[Dermatology and polyposis of the gastro-intestinal tract (author's transl)]. 1978

A Bazex, and J Bazex

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.

UI MeSH Term Description Entries
D007417 Intestinal Polyps Discrete abnormal tissue masses that protrude into the lumen of the INTESTINE. A polyp is attached to the intestinal wall either by a stalk, pedunculus, or by a broad base. Intestinal Polyp,Polyp, Intestinal,Polyps, Intestinal
D009455 Neurofibroma A moderately firm, benign, encapsulated tumor resulting from proliferation of SCHWANN CELLS and FIBROBLASTS that includes portions of nerve fibers. The tumors usually develop along peripheral or cranial nerves and are a central feature of NEUROFIBROMATOSIS 1, where they may occur intracranially or involve spinal roots. Pathologic features include fusiform enlargement of the involved nerve. Microscopic examination reveals a disorganized and loose cellular pattern with elongated nuclei intermixed with fibrous strands. (From Adams et al., Principles of Neurology, 6th ed, p1016) Neurofibromas
D010016 Osteoma A benign tumor composed of bone tissue or a hard tumor of bonelike structure developing on a bone (homoplastic osteoma) or on other structures (heteroplastic osteoma). (From Dorland, 27th ed) Osteomas
D010580 Peutz-Jeghers Syndrome A hereditary disease caused by autosomal dominant mutations involving CHROMOSOME 19. It is characterized by the presence of INTESTINAL POLYPS, consistently in the JEJUNUM, and mucocutaneous pigmentation with MELANIN spots of the lips, buccal MUCOSA, and digits. Lentiginosis, Perioral,Periorificial Lentiginosis Syndrome,Peutz-Jegher's Syndrome,Peutz-Jeghers Polyposis,Polyposis, Hamartomatous Intestinal,Polyps-and-Spots Syndrome,Hamartomatous Intestinal Polyposes,Hamartomatous Intestinal Polyposis,Intestinal Polyposes, Hamartomatous,Intestinal Polyposis, Hamartomatous,Lentiginoses, Perioral,Perioral Lentiginoses,Perioral Lentiginosis,Periorificial Lentiginosis Syndromes,Peutz Jegher's Syndrome,Peutz Jeghers Polyposis,Peutz Jeghers Syndrome,Peutz-Jegher Syndrome,Polyposes, Hamartomatous Intestinal,Polyposis, Peutz-Jeghers,Polyps and Spots Syndrome,Polyps-and-Spots Syndromes,Syndrome, Periorificial Lentiginosis,Syndrome, Peutz-Jegher's,Syndrome, Peutz-Jeghers,Syndrome, Polyps-and-Spots,Syndromes, Periorificial Lentiginosis,Syndromes, Polyps-and-Spots
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000505 Alopecia Absence of hair from areas where it is normally present. Alopecia, Androgenetic,Baldness,Male Pattern Baldness,Pseudopelade,Alopecia Cicatrisata,Alopecia, Male Pattern,Androgenetic Alopecia,Androgenic Alopecia,Baldness, Male Pattern,Female Pattern Baldness,Hair Loss,Pattern Baldness,Alopecia Cicatrisatas,Alopecia, Androgenic,Alopecias, Androgenic,Androgenic Alopecias,Baldness, Female Pattern,Baldness, Pattern,Hair Losses,Loss, Hair,Losses, Hair,Male Pattern Alopecia
D012877 Skin Manifestations Dermatologic disorders attendant upon non-dermatologic disease or injury. Manifestation, Skin,Manifestations, Skin,Skin Manifestation
D012880 Skin Pigmentation Coloration of the skin. Skin Color,Skin Tone,Color, Skin,Colors, Skin,Pigmentation, Skin,Pigmentations, Skin,Skin Colors,Skin Pigmentations,Skin Tones,Tone, Skin,Tones, Skin
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

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