Primary cutaneous CD30+ T-cell lymphoproliferative disease

Primary cutaneous CD30+ T-cell lymphoproliferative disease (also known as primary cutaneous CD30-positive T-cell lymphoproliferative disorder) is a spectrum of related conditions that represent the second most common group of cutaneous T-cell lymphomas. This spectrum primarily includes lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (pcALCL), with borderline cases that may not fit neatly into either category. These conditions are characterized by the proliferation of T-lymphocytes that express the CD30 antigen (also known as Ki-1) in the skin. Lymphomatoid papulosis typically presents as recurrent, self-healing papular or nodular skin lesions that may ulcerate and resolve spontaneously over weeks to months, often leaving small scars. Primary cutaneous anaplastic large cell lymphoma usually presents as solitary or localized firm, often ulcerated nodules or tumors on the skin. Both conditions primarily affect the skin, though pcALCL can occasionally involve regional lymph nodes. Despite their histological appearance, which may resemble aggressive lymphomas, these conditions generally follow an indolent clinical course with an excellent prognosis, particularly lymphomatoid papulosis, which has a 10-year disease-specific survival rate exceeding 90%. However, patients with LyP have a small but notable risk of developing secondary lymphoid malignancies, including mycosis fungoides, pcALCL, or systemic anaplastic large cell lymphoma. Treatment depends on the specific subtype and extent of disease. For lymphomatoid papulosis, options include observation (watchful waiting), topical therapies such as corticosteroids or nitrogen mustard, low-dose methotrexate, phototherapy (PUVA), or in some cases, bexarotene. For primary cutaneous anaplastic large cell lymphoma, surgical excision or local radiation therapy is often effective for solitary or localized lesions. Multiagent chemotherapy is generally reserved for cases with extracutaneous spread. The targeted antibody-drug conjugate brentuximab vedotin, which targets CD30, has shown efficacy in refractory cases of both conditions.

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