|Year : 2019 | Volume
| Issue : 2 | Page : 120-121
Congenital idiopathic calcinosis cutis with aplasia cutis congenita
Jerin C Sekhar1, Ashish Jain2, Sataroopa Mishra1, Abhishek Chopra2
1 Department of Pediatrics, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India
2 Department of Neonatology, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India
|Date of Web Publication||25-Apr-2019|
Dr. Ashish Jain
601 A, Sukhsagar Apts, Plot No. 12, Sector 9, Dwarka, New Delhi - 110 077
Source of Support: None, Conflict of Interest: None
Calcinosis cutis is a disorder characterized by the deposition of insoluble calcium salts in the skin and subcutaneous tissue. It is separated into five subtypes, namely dystrophic calcification, metastatic calcification, idiopathic calcification, iatrogenic calcification, and calciphylaxis. Idiopathic calcification occurs without any underlying tissue damage or metabolic disorder. We here report a rare case of congenital idiopathic calcinosis cutis associated with aplasia cutis congenita.
Keywords: Aplasia cutis, calcinosis cutis, idiopathic, neonate
|How to cite this article:|
Sekhar JC, Jain A, Mishra S, Chopra A. Congenital idiopathic calcinosis cutis with aplasia cutis congenita. J Clin Neonatol 2019;8:120-1
|How to cite this URL:|
Sekhar JC, Jain A, Mishra S, Chopra A. Congenital idiopathic calcinosis cutis with aplasia cutis congenita. J Clin Neonatol [serial online] 2019 [cited 2019 Jul 22];8:120-1. Available from: http://www.jcnonweb.com/text.asp?2019/8/2/120/257140
| Introduction|| |
Calcinosis cutis is characterized by abnormal deposits of calcium salts in the dermis and/or hypodermis. It is classified into five subtypes, namely dystrophic calcification, metastatic calcification, idiopathic calcification, iatrogenic calcification, and calciphylaxis. Dystrophic calcification appears as a result of local tissue damage with normal calcium and phosphate levels in serum. Metastatic calcification is characterized by an abnormal calcium and/or phosphate metabolism, leading to the precipitation of calcium in cutaneous and subcutaneous tissues. Skin calcification in iatrogenic calcinosis cutis is a side effect of therapy. Calciphylaxis presents with small-vessel calcification mainly affecting blood vessels of the dermis or subcutaneous fat. Idiopathic calcification occurs without any underlying tissue damage or metabolic disorder. We here report a case of congenital idiopathic calcinosis cutis associated with aplasia cutis congenita.
| Case Report|| |
A 34-week-old preterm male, weighing 1800 g, an outcome of a nonconsanguineous marriage, was born by cesarean delivery done for antepartum hemorrhage to a 32-year-old third gravida mother with an uneventful supervised antenatal period. He was born through clear liquor with Apgar scores of 9 at 1 and 5 min, needing only initial steps of resuscitation at birth. On examination at birth, he was alert, warm, and pink with no dysmorphism, and his systemic examination was essentially normal. A head-to-toe examination revealed an area of alopecia over the parieto-occipital region (1 cm × 1 cm) that was circumscribed and associated with cutis aplasia. In addition, on examination of the limbs, there was a firm irregular swelling over the dorsum of the right wrist measuring 3 cm × 2 cm, with no signs of inflammation or discharge over the swelling [Figure 1]. The swelling was adherent to the skin, but moved freely over the bone and resulted in partial limitation of flexion of fingers of the right hand. A plain radiograph of the affected wrist revealed areas of calcification in the subcutaneous tissue and muscle of the limb [Figure 2]. Laboratory workup revealed normal blood counts, serum calcium, phosphorous, and alkaline phosphate levels. Lack of dysmorphism and malformations of the great toe made genetic causes such as fibrodysplasia ossificans progressiva unlikely and hence the child did not warrant a karyotype as advised by the geneticist. The parents could not afford a magnetic resonance imaging of the wrist. The course of stay in the hospital was uneventful, and there was no increase in the size of the swelling. He was given care for low birth weight and discharged on day 12 of life. There was no significant change in the size of the swelling at discharge. The child was followed up in high-risk clinic at 9 months of age. He had no residual wrist swelling, and a radiograph done was normal.
|Figure 2: Plain radiograph of the wrist showing calcification in the soft tissue|
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| Discussion|| |
Calcinosis cutis is characterized by deposition of hydroxyapatite crystals of calcium phosphate in the skin. In our case, idiopathic calcification was diagnosed as no underlying metabolic defect was detected (metastatic calcification) and no history of tissue injury (dystrophic calcification) was evident. To the best of our knowledge, there is no reported association of calcinosis cutis with alopecia. Intrauterine trauma, infections, or ischemia can cause both calcinosis cutis and aplasia cutis.
These lesions may be confused with pyogenic infections and may be subjected to unnecessary surgical intervention.,,, Blood investigations are done to detect any underlying metabolic disorder. Plain radiograph clinches the diagnosis. In most neonates, both scalp and calcified lesions are self-resolving by 6 months of onset; hence, wait-and-watch approach is most appropriate in these cases.,
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]