M. Beylotbarry et al., LICHENOID PARANEOPLASTIC PEMPHIGUS ASSOCIATED WITH A LOW-GRADE NODAL LYMPHOMA, Annales de dermatologie et de venereologie, 125(10), 1998, pp. 720-723
Background. The precise diagnostic criteria for paraneoplastic pemphig
us defined by Anhalt are not always present. In many cases, the clinic
al and histological features are misleading. Case report. We observed
a case of paraneoplastic pemphigus in a 60-year-old mon followed for l
ow-grade inactive nodal lymphoma. The clinical course was long. The ma
in features were lichenoid cutaneous and mucosal lesions without bulla
e. The only histology finding was basal vacuolization, lymphocyte infi
ltration and suspended keratinocyte necrosis without acantholysis. Thi
s clinical and pathological situation continued for one year. Just a f
ew days prior to the patient's death, the clinical situation suddenly
worsened with major bullae involving most of the skin surface and muco
sal areas including the esophagus and respiratory tract. Cyclosporine
and bolus injections of corticosteroids did not prevent the fatal outc
ome. Discussion. This unusual lichenoid presentation has not been prev
iously described. Associated skin erosions usually occur early in the
disease course. The long lichenoid presentation for several months wit
hout acantholysis emphasizes the difficulties encountered in the diagn
osis of paraneoplastic pemphigus. This diagnosis should be entertained
in cancer patients who develop lichenoid lesions.