Aplasia Cutis Congenita | Figure 2.2
A: Seborrheic dermatitis
B: ACC, cicatricial (Courtesy of
Dr. Michelle B. Bain)
C: ACC, bullous (Courtesy of
Dr. Michelle B. Bain) |
- Presents with erosion, ulceration, scar, or membranous defect (ovoid lesion covered by an epithelial membrane)
- Hair collar sign: ring of dark long hair encircling lesion; ± marker of underlying neural tube defect
- Typically isolated abnormality, but may be associated with developmental anomalies or following disorders:
| | | | | | | | Bart Syndrome | | ACC of lower extremities + epidermolysis bullosa (dominant dystrophic) | | | Adams–Oliver Syndrome | | ACC on scalp (with skull ossification defect) + extensive CMTC + limb defects (reductions, syndactyly) + cardiac abnormalities | | | Seitles Syndrome | | Bilateral temporal ACC + abnormal eyelashes, “leonine” facies, upward-slanting eyebrows |
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