Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder, characterized by skeletal anomalies and multiple keratocystic odontogenic tumors of the jaws. The skeletal anomalies of this syndrome are mandibular prognathism, bossing of frontal and parietal bones, high-arched palate, and bifid rib. We report three cases with NBCCS, emphasizing the clinical and radiographic findi...

The first three disorders discussed are abnormalities of bone: too little bone in cleidocranial dysplasia caused by mutations in RUNX2; too much bone in fibrodysplasia ossificans progressiva with overexpression of BMP4; and abnormal bone in McCune-Albright syndrome and fibrous dysplasia caused by mutations in GNAS1. Disorders of the sonic hedgehog signaling network are discussed next, including...

INTRODUCTION Nevoid hyperkeratosis of the nipple and areola (NHNA) is a rare, benign condition of unknown etiology characterized by verrucous hyperpigmented plaques on the nipple and/or areola that was first described in 1923. Since then many cases have been reported in the literature and some have argued whether it is a distinct entity. Since the pathogenesis is unknown and it is a rare dermat...

Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder and is characterized by a great variety of signs and symptoms. The most important are a characteristic facies, the occurrence of basal cell carcinomas and odontogenic keratocysts. In view of the neoplastic skin change, constant review of the patients is indicated. Any jaw film revealing two or more dentigerous or follicular ...

Nevoid basal cell carcinoma, known as Gorlin Goltz Syndrome, is a rare hereditary condition, characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. In this report, we discuss a case of a patient with Gorlin Goltz Syndrome, who was 16 years old when first admitted for an initial appointment. The patient was diagnosed, treated and followed up for 7 years t...

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare, autosomal-dominant, cancer-predisposing, multisystem disorder. The clinical manifestations of NBCCS include multiple basal cell carcinomas (BCCs), odontogenic keratocysts, palmar or plantar pits, and calcification of the falx cerebri. We present a case of an 11-year-old boy with Fitzpatrick skin type V who presented with multiple facial le...

A 14 year old female presented with a patch of hair on her lower back since infancy (Figure 1). There was history of prolonged low backache. The antenatal and birth history was uneventful. Developmental milestones achieved by her were normal for age. There was no history of urinary or fecal incontinence or lower limb weakness. On examination, a well-defined patch of terminal, dark hair was note...