Background A retrospective clinical, histologic, and immunohistochemic
al study was performed in 37 cases of isolated primary cutaneous lymph
oma (PCL) (22 B and 15 T phenotype). Patients with epidermotrophic inf
iltrate (mycosis fungoides and Sezary syndrome) were excluded. Methods
Patients with PCL were selected according to strict criteria: isolate
d cutaneous involvement for at least 6 months and a negative exhaustiv
e study of possible spread. Lesions were either limited to a single cu
taneous region or were disseminated, involving at least two nonadjacen
t regions. The diagnosis was confirmed histologically, and an immunohi
stochemical study was performed. Results On the basis of the new Wille
mze classification for prognostic criteria, this study showed similari
ties between lymphomas of B and T phenotype in clinical features, ther
apeutic response, course, and overall prognosis. The clinical lesion w
as usually an erythematous nodule associated, or not, with an infiltra
ted layer and generally limited to a single cutaneous region. PCLs wer
e highly sensitive to nonaggressive treatment, showing complete or mor
e than 50% partial remission in all cases. Conclusions The overall pro
gnosis for these lymphomas was good, even for disseminated cutaneous f
orms. Patient survival at 48 months was 78% for T and 89% for B phenot
ype. In the latter group, the prognosis was comparable for CD30+ and C
D30- T lymphomas; however, the course of PCL involved frequent cutaneo
us relapses, particularly with the disseminated forms, raising the pro
blem of adjuvant treatment after complete remission was obtained. Extr
acutaneous involvement was rare, but always indicative of poor prognos
is.