“…Occasional reports of its occurrence in two or more members of a family exist, nevertheless most cases are sporadic [17], Recently the clinical features have been discussed as an abnormal host reaction towards Pityrosporum orbiculare [14,20]. Furthermore endocrino logical disturbances, especially a thyroid deficiency, are regarded as a possible cause [1,8,21], Skin alterations which are discussed with respect to a possible differential diagnosis are acanthosis nigricans [3,4,8], pseudoacanthosis nigricans [18], Darier's disease [1,3,4,21], verrucae planae juveniles [3], epi dermodysplasia verruciformis Lewandowski-Lutz [3,4] and pseudoatropho derma colli [12], Until now the disease is regarded as a separate pathological entity.…”