Science and Education Publishing American Journal of Medical Case Reports 2 12 2014 12 03 Punctate Palmoplantar Keratoderma (Brauer-Buschke Fischer Syndrome) and Pituitary Adenoma 276 279 EN Mouna ELLEUCH Raouf HAJJI Internal Medicine Department, Regional Hospital of Sidi Bouzid, Sidi Bouzid, Tunisia Fatma DERBALI Kais MNAFGUI Noureddine LITAIEM Naourez KAMMOUN Nadia CHARFI Mohammed ABID AJMCR20142125 10.12691/ajmcr-2-12-5 2014 10 30 2014 11 28 2014 12 03 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.