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Paciente femenina de 41 años de edad con antecedente de dermatomiositis diagnosticada desde hacía 6 años, en tratamiento con prednisona e hidroxicloroquina. Consultó por presencia de lesiones dolorosas en la piel de 3 meses de evolución, múltiples, en región axilar y cara dorsal de manos, con presencia de secreción blanca tipo tiza, por lo que se diagnostica calcinocis cutis. Este es un hallazgo extremadamente inusual en pacientes con dermatomiositis clásica.

Introduction: Fine needle aspiration cytology (FNAC) forms one of the first diagnostic tools in evaluation of soft tissue lesions. Aim of this study was to evaluate the role of FNAC in the primary diagnosis of soft tissue tumors and tumor like lesions and to assess the effectiveness of FNAC in the histological subtyping and grading of soft tissue tumors. Materials and Methods: We reviewed FNAC smears of 170 soft tissue tumors and tumor like lesions over a 3 years period and correlated with the histopathological features which were available in 117 cases. Conventional Papanicolaou (PAP) and Giemsa staining was done in all cases. Results: 152 out of 170 cases [89.41%] were neoplastic, of which 130 tumors were labelled as benign. Lipoma constituted 83.07% of benign tumors. Based on the predominant cytomorphological features and background, the tumors were classified into myxoid (4), spindle cell (20), pleomorphic (2), small round cell(4), epithelioid /polygonal cell (7), lipomatous (108) and miscellaneous (7). The non-neoplastic lesions included tuberculosis, filariasis, actinomycosis, scar endometriosis and calcinosis cutis. Conclusion: FNAC proved to be helpful in distinguishing between neoplastic and non-neoplastic soft tissue lesions, differentiating metastatic carcinoma and melanoma in soft tissue from primary soft tissue tumors and differentiating benign and malignant soft tissue tumors. Accurate subtyping was possible in many tumors of myxoid category and round cell tumors. Spindle cell tumors of intermediate grade were difficult to grade on FNAC. Pleomorphic sarcomas were of high grade and their subtyping could not be done on FNAC. Keywords: FNAC, Soft tissue lesions, Soft tissue tumors.

Objective The occurrence of calcinosis cutis as a clinical feature of dermatomyositis in adult patients is not well understood. Cohort studies of adult patients with calcinosis are rare. We systematically describe the clinical features, treatments and outcomes of adult patients with calcinosis. Methods We initially enrolled 627 adult DM patients. Of those enrolled, 35 (5.6%) were found to have calcinosis. We analysed the clinical features associated with calcinosis in this subgroup. The risk factors associated with calcinosis were analysed using the Poisson regression model. Results Multivariate analysis showed that a younger age at disease onset [odds ratio (OR) = 0.945, 95% CI 0.925, 0.966, P Conclusion Calcinosis is an uncommon, but significant clinical feature in adult patients with DM. Bisphosphonates were not found to effectively treat calcinosis, however, the overall health outcomes of adult DM patients with calcinosis were favourable.

Calcinosis cutis (CC) is defined as the deposition of calcium salts on the skin and subcutaneous tissue. It is associated with different conditions, including some autoimmune diseases, and it can generate significant inflammation, pain, and functional impairment. Different therapies have been tried with limited results. Intralesional sodium thiosulfate seems a promising therapeutic option. We report a patient with diffuse systemic sclerosis who presented with two symmetrical plaques on both axillae, which caused pain and skin retraction. The clinical diagnosis was consistent with CC, which was confirmed by skin biopsy and ultrasound. The patient was treated with a 250 mg/ml solution of sodium thiosulfate injected into the plaques. Complete resolution was achieved after three monthly sessions. The only reported adverse effect was a transient burning sensation during the injections. Given its effectiveness and safety, we believe that intralesional sodium thiosulfate could become a valid first-line option for the treatment of CC.

Calcinosis cutis is the aberrant deposition of insoluble calcified salts in cutaneous tissue that occurs in association with a variety of diseases. A relatively uncommon disorder, it can range in severity from mild to debilitating. This article describes a patient with a long history of dermatomyositis who developed multiple calcific lesions in the extremities that did not respond to conservative therapies and eventually required multiple surgical interventions.

A 9-year-old boy presented for evaluation of variegated skin pigmentation. Palms and soles revealed honeycombed hyperpigmented hyperkeratosis. Irregular, firm, skin coloured nodules suggestive of cutaneous calcification were present on both elbows. Total leucocyte count and absolute neutrophil count were 3720/mm3 and 420/mm3 respectively. The neutropenia was not cyclical. Systematic analysis of the whole exome data revealed a homozygous mutation in USB1 gene; chr16:g.58043892TA>-[1/1]. A final diagnosis of poikiloderma with neutropenia- Clericuzio type (PNC) was made. Naegeli Franceschetti Jadassohn, dermatopathia pigmentosa reticularis, PNC and dyskeratosis congenita, all can present with overlapping cutaneous manifestations. Subtle clinical details like thickened nails, hyperextensible joints, calcinosis cutis, characteristic facies and a preceding erythematopapular rash strongly favor the diagnosis of PNC. The index case highlights two novel findings: obliterated dermatoglyphics and mucin deposition (features not described hitherto in PNC).

Dermatomyositis is a rare disease characterized by classic skin lesions and muscle weakness. In rare cases, life-threatening hypercalcemia may develop caused by regression of dystrophic calcifications. Here we report a 36-year-old man who presented with progressive proximal weakness, difficulty in ambulation, and weight loss. He had the V-sign, Gottron’s papules, and hard, chalky nodules on both antecubital, thigh, and hip areas. Laboratory examinations revealed hypercalcemia (3.47 mmol/L) and shortened QT interval. Workup for malignancy and tuberculosis yielded negative results. Biopsy of the antecubital areas revealed calcinosis cutis. Serum calcium levels gradually normalized with hydration and steroids. Our case illustrated that a high index of suspicion for dermatomyositis is warranted for early diagnosis and ascertaining the etiology of hypercalcemia is vital in the management of this life-threatening complication. While hypercalcemia from dermatomyositis may respond to steroids, to date, individualization of treatment remains the standard of care. (J Rheum Dis 2020;27:285-289)

Objectives To describe consecutive vulvar biopsy cases and to create an educational template for pathology trainees and practicing pathologists. Methods We reviewed 189 consecutive biopsies from the female genital area skin and mucosa. We classified them based on etiologies and examined limited clinical information. Results We classified diagnoses as squamous intraepithelial neoplasia (21.5%), melanocytic neoplasia (17.9%), lichenoid dermatoses (15.9%), nonlichenoid dermatoses (11.3%), infectious (6.2%), reparative (4.6%), or miscellaneous (22.6%). The miscellaneous diagnoses included common entities (polyps and cysts) and rarer entities (calcinosis cutis, adnexal neoplasms, or basal cell carcinoma) and nonspecific descriptive diagnoses. Clinicians most often included the actual diagnosis in their differential for melanocytic lesions (83%) and least often for inflammatory lesions (32%). However, some cases included a clinical description without a differential diagnosis (14%) or no helpful clinical information (4%). The distribution of whether correct diagnoses were included in the clinical differential was similar between submitting physicians and midlevel providers. Conclusions Understanding squamous and melanocytic pathology and the various lichenoid and other inflammatory diagnoses is critical for signing out female genital tract skin pathology. The cases examined in this report can serve as an educational template for trainees and practicing pathologists.

Background: A wide range of inflammatory as well as neoplastic lesions can present as nodular, soft, mobile subcutaneous swellings all over the body. FNAC(fine needle aspiration cytology) is considered to be the first reliable technique for diagnosing them. Aim: To study the cytomorphological patterns of subcutaneous nodular lesions and correlate with histopathology. Materials and Methods: A three year prosective study of all palpable subcutaneous swellings presenting to cytology section with histopathological correlation. Statistical Analysis: The data are expressed in descriptive statistics measures such as percentages and proportions. A continuous data was presented as mean and standard deviation (SD). Results: Out of 1427 total cases, most common were benign lesions (97.4%) including lipomas (35.24%) followed by benign spindle cell lesions (17.02%). Malignant cases comprised only 2.7%. Many rare lesions like gouty tophi, calcinosis cutis, filariasis, myositis ossificans were also encountered. Biopsy correlation could be done in 12% of cases. Conclusion: Small subcutaneous swellings though appear benign in most of the cases, can be due to an interesting and grave aetiology which must be kept in mind of clinicians as well as cytopathologists. Keywords: Biopsy, Cytodiagnosis, Subcutaneous nodule.

Background Intralesional injection of sodium thiosulfate has emerged as a promising therapy for calcinosis cutis, but to our knowledge there are no randomized controlled trials evaluating its efficacy as a treatment. Objective Conduct a prospective, double-blinded investigation of intralesional sodium thiosulfate versus normal saline in the treatment of dystrophic calcinosis cutis. Methods This prospective pilot study injected normal saline or sodium thiosulfate at 0.1 mL/cm2 into lesions at baseline and at 1- and 2-month follow-up. Subjects were followed for a total of 12 weeks. An in-person Physician Global Assessment score was assigned by the injecting physician at each visit and was repeated by an independent observer. Results Of 4 subjects who completed the study, only 1 experienced improvement in the size and Physician Global Assessment score of the lesion. By 3-month follow-up, there was no difference between the average size of the treatment and control lesions (P = .39). Limitations This was a small single-center study with limited demographic diversity and a short follow-up period. Only dystrophic calcinosis cutis subjects were included, and subjects received only 3 monthly injections of sodium thiosulfate. Conclusions With only 1 positive response, our results highlight the need for further study of sodium thiosulfate treatment for dystrophic calcinosis.

Soft tissue calcifications associated with various connective tissue diseases such as dermatomyositis and scleroderma have been well documented Plaque-like sheets of subcutaneous calcifications presenting as an indurated soft tissue mass in a patient with primary Sjogren syndrome have been rarely documented in the literature. We present the magnetic resonance and conventional radiographic findings of calcinosis cutis and calcinosis circumscripta of a 47-year-old woman with biopsy proven Sjogren syndrome. We also delineate various types of soft tissue calcification, histopathology of calcinosis cutis, and current treatment options. Recognizing the magnetic resonance characteristics of this phenomenon may prove useful to radiologists, especially in the absence of clinical history and conventional radiographs.

We present a rare case of calcinosis cutis associated with chronic sclerodermoid graft versus host disease in a 59-year-old male, 13 years following allogenic bone marrow transplantation. The etiology of calcification was thought to be dystrophic. Further research is needed to understand the link between calcinosis cutis and chronic sclerodermoid graft versus host disease to assist with selecting appropriate management for these patients.

Background Calcinosis cutis is a rare condition associated with different diseases, which is difficult to manage. Aims and objectives In this retrospective study, the epidemiology of calcinosis cutis and the effectiveness of various treatment regimens in its management were assessed in a single center. Materials and methods The data of 34 patients suffering from calcinosis cutis (male:female = 12:22; mean age = 48.6 ± 18.6 years) treated at our department between 2003 and 2016 were analyzed retrospectively. Results Dystrophic, idiopathic, metastatic subtype, and calciphylaxis occurred in 70.6%, 11.8%, 5.9%, and 11.8% of the cases, respectively. Underlying diseases of dystrophic calcinosis included autoimmune connective tissue disease, skin trauma, cutaneous neoplasm, and inherited disorder in 58.3%, 20.8%, 12.5%, and 8.3% of the cases, respectively. Extremities were most frequently affected (n = 18). In the management, diltiazem was most frequently used in monotherapy with partial response in five of eight cases. Other drugs in monotherapy or in combination were administered in single cases. Surgical treatment resulted in least partial response in all of the cases followed (n = 7). Conclusion Dystrophic was the most common subtype and autoimmune connective tissue disease was the most frequent underlying disease. We conclude that lower doses of diltiazem have only partial efficiency, and surgical therapy is at least partially effective in localized calcinosis.

Objectives Calcium depositions are frequent in multiple inflammatory dermatosis, they can be explored by ultrasound (US) but the patterns of these depositions have not yet been described. The aim of this study is to describe different patterns of calcium deposition in inflammatory dermatoses. Methods The clinical and US data of 58 patients from 7 different centers with inflammatory dermatosis showing ultrasonography-detected calcium depositions was retrospectively reviewed. Results Dystrophic calcinosis represented 86.2%, calciphylaxis 8.6%, and metastatic calcinosis 5.2%. Three different sonographic patterns of calcium deposition were found: 1) thin hyperechoic bands, parallel to the surface of the epidermis, generating a strong and wide posterior acoustic shadow; 2) hyperechoic spots or lumps with a narrow acoustic shadow; and 3) a linear hyperechoic band parallel to the walls of a blood vessel with also a narrow acoustic shadow. The predominant pattern in metastatic calcifications was type 1, in dystrophic calcifications type 2, and in calciphylaxis type 3. In dystrophic calcinosis, cutis deposits were longer and wider than in calciphylaxis (P < .05). Conclusion New data on inflammatory dermatoses with calcium deposition may be useful for the diagnosis and monitoring of calcium deposits and could avoid the performance of more invasive tests, such as a skin biopsy.

Dermatomyositis is an inflammatory myopathy that affects children and adults. It can present without muscle involvement in the case of amyopathic dermatomyositis. Whereas the juvenile variant is often associated with calcinosis cutis, the adult variant is typically associated with malignancies.1 Approximately 94% of the patients with dermatomyositis develop cutaneous manifestations.2,3 Typical skin findings include the Gottron sign, which refers to a symmetrical heliotropic livid-erythematous macule on the face often including the eyelids, Gottron papules on the elbows and metacarpal or interphalangeal joints of the hands, nailfold changes characterized by ragged cuticulae and enlarged telangiectasias in the nail bed, and poikilodermatous skin changes in the neck and on the chest (shawl sign). Histopathology reveals epidermal atrophy, vacuolar changes of the basal epidermal cell layer, hyperkeratosis, mucin deposition, basement membrane thickening, dermal edema, pigment incontinence, and a perivascular infiltrate.4 Treatment includes topical and/or systemic corticosteroids. Hereafter, steroid-sparing therapy is initiated with methotrexate, hydroxychloroquine, azathioprine, tacrolimus, cyclosporine A, mycophenolate mofetil, intravenous immunoglobulin, or rituximab.5 Despite therapy, symptoms such as pain and burning sensation associated with skin lesions can be therapy-resistant and affect the quality of daily life. Laser therapy is an emerging treatment in inflammatory skin diseases for which only very few reports exist on dermatomyositis. Here, we report the resolution of the shawl sign with 2 sessions of pulsed dye laser (PDL).

Hyperphosphatemic familial tumoral calcinosis (HFTC) is an extremely rare autosomal recessive disorder caused by variants in the GALNT3 (N-acetylgalactosaminyltransferase 3), FGF23 (Fibroblast Growth Factor-23) and αKL (α-Klotho) genes, which results in progressive calcification of soft tissues. We describe the case of a 9-year-old girl who presented with recurrent hard nodular swellings on her feet and knees which intermittently discharged chalky white material. Her younger brother also had a similar condition. Both siblings showed hyperphosphatemia, but the parentsbiochemical parameters were normal. The histological features of the material aspirated from a skin lesion were consistent with tumoral calcinosis. Sanger sequencing identified a novel homozygous non-synonymous sequence variant in exon 10 of the GALNT3 gene (NM_004482.3:c.[1681T>A];[1681T>A], NP_004473.2:p. [Cys561Ser];[Cys561Ser] in the proband and her affected brother. The parents were heterozygous carriers for the same sequence variant. In conclusion, we report a new variant in the GALNT3 gene that caused HFTC in a North Indian family.

Calcinosis cutis is very rare disorder characterized by the deposition of calcium in the skin and subcutaneous tissue. Idiopathic calcinosis cutis has only rarely been reported in the literature. Here, we report cases of two healthy females 40 year old and 55 year old who presented with multiple asymptomatic hard nodules on the fingertips of their hands. Histopathological, radiological, and extensive blood investigations confirmed the diagnosis of idiopathic calcinosis cutis.

Endocrine disorders are known to involve all organ systems of the body, including the skin. The cutaneous manifestations of endocrine disorders can range from common findings such as acanthosis nigricans, pretibial myxedema, acne, hirsutism, hyper or hypopigmentation to rare cutaneous findings such as miliaria rubra, calciphylaxis, lentigines, and calcinosis cutis. These cutaneous symptoms can sometimes be the presenting symptoms or can even be pathognomonic of the underlying endocrine condition. In some cases, the cutaneous symptoms from an underlying endocrine abnormality may be the most prominent. These symptoms can significantly affect the quality of life of individuals. Often, individuals may seek health care from a dermatologist or primary care physician for isolated skin symptoms. Therefore, it is imperative for physicians to recognize the skin symptoms as the manifestation of the endocrine disorder for prompt diagnosis and treatment of the underlying endocrine disorder.

We report an 11-year-old girl who presented with white papules on the dorsal and palmar region of the hands bilaterally. The parents reported that the lesions had appeared four months before and some had resolved spontaneously. The girl was suffering from celiac disease, Down syndrome, and alopecia areata treated with topical corticosteroids. At the first visit, the girl presented with alopecia areata, corticosteroid acne, and a dozen white papules located on the hands. On dermoscopy, a whitish structureless area was seen. Histological examination showed the presence of calcium deposits without tissue damage, thus confirming the diagnosis of milia-like idiopathic calcinosis cutis. At 6-month follow up, the lesions had completely disappeared. Milia-like idiopathic calcinosis cutis is a benign cutaneous disorder consisting of calcium deposits in an apparently undamaged dermis and is typically associated with Down syndrome. Up to a quarter of patients have coexisting syringomas. The milia-like papules tend to self-resolve as patients reach adulthood, so a wait-and-see approach is recommended.

A dystrophic nail is an alteration of the physical appearance and structural properties of the nail from its shape, color, and texture, which can result from multiple etiologies. Calcinosis cutis is a condition that refers to the calcium deposition in the skin and underlying tissue. A 55-year-old man who presented with a split right thumbnail associated with subungual calcinosis cutis affecting the same digit for a duration of nine years is described. He did not recall trauma to the affected area. Microscopic evaluation of the tissue specimen obtained following a biopsy of the affected digit's nail matrix showed foci of calcium in the dermis. The correlation of his clinical presentation and biopsy findings established a diagnosis of subungual calcinosis cutis. Subungual calcification has been observed in several clinical settings. In addition to subungual calcinosis cutis, it has been noted as a normal finding in elderly individuals. In addition, it has been observed in patients with scleroderma or following trauma to the site. Less commonly, subungual calcification can be associated with idiopathic conditions: calcifying aponeurotic fibroma, digital calcinosis circumscripta, subepidermal calcified nodule, and calcified subungual epidermoid inclusion.