Long-term benefit of electron beam radiation therapy in the treatment of scleredema of Buschke

INTRODUCTION: Scleredema of Buschke is a rare connective tissue disorder presenting with woody thickening and induration of the nuchal and shoulder regions resulting in progressive decrease in the range of motion of the neck. Treatment options include several forms of systemic therapy with variable results. Local radiation therapy (RT) is often thought of as a secondary form of therapy. Few reports exist in the literature about the durability of its benefit, however. Here, we present a case report with the longest known follow-up after primary treatment with electron beam RT. METHODS: The patient was treated using 8-MeV en face electrons with 2000 cGy in 10 fractions with 2 separate but matched electron fields. The treatment fields included the posterior neck from the occiput superiorly to the mid-thoracic spine inferiorly with the lateral borders extending to the scapulae. The patient received no additional therapy either pre- or post-RT. Clinical follow-up was obtained at regular intervals. Published literature regarding RT for this disease was reviewed and consolidated. RESULTS: The patient was followed at regular intervals for 6 years with significant softening of the plaque starting at 2 months after RT, resulting in decrease in plaque size by 50% after 18 months. The patient regained 45° of lateral, bidirectional cervical motion from central axis and 50% improvement in neck extension that has remained durable 6 years after treatment with no additional therapy. Quality of life was restored with a simple nontoxic treatment limited to transient, grade 1 fatigue. CONCLUSION: Scleredema of Buschke is a rare connective tissue disorder commonly treated with multimodal therapy, but it can be effectively and durably controlled with RT alone. This case report documents the durability of the benefit achieved with RT and suggests that RT should be considered earlier in the treatment of this disease.