Juvenile dermatomyositis in Thai children.

Juvenile dermatomyositis is a rare, chronic multisystemic inflammatory disorder of unknown etiology, characterized by a typical skin rash and proximal muscle weakness. A retrospective study from the medical records of patients diagnosed as juvenile dermatomyositis was performed at Queen Sirikit National Institute of Child Health from 1988 to 1998. There were seven cases of juvenile dermatomyositis diagnosed according to the criteria of Bohan and Peter. Six cases were female and one case was male. The age of diagnosis ranged from 2.5 years to 11 years. (mean age was 7 +/- 3.6 years). The presenting symptoms were muscle weakness (6 cases), muscle pain (2 cases) and skin rashes (4 cases). All of the patients developed proximal muscle weakness of the lower extremities varying from grade 3 to grade 4. The cutaneous manifestations were heliotrope signs (6 cases), gottron's papules (2 cases), photosensitivity (2 cases) and calcinosis cutis (4 cases). Electromyography (EMG) was performed in 6 cases and revealed typical change of myopathic type. Elevated muscle enzymes were noted in all cases. Muscle biopsy was performed in 6 cases and was compatible with myositis. Oral prednisolone (1-2 mg/kg/day) was given in 6 cases and the muscle weakness improved. There was no mortality in this study. Four cases developed calcinosis cutis 1 to 3 years after muscle weakness and did not respond to any treatment. In conclusion, juvenile dermatomyositis is a disease which causes chronic disability in children. Early diagnosis and treatment can prevent morbidity and mortality. Calcification at the skin usually occurs after the onset of muscle weakness several months to years after diagnosis.

Proteus syndrome: a case report.

Proteus syndrome is a rare genetic disorder, characterized by partial gigantism of the hands and/or feet, asymmetry of the limbs, plantar hyperplasia, multiple hamartomatous subcutaneous tumors, hyperostoses, and long bone overgrowth. A one day old Thai male infant is reported with macrosomia, hemihypertrophy of the left side of the face and left leg, large feet, macrodactyly of toes, plantar hyperplasia, large subcutaneous mass with a violet-red surface over the left side of the chest wall and a large port-wine stain involving the lateral aspect of the right chest wall. The clinical findings, diagnostic criteria, differential diagnosis, and management of the Proteus syndrome are reviewed.

Neonatal varicella: a report of 26 cases.

Varicella infection usually occurs in childhood and is uncommon in neonates. We reported 26 cases of neonatal varicella seen at the Queen Sirikit National Institute of Child Health, Bangkok, from 1988 to 1995. The sex ratio of male to female was equal. The age of onset was between 6 to 27 days. Twelve cases contracted varicella from mothers who were infected between 6 days before delivery to 2 days after delivery (perinatal varicella) and fourteen cases contracted varicella from mothers or siblings in the postnatal period (postnatal varicella). All babies developed vesicular rash. Intravenous acyclovir was given in high risk and severe cases (nine perinatal and three postnatal varicella patients). Complications of neonatal varicella included clinical sepsis 8 cases (30%), pneumonia 7 cases (26%), pyoderma 9 cases (35%) and hepatitis 1 case (4%). There was no statistical difference between the complications of perinatal and postnatal group (p > 0.05). No death was observed during this study. Clinical manifestations of neonatal varicella varied from mild to severe, depending on the onset of rash in the mother and baby and mode of transmission of the disease. Although we have no varicella-zoster immunoglobulin (VZIG), acyclovir therapy is beneficial in the treatment of neonatal varicella.