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CASE REPORT
Year : 2015  |  Volume : 16  |  Issue : 1  |  Page : 48-49

Aplasia cutis congenita with fetus papyraceus


Department of Dermatology, PGIMER, Dr. Ram Manohar Lohia Hospital, New Delhi, India

Date of Web Publication16-Jan-2015

Correspondence Address:
Neha Meena
Department of Dermatology, PGIMER, Dr. Ram Manohar Lohia Hospital, Baba Kharak Singh Marg, New Delhi - 110 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.149432

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  Abstract 

We present a case of 8 h old newborn girl with symmetrical, stellate type of truncal aplasia cutis congenita. She was the survivor twin as the other fetus died at gestational age of 13 weeks and 5 days.

Keywords: Aplasia cutis congenita, fetus papyraceus, stellate, symmetrical, twin pregnancy


How to cite this article:
Meena N, Saxena AK, Sinha S, Dixit N. Aplasia cutis congenita with fetus papyraceus. Indian J Paediatr Dermatol 2015;16:48-9

How to cite this URL:
Meena N, Saxena AK, Sinha S, Dixit N. Aplasia cutis congenita with fetus papyraceus. Indian J Paediatr Dermatol [serial online] 2015 [cited 2019 Dec 7];16:48-9. Available from: http://www.ijpd.in/text.asp?2015/16/1/48/149432


  Introduction Top


Aplasia cutis congenita (ACC) is a rare disorder characterized by congenital absence of skin on the scalp, extremities, and trunk. [1] We report a case of 8 h old newborn girl with the symmetrical, stellate pattern involvement of the trunk, both arms till elbow joints and thighs till knee joints. She was a survivor of twin pregnancy, the other fetus died at gestational age of 13 weeks and 5 days. She was managed conservatively, and her lesions healed with atrophic linear scars.


  Case report Top


A newborn (8 h old) girl presented in the emergency of our hospital with congenital absence of skin on the trunk, both arms, and thighs. On examination, there was symmetrical absence of skin in a stellate or butterfly pattern on back, radiating laterally on both arms till elbow joints, both thighs till knee joints [Figure 1] and anteriorly to involve trunk sparing the subcostal angle [Figure 2]. Instead of skin, there was a thin, gelatinous, shiny membrane with telangiectasia. Her vitals were stable with normal systemic examinations. She was the product of twin pregnancy with intrauterine demise of co-twin at gestational age of 13 weeks and 5 days as evident by the antenatal ultrasonography records. She was delivered at term by elective caesarean section. There was no history of consanguinity in parents, intrauterine infection or drug intake. She was managed conservatively with topical antibiotics and dressing with nonadherent paraffin gauges. The lesions healed with atrophic linear scarring and depigmented margins.
Figure 1: Symmetrical absence of skin with gelatinous, shiny, telangiectatic membrane present in stellate pattern involving proximal limbs

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Figure 2: Truncal aplasia cutis congenita with sparing of subcostal angle

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  Discussion Top


Aplasia cutis congenita is a rare disorder characterized by congenital absence of skin. Frieden classified ACC in 1986 into 9 groups on the basis of location of the lesions and associated congenital anomalies. [1],[2] The scalp is the most commonly involved area with lesser involvement of trunk and extremities. Frieden classified ACC with fetus papyraceus as type 5. This type presents as truncal ACC with symmetrical absence of skin in stellate or butterfly pattern with or without involvement of proximal limbs. [1] There are 45 case reports of ACC with fetus papyraceus in the English literature. [2] The death of co-twin in late first or early second trimester leads to complete resorption of fetus papyraceus as in the present case while later death results in mummification of the dead fetus. [3]

The exact etiology of ACC is still unclear but intrauterine infection by varicella or herpes virus, drugs such as methimazole, misoprostol, valproate, cocaine, marijuana etc., fetus papyraceus, feto-fetal transfusion, vascular coagulation defects, amniotic membrane adherence, abnormal elastic fiber biomechanical forces and trauma are implicated. [1],[2] In ACC with fetus papyraceus, cutaneous infarction occurs due to the release of thromboplastin into the placenta after the intrauterine death. [1] Hypotension and hypovolemia in the viable twin also results in ischemia of the skin and other organs leading to loss of the skin. [2],[3]

Diagnosis of ACC is clinical with a few reports on histopathology, which reveal absence of epidermis, dermis, adnexa and sometimes subcutaneous tissue depending upon the depth of the defect. [1] Regular ultrasonography during antenatal checkup sometime helps in early diagnosis of ACC. The normal fetal skin generate strong echoes on ultrasound while in ACC such echoes are absent. [4] Early diagnosis help in counseling the parents and give time and information for planning prenatal treatment. [4] Management of ACC is conservative. [1],[2]



 
  References Top

1.
Moss C, Shahidulla H. Naevi and other developmental defects. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8 th ed. United Kingdom (UK): Wiley-Blackwell Publication; 2010. p. 18, 18.98-18.106.  Back to cited text no. 1
    
2.
Tempark T, Shwayder TA. Aplasia cutis congenita with fetus papyraceus: Report and review of the literature. Int J Dermatol 2012;51:1419-26.  Back to cited text no. 2
    
3.
Klein RQ, Robinson DM, Lieber CD, Antaya RJ. Symmetric aplasia cutis congenita associated with fetus papyraceus: Report of two cases. Pediatr Dermatol 2011;28:467-9.  Back to cited text no. 3
    
4.
Liu F, Chen X, Tu R, Liu S. Prenatal diagnosis of aplasia cutis congenita of the trunk. Int J Dermatol 2014;53:1269-71.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2]



 

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