Bazex A, Bazex J
Ann Dermatol Venereol. 1978 Oct;105(10):851-8.
The authors study three rare syndromes which are characterized by the association of cutaneous manifestations with an intestinal polyposis: Gardner's syndrome, Peutz-Jeghers-Touraine's syndrome, Cronkhite-Canada's syndrome. The Gardner's syndrome is transmitted with an autosomal prevalence, and its vital prognosis remains very porr. It is characterized by the association of various cutaneous manifestations such as fibromas, freckles, etc. with osteomas, neuro-fibromas and polyps of the large bowel. Its severity is related to the very early malignant degeneration of digestive polyps. The Peutz-Jeghers-Touraine's syndrome is transmitted in an autosomal prevalence and its vital prognosis is benign. The cutaneous manifestations are the very early occurrence of lentigines on the face, around the hiatus, and on the lips. The polyps are situated on the small bowel, and are the source of important functional phenomenons; their malignant change is rare. The Cronkhite-Canada's syndrome is rare. Its etiology is unknown and its prognosis is very poor. Its manifestation is the association of more or less wide-spread cutaneous pigmentations, alopecia, and onyxis with a digestive syndrome secondary to a pseudo-polyposis which is the origin for afecal and serous diarrhea, a cause for very severe denutrition. The diagnosis and the treatment of these three syndromes are discussed.
作者研究了三种罕见综合征，其特征为皮肤表现与肠道息肉病相关联：加德纳综合征、佩-杰-图综合征和克-卡综合征。加德纳综合征以常染色体显性方式遗传，其生命预后仍然很差。它的特征是各种皮肤表现如纤维瘤、雀斑等与骨瘤、神经纤维瘤和大肠息肉相关联。其严重程度与消化性息肉的极早期恶性变有关。佩-杰-图综合征以常染色体显性方式遗传，其生命预后是良性的。皮肤表现为面部、乳晕周围和嘴唇上很早就出现雀斑样痣。息肉位于小肠，是重要功能现象的根源；其恶变罕见。克-卡综合征很罕见。其病因不明，预后很差。其表现为或多或少广泛分布的皮肤色素沉着、脱发和指甲营养不良与继发于假息肉病的消化综合征相关联，假息肉病是无粪性和浆液性腹泻的根源，是非常严重营养不良的一个原因。文中讨论了这三种综合征的诊断和治疗。
