An unusual generalized form of hyperkeratosis lenticularis perstans (Flegel's disease)

An 82-year-old female presented with an 11-year history of multiple, symmetrically disseminated hyperkeratotic red-brown papules 3-5 mm in diameter on the dorsal aspects of the upper and lower extremities. The eruption first appeared on the dorsa of the feet and on the lower parts of the legs. During the last few years, the lesions extended gradually to the upper part of the legs and thighs, also disseminating over the trunk. There was no family case history of Flegel's disease. Histopathological examination revealed a hyperkeratosis with a focal parakeratosis overlying the thinned epidermis. In the papillary dermis there was a dense band-like infiltrate composed of lymphocytes and some histiocytes. Electron microscopy showed a normal number of membrane-coated granules (Odland bodies). The clinical and histopathological findings in the patient corresponded with the diagnosis of hyperkeratosis lenticularis perstans. The patient was treated with PUVA (psoralen plus ultraviolet A)-bath therapy, retinoid plus PUVA and calcipotriol. However, all of these treatments were unsuccessful.