Paget's Disease of T H E Skin and Its Relation to Carcinoma of T H E Apocrine Sweat Glands

More than sixty years has elapsed since Sir James Paget ( I ) , with admirable succinctness, described a peculiar affection of the skin now called Paget's disease of the nipple. Paget himself had noted the possibility of an extramammary location, and his casually created dichotomy of malnmary and extramammary has persisted to the present time, even though, before long, the histologic identity of the lesions became evident. The proper justification for maintaining this division was the number of clues which the extramammary cases might offer towards the solution of this long baffling affection. For the history of the disease, as recorded in a voluminous literature, is a history of prolonged controversy. Is Paget7s disease a chronic eczema? Is it a dyskeratosis, or precancerous? Is it always associated with cancer? Is it itself cancer? If so, what sort of cancer? These and a host of similar questions regarding both the etiology and histogenesis have been raised constantly. Unfortunately, the answer-presumably histological-has too frequently been sought in either clinical or incomplete surgical material. Of the 57 extramammary cases recorded in the literature, not a single one includes necropsy findings. Such a situation in turn has tended to discredit a method inadequately utilized. Cheatle (2 ) , himself an advocate of a more complete histological technic, says: ". . . morphological appearances alone are insufficient to determine beyond all doubt the site of the epithelium in which the neoplasia originated. Whether unanimity of opinion on this matter will ever be reached does not concern the problem. . . ." That such a view is not justified has been amply demonstrated by many of the more recent detailed studies, as those of Muir ( 3 ) , Pautrier (4), and Sirnard (5). In the belief that the histogenetic mystery of Paget's disease might be elucidated by detailed histological studies, the following case, occurring in the vulva, was studied. As the study progressed, it became increasingly evident (a) that the extramammary site was not completely haphazard, (6 ) that the relation between the skin lesion and the underlying cancer was fairly definite, and ( c ) that the nature of the carcinoma could be determined with reasonable certainty. Furthermore, interesting general relationships were discovered between the apocrine sweat glands and Paget's disease.