Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a...

Congenital melanocytic nevus syndrome (CMNS) is the result of an abnormal proliferation of melanocytes in the skin and central nervous system caused by progenitor-cell mutations during embryonic development. Mutations in the NRAS gene have been detected in many of these cells. We present 5 cases of giant congenital melanocytic nevus, 3 of them associated with CMNS; NRAS gene mutation was studie...

Giant congenital melanocytic nevi are rare and occur in about one out of every 2,00,000 to 5,00,000 births. There is a significant association between bathing trunk nevus and neurofibromatosis and lipomatosis. Apart from this, association of bathing trunk nevus with abnormalities like spina bifida occulta, meningocele, club foot and hypertrophy or atrophy of deeper structures of a limb, have be...

Herein reported is a case of congenital malignant melanoma in a premature male baby from a 25-year-old healthy mother who was found to have hydramnios at the 29th week of gestation. The pregnancy was interrupted because of a large posterior neck mass detected by ultrasonography. The large neck mass of the baby was a malignant melanoma involving deep dermis and subcutaneous tissue. The skin over...

Epidermal nevus syndrome is a kind of neurocutaneous syndrome that is associated with epidermal nevus and a variety of congenital CNS disorders. Clinical presentations include seizures, paresis, mental retardation, and developmental delay. We report three cases with MR imaging and magnetoencephalography findings; one patient underwent ictal and interictal single photon emission CT. Both structu...

CHOROIDAL NEVUS Choroidal nevus (Figure 1), the most common primary intraocular tumor,1 is a benign tumor that arises from melanocytic cells derived from the neural crest. Choroidal nevi are more common in white people, with 4% of the population having at least one.1 Malignant transformation into melanoma is possible; therefore, choroidal nevi should be monitored even though the rate is 1 in 4,...

Malignant melanoma in children is rare. It can arise from congenital melanocytic nevi. In pediatric patients, diagnosis of melanoma is difficult and challenging because the physicians have a low index of suspicion. Marrow metastasis in malignant melanoma especially in children is extremely uncommon. Here, the authors reported a 5 year old girl who was presented with a 4 month history of pelvic ...

Nevus comedonicus is a rare cutaneous anomaly, characterized by the clustering of papules with firm, blackened horny buffers. It in general has a zosteriform pattern and affects both genders equally. In some patients, there is formation of cysts, abscesses, and fistulas, aggravating its psychosocial impact. The association with extracutaneous lesions characterizes the nevus comedonicus syndrome...

Nevus sebaceous (NS) is a type of classical nevus or congenital malformation that is often present at birth and commonly involves the scalp or face. The lesion usually presents as a linear, yellow, hairless, and verrucous plaque. It has been well-established that several benign and malignant tumors can develop from the NS; however, there have been no reports about ectopic fat cells in the dermi...