A case of disproportionate macrodactyly or a mild form of Proteus syndrome? An interesting case.
(19/31)
We present a 20-year-old Malay male whom we believe has Proteus syndrome, a rare congenital disorder of asymmetrical overgrowth of body tissues. There are fewer than 100 confirmed cases reported worldwide thus the clinical presentation and histopathological findings are of significance. Our patient presented with an overgrown right small finger and subcutaneous purplish pigmentation over his left upper arm and chest since birth. His small finger gradually increased in size. He had no abnormalities in sensation or power. Radiographs revealed a delta shaped middle phalanx of the small finger. His activities of daily living were uninterrupted but he requested debulking surgery for cosmetic reasons. Histopathological examination reported hypertrophic fatty tissue composed of well formed lobules of mature adipocytes interspersed with fibrous elements. (+info)
An unusual initial presentation of Proteus syndrome: macrosyndactyly.
(20/31)
Proteus syndrome is a complex disorder characterized by a wide variety of deformities including macrodactyly. In the present report, we present a case with complex macrosyndactyly in his hand. The patient was surgically treated. However, he admitted with lymphangiomas on his body during the follow-up period, leading to the diagnosis of Proteus syndrome. This article discusses the differential diagnosis of Proteus syndrome and the treatment methods used for macrodactyly along with the presentation of the case. (+info)
Macrodactyly with skin hypertrophy: a minimal form of the Proteus syndrome.
(21/31)
Chronic rectal bleeding in Proteus syndrome.
(23/31)
Proteus Syndrome is a rare congenital hamartomatous disorder that typically manifests itself in overgrowth, vascular malformation and disregulation of fatty tissue. The tissues affected are commonly the limbs but can be of any tissue. Vascular anomalies are common and appear at random sites on the body. Diagnosis is often difficult leading to wrong treatment. We describe a case of a 17-year-old girl with Proteus syndrome presented with symptomatic anaemia secondary to chronic rectal bleeding. Computed Tomography Angiogram of Abdomen and Pelvis confirmed the presence of rectal vascular malformations. (+info)