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Published on: October 14, 2021
Primary cutaneous marginal zone lymphoma presenting with secondary chilblains of the toes
Ali Zagham Nasir1, Ryan Scott Burmeister2
1Internal Medicine Residency, Trinity Health Saint Mary's - Grand Rapids, Grand Rapids, Michigan, USA ali.nasir@trinity-health.org.
Insights
Primary cutaneous marginal zone lymphoma (PCMZL) can manifest as chilblains. Early diagnosis via skin biopsy and treatment with rituximab can lead to resolution of these refractory dermatological symptoms.
Area of Science:
- Dermatology
- Hematology
- Oncology
Background:
- Cutaneous lymphoproliferative disorders encompass systemic lymphoma manifestations and primary cutaneous lymphomas.
- Primary cutaneous B-cell lymphomas are classified into four types, including primary cutaneous marginal zone lymphoma (PCMZL).
- Accurate diagnosis and staging are crucial for effective treatment of these conditions.
Observation:
- A case of PCMZL presented atypically as secondary chilblains on the toes.
- Diagnosis was confirmed through a punch skin biopsy.
- The patient's chilblains resolved completely following treatment with rituximab.
Findings:
- This case highlights PCMZL as a potential cause of refractory chilblains.
- Skin biopsy is essential for diagnosing cutaneous lymphoproliferative disorders.
- Rituximab demonstrated efficacy in treating this manifestation of PCMZL.
Implications:
- Clinicians should consider PCMZL in the differential diagnosis of patients with persistent chilblains or skin lesions.
- Early recognition and appropriate biopsy can lead to timely diagnosis of primary cutaneous lymphomas.
- This case broadens the understanding of PCMZL's clinical presentations and treatment responses.
Abstract:
Cutaneous lymphoproliferative disorders include cutaneous manifestations of systemic B-cell or T-cell lymphoma and primary cutaneous lymphomas. Primary cutaneous B-cell lymphomas are subcategorised into four groups: primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle centre lymphoma, primary cutaneous diffuse large B-cell lymphoma, leg-type and EBV-positive mucocutaneous tumour. Each of these cutaneous lymphoproliferative disorders has unique clinical and pathological features necessitating biopsy and staging to establish proper treatment. We present a case of PCMZL manifesting as secondary chilblains of the toes that was diagnosed with punch skin biopsy. The patient's chilblains resolved with rituximab. This case underscores the importance of considering PCMZL and other cutaneous lymphoproliferative disorders in patients with dermatological manifestations and wounds refractory to first-line treatment.
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