego de un accidente en el hogar, presentó tumoraciones con flogosis que no remitieron con medicación sintomática. En la radiografía se evidenció dos lesiones calcificadas, una en cara lateral del brazo izquierdo y otra en la pared torácica a nivel de octava costilla derecha. Los estudios metabólicos e inmunológicos fueron normales. Fifty four year-old woman, housewife, with hypertension and past history of right breast cancer. After a household accident, warm tumors appeared that were resistant to symptomatic treatment. Radiographic studies showed two calcified lesions, one in the lateral aspect of the left arm and other in the chest wall at the level of eighth right rib. Metabolic and immunologic studies were normal.

Background Calcinosis includes a rare group of disorders characterized by abnormal intracutaneous, subcutaneous, fascial or intramuscular calcium deposits. Idiopathic calcinosis is diagnosed after the exclusion of secondary forms related to trauma, abnormal calcium/phosphorus metabolic disorders, inflammatory processes, neoplasms, connective tissue diseases or renal insufficiency. There are very few cases reported, especially in childhood. Objectives We report the case of idiopathic calcinosis cutis in a young girl. Methods A 15-year-old female presented an abrupt onset of 2 painful swollen lesions on the left trochanteric region and over the posterior side of her right elbow. The patient denied any trauma or associated symptoms such as muscle weakness or joint stiffness. On physical examination, a large hard mass was present over her left trochanteric region, with hyperemic skin, and a similar one was palpable over her right elbow. Results Radiographs of the two sites showed well-defined calcified masses, one (9x5 cm) over the trochanteric region, and the other (3x2) cm over the posterior aspect of the distal end of the humerus extending up to the elbow. MRI confirmed the presence of calcified intramuscular lesions (Figure 1). Serologic evaluation showed increased levels of CRP and ESR, while autoimmune screening was negative. Calcium/phosphorus metabolism, creatinine kinase, aldolase and ferritin levels were within the normal limits. Therefore, a clinical diagnosis of idiopathic calcinosis was made. After 3 months, the lesion on the elbow had spontaneously reabsorbed, while the other one kept on growing, with 3-4 episodes per month of febrile painful swelling. A CT-guided biopsy was then performed, confirming the diagnosis of calcinosis. No infectious causes were detected. One year later, since there was no local amelioration, the lesion was surgically removed with apparent complete local remission. No other new lesions were detected during the 3 months follow-up period. Conclusion Idiopathic calcinosis is very rare and no specific medical therapies are available. In our patient no causative factors were identified and thus the diagnosis of idiopathic calcinosis was made. Prognosis seems to be variable, since one lesion risolved spontaneously while the other needed surgical excision. References [1] Alabaz D, Mungan N, Turgut M, Dalay C: Unusual Idiopathic Calcinosis Cutis Universalis in a Child. Case Rep Dermatol2009;1:16-22. doi: 10.1159/000227285 [2] N. Reiter, L. El-Shabrawi, B. Leinweber, A. Berghold, and E. Aberer, “Calcinosis cutis. Part I: diagnostic pathway,”Journal of the American Academy of Dermatology, vol. 65, no. 1, pp.1–12, 2011. [3] [3] Hwang ZA, Suh KJ, Chen D, Chan WP, Wu JS. Imaging Features of Soft-Tissue Calcifications and Related Diseases: A Systematic Approach. Korean J Radiol. 2018Nov-Dec;19(6):1147-1160. [5] S. K. Venkatesh Gupta, Ramana Rao Balaga, and Suman Kumar Banik, “Idiopathic Calcinosis Cutis over Elbow in a 12-Year Old Child,”Case Reports in Orthopedics, vol. 2013, Article ID 241891, 4 pages, 2013. Disclosure of Interests Lisa Gamalero: None declared, Ilaria Pagnini: None declared, Gabriele Simonini Grant/research support from: Abbvie, Speakers bureau: Abbvie, Rolando Cimaz: None declared, Teresa Giani: None declared

Background Juvenile dermatomyositis (JDM) is characterized by the presence of microangiopathy which can be assesed by nailfold capillaroscopy (NFC), a simple and noninvasive imaging technique. Morphological abnormalities in scleroderma NFC are well described, but it is known that they may be present in other autoimmune diseases, such as JDM. Objectives To describe the capillary morphological abnormalities assessed by NFC in a series of patients diagnosed with JDM in contrast with patients diagnosed with juvenile idiopathic arthritis (JIA). Methods A cross-sectional descriptive study in which 34 patients were recruited sequentially and randomly, 15 with JDM and 19 with JIA, from the Rheumatology department of Nino Jesus Hospital between September-November (2018). Demographic, clinical, analytical data, presence and subtype of calcinosis, active treatment, HAQ, patient and physician global assessment (PGA), cutaneous assessment tool (CAT) and manual muscle testing (MMT8) validated for JDM were collected. One observer performed blindly, both for clinic and diagnosis, a NFC initially using a videodermatoscope in order to obtain a wide view of the vascular bed and then with a videocapillaroscope (x500), which recorded 2-4 images per finger (except thumbs) to be analyzed far ahead. The presence of a decreased nailfold capillary density (NCD, capillary n°/mm≤6), architectural derangement (disorganization), neoangiogenesis, giant capillaries (o>50μm, pathological if ≥1), enlarged loops (o20-50μm) and microhaemorrhages, both pathological present in ≥2 fingers were evaluated. Neovascular pattern was defined as the sum of NCD, disorganization and neoangiogenesis with frequent haemorrhages and giants (active and late scleroderma pattern). Results The descriptive analysis of the main variables is shown in table1. Patients with JIA: all received active treatment, 95% MTX (33% concomitant with anti-TNF, 1 TCZ and 16% CE) and 1 anti-TNF. 26% active uveitis. Two patients presented morphological abnormalities: 1 neoangiogenesis and another enlarged loops. Patients with JDM: 73% received MTX (18% concomitant with CE, 73%Igs and 1 RTX). Three patients had active calcinosis: 2 mixed (circumscribed and cutis) and 1 calcinosis cutis. 2 had previous calcinosis (both circumscribed). 87% presented for the first-time weakness but none had current weakness (MMT8 40). 67% presented morphological abnormalities: NCD 33%, disorganization 40%, neoangiogenesis 53%, enlarged loops 27%, giants 20% and microhaemorrhages 13%. All patients with calcinosis (active or previous) presented disorganization and neoangiogenesis, NCD 80%, giants 60%, enlarged loops and microhaemorrhages 40%. 50% of patients with JDM had a neovascular pattern, of which 80% had a previous or current history of calcinosis. Conclusion Patients with JDM presented more abnomalities in NFC compared to patients with JIA. Neovascular pattern was observed in half of patients, who have previous or current history of calcinosis. Consequently, NFC could be a useful imaging technique in routine clinical practice to evaluate skin activity in patients with JDM. References [1] Wu Q. Juvenile dermatomyositis: Latest advances Best Pract Res Clin Rheumatol.2017 Aug;31(4):535-557. 2.Barth Z. Assesment of microvascular abnormalities by Nailfold Capillaroscopy in Juvenile Dermatomyosistis after medium to long-term follow up. Arthritis Care Res (Hoboken). 2018 May;70(5):768-776. Disclosure of Interests None declared

A case of a 15-year-old male patient with a 3-year history of linear, segmental amyopathic dermatomyositis with calcinosis cutis is presented. The calcinosis was recalcitrant to treatment with topical steroids and hydroxychloroquine. Topical 10% sodium thiosulfate use for 8 weeks resulted in improvement. The use of topical sodium thiosulfate for patients in whom surgical extraction is not an option is detailed.

The purpose of this case study is to describe the surgical treatment of idiopathic ulcerative cutaneous calcinosis or calcinosis cutis of the lower extremity. A 77-year-old Latin American female who reported no significant past medical history presented to our hospital's emergency department from her home complaining of worsening right lower extremity erythema, edema, increased temperature, and pain. It was noted that the patient presented with multiple cutaneous calcified nodules to bilateral lower extremities, which she stated has been present for approximately 40 years. At the time of evaluation, 1 of the nodules on the lateral aspect of the right lower extremity ulcerated and became infected with unknown etiology, which lead to cellulitis of this limb. Radiographic imaging studies of the bilateral lower extremities showed extensive sheetlike soft tissue calcification overlying the middle to distal lower extremities. Serology reports showed the patient was positive for rheumatoid factor, antinuclear antibodies, SS-A/Ro antibody, and SS-B/La antibody. Because of the evidence of frank purulence and cellulitic changes to the infected nodule, the patient was taken to surgery the following day for sharp debridement and biopsy of the site. Postoperatively, there were minimal signs of improved healing to the wound base, although there was evidence of decreased erythema and edema to the extremity after the initial debridement and biopsy. Four days after the initial surgical invention, the patient was taken for a second operative procedure, which included a wide excisional biopsy with application of acellular dermal matrix and negative-pressure therapy. It was during this secondary debridement that further calcified deposits were encountered and specimens were submitted to pathology. Pathologic examination diagnosed the submitted specimen as cutaneous calcinosis. At this time, the patient is currently undergoing local wound care of the soft tissue deficient to her right lower extremity with the assistance of negative-pressure wound therapy with biweekly clinical follow-up.

International audience; Background: We have limited data on the treatment of calcinosis cutis associated with systemic sclerosis and dermatomyositis.Objective: To assess the efficacy and tolerance of available treatments for calcinosis cutis based on previously published studies.Methods: We performed a systematic review of studies published in Medline, Embase, and the Cochrane library during 1980-July 2018. The strength of clinical data was graded according to the modified Oxford Centre for Evidence-Based Medicine levels of evidence.Results: In all, 30 studies (288 patients) were included. Eleven therapeutic classes, surgery, and physical treatments were identified as potential treatment options for calcinosis cutis. On the basis of results of a small randomized controlled trial and 4 retrospective studies, low-dose warfarin should not be used for calcinosis cutis (level IB evidence). The results of several studies suggest diltiazem and bisphosphonates might be useful treatment options (level IV). Considering biologic therapies, rituximab has shown promising results in treating both dermatomyositis and systemic sclerosis, whereas tumor necrosis factor inhibitors might be useful for treating juvenile dermatomyositis (level IV). Intralesional sodium thiosulfate might be a promising alternative (level IV).Limitations: Few included studies had a high level of evidence.Conclusion: This study highlights the efficacy and tolerance profiles of available treatments for calcinosis cutis, with a focus on level of evidence.

Juvenile dermatomyositis (JDM) is a systemic inflammatory disease involving children, which primarily affects the skin and the musculoskeletal system. The characteristic findings include Gottron papules, heliotrope rash, calcinosis cutis, and symmetric proximal muscle weakness. Histologically, it is characterized by the presence of lymphocytic vascular inflammation and endothelial swelling. Herein, we report a case of a 10-year-old girl of Indian origin, who presented to us with classical clinical and histological features of JDM.

Idiopathic subepidermal calcinosis, also known as idiopathic calcinosis cutis, is a rare condition, which typically presents as a small, hard, subepithelial mass in the absence of systemic disease and trauma. We report on an atypical case of idiopathic calcinosis, which appeared in an otherwise healthy 61-year-old female along the tarsus of her right upper eyelid. The purpose of this case report is to demonstrate idiopathic calcinosis in an unusual location that is deep to the dermis and to discuss the vast differential diagnosis of this entity.

Calcinosis cutis is an unusual disorder characterised by calcium–phosphate deposition into cutaneous and subcutaneous tissues. There are five subtypes: dystrophic, metastatic, idiopathic, iatrogenic and calciphylaxis. Our objective was to report a patient with a history of haemodialysis-dependent end-stage renal disease (ESRD) who developed metastatic calcinosis cutis, which is usually associated with abnormalities of calcium–phosphate metabolism in renal failure. In our patient, the serum calcium and phosphate levels were normal. Additionally, we describe the diagnostic approach and the difficulties involved in the management of calcinosis cutis in patients with haemodialysis-dependent ESRD.

Juvenile Dermatomyositis (JDM) is an idiopathic inflammatory myopathy characterized by the weakness of skeletal muscle and pathognomonic skin rashes. Studies focusing on the cutaneous manifestations of JDM are sparse. The objectives of the study were (1) to describe cutaneous changes seen in JDM and (2) to observe nailfold capillaroscopy (NFC) changes in patients with JDM. This was a retrospective case series. All children under the age of 15 years who were diagnosed to have dermatomyositis according to the Bohan and Peter criteria and on histopathology presenting to the dermatology outpatient department during a period of 1.5 years (January 2017–June 2018) were included in the series. Details including clinical features and finding from investigations such as NFC, muscle magnetic resonance imaging, electromyography, muscle enzymes, and biopsy were noted. Statistical analysis was performed on the basis of measures such as percentage and frequency of cutaneous manifestations. A total of eight cases of JDM were included, with average age at presentation being 7.62 years. Among the cutaneous manifestations, the most common presenting features were heliotrope rash and Gottron's papule. Facial telangiectasias, calcinosis cutis, and hypertrichosis were seen in 37.5% of cases. Other cutaneous features seen were confluent and reticulate violaceous erythema, cutaneous ulcer, and periungual telangiectasia with cuticular overgrowth. NFC was suggestive of scleroderma-dermatomyositis pattern in all six patients in whom it was conducted. JDM has classic, pathognomonic skin manifestations that may or may not correspond with muscle inflammation. To avoid complications, early diagnosis and prompt treatment is the mainstay. NFC acts as an important diagnostic tool and also helps to characterize the phase of disease.

Idiopathic calcinosis cutis is characterized by multiple nodules present on scrotal skin. They are usually seen in young adults and present as painless, asymptomatic, round, and firm nodules. A 30-year-old male presented with painless, multiple scrotal nodules. The serum levels of calcium, phosphorus, calcitonin, and parathyroid hormone were within normal limits. There was also no history of trauma. Clinically, it was diagnosed as sebaceous cyst. Fine-needle aspiration cytology examination was suggestive of epidermal inclusion cyst. Histopathology examination confirmed the diagnosis of idiopathic scrotal calcinosis cutis, thus suggesting that the dystrophic calcification of epidermal inclusion may be an initiating event in the pathogenesis of the idiopathic scrotal calcinosis cutis. The documentations regarding the association of idiopathic calcinosis cutis with epidermal cyst are not enough. Hence, this case report may enrich the scanty existing data.