Xeroderma Pigmentosum with Affection of the Eye.

IN the present article an account is given of a skin disease with ocular manifestations. At the beginning the skin disease was diagnosed as lentigenes and the eye-affection was suggested to be a melanoma bulbi. Observation of the clinical picture, however, and later familial investigations, gave clear evidence of a close connection between the two affections, and the condition became recognised 'as xeroderma pigmentosum (x.p.). X.p. belongs to the so-called actinic dermatoses, and is a rare disease in this part of the world. From the present work it clearly appears that in the initial stage of the disease the diagnosis may be difficult. Here the condition has been described' from its first manifestation up to the fully developed clinical picture. The term lentigenes has been made to describe from pinhead to lentil-seed-sized, brownish maculae, considerably darker than freckles. They may be sitting close together or. singly, and so they may be scattered all over the body. They are not congenital, but appear in the growing age. Lentigenes, in contrast to ephelides, are fairly uninfluenced by light. A melanoma is, according to G. Miescher, a new-growth having the power of forming melanin. There are malignant and benign forms of these tumours. Distinction is further made between epidermogenic and cutaneous forms and those proceeding from the mucous membranes. Examples of epidermogenic melanomas are naevi spili, ephelides and lentigenes. " Mongolenfleck ", and, " Der blaue Naevus " (Jadassohn) are cutaneous forms. Melanomas also occur on the eye, and seem to have three sites of predilection: the corneal limbus, the lacrimal caruncle and the palpebral border. They may be malignant or benign. A benign melanoma may suddenly become malignant, and in these conditions it has been claimed that external factors (traumas) are of some importance in the transition to malignancy. Schieck declares that the suspicibn of malignancy should be specially strong where a macula suddenly begins to grow in an individual past the age of 30 years. Special attention should also be paid to tumours presenting marked pigmentary irregularities or