American Journal of Medical Case Reports
ISSN (Print): 2374-2151 ISSN (Online): 2374-216X Website: Editor-in-chief: Samy, I. McFarlane
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American Journal of Medical Case Reports. 2014, 2(12), 276-279
DOI: 10.12691/ajmcr-2-12-5
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Punctate Palmoplantar Keratoderma (Brauer-Buschke Fischer Syndrome) and Pituitary Adenoma

Mouna ELLEUCH1, Raouf HAJJI1, , Fatma DERBALI1, Kais MNAFGUI2, Noureddine LITAIEM3, Naourez KAMMOUN1, Nadia CHARFI4 and Mohammed ABID4

1Internal Medicine Department, Regional Hospital of Sidi Bouzid, Sidi Bouzid, Tunisia

2Pathophysiology Laboratory, FSS, Tunisia

3Dermatology Department, La Rabta University Hospital, Tunis, Tunisia

4Endocrinology Department, Hedi Chaker University Hospital, Sfax, Tunisia

Pub. Date: December 03, 2014

Cite this paper:
Mouna ELLEUCH, Raouf HAJJI, Fatma DERBALI, Kais MNAFGUI, Noureddine LITAIEM, Naourez KAMMOUN, Nadia CHARFI and Mohammed ABID. Punctate Palmoplantar Keratoderma (Brauer-Buschke Fischer Syndrome) and Pituitary Adenoma. American Journal of Medical Case Reports. 2014; 2(12):276-279. doi: 10.12691/ajmcr-2-12-5


The type 1 punctate palmoplantar keratoderma/ Brauer-Buschke-Fischer syndrome (PKK 1) is a rare genodermatosis with a prevalence estimated at 1.17/100000. We report the case of a 70-year old patient, with type 2 diabetes, who presents a PPK 1 since the age of 30. Examination revealed an important family inbreeding, a significant family history as 38 members were affected by this disease: it was associated with different tumors (colorectal cancer, hepatocellular cancer and melanoma). Clinical examination showed multiple papular hyperkeratotic lesions with variable diameter between 2 and 10 mm on the palms and the soles. The histology confirmed the diagnosis of punctate palmo-plantar keratoderma. This patient presented at the age of 65 an acute adrenal insufficiency. Different explorations showed a non-secreting necrotic pituitary adenoma. In our knowledge, it is the first observation of association of PKK1 and pituitary adenoma. Its pathophysiological mechanism is still unclear. More studies are needed to will have to be clarify it.

punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome) pituitary adenoma

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