The skin often signals systemic changes. Some neoplastic diseases that affect
internal organs may trigger several cutaneous manifestations. Although these
dermatoses are relatively unusual, the recognition of some typical paraneoplastic
dermatoses may lead to the early diagnosis of a neoplasm and determine a better
prognosis. In this review article, we discuss the paraneoplastic cutaneous
manifestations strongly associated with neoplasms, which include acanthosis nigricans
maligna, tripe palms, erythema gyratum repens, Bazex syndrome, acquired
hypertrichosis lanuginosa, necrolytic migratory erythema, Leser-Trélat sign and
paraneoplastic pemphigus. We also review the clinical manifestations of each
condition and include updated knowledge on disease pathogenesis.
Endemic pemphigus foliaceus (EPF), is an autoimmune disease associated with production of IgG antibodies against epidermal antigens. We have tested 38 patients and 50 control subjects living in endemic areas to investigate whether HLA genes are associated with host factors that determine whether or not exposed individuals will develop this disease. A variant of HLA-DR1, an antigen common in Blacks (DRB1*0102), was found to be the main susceptibility factor (relative risk = 7.3, P less than 0.0002). Two amino acids, in positions 85 and 86 of DRB1, distinguish DRB1*0102 from DRB1*0101. These residues appear to be involved in the formation of a functional epitope that causes T cell recognition and determines disease susceptibility. Moreover, subjects having DQw2 did not develop the disease, while the frequency of DQw2 in controls was 22% (RR = 0.04, P less than 0.006). Thus HLA genes appear to play a crucial role in the response to an environmental factor which in this setting frequently leads to the development of autoimmune disease. An HLA-DQ allele, DQw2, appears to be associated with factors that prevent the development of the disease in exposed individuals.
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