Punctate Palmoplantar Keratoderma (Brauer-Buschke Fischer Syndrome) and Pituitary Adenoma

Mouna ELLEUCH¹, Raouf HAJJI^1, , 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

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

Abstract

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.

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

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