The problem of congenital defects of the scalp has been thoroughly discussed in the European1,2 as well as American literature.3,4 The defect may be initially membranous, or bullous, or a "punched out" ulcer. The lesions are circular or linear, usually single, but they can be multiple. The defect may extend through the periosteum to the cerebral meninges. The lesions heal with a thin, hairless, atrophic cicatrix. In the larger defects, especially of the linear type, if they lie over the superior sagittal sinus there is danger of necrosis followed by hemorrhage and/or meningitis and subsequent death. The defects may appear raised because of an underlying exostosis, glial rest,5 or keloid formation. Visceral as well as other cutaneous congenital anomalies have been described in association with these defects. Cases have been reported in families. The current opinion is that these defects are due to a developmental abnormality whose
MOSCHELLA SL. Congenital Defects of Scalp with Keloid Formation: Cousins Show Similar Defects. Arch Dermatol. 1962;86(1):63–64. doi:10.1001/archderm.1962.01590070069010
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