ABSTRACT

In PCT, hyperfragility in sun-exposed areas leads to vesicobullous lesions after even minor trauma (dorsa of the hands, forearms, face and ears) (Figure 21.1)

Figure 21.1 ● Blisters evolve in erosions, leaving milia and scarring (Figure 21.2) ● Malar and periorbital hypertrichosis is common, with facial hirsutism even in females

(Figures 21.3 and 21.4) ● Hyperpigmentation of different degrees in sun-exposed areas occurs without

photosensitivity ● In men, large and dark comedones may appear in malar areas (Figure 21.4)

light hypersensitivity cutaneous fragility, bullae and erosions (Figure 21.5). During adulthood sclerodermiform changes, scarring, atrophy and scleromalacia lead to a clinical picture resembling Gunther’s disease (Figures 21.6-21.8).