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

ABSTRACT

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.