Extensive Erosions and Ulcerations in a Newborn

A two-day-old male presented with symmetric, stellate erosions and ulcerations located on the lower extremities and axillae (Figures 1-3). The lesions were present from birth. He had no mucosal involvement or nail changes. He was born at 39 weeks gestation via vaginal delivery. The pregnancy was initially of monochorionic diamniotic twins; however, the family elected to undergo selective fetal reduction at 17 weeks gestation to minimize complications. Ultrasound monitoring of the surviving fetus was reportedly normal thereafter. There was no family history of blistering disorders or herpes simplex virus (HSV). Infectious workup was negative, including HSV polymerase chain reaction (PCR), varicella zoster virus PCR, enterovirus PCR, and an aerobic bacterial culture of the ulcerations. Genetic testing for epidermolysis bullosa was negative. Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by areas of absent epidermis, dermis, and sometimes also involving the subcutaneous tissue. ACC is classified into nine subtypes based upon presentation, associations, and etiology. Type V ACC is a rare subtype associated with fetus papyraceus or placental infarction [1]. Fetus papyraceous describes a phenomenon of multiple gestation pregnancy with intrauterine demise of a fetus. The nonviable fetus undergoes mummification and compression by the viable fetus, resulting in a parchment paper-like appearance [2]. The viable fetus then classically presents at birth with multiple symmetric areas of absent skin, with lesions often stellate or linear in shape. Locations commonly affected include the scalp, chest, flanks, axillae, and extremities. This symmetric and extensive distribution is in contrast to type I ACC, which is the most common type of ACC that usually presents as a solitary scalp lesion [1]. Type V ACC is thought to occur sporadically in multiple gestation pregnancies [3]. Most prior reports of type V ACC have been in twin gestations; however, there are several cases also reporting this condition in triplet and sextuplet gestations [4]. In each of these cases, only one infant was affected with ACC [1, 5]. As for chorionicity, there is a potential association between type V ACC and monochorionic pregnancies. Among type V ACC cases that specified details of chorionicity, 95% were from monochorionic twin gestations [5]. No significant maternal comorbidities have been identified as risk factors [4]. Epidemiologic data remain limited; however, a study conducted in Spain found that the incidence of ACC was 2.8 per 10,000 live births, and a study in Korea found that 3.39% of ACC patients had the type V variant [6, 7]. No sex predisposition has been found [4, 6]. Several mechanisms for the pathogenesis of type V ACC have been proposed; one theory is that fetal demise causes pro-thrombotic material to enter the viable fetus, leading to infarcts in the skin. This mechanism was suggested based upon the observation of disseminated intravascular coagulation in cases of late-gestation fetal demise [3]. Recent evidence, including ultrasound studies, instead suggests that fetal demise causes hypovolemia in the viable fetus. Areas of the skin with watershed blood supply may be particularly vulnerable to this period of ischemia, thus potentially explaining why type V ACC tends to be distributed across the flanks and lateral chest [5, 8]. Of note, with the increase of multifetal births, commonly attributed to increasing use of fertility treatments, there has been a concurrent increase in fetal reduction. Kim et al. proposed considering type V ACC as an adverse event resulting from selective reduction, and encouraged greater caution when counseling parents [9]. The differential diagnosis of congenital erosions and ulcerations includes infectious entities, epidermolysis bullosa, focal dermal hypoplasia, and incontinentia pigmenti among others. In cases of severe epidermolysis bullosa (EB), patients may similarly present with erosions and ulcerations of the trunk and extremities. However, EB patients typically also present with blistering and may also have additional findings including dystrophic nails and mucosal involvement. In contrast to type V ACC, focal dermal hypoplasia commonly presents as patches of hypopigmentation or atrophy with associated craniofacial or limb abnormalities. Lastly, most infants with incontinentia pigmenti initially present with blistering, followed by verrucous lesions and finally with pigmentary changes. This contrasts with the absent skin characterizing type V ACC. With neonatal cutaneous erosions and ulcers, it is important to include type V ACC in the differential diagnosis, especially with a maternal history of multi-gestational pregnancy. While the diagnosis of type V ACC is clinical, workup to rule out infectious entities, as well as genetic testing to evaluate for congenital blistering diseases, may be required. Management of type V ACC relies on proper wound care and prevention of potential complications, which can include secondary infection, fluid loss, and electrolyte shifts [10]. Prognosis is generally favorable with gradual wound healing and scar formation. Our patient was treated with topical mupirocin 2% ointment, petroleum jelly, silicone transfer dressing, and rolled gauze with good response. After a few months, his skin had fully re-epithelialized with some areas of contracted scarring and without development of new lesions. The authors declare no conflicts of interest.