DOI: http://dx.doi.org/10.18203/issn.2455-4529.IntJResDermatol20204574

Aplasia cutis congenita with fetus papyraceus: an uncommon case report

Vivekananda Ittigi, Aparna Anto

Abstract


Aplasia cutis congenita is a condition characterized by congenital absence of all skin layers in a localized or widespread area. Frieden's classification recognized a rare subtype, type V, that is associated with multiple gestations in which there is the in-utero demise of a twin with resultant fetus papyraceus or mummification. A twin pregnancy was complicated by in-utero death of one twin at 5 months. On full term birth of the other twin with fetus papyraceus large defects in the skin of knees were noted bilaterally. Conservative management with topical antibiotics and emollients helped in complete re-epithelialization in few months. As the use of reproductive technologies increase the incidence of multiple gestation and associated conditions are expected to increase. This type is clinically unique in that it is characterized by stellate lesions in a symmetrical distribution over the trunk and extremities, differing from other subtypes, which are typically localized to scalp (70%-85% of cases) or extremities. Management ranges from conservative to surgical grafts.


Keywords


Aplasia cutis, Fetus papyraceus, Congenital absence of skin

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References


Maccario S, Fasolato V, Brunelli A et al. Aplasia cutis congenita: an association with vanishing twin syndrome. Eur J Dermatol. 2009;19:372-4.

Simman R, Priebe CJ JR, Simon M. Reconstruction of aplasia cutis congenita of the trunk in a newborn infant using acellular allogenic dermal graft and cultured epithelial autografts. Ann Plast Surg. 2000;44:451-4.

Taifour Suliman M, Quazi A. Aplasia cutis congenita of the trunk in a Saudi newborn. Br J Plast Surg. 2004;57:582-4.

Bui D, Ikeda C. Reconstruction of aplasia cutis congenita (group V) of the trunk in a newborn. Plast Reconstr Surg. 2003;111:2119-20.

Prager W, Scholz S, Rompel R. Aplasia cutis congenita in two siblings. Eur J Dermatol. 2002;12:228-30.

Verhelle NA, Heymans O, Deleuze JP et al. Abdominal aplasia cutis congenita: case report and review of the literature. J Pediatr Surg. 2004;39:237-9.

Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol. 1986;14:646-60.

Mannino FL, Jones KL, Benirschke K. Congenital skin defects and fetus papyraceus. J Pediatr. 1977;91:559-64.

Schaffer JV, Popiolek DA, Orlow SJ. Symmetric truncal aplasia cutis congenita following multifetal reduction of a sextuplet pregnancy. J Pediatr. 2008;153:860-3.

Classen DA. Aplasia cutis congenita associated with fetus papyraceous. Cutis. 1999;64:104-6.

Joshi RK, Majeed-Saidan MA, Abanmi A et al. Aplasia cutis congenita with fetus papyraceus. J Am Acad Dermatol. 1991;25:1083-5.

Qureshi UA, Ahmed N. Type V aplasia cutis congenita. Ann Saudi Med. 2010;30:171-2.

Lewi L, Van Schoubroeck D, Gratacos E. et al. Monochorionic diamniotic twins: complications and managementoptions.Curr Opin Obstet Gynecol. 2003;15:177-94.

Rhee ST, Colville C, Buchman SR. Complete osseous regeneration of a large skull defect in a patient with cutis aplasia: a conservative approach. Journal of Craniofacial Surgery. 2002;13(4):497-500.

Ploplys EA, Muzaffar AR, Gruss JS, Ellenbogen RG. Early composite cranioplasty in infants with severe aplasia cutis congenita: a report of two cases. Cle Palate-craniofac J. 2005;42(4):442-7.

Basterzi Y, Bagdatoglu C, Sar A, Demirkan F. Aplasia cutis congenita of the scalp and calvarium: conservative wound management with novel wound dressing materials. Journal of craniofacial surgery. 2007;18(2):427-9.

Ross DA, Laurie SW, Coombs CJ et al. Aplasia cutis congenita: failed conservative treatment. Plast Reconstr Surg. 1995;95:124-9.

Lewis N, Kolimarala V, Lander A. Conservative management of exomphalos major with silver dressings: are they safe? J Pediatr Surg. 2010;45:2438-9.

Lee SL, Beyer TD, Kim SS, Waldhausen JH, Healey PJ, Sawin RS, Ledbetter DJ. Initial nonoperative management and delayed closure for treatment of giant omphaloceles. J Pediat Surg. 2006;41(11):1846-9.

Charlesworth P, Ervine E, McCullagh M. Exomphalos major: the Northern Ireland experience. Pediatr Surg Int. 2009;25:77-81.