A 15-month-old girl presented with developmental delay, intractable right focal motor seizures, hyperpigmented patch on the left side of the face, facial asymmetry, and right hemiparesis (figure 1). A diagnosis of linear nevus sebaceous syndrome (LNSS) was considered. Brain MRI revealed hemimegalencephaly (figure 2). EEG showed spike/wave discharges in left occipital leads. Ophthalmologic and s...

pachyonychia congenital (pc) is a rare autosomal dominant genodermatosis characterized hyperkeratosis affecting the nails and palmoplantar areas, oral leukokeratosis, and cystic lesions. a 39-year-old woman with pc type 1 (jadassohn-lewandowsky syndrome) and b-cell lymphoma is described. no similar disorders or parental consanguinity were found in her family. typical features of pc developed si...

A lesion of nevus sebaceus with multifocal basal-cell carcinomas was excised by Mohs' fresh-tissue technique. Histologically, most of the foci of basal-cell carcinomatosis were of the superficial type, but the few more deeply situated foci had adamantinoid features. The malignancies extended well beyond the clinically discernible margins of the nevus sebaceus. Spontaneous healing of the wound o...

Pachyonychia congenital (PC), is a rare genetic disorder, autosomal dominant, disorder of keratinization. This condition characterized by cutaneous manifestation mainly hyperkeratosis skin and mucosae hypertrophy nails. In this condition, almost 50% the patients will have oral leukokeratosis. The case report here 15 years old girl, presented with dystrophic, thickened fingernails toenails subun...

CONGENITAL lack of lashes and eyebrows, whether total or partial, is seldom encountered. Associated at times with other developmental defects, mostly ectodermal, it may appear in conjunction with general alopecia, faulty dentition, and/or cataract formation (Duke-Elder, 1952). In the so-called Ullrich-Dohna syndrome, it has been seen in combination with multiple ocular malformations, strabismus...