Encephalocraniocutaneous Lipomatosis (Haberland’s Syndrome) - A Case Report of a Neurocutaneous Syndrome and a Review of the Literature

We present the case of a full-term girl born to nonconsanguineous parents. Physical examination revealed a large (14 x 6 centimeters) hairless lesion on the right frontalparietal scalp (Fig.1), multiple nodular lesions of the right upper eyelid and eyebrow, and a reddish bulbar conjunctival lesion on the right eye consistent with choriocystoma (Fig.2). A CT scan of the brain showed cranial asymmetry, an arachnoid cyst of the right middle fossa, a right frontal subdural collection, a porencephalic cyst, hemiatrophy of the right hemisphere, and cortical calcifications of the parietal and occipital lobes (Fig.3). At the age of two years, the patient underwent a complete excision of the scalp lesion after tissue expansion and excision of the nodules on the eyelid (Fig.4). The histopathological examination of the scalp lesion showed absence of hair follicles, a thinned dermis, and extended adipose tissue into the dermis; the cutaneous nodules were Figure 1 The hairless lesion (“naevus psiloliparus”)