Bilateral Skin Lesions

 
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Question :
Vivek Sharma, Smriti Nath, Venkat Reddy, Santosh Kumar
Department of Pediatrics, Tata Motors Hospital, Jamshedpur, India.

Address for Correspondence: Dr Vivek Sharma, M-36, 4, Telco colony, Jamshedpur, Jharkhand- 831004, India. Email: dr_viveksharma@yahoo.com
A near term male appropriate for gestational age baby delivered by lower segment caesarean section presented with bilateral skin defects on the trunk. Lesions were gelatinous, symmetric covered only by a thin membrane. Antenatal ultrasonogram in first trimester showed twin pregnancy but in second trimester it suggested only singleton pregnancy. The other fetus had died in utero and was lost as fetus papyraceous at birth. Radiological examination and ultrasonography of the baby showed no abnormalities. Baby was managed conservatively with antiseptic dressing and at 3 months of age showed resolution of the lesions with scar formation.

What is the diagnosis_?
Expert Opinion :
Aplasia cutis congenita {ACC} is a rare condition characterized by congenital absence of skin and sometimes subcutaneous tissue as well. Incidence of ACC is 2.8 per 10,000 newborns. {1} Usually the defect is found in scalp where it is often solitary. {2} Non scalp lesions are rare, may involve the trunk and, or extremities and are usually bilaterally symmetrical. These non scalp lesions are sometimes associated with twin pregnancy in which the other twin dies in utero and is lost as fetus papyraceous {3} as in our case. Possible reason being vascular disruption caused by release of some prothrombotic materials by the dead fetus. {3} Management of ACC is mainly conservative with proper antiseptic dressings. Larger lesions may sometimes require surgical intervention with skin grafts, local skin flaps. {4}

References:
1} Martinez-Regueira S, Vazquez-Lopez ME, Somoza-Rubio C, Morales-Redondo R, Gonzalez-Gay MA. Aplasia cutis congenita in a defined population from northwest Spain. Pediatr Dermatol 2006`23:528–532.
2} Crowe MA. Aplasia Cutis Congenita. Available at website: emedicine.medscape.comperrticle, 1110134-overview. Accessed on 15th February 2015
3} Cambiaghi S, Schiera A, Tasin L, Gelmetti C. Aplasia cutis congenita in surviving co-twins: four unrelated cases. Pediatr Dermatol. 2001`18: 511-515
4} Ahcan U, Janezic T. Management of aplasia cutis congenita in a non-scalp location. Br J Plast Surg. 2002` 55: 530–532

Correct Answers : yes  20%

Last Shown : Jul 2015
 
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