DOI: 10.36347/sjmcr.2013.v01i01.006

Pages: 15-17

Abstract: Olmsted Syndrome, OS, (congenital palmoplanter and periofical karetoderma) is a very rare condition and is associated with palmoplanter karetoderma and perioral hyperkaratotic plaques, deformation and loss of the flexibility of hands. On rare occasions it could lead to amputation of the severely affected digits or hands. The condition has also been associated with growth impairement and joint laxity and described to be inherited from the famility or effect patient’s sporadicaly. Specific types of skin lesions are formed with OS. New types of leasions and plaques are still being identified with new patients. In our sunjest we have seen depletion of IgG2 levels (immune deficiency) severe pneumonia and empyema during skin deformation. Although all reported cases have been described to have genetic links or appear sporadicly, our findings show that the depletion of the immune responses should also be considered and not ignored when identifying and diagnosing the condition.