Walter H.-S. , Mittelviefhaus H.

Universitäts-Augenklinik, Killianstr. 5, D-79106 Freiburg

We report on a 64 year old patient with a eruptive Grzybowski-type keratoakanthoma, who developed cicatricial ectropion of all four eye lids and exposure keratitis due to progressive sclerodermiform scarring.
Case: The patient developed induration, redness and scaling of the facial and truncal skin at age 51. Especially in sun-exposed skin regions hundreds of 5-15 mm large follicular papules were observed. Due to progressive scarring of the skin cicatricial ectropion of all four eye lids and amimia developed in a 2 year time period. Keratinisation of the lower and upper tarsal conjunctiva, corneal vascularisation and superior limbic chemosis as well as repeated bacterial contaminations of the tear film were indications for ectropion operations, which had to be repeated after 2,4,6 and 9 years. Free skin grafts were harvested from the severely involved inside of the upper arms. Despite various trials of medical treatment progressive sclerodermiform changes of the skin grafts with scarring reoccurred.
Discussion: Since the first published case (1) only 28 additional patients with generalised eruptive Grzybowski-type keratoakanthoma have been described. In this disease cicatricial ectropion can cause severe loss of vision within a few months. The presented case describes for the first time the course of the disease with more than 13 year follow-up. Despite severe and recurrent cicatricial ectropion visual function in this patient could be preserved by timely and repeated operations. (1) Grzybowski M. 1950: A case of peculiar generalized epithelial tumours of the skin. Br J Dermatol Syphilol 62: 310-13

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