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Volume 16/Issue 5, September 2020

A rare and unusual presentation of aplasia cutis congenita

Marika Lasokova, Ourania Kaltsogianni, Jogesh Kapadia


Aplasia cutis congenita (ACC) is a heterogenous group of disorders characterised by the absence of a portion of skin in a localised or widespread area at birth. ACC manifests as a solitary defect on the scalp in the majority of cases, however, extensive lesions on the trunk and limbs are reported in the literature as rare presentations. Most cases are sporadic but ACC has also been associated with twin pregnancies, in which one twin died during early pregnancy. In this case study, we report a rare case of extensive cicatricial ACC on the trunk and lower limbs of a male infant.

Extensive ACC lesions on the trunk and limbs are rare. ACC can be associated with placental infarcts or the in utero death of a twin fetus, as in the case presented here. Obstetric history should review maternal medications/infections during pregnancy, determine an initial multiple pregnancy with death of a co-twin and investigate any placental anomalies. Despite their large size, truncal and limb ACC lesions usually resolve within the first few months of life.
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