Report on a patient suffering from keratosis follicularis spinulosa decalvans (Siemens I syndrome). This ia a very rare clinical picture, at least in its complete form. The patient's father and brother are suffering from an abortive Siemens I syndrome. The condition is a form of hereditary parakeratosis, a spinulose keratosis with typical localization in the eyes and on the skin. Causal therapy is not possible; attempts at conservative and surgical treatment are described. Prognostically, the condition will probably come to a standstill at puberty. The description of this case is intended to illustrate the necessity of close cooperation between dermatologist and ophthalmologist.
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