Pink plaque on the ear
A 65-year-old woman presented to Dermatology with a soft, vascular-appearing, tender, and painful pink plaque on her left proximal helix. Dermoscopy revealed a salmon-colored background and nonspecific vascular network.1 The 2-cm plaque had first appeared 1 month earlier. Her primary care physician had previously attempted a biopsy, but it was aborted due to significant bleeding. The patient had a history of stage III marginal zone lymphoma with stable pulmonary tumors that remained unchanged after 4 cycles of rituximab more than a year earlier.
What’s your diagnosis?
A 4-mm punch biopsy was performed and revealed B-cell lymphoma, consistent with extranodal marginal zone lymphoma. The plaque was palpated carefully and the location of branches of the superficial temporal artery, which usually course anterior to the helix, were mapped, and avoided as a biopsy site.
Marginal zone lymphoma is a relatively indolent B-cell lymphoma that occurs in adults in mucosal associated lymphoid tissue, most often in the gastrointestinal (GI) tract. Neoplasms also occur in the lungs, eyes, and skin. Initial symptoms vary according to the site of manifestation. Patients with GI tumors may present with GI bleeding, abdominal pain, and weight loss. Pulmonary lesions are often asymptomatic and picked up on chest imaging for other indications. Chronic gastritis associated with Helicobacter pylori contributes to cases and occasionally eradication of H. pylori may clear patients of disease. Autoimmune diseases, particularly Sjögren disease and Hashimoto thyroiditis, also have a causative association. Rarely, transformation into high-grade disease occurs.
On the skin, marginal zone lymphoma may be exhibited as soft salmon-colored patches with serpentine vascular markings, as seen here on dermoscopy.1 The differential diagnosis includes keloid scar, basal cell carcinoma, Merkel cell carcinoma, arthropod bites, and amelanotic melanoma.
This patient had been under the care of Medical Oncology for surveillance and was offered observation as a potential treatment strategy because of the relatively indolent nature of the tumor. However, because the tumor was painful and growing, she opted for focal palliative radiation therapy.
Text courtesy of Jonathan Karnes, MD, medical director, MDFMR Dermatology Services, Augusta, ME. Photos courtesy of Jonathan Karnes, MD (copyright retained).