What in the world is proteus disease and where did it come from?
Background Proteus syndrome is a rare condition that can be loosely categorized as a hamartomatous disorder. It is a complex disorder with multisystem involvement and great clinical variability. Once thought to have neurofibromatosis, Joseph Merrick (also known as “the elephant man”) is now, in retrospect, thought by clinical experts to actually have had Proteus syndrome. This condition is characterized by a variety of cutaneous and subcutaneous lesions including vascular malformations, lipomas, hyperpigmentation, and several types of nevi. Partial gigantism with limb or digital overgrowth is pathognomonic with an unusual body habitus and, often, cerebriform thickening of the soles of the feet. Because cutaneous lesions tend to appear over time, the diagnosis may be delayed until late infancy, childhood, or even adulthood. Orthopedic complications often pose the most challenging medical problems, although vascular complications also contribute to overall morbidity. Severe disfigurement
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- What in the world is proteus disease and where did it come from?
