Description, Causes and Risk Factors:
A rare nonfamilial disease characterized by enlargement of the fingers by lipomas, with painful degenerative arthropathy of the metacarpophalangeal and interphalangeal joints.
Macrodystrophia lipomatosa is a rare congenital anomaly characterized by an abnormal overgrowth of the mesenchymal elements resulting in gigantism of single or multiple digits or the entire limb. Sometimes it may have bilateral involvement of the limbs. Although there have been only few anecdotal reports of involvement of entire limb and even the abdominal wall involvement has been reported. The age of presentation is in consistent and the clinical findings can be recognized as early as in the neonatal period to late adulthood. Although there is no gender predilection, slight male preponderance is seen. The overgrowth appears to develop in a specific sclerotome region of the body. Generally, the lateral aspect of the upper limb (i.e. along the median nerve distribution) and the medial aspect of the lower limb (i.e. along the plantar nerve distribution) are affected. Distal limb involvement is seen predominantly. The lower limb is more often involved than the upper limb and the 2nd and 3rd digits are more commonly affected. The affected region continues to grow only until puberty at which time it reaches a plateau.
Exact etiology is not known. However there are many hypothesis suggesting that it is due to fibrofatty tumors impinging on nerves supplying the enlarged digit or due to alteration of somatic cells during limb bud development. It is now believed to be a part of generalized hamartomatous disorder known as proteus syndrome.
Macrodystrophia lipomatosa is a congenital anomaly but it is certainly not a hereditary disease. Various hypotheses have been proposed regarding the etiopathogenesis of ML. These include lipomatous degeneration, fetal circulation abnormality, and damage of extremity bud and errors in the segmentation in intrauterine life and hypertrophy of the concerned nerve. The gamut of nomenclature, as given by different authors, produces considerable confusion regarding the exact terminology and also difficulty in setting up the criteria for diagnosing the disease especially vis-
Researchers from the University of Louisville in Kentucky suggest that natural polyphenols found in pomegranate can fight symptoms of inflammatory bowel disease (IBD). Polyphenols are also present in berries such as strawberries, blackberries, and raspberries. The...
A recent study, published in the journal Nature Medicine, shows that microbiota, aka gut bacteria, play a crucial role in protecting humans against food allergies. During the study, two groups of mice without their own bacteria received gut bacteria from either...
In childhood, many of us dreamed of learning to jump high. Now, after years, it became easier - Kangoo Jumps has appeared. This is one of the relatively new, but quickly gaining popularity types of fitness training. There are several advantages of jumpers. ...read more
It is time for us to find the solution how to deal with extra weight which is coming with Christmas and New Year’s celebrations because 2018 is almost over and 2019 is getting closer. The main aim is obviously staying in shape and today will find an effective way to...read more
We know that moderate exercise strengthens our health and makes us more beautiful. But few know about the importance of doing sports as psychotherapy. And, nevertheless, physical loads can save from depressions, reduce anxiety, relieve from professional burnout,...read more