Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case

Abstract

Aplasia cutis congenita (ACC) is a congenital disorder characterized by the absence of epidermis, dermis, and sometimes subcutaneous tissue and bone. There are nine types of ACC based on the number and location of the lesions as well as the presence or absence of associated deformities, with type I ACC being the most common. Type V affects the trunk with a characteristic pattern resembling the "H" letter of the alphabet, generally accompanied by fetus papyraceus (death of one of the twin fetuses) or placental infarction. Type V ACC without fetus papyraceus and placental infarction is a rare case. We reported a case of type V ACC in a 3-day-old baby girl, with clinical manifestation of extensive ulcers on the scalp, back, buttocks, and both lower extremities, in an "H"-shaped pattern, with history of placenta accreta, but no fetus papyraceus or placental infarction was found. The patient received dialkyl carbamoyl chloride hydrophobic swab with hydrogel (Sorbact ® gel dressing-BSN Medical) and hydrocolloid wound dressing with good response, as indicated by the wounds becoming dried up, leaving an atrophic scar. Complete epithelialization was achieved in 72 days. The cause of type V ACC remains not completely understood. The diagnosis of ACC can be established based on clinical manifestations. Further examination is required if extracutaneous involvement is suspected. Early identification of the etiology of the lesions and appropriate investigation and treatment, including conservative wound management, are needed so that aplastic lesions can heal successfully, although cosmetic repair may be required at a later stage.

Challenges in the management of extensive aplasia cutis congenita

“…Although there are numerous case reports of ACC, only a handful describe ‘extensive ACC’, including seven that involve the scalp,21 26–31 four that affect the trunk,32–35 and three that affect both the head and trunk . Among these, only the latter three36–38 are nearly as extensive as our patient, with only one of them having survived 38. This particular patient was similar to our patient, and had both scalp and trunk involvement, although the exact extent in terms of BSA was not reported.…”

“…The above-mentioned cases of extensive ACC with truncal involvement were classified as Group V ACC,32–35 38 even though two of them, namely Boente et al 32 and Effendi et al ,38 did not have a history of fetus papyraceous or vanishing twin, as was the case in our patient. In these cases, the lesion had a highly characteristic, symmetric distribution pattern that encircled the umbilicus and formed an ‘H’ pattern on the anterior or posterior trunk, which was very similar to the presentation in our patient.…”