El síndrome de Peutz-Jeghers es una condición familiar caracterizada por la presencia de manchas cutáneas hiperpigmentadas y poliposis intestinal. La invaginación intestinal es la complicación abdominal más frecuente, pero es muy rara. Éste es el segundo reporte que se comunica en nuestro país. En este artículo presentamos cuatro pacientes que sufrieron el síndrome, que se complicó con invagina...

IN 1921 Peutz described a family with intestinal polyposis and spots of melanin pigmentation in and around the mouth. Prior to this, in 1896, Sir Jonathan Hutchinson described two girls with pigmented patches in the oral mucosa and around the mouth. Later, Parkes Weber (1919) reported that one of these twins died of what appeared to be an intussusception. Since 1921 over one hundred cases have ...

We report a rare co-occurrence of an adenoma malignum and an adenocarcinoma in a 30-year-old woman with Peutz-Jeghers syndrome. The woman was diagnosed with Peutz-Jeghers syndrome based on an endoscopic biopsy after vaginal bleeding. A pelvic examination and an MRI revealed the co-occurrence of a 4×5 cm protruding adenocarcinoma of FIGO stage Ib2 based on a punch biopsy and a 4.5×5.7 cm multilo...

Laugier-Hunziker syndrome is a rare, hereditary pigmentary disorder characterized by mucocutaneous lentiginous lesions and melanonychia. The condition regarded as benign since underlying malignancies or systemic disorders were not reported. Since various malignancy syndromes including Peutz-Jeghers Cronkhite-Canada cause of hyperpigmentation such Addison disease drugs are presented with similar...

Peutz-Jeghers syndrome (PJS) is an unusual hamartomatous polyposis of the gastro intestinal (GI) tract, with pigmentation around lips and macules on the buccal mucosa. The case of a 10-year-old girl who presented with intussusception is reported. A polyp was found to be the cause of an invagination. Histologically it was a hamartoma. PJS is a rare syndrome inherited in an autosomal dominant pat...

Introduction: Infrequent and characterized by mucocutaneous pigmentations, gastrointestinal polyposis, an increased risk of malignancy, Peutz-Jeghers syndrome is autosomal dominant genetic condition. The usual perioral macules patches that are colored in the buccal mucosa present 90% patients, numerous but not continuously contiguous lesions, primarily (G.I.) tract, with rarely more than 20 ham...

Laugier-Hunziker syndrome (LHS) is a rare sporadic disorder, which shares some dermatological features with Peutz-Jeghers syndrome (PJS). However, whereas PJS is associated with hamartomatous gastrointestinal polyposis and carries a high risk of malignancy justifying intensive screening protocols, LHS is known to be an entirely benign disease with no systemic manifestations, which requires pati...

Cutaneous hyperpigmentation may be caused by a number of diseases, such as Addison’s disease, scleroderma, haemochromatosis, Peutz-Jeghers syndrome, lichen planus, and adverse reactions to some medications such as oral contraceptives, cyclophosphamide, chloroquine, minocycline, bleomycin, ketoconazole and methyldopa (1). Hyperpigmentation of the tongue, face and body skin is a rare adverse effe...