Malte Stoffregen und Johannes Baar: Ein vernachlässigter Quellentext zur Geschichte des adrenogenitalen Syndroms. Jean-Baptiste Bouillauds Klassifizierung des Hermaphrodisme Valmontien (1833)

In the early history of the discovery of the adrenogenital syndrome one occasionally comes across case histories that can only be connected with this endocrinological disease because of clinical criteria. In these cases, it it impossible to prove the diagnosis because the adrenal glands and their pathological changes, which constitute the basis of this disease, are not mentioned. One of these uncertain cases claimed to testify to an early knowledge of the adrenogenital syndrome is a hermaphrodite described by the French clinican Bouillaud in 1833. The subject presented as a male whose genitalia were unremarkable, apart from slight hypospadia and an empty scrotum. On dissection, the pelvis was found to contain a uterus, tubes, ovaries and – a prostate. In spite of the absence of male gonads, i.e. tissue producing androgens, the phenotype was male. In the usual classification of hermaphroditism, this is female pseudohermaphroditism, and we must ask ourselves wether this was an instance of the adrenogenital syndrome, i.e. virilisation of a female caused by androgens. Because the adrenal glands are not mentioned, direct proof is impossible.

A close reading of the report and the plates showed, that a definitive diagnosis could be arrived at even without an examination of the adrenal glands: Abnormal androsteroids (which are causative for this pathology) produced by the adrenal glands during the develoment of the female embryo cause the development of a ‚prostate‘, which has its origin in the paraurethral glands, the homologue of the male portion of the bisexually preformed prostate. The characteristic position of this organ normally absent in the female in relation to other parts of the female genitourinary tract, which is only present in the adrenogenital syndrome and is documented in one of Bouillaud’s plates, dispells any doubts about the diagnosis and allows us to add another case to the small number of certain instances during the early history of the discovery of this rare pathology.

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