Successful treatment of a 9-year-old boy affected by acrodermatitis continua of Hallopeau with targeted ultraviolet B narrow-band phototherapy.

Acrodermatitis continua of Hallopeau (ACH) consists of a relapsing pustular eruption of the distal portions of hands and feet. We described a case of a 9-year-old boy affected by ACH, successfully treated with targeted ultraviolet B 311 nm phototherapy, which seems to be an effective and safe therapy for this condition.

Dental and anaesthetic management of children with dystrophic epidermolysis bullosa.

BACKGROUND: Epidermolysis bullosa (EB) is a genetic disease associated with fragility and bullous lesions of the skin and mucous membranes. There are various patterns of inheritance and histopathology. The disease is associated with systemic and oral manifestations, among which may be dental decay necessitating oral rehabilitation. General anaesthesia is frequently required for oral rehabilitation in children with dystrophic EB. Paediatric dentists should be aware of the implications of dystrophic EB for dental and anaesthetic management. CASE REPORT: Two siblings with moderate to severe systemic and oral manifestations of dystrophic EB requiring extensive oral rehabilitation for rampant decay are described, including special anaesthetic techniques required for airway management and maintenance of skin integrity. CONCLUSION: Dystrophic EB is a rare genetic disorder in which vesiculobullous lesions occur with erosions and scarring of cutaneous and extracutaneous surfaces in the oral cavity. Poor oral hygiene results from efforts to avoid mucosal trauma, resulting in decay. The comprehensive care of children with dystrophic EB impacts not only dental management but anaesthesia administration as well.

A case of dystrophic epidermolysis bullosa: surgical treatment for hand contracture using abdominal skin flap.

The patient was a 15-year-old male. Since birth, he has suffered from recurrent blistering, especially on the extremities. Histology and electron microscopic finding of the cutaneous lesions were compatible with those of dystrophic epidermolysis bullosa. Contracture and mobility limitation of the hands had gradually deteriorated. Prior to the admission to our clinic, both hands' contracture had been surgically treated 4 times in another hospital; however, after treatment with skin grafting, hand contracture recurred each time within one year. This time, surgical treatment was carried out for his left hand contracture using an abdominal skin flap under local anesthesia. In comparison with the free skin graft the abdominal skin flap improved hand contracture for a longer time. Abdominal skin flap is recommended as a method for the treatment of hand contracture of patients with dystrophic epidermolysis bullosa.

High-dose tocopherol acetate therapy in epidermolysis bullosa siblings of the Cockayne-Touraine type.

We have employed a successful therapy for epidermolysis bullosa with high-dose oral tocopherol acetate. Two siblings with dominant dystrophic epidermolysis bullosa of the Cockayne-Touraine type were reported. Both siblings suffered from tense blisters and erosions healing with scars and transient milia on the extremities. Electron microscopic study of the blisters revealed a cleavage beneath the lamina densa in both siblings. High-dose oral tocopherol acetate therapy was administered to them with satisfactory clinical reduction of the blister formation.