Thursday, July 19, 2012

Regional Scleroderma (Sclérodermie Monomélique)

Regional scleroderma is a puzzling malady in that it is different from the usual systemic scleroderma, but has many common features.  It is usually younger in onset, has often a limb that it affects, it is predominant in females primarily and has a rapid development phase, rather than the more gradual systemic scleroderma.

The clues to this diagnosis lie in the hypoplastic breast and the scarred down appearance of the tissue of the RUE.  This differentiates it from, say, Becker's Nevus, in which the skin may appear bound down, but it does not have the hypoplasia associated with it.  Other variants of this condition are called regional morphea, variant pansclerotic morphea, and as above, in the Francophone world, Sclerodermie monomelique.  

There are some data that support a causative link between this condition and an acid-fast bacillus, but I would not be surprised if some of these are also associated with borreliosis.

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