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Resumen El calcio participa en muchos procesos fisiológicos. La calcinosis cutis es una enfermedad de depósito de calcio en la dermis. La calcicosis escrotal idiopática (CEI) es una forma común de calcinosis cutis idiopática. Se presenta el caso de un paciente masculino de 28 años de edad, con clínica de masas múltiples en escroto, de color blanco, tamaños variados, no dolorosas. Los niveles de calcio y fósforo se mostraron dentro de los rangos normales, característico de calcinosis de tipo idiopática, mientras que la vitamina D y la PTH se mostraron fuera de los límites normales. Se realiza una cirugía menor de las lesiones nodulares circunscritas, de diversos tamaños y de bordes regulares. El examen histológico post-biopsia confirmó el diagnóstico de calcinosis cutis. Se prescribió una dieta baja en calcio como método preventivo a largo plazo. A pesar de lo infrecuente de esta condición debe tenerse en cuenta al realizar un diagnóstico diferencial. Su etiología es aún desconocida y puede presentarse de manera asintomática. Lo importante es brindar al paciente una mejor calidad de vida, evitar incomodidades y baja autoestima debido al aspecto estético.
Abstract Calcium participates in many physiological processes. Calcinosis cutis is a disease caused by calcium deposition in the dermis. Idiopathic scrotal calcinosis (ICS) is a common form of idiopathic calcinosis cutis. A 28-year-old male patient was evaluated, with symptoms of multiple white masses in the scrotum, of various sizes, not painful. Calcium and phosphorus levels were within normal ranges, characteristic of idiopathic calcinosis, while vitamin D and PTH were outside the normal limits. Minor surgery was performed on circumscribed nodular lesions of various sizes with regular borders. Post-biopsy histological examination confirmed the diagnosis of calcinosis cutis. A low-calcium diet was prescribed as a long-term preventive method. Despite the infrequency of this condition, it should be taken into account when making a differential diagnosis. Its etiology is still unknown and it can present asymptomatically. The important thing is to provide the patient with a better quality of life, avoid discomfort and low self-esteem due to the aesthetic aspect.
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Background: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. Aim: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. Method: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. Results: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron’s rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. Limitations: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. Conclusion: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur
ABSTRACT
Summary@#Calcinosis cutis is characterized by the accumulation of insoluble calcium salts in the cutaneous and subcutaneous tissue. This condition is classified into four subtypes of calcification: dystrophic, metastatic, idiopathic and iatrogenic. Here, we describe a case of calcinosis cutis in a patient with systemic lupus erythematosus (SLE) who was recently diagnosed with ovarian cancer. Following total hysterectomy with bilateral salphingo-oophorectomy, she developed a painful erythematous plaque over the anterior aspect of the left forearm which turned into single thin yellowish plaque over the following days. Multiple attempts of intravenous cannulation for intravenous fluids occurred at the site. Skin biopsy revealed calcification in the dermis and subcutaneous fat associated with altered dermal collagen. Von Kossa stain demonstrated the presence of calcium deposition. She responded to topical calcineurin inhibitor which resulted in a complete resolution of the skin lesion.
Subject(s)
Calcinosis Cutis , Lupus Erythematosus, Systemic , Ovarian NeoplasmsABSTRACT
Resumen Introducción: La calcinosis cutis es el depósito de sales insolubles de calcio en la piel y se clasifica, de acuerdo con su patogénesis, en distrófica, metastásica, idiopática, iatrogénica y calcifilaxis. La calcinosis idiopática se presenta en pacientes sanos y es asintomática; incluye la calcinosis escrotal, la calcinosis nodular de Winer o nódulos calcificados subepidérmicos y la calcinosis tumoral familiar. Esta última es una condición rara que se caracteriza por el depósito de calcio periarticular en pacientes normocalcémicos sin conexión al hueso. Caso clínico: Paciente de sexo masculino de 5 meses de edad, quien al séptimo día de vida fue hospitalizado por ictericia multifactorial, sepsis neonatal tardía y apnea con crisis epilépticas. La evolución fue tórpida, con ingresos hospitalarios por crisis epilépticas de difícil manejo, respuesta parcial a la difenilhidantoína y descontrol electrolítico. Mediante la secuenciación del exoma dirigido se detectó una variante patogénica de sentido equivocado en FGF12 que confirmó el diagnóstico de encefalopatía epiléptica temprana número 47. Además, el paciente presentó dermatosis congénita diseminada a las extremidades inferiores con afección en muslos, asintomática, bilateral y simétrica, constituida por hipopigmentación y fóveas duras a la palpación profunda. La biopsia mostró calcificación distrófica. Conclusiones: Se presenta el caso de un lactante con calcinosis cutis congénita profunda asociada con una variante patogénica en el gen FGF12 y con encefalopatía epiléptica, situación clínica que, a la fecha, no había sido reportada en la literatura.
Abstract Background: Calcinosis cutis is the deposit of insoluble calcium salts in the skin. It is classified according to its pathogenesis in dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. Idiopathic calcinosis is asymptomatic, occurs in healthy patients, and includes scrotal calcinosis, Winer's nodular calcinosis or subepidermal calcified nodules, and familial tumor calcinosis. The latter is a rare condition characterized by periarticular calcium deposition in normocalcemic patients with no bone connection. Case report: The case of a 5-month-old male patient, who on the seventh day of life was hospitalized for multifactorial jaundice, late neonatal sepsis, and apnea with epileptic seizures is described. His evolution was torpid, with hospital admissions due to epileptic seizures that were difficult to manage with partial response to the use of diphenylhydantoin and electrolyte alterations. By means of exome sequencing directed, a pathogenic variant of wrong direction in FGF12 was detected and the diagnosis of early epileptic encephalopathy number 47 was confirmed. Also, the patient showed disseminated congenital dermatosis to lower extremities affecting thighs, asymptomatic, bilateral and symmetrical, constituted by hypopigmentation and fovea hard to deep palpation. The biopsy showed dystrophic calcification Conclusions: The case of an infant with deep congenital cutis calcinosis associated with a pathogenic variant in the FGF12 gene with epileptic encephalopathy is described. To date, this clinical situation has not been previously reported in the literature.
Subject(s)
Humans , Infant , Male , Skin Diseases , Brain Diseases , Calcinosis , Epilepsy , Skin Diseases/complications , Skin Diseases/diagnosis , Skin Diseases/genetics , Brain Diseases/diagnosis , Brain Diseases/genetics , Calcinosis/complications , Calcinosis/congenital , Calcinosis/genetics , Epilepsy/diagnosis , Epilepsy/genetics , Fibroblast Growth Factors/geneticsABSTRACT
Calcinosis cutis is a condition, where there is deposition of calcium salts in skin and subcutaneous tissue, seen often in the middle to elderly aged population and is rare in neonates and infants. There are many aetiological factors, but in neonates and infants it is mostly seen as complication of extravasation of intravenous calcium infusion or trauma. For uncomplicated cases supportive treatment has been advocated. Authors describe a case presented with calcinosis cutis, who was treated for transient hypoparathyroidism in neonatal period.
ABSTRACT
Metastatic calcinosis cutis refers to the deposition of calcium salts in normal tissue secondary to an underlying defect in calcium and/or phosphate metabolism. It commonly affects periarticular areas in patients with chronic renal failure. We report a case of a 51-year-old man with a past medical history of peritoneal dialysis for chronic renal failure, who presented with multiple yellowish nodules on his right thumb. In view of the asymptomatic non-inflamed fluctuating nodules, the differential diagnoses included bacterial, tuberculous, atypical mycobacterial, or fungal infections. Histopathological and radiological examinations revealed calcifications in the right thumb and shoulder with elevated serum phosphorus and parathyroid hormone levels. The lesions improved after the patient was switched from peritoneal dialysis to hemodialysis. We report a case of metastatic calcinosis cutis in a patient with chronic renal failure. We emphasize the importance of imaging for accurate diagnosis and follow-up of calcinosis cutis and that hemodialysis scores over peritoneal dialysis in the treatment of this condition.
Subject(s)
Humans , Middle Aged , Calcinosis , Calcium , Diagnosis , Diagnosis, Differential , Follow-Up Studies , Kidney Failure, Chronic , Metabolism , Parathyroid Hormone , Peritoneal Dialysis , Phosphorus , Renal Dialysis , Salts , Shoulder , ThumbABSTRACT
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.
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@#Calcinosis cutis is a rare presentation and not many caseshave been reported especially of idiopathic type. We arereporting a case of idiopathic calcinosis cutis of lower limbsin a 33-year old female who presented to our clinic formultiple painless swellings over her lower limbs for the pastsix months, without any history of trauma or infection. Wehave decided to observe her condition on regular follow-upand conservative management.
ABSTRACT
Calcinosis cutis is an uncommon disorder and defined as the deposit of insoluble calcium salts in the skin. It is classified as dystrophic, metastatic, idiopathic, and iatrogenic according to etiology. The dystrophic type is the most common and occurs in the previously damaged tissue, including connective tissue diseases, panniculitis, inherited disorders, benign and malignant tumors, a variety of scarring caused by burn, radiation, trauma, surgery, and keloid. Treatment options are limited. Whereas medical therapy is usually not very effective, surgical intervention has shown to be beneficial and is indicated when painful masses, recurrent infection, ulcerations, functional impairment, and cosmetic concerns exist. Herein, we report two cases of dystrophic calcinosis cutis associated with longstanding, painful, ulcerated cutaneous lesions in the extremities successfully treated with surgical excision.
Subject(s)
Burns , Calcinosis , Calcium , Cicatrix , Connective Tissue Diseases , Extremities , Keloid , Panniculitis , Salts , Skin , UlcerABSTRACT
Idiopathic calcinosis cutis is a rare disorder and occurs in the absence of known tissue injury or systemic metabolic defect. It is a skin calcification process with abnormal deposits of calcium phosphate in the skin in various parts of the body. It is a rare and benign syndrome which does not cause any late complication and whose prognosis is therefore favourable. Hereby, I am reporting a case of 40 years old male who presented with multiple asymptomatic calcified nodules in the scrotum with normal lab findings. Excision of the nodule on histopathological examination revealed foci of calcification separated by fibrous septa in the dermis.
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@#Metastatic calcinosis cutis of the penis and scrotum seen in a patient with End Stage Renal Disease (ESRD) on hemodialysis is a case not usually encounter in urology clinics. Review of the available literature mostly showed compromised erectile function of patients with metastatic calcinosis cutis of the penis. Presented is a case of a patient with ESRD on hemodialysis for five years who developed metastatic calcinosis of the penis and scrotum causing dysmorphic changes, however maintaining full erectile function. Preserving the erectile function after successful excision of the penile and scrotal calcification is imperative to maintain good quality of life.
Subject(s)
Erectile Dysfunction , Hyperparathyroidism, SecondaryABSTRACT
No abstract available.
ABSTRACT
La calcinosis cutis es un trastorno raro causado por el depósito anormal de sales de calcio en la piel y tejido subcutáneo. Clínicamente se caracteriza por pápulas, placas o nódulos calcificados. Según su etiopatogenia se distinguen cinco tipos clínicos: distrófica, metastásica, iatrogénica, idiopática y calcifilaxis.Comunicamos el caso clínico de un paciente en edad pediátrica con una calcinosis cutis idiopática universal, sin afectación extra-cutánea. Esta publicación reviste importancia debido a lo infrecuente de esta enfermedad y a que su diagnóstico nos permite detectar padecimientos asociados, que son determinantes para el pronóstico y tratamiento.
Cutaneous calcinosis is a rare disorder caused by the deposit of abnormal calcium salts in the skin and subcutaneous tissue. Itpresents with calcified papules, plaques or nodules. Five clinical types are distinguished: dystrophic, metastatic, iatrogenic, idiopathic and calcifilaxis. This paper reports a pediatric patient with aidiopathic calcinosis cutisuniversalis, without extracutaneous manifestations. This publication is important because of the rarity of this disease. The diagnosis allows us to investigate the possible associated diseases that determine patient prognosis and treatment.
Subject(s)
Humans , Male , Child , Calcinosis , Durapatite , Finger Joint , Knee JointABSTRACT
La calcinosis cutis idiopática (CCI) es una afección dermatológica poco frecuente en pediatría, caracterizada por la presencia de nódulos cutáneos calcificados, sin evidencia de lesiones tisulares previas o defectos metabólicos subyacentes. Afecta por igual a ambos sexos y su tratamiento depende del grado de compromiso estético o funcional. Presentamos 8 niños con lesiones de CCI. Hubo un leve predominio de afectación del sexo femenino, y la edad promedio de presentación fue de 5 años. Las lesiones se ubicaron preferentemente en la cara, en coincidencia con lo relatado en la literatura. Si bien es una patología benigna, existen muchos diagnósticos diferenciales, por lo que creemos que publicar nuestra experiencia podría ayudar a considerar su diagnóstico en la práctica diaria y así evitar la realización de estudios complejos innecesarios.
Idiopathic calcinosis cutis (ICC) is a rare pediatric condition characterized by the presence of skin calcified nodules without evidence of previous tissue injury or underlying metabolic defects. It affects both sexes equally and treatment depends on the aesthetic or functional compromise. We herein present 8 children with lesions of ICC. A slight female predominance was observed and the median age of presentation was 5 years. Lesions were located mainly on the face, in accordance with the reported data in the literature. Although it is a benign disease, many differential diagnoses must be considered, therefore we believe that publishing our experience may help to consider its diagnosis in daily practice and avoid performing unnecessary complex studies.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Calcification, Physiologic , Calcinosis , FaceABSTRACT
Neonatal hypocalcemia is not uncommon, especially in premature neonates. It is usually treated by intravenous calcium gluconate administration. However, complications caused by the extravasation of intravenous calcium gluconate include calcinosis cutis, calcium deposition in the dermis, which is irritating to soft tissues and can produce tissue necrosis. We report a case of iatrogenic calcinosis cutis in a 27-day-old preterm baby following the extravasation of calcium gluconate. In this case, calcinosis cutis occurred along the venous pathway in a rare vascular calcification pattern but spontaneously resolved 2 months later. Although iatrogenic calcinosis cutis is generally benign, early recognition of the extravasation of calcium gluconate is important to avoid severe complications. This report aimed to provide a warning about the risks associated with intravenous calcium gluconate and information about the course of a rare presentation of the common disease calcinosis cutis with vascular calcification.
Subject(s)
Humans , Infant, Newborn , Calcinosis , Calcium , Calcium Gluconate , Dermis , Hypocalcemia , Necrosis , Vascular CalcificationSubject(s)
Humans , Female , Child, Preschool , Skin Diseases/pathology , Calcinosis/pathology , Skin Diseases/therapy , Calcinosis/therapy , Diet TherapyABSTRACT
Dystrophic calcinosis cutis associated with spontaneous hyperadrenocorticism was diagnosed in a 8-year-old female Chihuahua dog with erythematous, erosive, numerous papules, plaques, and crusts on the bilateral trunk, and inguinal region. Serum biochemical abnormalities included increases in alkaline phosphatase (ALP), alanine aminotransferase (ALT), gamma-glutamyl transpeptidase (GGT), and cholesterol. Radiographs showed mild hepatomegaly and subcutaneous lobulated calcific deposits. Histopathologic examination demonstrated diffuse deposition of basophilic calcified material in the dermis. Von Kossa's stain confirmed calcium deposition. Therapy with diltiazem was useful in resolving calcinosis.