Dermatologic Syndromes

Paraneoplastic pemphigus is a severe dermatologic syndrome that is most commonly associated with NHL,111 but can also be seen in the course of CLL, multiple myeloma, Waldenstrom's macroglobulinemia, HD, and other solid tumors. It presents as painful mucocutaneous erosions of the lips, oropharynx, and skin. In paraneoplastic pemphigus, the antibodies target the plakin proteins of desmosomes that are found in all epithelial tissues, including the respiratory tract.112113 As a consequence, bronchiolitis obliterans with respiratory failure may develop in about 30% of these patients. Immunosuppressive therapy with cyclosporine and rituximab,114115 in some cases, has been associated with good responses to the skin and mucosal lesions, but does not seem to alter the eventual outcome, even when good control of the underlying neoplasm is achieved. Nousari et al.113 noted a mortality rate of greater than 90% in 84 patients with paraneoplastic pemphigus, the leading cause of death in the absence of respiratory failure being infection.114 Sweet's syndrome is a prototype of the neutrophilic dermatoses and is found more often with AML, but is also reported in NHL, HD, and a variety of other hema-tological malignancies.116117 It manifests with an acute onset of fever, leukocytosis, and cutaneous plaques which show dermal infiltration with neutrophils.

T-cell lymphoma and HD can rarely be associated with eosinophilic fasciitis, which presents with painful sclerotic lesions on the extremities and trunk.118-120 Treatment with corticosteroids has modest results, but as with any other paraneoplastic manifestation, control of the underlying malignancy is crucial. Other conditions that have been rarely associated with lymphoma are pyoderma gangrenosum,121 Bazex syndrome,122 granuloma annulare,123 and molluscum contagiosum.124

0 0

Post a comment