Ankylosing spondylitis (AS) is a systemic disease primarily characterized by the inflammation of sacroiliac joints and axial skeleton. Neurofibromatosis type 1 (NF1) is a multisystem genetic disease which is characterized by cutaneous findings, most importantly café-au-lait spots and axillary freckling, by skeletal dysplasia, and by the growth of both benign and malignant nervous system neoplas...

Type I Neurofibromatosis (NF1) is a dominant autosomal disease. Its prognosis is related to the development of tumors that may evolve to malignancy. It is characterized by multiple café-au-lait spots, skeletal defects, optical gliomas, Lisch nodules and neurofibromas. Plexiform neurofibromas (PN) are one of the many possible findings on patients affected by the disease. This paper aims at repor...

Neurofibromatosis type 1 (NF1) and AIDS are risk factors for the development of malignant neoplasms, including hematological malignancies, such as non-Hodgkin lymphoma. NF1 is an autosomal dominant disease that primarily manifests as café-au-lait spots, dermal neurofibromas, axillary and/or inguinal ephelides or freckles, plexiform neurofibromas, Lisch nodules, and bone deformities. In this rep...

We report a case of sudden death in a 28-year-old male diagnosed with neurofibromatosis at the age of 6 years. The patient had multiple café-au-lait spots which, although being benign, were an ominous sign in this context. The immediate cause of death was intrathoracic neurofibroma causing compression that led to fracture of the thoracic vertebrae and laceration of the aorta with massive hemoth...

McCune Albright syndrome is diagnosed by a constellation of Café-au-lait spots, multiostosis fibrous dysplasia and precocious puberty. Other endocrine dysfunctions which are often associated are growth hormone excess, hyperthyroidism, Cushings syndrome, hyperprolactinemia and phosphate wasting. Its prevalence is 1:100000–1:1000000 in different ethnicity [1]. It is a rare cause of precocious pub...

A 13-year-old girl presented with features of intestinal obstruction. At surgery, the terminal 25 cm of ileum, which was resected along with the right colon, showed plexiform neurofibromatosis of the serosa and mesentery, hyperplastic submucosal and myenteric nerve plexuses and proliferation of neural tissue in the lamina propria, which manifested as diffuse polyposis of the ileal mucosa. The p...

background: mccune albright syndrome is rare with an estimated prevalence of 1 in 100,000 to 1 in 1,000,000 persons. the classical clinical triad consists of fibrous dysplasia of the bone, café-au-lait skin spots and precocious puberty. however, in rare cases, there may be primary hypogonadism and amenorrhea. case presentation: an eighteen-year-old female presented with amenorrhea. she had a sh...

The syndrome of McCune-Albright syndrome (MAS) is clasically defined as a triad presentation with the findings of polyostotic fibrous dysplasia, café-au-lait spots, and sexual precocity. However, not all patients present with complete symptoms. A 52-year-old man was diagnosed as having a variant of McCune-Albright syndrome with the following findings: polyostotic fibrous dysplasia, acromegaly d...

McCune-Albright syndrome is characterized by the triad café-au-lait cutaneous spots, polyostotic fibrous dysplasia and endocrinopathies. This article presents two cases of McCune-Albright syndrome in a middle-aged woman and a young girl. Both patients presented café-au-lait spots on the face and other parts of the body and expansion of the mandible with radiopaque-radiolucent areas with ground-...

McCune-Albright Syndrome is a rare syndrome characterized with excessive function of peripheral endocrine organs and activating mutations of the stimulatory G protein alpha subunit are involved in the pathogenesis. The three main findings of the disease include hyperpigmented café au lait spots, fibrous dysplasia and increased endocrine functions and excessive secretion of growth hormone is obs...