Histiocytosis (LCH)

There are generally first signs of LCH and frequently become manifest as scaly, erythmatous, seborrhea like brown to red papules, especially pronounced in inter-triginous zones. Hepatosplenomegaly is the indication of presence of organ involvement by LCH or it may indicate obstructive disease caused by enlarged nodes in the porta hepatis. It may also reflect kupffer cell hypertrophy and hyperplasia as an indicator of generalized activation of the cellular immune system. Cough, tachypnea/dyspnea, cyanosis, pneumothorax, or pleural effusion are attributable to the disease rather than to superimposed infection. Increasing number of cysts form honeycomb lungs and in later stages large bullae. This child had fever and was pale. The seborrhoeic dermatitis lesions were seen over scalp and abdomen. He had cervical lymphadenopathy. Abdomen was soft on palpation with liver 8 cm below costal margin and spleen 4 cm below costal margin. Preliminary blood counts show hemoglobin 6.5 gm%, TLC 6300, Platelet count 68,000, DLC N49 L46 M02 My01 MM02.