A Case of Aplasia Cutis Congenita, Type VII: DISCUSSION part 2

Leg

Aside from other differential type of ACC, we made a distinction with drug-induced ACC, Adams- Oliver syndrome, Bart syndrome, and traumatic origin ulceration. As there were no signs of syndactyly or transverse nail dystrophy and no defects in the central nervous system, Adams-Oliver syndrome, an ACC with transverse limb defects, can be excluded. Bart syndrome which was first described by Bart in 1996, has to be considered; it represents the combination of congenital epidermolysis bullosa, congenital localized absence of skin affecting the extremities, and shedding or dystrophy of the nails. It sometimes also involves the oral mucous membrane. Our patient did not have any nail abnormality or mucus membranes involvement.

When ACC occurs as a small, focal scalp ulcer, it heals spontaneously. In the case of the large lesion with or without underlying tissue and associated with exposed dura or sagittal sinus, prompt intervention should be considered. While some evidence exists to support conservative dressing and the avoidance of active surgical intervention, several cases of dramatic and often fatal sagittal sinus hemorrhage associated with a large full thickness scalp and skin defects over the sagittal sinus have been reported. Therefore, consultation with the surgical department, such as neurosurgery, is of critical importance in the treatment of these patients. After our patient was given conservative treatment with prophylactic topical antibiotics and wet dressing, the ulcer was healed with atrophic scar formation. viagra 50 mg

In conclusion, localization in the pretibial area of the right limb and the absence of associated malformation suggests that our case should be classified as type VII of the classification accordin- g to Frieden. Almost all previously reported cases had scalp ACC. However our case is limited to unilateral lower extremities. This is the first case of ACC on the leg without association with epider- molysis bullosa in Korea. The follow-up after nearly 2 years showed a very favorable outcome, with little scar formation and no functional impairment of the leg.

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