Untreated relapsing secondary syphilis with meningitis
1989
Another important zone is the superior extremity, whereas the inferior extremity is only affected in one case. 5 To our knowledge, no case of segmental neurofibromatosis has been reported with development of malignant schwannoma, pheochromocytoma, or neurofibrosarcoma, as occurs in classic neurofibromatosis. Moreover, none of the patients was neurologically affected. Only 15 cases of all segmental neurofibromaloses published may be called true segmental neurofibromatosis, following the strict definition of Riccardi. As occurs in classic neurofibromatosis, segmental neurofibromatosis may present great variability in its clinical expressions. For this reason, Roth et al? proposed classifying segmental neurofibromatosis into four different groups, in which the first group would only include the true cases of segmental neurofibromatosis. In the other groups, family history, bilateral disposition, deep soft tissue, or bone involvement can be found. 6 Our case presents some interesting details. It is the first case of segmental neurofibromatosis described in black African people. In addition, our patient had mental retardation; this association has been previously observed in one case. 4 Finally, we find the pain he had when any of his skin lesions were pressed a significant detail, and the fact that this pain irradiates to an ipsilateral paravertebral region of D12, L1-2 is interesting. This clinical sign, which would refer to an underlying neural affection in these zones and would explain its metameric arrangement, has not been previously described in the literature. Nevertheless, we find it necessary to have this noted and encourage its exploration in further eases.
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