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
BACKGROUND: Idiopathic calcinosis cutis (CC) is currently sub-classified as tumoral CC, sub-epidermal calcified nodules, idiopathic CC of the scrotum, and milia-like CC. This sub-classification system is obscure and can be confusing at times. OBJECTIVE: This study was designed to characterize the clinical and histologic features of idiopathic CC, to evaluate the adequacy of the current sub-classification system, and to propose a new sub-classification system. METHODS: Thirty patients with idiopathic CC were sub-classified according to the Lever classification and the Walsh and Fairley classification and were also categorized based on the type of the primary lesion. RESULTS: Idiopathic CC lesions were most common on the buttock (n=7), followed by the scrotum (n=5), arms and legs (n=5), hands and feet (n=4), inguinal area (n=3), ears (n=2), neck (n=2), trunk (n=1), and face (n=1). With the Lever classification, twenty-three of 30 cases were categorized as "unclassified". With the Walsh and Fairley classification, twenty-three cases were categorized as "unclassified", whereas with our new classification system, we managed to classify all cases as either nodular or papular calcification according to the type of the primary lesion. CONCLUSION: In this study, we identified a common location and the primary morphological features of idiopathic CC. Our findings suggest that our new sub-classification system based on the type of the primary lesion would be useful.
Subject(s)
Humans , Arm , Buttocks , Calcinosis , Ear , Foot , Hand , Leg , Neck , ScrotumABSTRACT
Milia-like idiopathic calcinosis cutis is a rare entity. It is characterized by micronodular, whitish papules resembling milia. Histopathological examination shows small nodular foci of calcium within the superficial dermis, which are surrounded by thick collagen and inflammatory cells. Although the pathogenesis and treatment remain unclear, spontaneous resolution of the lesions may occur. We report a case of milia-like idiopathic calcinosis cutis in a 7-year-old girl who improved after treatment with tretinoin cream.
Subject(s)
Child , Humans , Calcinosis , Calcium , Collagen , Dermis , TretinoinABSTRACT
Milia-like idiopathic calcinosis cutis (MICC) is a peculiar subtype of idiopathic calcinosis cutis, characterized by clinically resembling milia, and the majority of the cases are associated with Down syndrome. We report a rare case of MICC which was not associated with Down syndrome. A healthy 10-year-old boy presented with whitish papules surrounded by erythema on the back. Some of the papules were umbilicated or crusted, therefore, they gave a clinical impression as molluscum contagiosum. Histopathologic findings of the papule showed calcium deposits as amorphous, basophilic mass on the upper dermis and focal transepidermal elimination of calcium, which were compatible with MICC.
Subject(s)
Child , Humans , Male , Basophils , Calcinosis , Calcium , Dermis , Down Syndrome , Erythema , Molluscum ContagiosumABSTRACT
Down syndrome is associated with a large number of abnormal skin features including syringoma. Milia-like idiopathic calcinosis cutis is one of the rare dermatoses associated with Down syndrome. It has been reported in children with Down syndrome and clinically suggested as milia. The histological features show small nodular foci of calcium within the papillary dermis and transepidermal elimination of calcium deposits occasionally. We describe a 14-year-old female affected by Down syndrome, who had whitish papules on both the dorsal hands and feet and periorbital syringomas. Histopathological findings of the papule on the dorsal hand showed calcium deposits as amorphous, basophilic masses in the upper dermis and focal discharge of calcium by means of transepidermal elimination.