Abstract

HIDALGO-MATLOCK, Benjamín; HINES-JACKSON, Norman; GEI-GUARDIA, Orlando  and  JARAMILLO-ANTILLON, Orlando. Síndrome de Touraine-Solente-Goulé (Paquidermoperiostosis primaria: breve revisión y reporte del primer caso en Costa Rica). Rev. costarric. cienc. méd [online]. 2001, vol.22, n.3-4, pp.163-170. ISSN 0253-2948.

The disease known as pachydermoperiostosis is of rare occurrence and is characterized by thickening of the skin folds on the forehead and eyelids and in the extremities. It is also accompanied by a gross thickening of the underlying the bony structures of hands and feet, raising the diagnostic possibility of acromegaly. It has two presentations, idiopathic, which is transmitted in an autosomic and dominant fashion with variability in its penetrance and starts in the teen years and halts progression in the third and fourth decades of life, the secondary type starts in the fourth and fifth decade and its secondary to a pulmonary or intrathoracic tumoral lesion. Treatment consists in either reconstructive surgery for the primary type, with cosmetic acceptable results and surgical excision in the secondary type with partial or complete regression of the disease. We present to our knowledge what is the first reported case in Costa Rica with the purpose of informing the scientific body in our country of the existence of the disease and to help detect any other possible cases.

Keywords : Primary pachydermoperiostosis.