General physical manifestations- Café au laît macules (CALMs)
The most common systemic sign of neurofibromatosis was the presence of hyperpigmented macules (patches) on the skin; known as Café au laît macules (CALMs)35. While most cases had the hyperpigmented (café) spots and patches, very few had the hypopigmented (laît) spots. Even among the patients with hyperpigmentation, this was sometimes difficult to see against the backdrop of their dark complexion (Figure 2a-c). Twenty-five out of 34 cases, had documentation on the presence or absence of CALMs. Café au laît macules were observed in 22 out of 25 (88%) of cases. The distribution was predominantly on the trunk, buttocks and limbs. Identification of CALMs required a general physical examination in most cases, as they are not readily visible. Axillary and inguinal freckling was very uncommon, seen in only 2 cases (5.9%). We observed multiple crops of hyperpigmented macules measuring about 2-3mm in diameter, on the palms of one patient.