PRIMARY CUTANEOUS HODGKINS-DISEASE - UNIQUE CLINICAL, MORPHOLOGIC, AND IMMUNOPHENOTYPIC FINDINGS

The authors studied five patients with primary cutaneous Hodgkin's dis ease (PCHD). Each patient presented with skin lesions without evidence of systemic HD. Skin lesions were papules or nodules, many of which r egressed spontaneously. Lesions were distinguished from lymphomatoid p apulosis (LyP) by the presence of numerous diagnostic Reed-Sternberg ( RS) cells that expressed CD30 and CD15 but were negative for CD45R; Ly P lesions usually are CD15 -, CD45R +. Anaplastic large cell lymphoma (ALCL) was excluded by the polymorphous background of inflammatory cel ls in PCHD. Three patients with PCHD had a benign course without syste mic disease with up to 20 years of follow-up, whereas two other patien ts developed mixed-cellularity HD in lymph nodes 2 months and 6 years following the onset of PCHD. This study indicates that PCHD does occur as a rare but distinct clinicopathologic entity morphologically and i mmunophenotypically indistinguishable from nodal HD but with an unexpe ctedly indolent course in some patients. Patients with PCHD should be observed for development of systemic HD, but unlike patients with LyP or ALCL, an association of PCHD with mycosis fungoides or cutaneous T- cell lymphoma has not yet been observed.