Congenital linear ulcerative and scarring lesions associated with fetus papyraceus. Unilateral aplasia cutis congenita type V?

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Milano A.
Dermatologia Pediatrica, Bari, Italy

Keywords: aplasia cutis congenita, fetus papyraceus.

Abbreviations: ACC = aplasia cutis congenita.

Case report. A 20-day-old infant was examined for scarring lesions on the trunk. The mother reported that he was born of a biamniotic monochorionic twin pregnancy and that a prenatal ultrasound had highlighted the death of the twin at 13 weeks. The medical history excluded infections, endocrine disorders, use of medications or drugs, spontaneous rupture of the amniotic sac, and trauma during pregnancy. She also reported that at birth there were irregular scars on the trunk and also an exudating ulcerative lesion. Physical examination (Fig. 1) showed erythematous-infiltrated, linear, K-shaped lesions without appendages on the left side, and inside one of them a circular lesion, more intensely red with a depressed bottoms of about 1 mm; on palpation the lesions appeared hard-scarred and were not painful. The rest of the skin, especially of the right flank, and physical examination of other organs appeared normal. Laboratory tests were within normal limits. Abdominal and encephalic ultrasound showed no alterations. These data led to the diagnosis of unilateral aplasia cutis congenita associated with fetus papyraceus.

Fig. 1: Unilateral aplasia cutis congenita associated with fetus papyraceus.

Discussion. The current patient has linear scarring lesions since birth. We excluded the diagnosis of amniotic bands due to the absence of anamnestic data such as spontaneous rupture of the amniotic sac, of grooves, constriction, lymphedema, and to the presence of a concomitant ulcerative lesion and above all due to the history of fetus papyraceus in the twin. There is only one report in the literature (1) that associates amniotic bands and fetus papyraceus in a bichorionic diamniotic pregnancy; however, the fetus papyraceus was secondary to selective termination, due to maternal will, of one of the twins who presented edema of the lower body because of an amniotic band that constricted the navel; the other twin did not present any skin lesions at birth.
In our patient, the history of fetus papyraceus in the presence of congenital linear scarring and ulcerative lesions led us to the diagnosis of ACC, type V of the Frieden classification (2). This rare condition rarely occurs without any triggering factor (3, 4), in case of placental infarcts, placenta accreta (5) but above all in twin pregnancies (6, 7), which nowadays have become more frequent with assisted fertilization practices, and particularly in the presence of monochorionic diamniotic pregnancies: if one of the twins dies in the uterus around 13-15 weeks, the surviving twin may have aplasia cutis congenita. From a pathogenetic point of view various hypotheses have been put forward, but the one that today seems more likely is that the post-mortem blood pressure drop causes a blood transfer from the surviving twin to the dead one, with secondary hypovolemia, ischemia and consequent aplasia cutis. From a clinical point of view, ACC type V is characterized at birth by ulcerative or scarring lesions, more or less extensive and more or less deep, linear, characteristically bilateral and symmetrical, with a starry appearance or sometimes in the shape of a butterfly or H if communicating centrally with each other (8).
We have not found in the literature cases of unilateral and asymmetric type V ACC as in the present case, which could be linked to a more localized ischemic event in presence of an anatomical variant in the distribution of the arterial vessels.
Conclusion. The present case of aplasia cutis congenita associated with fetus papyraceus was presented for its unique asymmetric, unilateral distribution, which to our knowledge has never been reported in the literature in type V of Frieden classification.

Conflicts of interest
The Author declares that she has no conflicts of interest.

Address to:
Dr. Antonella Milano
Via Bitritto 131, 70124 Bari, Italy

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