Skin Disorders Guide

Diagnostic Hallmark

1. Distribution: random
2. Flat, nonpalpable
3. Homogeneous, light hrown color

Clinical Presentation

Individual cafe-au-lait patches are flat, light-brown, evenly pigmented patches 1.5 to 10 cm in diameter. Most lesions are round in configuration, but irregular and angular shapes are also seen. Cafe-au-lait patches may be present at birth, but sometimes they do not develop until childhood. Clinically it is not possible to distinguish a solitary cafe-au-lait patch from the flat, brown birthmarks that occur in about 10% of the population.

Course and Prognosis

Patients with more than four or five pigmented patches greater than 1 cm in diameter are rather likely to develop associated features of neurofibromatosis. Lesions somewhat similar in appearance to cafe-au-lait patches are also found in patients will Albright’s syndrome.Cafe-au-lait patches are permanent. Once present, they remain stable in size and color indefinitely. There is no predisposition to melanoma formation in these pigmented lesions, even when they have been present from birth.

Pathogenesis

The cause of cafe-au-lait patches is unknown. Electron microscopy reveals the presence of larger-than-normal melanin granules within the melanosomes of the melanocytes.

Therapy

No therapy is availahle or necessary.