EPIDERMOLYSIS BULLOSA (CASE REPORT)
Lubis M, Lubis CP, Pasaribu SP, Siregar C
Haji Adam Malik Hospital,
Medan, Indonesia
Epidermolysis bullosa are heterogenous group of
congenital, hereditary blistering disorders, that can be categorized under
three major headings: simplex epidermolysis bullosa, junctional
epidermolysis bullosa, and dystrophic epidermolysis bullosa. It is vary
rare case, where the incidence of dystrophic epidermolysis bullosa is about
1 in 50.000 live births.
Objective: to report a case of epidermolysis
bullosa in newborn infant.
Methods: descriptive report. The patient was
admitted at Haji Adam Malik Hospital, Medan, Indonesia since July 4th
until July 24th 2001.
Results: B/o L, a 10 days old boy, was referred
to the hospital with the complaint of blistering lesions on his hands,
feet, legs, neck, mouth and scalp that firstly appeared since birth. When
he was 5 days of birth, traditional healer treated the lesions, but it
tends to be severe. The body weight was 3000 grams, body length was 50 cm
and body temperature was 38.5¡ãC. There were no pale,
icteric, cyanosis, oedema nor dyspnoe.
Based on clinical and histologic features we suggested
that the lesion is dystrophic type of epidermolysis bullosa, with secondary
infection caused by Staphylococcus aureus. We gave phosphomycin 250 mg in
100 ml of 5% dextrose solution every 12 hours, hydantoin, skin care,
nutritional support and physiotherapy for preventing skin contracture.
Conclusion: It is the first case in Haji Adam
Malik Hospital during 8 years. The lesion had response by treatment, and
after 20 days of admission he got clinical improvement.