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.