Clinical Features

GSS manifests usually in the third or fourth decade, but occurs also in childhood (4). Men seem to be more often affected than women (male:female ratio = 2:1). GSS is seen almost exclusively in whites. The disease starts with slightly infiltrated, poikilo-dermatous plaques. Predilection sites are the intertriginous body areas, especially the axillary and inguinal folds (Fig. 1). Progressive destruction of elastic fibers results in bulky pendulous skin folds (Fig. 2). Occasionally ulceration develops. The same changes may develop in patients with pre-existing MF (5).

Figure 1 Large slightly infiltrated plaque in the groin as initial manifestation of GSS.

Figure 2 Bulky skin folds as final stage of the disease in a boy. (Courtesy of F.M. Camachoh M.D., Spain.)

Figure 3 Dense dermal lymphocytic infiltrate with scattered multinucleated giant cells.

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