Epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. Multicystic kidney disease has been very rarely reported in this syndrome. Here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with Wilms' tumor. According to this ...

Blue rubber bleb nevus syndrome (BRBNS) is a congenital disorder with characteristic venous anomalies that can present with varying degree of blood loss. The most clinically significant symptoms in adults include gastrointestinal (GI) bleeding and iron deficiency anemia. Severe complications can include intestinal torsion, intussusception, and even perforation, with each leading to significant ...

This 66-year-old Iranian gentle man had a one-year history of asymptomatic keratotic papules with a linear distribution on the skin of his right palm near the wrist. On histopathologic examination-cornoid lamella-like parakeratotic columns above eccrine ducts were observed. The acrosyringium was also dilated. The diagnosis was porokeratotic eccrine ostial and dermal duct nevus. The late-onset d...

BACKGROUND Sturge-Weber syndrome is a rare congenital neuro- oculo- cutaneous disorder. OBJECTIVE To report a very rare unusual case of bilateral manifestation of Sturge Weber syndrome. CASE We report an unusual case of a 17-year-old female with advanced stage of bilateral glaucoma associated with facial nevus extending to the other half of the face as well and bilateral intracranial calcif...

Port-wine stains (nevus flammeus, port-wine nevus) are congenital vascular lesions that have psychological and physiological implications for patient care. The location and size of these lesions can lead to complications related to anesthetic management. Facial lesions have the most profound psychological effect on the patient with respect to behavior alterations and reluctance to disclose the ...

A 20-year-old woman presented with a 2-day history of headache. She had a congenital grayish-blue pigmented nevus on her left side face, which involved ipsilateral sclera and oral mucosal membrane, diagnosed as nevus of Ota (figure 1, A and B). Neuroradiologic manifestation revealed a lesion in the left cerebellar hemisphere, the etiology of the presenting tumor apoplexy (figure 1, C–F). A left...

Vol. 28, No. 1, 2016 123 Received January 17, 2015, Accepted for publication March 21, 2015 Corresponding author: Hoon Kang, Department of Dermatology, St. Paul’s Hospital, College of Medicine, The Catholic University of Korea, 180 Wangsan-ro, Dongdaemun-gu, Seoul 02559, Korea. Tel: 82-2958-2143, Fax: 82-2-969-8999, E-mail: [email protected] This is an Open Access article distributed unde...

The sebaceous nevus of Jadassohn is a congenital hamartoma that may develop into a malignant cutaneous neoplasia. Photodynamic therapy is used to treat actinic keratoses and superficial or nodular basal cell carcinomas, and the cutaneous field cancerization can be observed using the Wood’s lamp during the performance of the technique. This article describes a case of photodynamic therapy used i...

The term large plaque type blue nevus with subcutaneous cellular nodules (LPTBN-SN) refers to a huge blue nevus, usually located on the trunk, that develops subcutaneous nodules many years after the lesion has appeared. The potential malignancy of an LPTBN-SN was only discovered in 2012. We report the case of a 56-year-old Caucasian man that developed a cutaneous melanoma on an LPTBN-SN of the ...

In this issue, Bayer-Garner et al.1 report on the immunopathology of regression in benign lichenoid keratosis, keratoacanthoma (KA), and halo nevus. They suggest that cytotoxic T-cells may be the common denominator of regression in these tumors. Other studies of halo nevi have shown abundant CD8+ T-cells and FXIIIa-positive histiocytes during regression, suggesting that the immune response is i...