Fetus Papyraceus: Congenital Pulmonary Anomalies Associated with Congenital Aplasia Cutis on the Surviving Twin

Abstract

Aplasia cutis congenita (ACC) can be associated with fetus papyraceus. We report here the first case of ACC linked to fetus papyraceus with pulmonary anomalies. At birth, the patient presented with skin lesions of the trunk consisting of well-defined, symmetrically distributed, bilateral atrophic ulcerations. Physical examination was otherwise normal. Persistent bronchospasm occurred at the age of 7 months; computed tomography images showed small bilateral pulmonary bullae. At the age of 5 years, skin and pulmonary lesions had not extended. Although the mechanisms of ACC linked to fetus papyraceus are unclear, vascular ischemia is strongly suggested, and could explain the bilateral and symmetric congenital skin and lung aplasia.

Aplasia cutis congenita: report of 22 cases

“…It may involve the epidermis, dermis, and/or subcutaneous fat . Only a few series are reported but none from a dermatology department. The scalp is commonly affected, but other sites can be involved.…”

“…The scalp is commonly affected, but other sites can be involved. Despite many pathogenesis theories, the etiology of ACC is still incompletely known . Treatment is usually controversial.…”

“…). Since then, more than 500 cases of varying severity but very few series have been reported . As expected, ACC is rare (0.01% in our study).…”

“…Treatment is based on either conservative methods or surgery. Local wound care (six of our patients) is recommended for small lesions of <3 cm. Reports include saline dressing, continuous saline drips, betadine solution, and silver sulfadiazine dressing .…”

Aplasia cutis congenita: A case report and literature review

“…Individuals affected by ACC most commonly exhibit a unique circular defect on the scalp. Skin defects are the primary symptom but can also be accompanied by congenital pulmonary (8) and other malformations, such as lumbosacral sinus tract (3), faun tail nevus and spinal cord malformation (9,10). An ACC scalp defect can be an indicator of internal organ involvement and increase the risk of complications (6).…”