Rapidly Progressive Acute Pustular Secondary Cutaneous Anaplastic Large Cell Lymphoma

A 71-year-old man presented with a two-week history of papules and pustules on his chest and back associated with fever and cough. He denied any previous similar lesions and was in his usual state of health prior to these findings. The patient’s past medical history included systemic anaplastic large cell lymphoma (ALCL), low-grade B-cell non-Hodgkin lymphoma (NHL), prostate adenocarcinoma, and non-small cell lung cancer. He had recently undergone CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone) for NHL, 2 weeks prior to admission.

Physical examination revealed numerous friable, bleeding erythematous papules with collarettes of scale predominantly on the trunk (Figure 1), as well as multiple pustules on the chest (Figure 2). A punch biopsy was performed on a representative lesion (Figure 3A-B). Histopathologic analysis revealed a pan-dermal infiltrate of large, atypical lymphocytes, found on immunohistochemistry to be positive for CD3, CD4, CD8 (partial), CD30, and anaplastic lymphoma kinase-1 (ALK-1) and negative for CD7, consistent with cutaneous anaplastic large cell lymphoma (ALCL). Unfortunately, before chemotherapy could be initiated the patient’s condition deteriorated rapidly, and he passed away within a few days.

ALCL is a type of CD-30-expressing T-cell lymphoma that accounts for approximately 12% of all T-cell non-Hodgkin lymphomas. Cutaneous ALCL exists on a clinical and pathologic spectrum, making definitive diagnosis and management sometimes challenging, and one must differentiate from other CD-30 positive entities such as lymphomatoid papulosis, which is histologically malignant but clinically benign unless an associated lymphoproliferative disorder develops.¹ Cutaneous ALCL typically presents as erythematous to brown nodules, tumors, and sometimes papules which frequently ulcerate. However, in rare cases cutaneous ALCL may also include pustular lesions, which will be further discussed.^2,3 Histopathology reveals sheets of large anaplastic lymphoid cells with over 75% CD30 positivity, typically with CD4 co-expression.⁴

Cutaneous ALCL may exist as a primary condition or occur as a secondary process in approximately one-fifth of patients with systemic disease.⁵ Primary cutaneous ALCL has an excellent prognosis with a five-year disease-specific survival above 90%.⁶ However, extensive limb disease, CD30/CD56 co-expression, and elevated fascin levels may portend a more aggressive course,^6-8 and extracutaneous dissemination occurs in about 10% of patients with primary cutaneous disease.³ Treatment centers around local control of discrete lesions via surgery or radiotherapy, but systemic treatment with chemotherapeutic regimens such as CHOP should also be considered in multifocal or refractory cases.^9,10

On the other hand, secondary cutaneous ALCL tends to be more aggressive and carries an overall five-year survival under 50%, which is worse than that of systemic ALCL without cutaneous disease.¹¹ Expression of anaplastic lymphoma kinase (ALK) protein has been extensively utilized as a prognostic determinant of systemic ALCL, usually involving a t(2;5)(p23;q35) translocation resulting in a fusion gene product of receptor tyrosine kinase ALK with nucleophosmin.12 The seminal work by Shiota et al. demonstrated that while the five-year overall survival was approximately 67% for all patients with systemic ALCL, patients with ALK positivity had a 93% five-year survival