Sidonia and Nicu de Barcsy: a famous mother with post-partum hirsutism after giving birth to a famous son with idiopathic short stature

Summary At the end of the 19th century, an 18-year-old lady gave birth to a well-proportioned, though very small, son. After delivery, the mother developed a full-grown beard, whereas the son always remained of small stature. The mother developed diabetes mellitus and died, aged 59, from a complicated severe cold. The son died at the age of 91 because of chronic kidney disease. The differential diagnosis in the son is isolated growth hormone deficiency. The mother might have suffered luteoma of pregnancy, polycystic ovary syndrome (PCOS), or Sertoli–Leydig cell tumor(s). The two cases are apparently coincidental/not related in pathophysiology. Learning points Hirsutism occurring directly postpartum can have several causes. Patients with isolated growth hormone deficiency can live a long life without the substitution of growth hormone. Coincidence does not necessarily imply correlation. In the past, patients with endocrine disorders like severe hirsutism or small stature were employed at circuses and fairs to entertain the audience as curiosities.


Background
Two different relatively rare endocrine disorders in a mother and son apparently seem to suggest some kind of pathophysiologic relationship.However, the presented cases, postpartum hirsutism and small stature/isolated growth hormone deficiency, seem to be unrelated and coincidental.Despite the fact that both disorders occurred in an era when endocrine testing was not possible yet, the clinical deduction can come close to probable differential diagnoses.
The appreciation of endocrine disorders leading to particular phenotyping (bearded female, small stature male) by the general public in the pre-World-War-II era was quite different from nowadays: patients were generally not seen as patients but as show objects, or curiosities.The impression, moreover, is that the bearded female patient was treated with dignity.

Discussion
Nicu de Barcsy was a normal-proportioned person of short stature (less than 1 m).Idiopathic short stature can have different causes (2).Interestingly, a historic German postcard from that time mentions that he originated from Veglia, which is the Italian name for the Croatian island Krk (Fig. 4B).Due to isolation and consanguineous marriages, this island is well-known because of the increased prevalence of specific hereditary diseases like idiopathic small stature.The Hanhart's dwarfs on the island of Krk, as they have been called, have recessively inherited multiple pituitary deficiencies (MPHD) due to a mutation in a pituitary transcription factor gene, the PROP1 gene (3,4,5,6).With adequate hormonal substitution, these patients can live a long life and it should be noted that Nicu de Barcsy also became 91 years old (3).According to the available information, Nicu never received any hormonal treatment.Also, there is no indication that he originated from the island of Krk, nor that he was adopted from that area.Therefore, despite the fact that a relationship with the island Krk and its small stature people had been suggested, it is more probable that Nicu de Barcsy suffered from isolated GH deficiency only.In the case of multiple pituitary insufficiencies, it is difficult to explain how he could survive for so long without any hormonal replacement therapy.It is also not plausible that renal insufficiency was the main cause of the growth delay since reports only mention that he suffered from kidney disease later in life.Growth retardation is one of the sequelae of chronic kidney disease in young children, but it is highly improbable that Nicu de Barcsy could have survived for such a long time with childhood-onset chronic kidney disease.
Sidonia de Barcsy developed severe hirsutism.There is no evidence that she developed other phenomena of virilization.Hirsutism can have many causes, but since she survived for 41 years after the first diagnosis, malignant adrenal and ovarian tumors seem the most improbable causes.Also, she did not develop the fullblown phenotype of Cushing's syndrome which excludes this diagnosis as well.The most probable causes of hirsutism in this case are luteoma of pregnancy, polycystic ovary syndrome (PCOS), or Sertoli-Leydig cell tumor(s) (7,8,9,10,11,12).Since PCOS is closely linked to insulin resistance, the development of diabetes at a later age fits well into this diagnosis (13,14,15).In patients with luteoma of pregnancy, signs and symptoms of androgen excess usually present during pregnancy and these tend to disappear post-partum, but exceptions have been described (11).The fact that the hirsutism started immediately after pregnancy does not seem to fit with the diagnosis of luteoma of pregnancy (11).Although maternal virilization in PCOS during pregnancy has been described, postpartum virilization is also not typical of PCOS (7,16).Late-onset (congenital) adrenal hyperplasia is another diagnosis which cannot be excluded.However, one would expect amenorrhea, decreased fertility and a relation between pregnancy and the development of hirsutism directly post-partum with this condition also cannot be easily explained (10).Androblastomas, or Sertoli-Leydig cell tumors are rare androgen-producing ovarian tumors that mostly occur between the ages of 20 and 40.The well-differentiated mostly unilateral tumors usually follow a benign course, but poorly differentiated malignant varieties which metastasize do also exist.These tumors have also been described in combination with pregnancy (12,17).
The case of Sidonia de Barcsy matches with another famous historical case of hirsutism occurring shortly after pregnancy in Magdalena Ventura from Abruzzi, Italy, who developed a beard at the age of 37.She gave birth to seven children: three were born before the beard started to grow and four were born after she had grown the beard.In 1631, at the age of 52, she and her husband were pictured in a painting by the Spanish artist José de Ribera (1591-1652).In the painting, which is currently on display in the Prado Museum in Madrid, Spain, she is breastfeeding her most recently born child in 1631 (18,19).
Bearded ladies were very popular actors in sideshows and traveling circuses in the 19th century and the beginning of the 20th century (20).Like the famous French bearded woman Clementine Delait, Sidonia de Barcsy was proud of her beard and did not feel the urge, nor the social pressure to shave and she was treated with dignity (20).The combination of severe post-partum hirsutism and offspring with short stature has not been described before and since a causal relationship is not evident this should be merely considered as coincidental.
A relationship between virilization of the female fetus and hyperandrogenism in women has been described, however (10,11,17).

Declaration of interest
The author declares that there is no conflict of interest that could be perceived as prejudicing the impartiality of the study reported.

Figure 1
Figure 1 Sidonia de Barcsy (1 May 1866 -19 October 1925) before she developed a beard.Picture from a picture postcard published by Mucke & Schaerf in Gera-Reuβ, Thüringen, Germany.Postcard in the collection of WW de Herder.

Figure 2
Figure 2 Sidonia de Barcsy (1 May 1866 -19 October 1925) after having developed a full-grown beard.Picture from a picture postcard published by Mucke & Schaerf in Gera-Reuβ, Thüringen, Germany.Postcard in the collection of WW de Herder.

Figure 3 Figure 4 A
Figure 3 Sidonia de Barcsy (1 May 1866 -19 October 1925) before and after having developed a full-grown beard.Fragment from a picture postcard.Postcard in the collection of WW de Herder.

Figure 5
Figure 5 Nicu de Barcsy (28 February 1885 -31 July 1976) dressed as Baron Capita(i)n.Fragment from a picture postcard.Postcard in the collection of WW de Herder.