Abstract
Primary Cutaneous Peripheral T-Cell Lymphoma NOS (PTL-NOS) is a rare, progressive, fatal dermatologic disease that presents with features similar to many common benign plaque-like skin conditions, making recognition of its distinguishing features critical for early diagnosis and treatment (Bolognia et al., 2008). A 78-year-old woman presented to ambulatory care with a single 5 cm nodule on her shoulder that had developed rapidly over 1-2 weeks. Examination was suspicious for malignancy and a biopsy was performed. Biopsy results demonstrated CD4 positivity, consistent with Mycosis Fungoides with coexpression of CD5, CD47, and CD7. Within three months her cancer had progressed into diffuse lesions spanning her entire body. As rapid progression is usually uncharacteristic of Mycosis Fungoides, her diagnosis was amended to PTL-NOS. Cutaneous T-Cell Lymphoma (CTCL) should be suspected in patients with patches, plaques, erythroderma, or papules that persist or multiply despite conservative treatment. Singular biopsies are often nondiagnostic, requiring a high degree of suspicion if there is deviation from the anticipated clinical course. Multiple biopsies are often necessary to make the diagnosis. Physicians caring for patients with rapidly progressive, nonspecific dermatoses with features described above should keep more uncommon forms of CTCL in mind and refer for early biopsy.
Highlights
Primary Cutaneous Peripheral T-Cell Lymphoma not otherwise specified (PTL-NOS) is an aggressive and life threatening dermatologic malignancy that often presents mimicking other less severe plaque-like skin conditions
Due to the nonspecific nature of these lesions, CD4-positive Cutaneous T-Cell Lymphoma (CTCL) is often misdiagnosed as either Mycosis Fungoides or Sezary Syndrome
We describe a patient who presented with a single malignant nodule which rapidly progressed provoking diagnosis with the rarest form of CTCL, known as PTL-NOS
Summary
Primary Cutaneous Peripheral T-Cell Lymphoma not otherwise specified (PTL-NOS) is an aggressive and life threatening dermatologic malignancy that often presents mimicking other less severe plaque-like skin conditions. Due to the nonspecific nature of these lesions, CD4-positive CTCL is often misdiagnosed as either Mycosis Fungoides or Sezary Syndrome It is not until the disease progresses that further investigation into alternative diagnoses is prompted. We describe a patient who presented with a single malignant nodule which rapidly progressed provoking diagnosis with the rarest form of CTCL, known as PTL-NOS. Several nodules became ulcerated and productive of purulent fluid (Figure 4) She developed a lesion suspicious for metastasis in her spleen that was seen on CT scan. After recognizing the aggressive nature of her disease, her diagnosis was amended to Primary Cutaneous Peripheral T-Cell Lymphoma, NOS. She was started on combination Cyclophosphamide, Hydroxydaunomycin (Doxorubicin), Oncovin (Vincristine), and Prednisone. She eventually succumbed to sepsis due to skin barrier breakdown six months after diagnosis
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