McCune-Albright syndrome: a case report in a male. 2010

Krina B Patel
Department of Dermatology, Smt. S.C.L. Hospital, Smt. NHL Municipal Medical College, Ahmedabad, India. y2k_kbpatel@yahoo.com

McCune-Albright syndrome (MAS) is a rare, heterogenous, clinical condition caused by a rare genetic mutation. The disorder is more common in females and is characterized by a triad of cutaneous, bone and endocrine abnormalities. We describe a male patient with MAS having multiple café-au-lait macules and deforming polyostotic fibrous dysplasia involving long bones of the limbs, skull and spine without any endocrine abnormality. Severe bone deformities involving almost all bones have not been described previously and this prompted us to present the current case.

UI MeSH Term Description Entries
D007866 Leg The inferior part of the lower extremity between the KNEE and the ANKLE. Legs
D008297 Male Males
D011859 Radiography Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film). Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D005359 Fibrous Dysplasia, Polyostotic FIBROUS DYSPLASIA OF BONE affecting several bones. When melanotic pigmentation (CAFE-AU-LAIT SPOTS) and multiple endocrine hyperfunction are additionally associated it is referred to as Albright syndrome. Albright's Syndrome,McCune-Albright Syndrome,Albright Syndrome,Albright's Disease,Albright's Disease of Bone,Albright's Syndrome with Precocious Puberty,Albright-Mccune-Sternberg Syndrome,Albright-Sternberg Syndrome,Fibrous Dysplasia with Pigmentary Skin Changes and Precocious Puberty,Polyostotic Fibrous Dysplasia,Dysplasia, Polyostotic Fibrous,Dysplasias, Polyostotic Fibrous,Fibrous Dysplasias, Polyostotic,McCune Albright Syndrome,Polyostotic Fibrous Dysplasias,Syndrome, Albright,Syndrome, Albright's,Syndrome, McCune-Albright
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001132 Arm The superior part of the upper extremity between the SHOULDER and the ELBOW. Brachium,Upper Arm,Arm, Upper,Arms,Arms, Upper,Brachiums,Upper Arms
D012886 Skull The SKELETON of the HEAD including the FACIAL BONES and the bones enclosing the BRAIN. Calvaria,Cranium,Calvarium,Skulls
D019080 Cafe-au-Lait Spots Light brown pigmented macules associated with NEUROFIBROMATOSIS and Albright's syndrome (see FIBROUS DYSPLASIA, POLYOSTOTIC). Cafe au Lait Spots,Cafe-au-Lait Spot,Spot, Cafe-au-Lait,Spots, Cafe-au-Lait

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