Severe Generalized Epidermolysis Bullosa Simplex in Two Hong Kong Children due to Variants in and .

We report two Hong Kong children with severe generalized epidermolysis bullosa simplex (EBS), the most severe form of EBS, without a family history of EBS. EBS is a rare genodermatosis usually inherited in an autosomal dominant fashion although rare autosomal recessive cases have been reported. Genetic studies in these patients showed that the first case was due to a novel heterozygous variant, c.

Neonatal epidermolysis bullosa: lessons to learn about genetic counseling.

Background: Epidermolysis Bullosa (EB) is a heterogeneous group of congenital blistering diseases that usually presents in the neonatal period. EB is classified into three major categories, each with many subtypes based on the precise location at which separation or blistering occurs, namely epidermolysis bullosa simplex (EBS), junctional epidermolysis bullosa (JEB) and dystrophic epidermolysis bullosa (DEB).

Methods: We describe genetics of neonatal EB in Hong Kong.

Vastus Lateralis Muscle Free Flap for Skull Base Osteoradionecrosis in Nasopharyngeal Carcinoma.

 To describe the first experience with a free vastus lateralis muscle-only flap to be used to cover and protect the exposed skull base and carotid artery from radiotherapy-induced skull base osteoradionecrosis (ORN).  Retrospective review of a case series.  Tertiary academic medical center.

Use of Intraoperative Indocyanine Green Videoangiography to Guide Cutaneous Angiosarcoma Excision.

Complete excision of cutaneous angiosarcoma, which is a rare, vascular, and aggressive tumor, is challenging. Its multifocal nature and propensity for lateral spread make the gross assessment of surgical margins difficult. Neither the use of frozen section analysis nor Mohs surgery consistently improves the probability of margin-free excision.

A validation study of scar vascularity and pigmentation assessment using dermoscopy.

Introduction: Vascularity and pigmentation are two important indicators of the maturing status of hypertrophic scars. We used the dermoscope to measure vascularity and pigmentation of hypertrophic scars to examine its validity and reliability.

Materials And Method: Eighteen subjects were assessed using the Vancouver Scar Scale (VSS), spectrocolorimeter and dermoscope.

A study of the maxillary and sphenopalatine arteries in the pterygopalatine fossa and at the sphenopalatine foramen.

Objectives: Arterial ligation remains a key option in the treatment of persistent epistaxis and clarification of the arterial configuration of the distal maxillary/sphenopalatine artery is important for understanding the rationale behind current surgical treatments. Greater understanding of the arterial anatomy will reduce the risk of technical failures and improve the reliability of surgical interventions for persistent epistaxis and will also be useful for surgeries involving the pterygopalatine fossa.

Study Design: Anatomical study in cadavers.

Urban temperatures in Hong Kong: thermal environmental safety and implications for city planning.

Cutaneous thermal injury caused by direct contact with objects heated solely by sun exposure is rare. We undertook to investigate factors that may influence the temperature of sun-exposed objects at the ground level. Two clinical cases of burns caused by contact with sun-exposed objects are described.

Prospective clinical study of bleeding sites in idiopathic adult posterior epistaxis.

Objectives: The precise identification of the bleeding point is important to the efficient management of epistaxis. Our hypothesis was that the septum was the commonest bleeding site.

Study Design And Setting: This was a prospective clinical study of 50 consecutive adult idiopathic posterior epistaxis patients carried out in a busy teaching hospital.

Extramammary Paget's disease in Chinese males: a 21-year experience.

Background: Extramammary Paget's disease (EMPD) is a rare disease in Chinese males. Our objectives were to examine the results of our experience, to correlate them with the published literature, and thus to formulate improved management guidelines.

Methods: This was a retrospective analysis of all male patients with EMPD treated at our institution.