ABSTRACT

Skin and mucosal lesions ● Rough, brownish keratotic papules on the face and upper part of the trunk (seborrheic

areas) coalescing to form plaques (Figures 3.2-3.5) ● Flat, wart-like papules on the dorsa of the hands and feet (Figure 3.6) ● Palmoplantar punctate keratoses (pits) (Figures 3.7 and 3.8)

Figure 3.1 ● Guttate leukodermatous macules ● Nail plate involvement: longitudinal splits, V-shaped notches of the distal part,

subungual hyperkeratosis (Figure 3.9) ● Less frequently major folds are involved (Figure 3.10) ● Oral, vaginal, anal involvement: white papules clustered in a ‘cobblestone’ pattern ● Lesions in a mosaic linear pattern are frequent (Figures 3.11 and 3.12)

● Epilepsy and mental retardation ● Corneal, bone, pulmonary, urogenital abnormalities ● Thyroiditis

Course and complications

There is increased susceptibility to widespread cutaneous infections, both bacterial (Figure 3.13) and

herpetic (Figure 3.14). It is a persistent and longstanding disease.