Skin involvement with Hodgkin disease: A rare condition late in the course of Hodgkin lymphoma
Authors: Garrett C. Lowe, Thomas L. Hocker, Nneka I. Comfere, Julia S. Lehman
Journal: American Journal of Hematology (2013)

Cutaneous Hodgkin disease typically presents late in the course of advanced lymphoma as papules, plaques, and nodules with or without ulceration. Incidence of cutaneous disease is decreasing, likely owing to improved treatment of patients with chemotherapy, radiation, and peripheral blood stem cell transplantation 1, 2. Recently, Introcaso et al. 3 reported a patient with skin involvement in Hodgkin lymphoma following nonmyeloablative allogenic stem cell transplantation for recurrent systemic disease. We now present the second case of cutaneous involvement by Hodgkin disease following stem cell transplantation. A 27-year-old otherwise healthy male presented in 2007 with pruritus and left axillary adenopathy. Shortly thereafter, he was diagnosed with classical Hodgkin lymphoma, stage IIA 4. His course was complicated by recurrences and disease progression despite cycles of Adriamycin, Bleomycin, Vinblastine, and Dacarbazine (ABVD) with adjuvant radiation, salvage Ifosfamide, Carboplatin, and Etoposide (ICE), autologous stem cell transplant, nonmyeloablative allogeneic sibling stem cell transplantation with subsequent acute graft versus host disease (GVHD), and clinical trial of sorafenib and everolimus. The cutaneous manifestations of the patient's Hodgkin disease were first noticed by the patient following lymph node biopsy from the right axilla in May 2011. He described two stable, asymptomatic subcutaneous nodules with increasing skin discoloration (Image 1) and was referred to dermatology. The presence of a mixed dermal infiltrate with large, binucleate tumor cells with prominent nucleoli, and a CD15 + CD30 + immunophenotype (Reed–Sternberg cells) on skin biopsy confirmed the clinical suspicion for cutaneous involvement by Hodgkin lymphoma (Image 1). Pathologic examination of skin specimen has proven helpful in differentiating cutaneous Hodgkin lymphoma from other entities, including infection, graft-versus-host disease, nonspecific skin conditions that often accompany Hodgkin lymphoma (such as pruritus, acquired ichthyosis, hyperpigmentation, urticaria, and erythroderma), and other lymphoid proliferative disorders associated with systemic lymphoma (such as mycosis fungoides, lymphomatoid papulosis, anaplastic large cell lymphoma, and granulomatous slack skin disease). In 1995, Cerroni et al. 5 summarized the immunohistochemical features of cutaneous Hodgkin's lymphoma. Binucleate tumor cells with prominent nucleoli (Reed–Sternberg cells) that are immunoreactive for CD30 and CD15 but negative for T- and B-lymphocyte markers are characteristic. Three mechanisms have been proposed to explain the cutaneous involvement in Hodgkin lymphoma: retrograde lymphatogenous spread from proximal lymph nodes, direct extension from involved lymph nodes, and hematogenous dissemination 3, 6. Our case likely represents retrograde spread from proximal lymph nodes. The patient was started on single-agent brentuximab in November 2011, and the cutaneous nodules in the right axilla resolved after two cycles. Follow-up Positron Emission Tomography (PET) scan after his third cycle of brentuximab is planned.