Congenital self-healing Langerhans cell histiocytosis with pulmonary involvement: spontaneous regression.

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by eruption of multiple, discrete and red-brown papules and nodules which may increase in size and number during the first few weeks of life with spontaneous regression. Systemic signs are usually absent except for occasional mild hepatomegaly. The authors report a case of CSHLCH associated with multiple lung cysts with spontaneous regression. A healthy full term male infant presented at birth with numerous diffuse discrete dark-red papules sized 0.2-1.0 cm scattered on the eyelids, temporal areas of the scalp, neck, palms and soles. Histologic findings were mixed inflammatory infiltration of numerous histiocytes. Immunochemistry findings were S-100 and CDla positive consistent with CSHLCH. Hepatomegaly and multiple lung cysts were detected at 1 month old. Since he was healthy, no medication was given except for close monitoring. At 2 months of age, hepatomegaly was resolved but the liver had sclerosing change. Skin lesions regressed completely at the age of 4 months. Lung cysts were markedly improved at 7 months old and completely resolved at 1 year old. Spontaneous regression in CSHLCH with lung involvement has never been reported in Thailand.

Henoch-Schonlein purpura: clinical manifestations and long-term outcomes in Thai children.

The clinical features of 47 children with Henoch-Schonlein purpura (HSP) are presented. The most common ages at presentation ranged from 3-5 years. Duration of data collection was 60 months. The peak incidence was from December to February. The organ involvements included skin (100%), gastrointestinal tract (74.5%), renal (46.8%) and joint (42.6%). Renal involvement was detected within the first 2 months in 16 cases (72.7%) but was delayed until 6 months after diagnosis in 6 cases. No risk factors for renal involvement could be identified. The mean duration of follow-up was 2.6 years (range 1-5 years). Six out of 16 (37.5%) patients had residual renal diseases but none were end stage. Recurrent episodes of abdominal pain and skin purpura were found in a few cases during the first year. Overall prognosis of HSP is good and long-term morbidity is predominantly associated with renal involvement. Patients with initially normal urinalysis should have sequential urinary examination at least for 6 months.