Unusual Idiopathic Calcinosis Cutis Universalis in a Child

Abstract

Calcinosis cutis is an uncommon disorder characterized by the progressive deposition of crystals of calcium phosphate (hydroxyapatite) in the skin in various areas of the body. It is classified into four types according to etiology, namely as dystrophic if calcium and phosphorus levels are normal and tissue damage is present, as idiopathic if calcium and phosphorus levels are normal and no tissue damage is present, or as metastatic if there is hypercalcemia or hyperphosphatemia. Medical and surgical treatments are options to cure calcinosis cutis. Medical therapy is not very effective. Surgical excision has shown to be beneficial, as it can provide a symptomatic relief. However, since calcinosis cutis limits are not always well defined, a recurrence of the lesions may occur. We dealt with a very rare form of calcinosis cutis in a healthy 6-year-old girl. There was no evidence of connective tissue disorder or abnormal mineral metabolism. Hence, she was diagnosed as idiopathic calcinosis cutis and, although calcifications in idiopathic cutis are most commonly localized to one area, our patient unusually exhibited widespread calcific deposits. Although the existing lesions showed slow improvement, systemic pamidronate therapy was effective in preventing the occurrence of new lesions. Surgical excision proved to be an effective and successful treatment. This report aims to raise doctors’ awareness on the presentation, etiopathogenesis, and course of the relatively rare idiopathic calcinosis cutis.

El Parmaklarına Lokalize İdyopatik Kalsinozis Kutis

“…Sklerodermaya yönelik bakılan antikorlar negatifti. Metastatik tip kalsifikasyon, normal dokuda meydana gelir ve anormal serum kalsiyum ve/veya fosfor seviyeleri gözlenir 2,8 . En sık nedeni böbrek yetmezliğidir 1,8 .…”

“…İdyopatik terimi doku kalsifikasyonuna sebep olacak altta yatan bir neden bulunmadığında kullanılır 9 . En nadir görülen kalsinozis kutis çeşididir [2][3][4][5] . Hastamızda travma ve parenteral tedavi öyküsünün olmaması, normal serum kalsiyum ve fosfor seviyelerine sahip olması, inflamatuvar ve metabolik hastalık olmaması nedeni ile idyopatik tip kalsinozis kutis tanısı konuldu.…”

Idiopathic calcinosis cutis of nasal dorsum

“…Primary hyperparathyroidism, hypervitaminosis D, renal failure, milk-alkali syndrome and destructive bone disease can cause metastatic calcinosis. [ 5 ]…”

Yetişkin bir hastada idiyopatik vulvar kalsinozis, olgu sunumu

“…Sistemik, metobolik hastalıkların yokluğun-da, doku hasarı olmadan diğer KK formların dışlanması ile tanı alır. Genellikle çocukluk ve ergenlik döneminde gözlenir [1,2]. Vulvar lezyonlar genellikle nodüler ya da plak şeklin-dedir.…”

“…Metastatik form genellikle kalsiyum ve fosfat metabolizması anormalliklerine bağlı, hiperkalsemi, hiperpotasemi ile ilişkili olarak ortaya çıkar. Bu durum sıklıkla böbrek yetmezliği, D vitamini aşırı alınması, süt alkali sendromu, destrüktif kemik hastalıkları kutonöz, subkutanöz ve derin dokularda spontan kalsifikasyon sonucudur [1]. Genellikle kemik dejenerasyonu ile beraberdir [2].…”