A Primary Systemic ALCL Present Initially as Cutaneous Localized Skin Lesions

Abstract

Anaplastic large cell lymphoma (ALCL) is classified into systemic and primary cutaneous subtypes. A primary systemic ALCL present initially as cutaneous localized skin lesions is a rare presentation. We present an unusual case of a 13-year-old boy with systemic ALCL who presented with cutaneous nodules. The systemic nature of this lymphoma was confirmed by immunohistochemical positivity of anaplastic lymphoma kinase and epithelial membrane antigen. Confirmation of this diagnosis will lead to a poorer prognosis and more aggressive systemic therapy.

Primary Cutaneous Anaplastic Large Cell Lymphoma With Positive ALK Expression and a Rapidly Progressive Cutaneous Nodule

“…This case appeared to be an isolated skin disease. In contrast with the report that ALK expression should not occur with pcALCL, 2 it resembled pcALCL clinically and histologically, but expressed ALK fusion proteins. EMA is positive in most sALCLs, 10 but is negative in cutaneous ALCL, 11,12 but this pcALCL case had a patchy positive EMA result.…”

“…1 ALCL encompasses at least 3 clinically distinct entities (primary cutaneous ALCL (pcALCL), anaplastic lymphoma kinase (ALK)+ systemic ALCL (sALCL), and ALK− sALCL). 2,3 pcALCL presents in the skin with no evidence of extracutaneous disease. 4 ALCL is otherwise categorized as sALCL.…”

“…sALCL is usually positive for ALK and epithelial membrane antigen (EMA) expression; these are negative in pcALCL. 2 Unlike sALCL, pcALCL only rarely affects children and young adults. 4,5 This patient was a young adult with pcALCL with positive ALK, patchy EMA expression, and a large, rapidly growing, cutaneous nodule.…”

Anaplastic Large Cell Lymphoma Mimicking Cellulitis: A Case Report

“…ALCL is a T-cell non-Hodgkin lymphoma that is diagnosed on the basis of biopsy specimens positive for CD30 on the cell membrane and in the Golgi bodies. 3 ALCL has 4 subtypes, namely, primary systemic ALK-positive ALCL, primary systemic ALK-negative ALCL, primary cutaneous ALCL, and breast implant-associated ALCL. 4 The systemic The International Journal of Lower Extremity Wounds 18 (2) ALCL subtypes usually present with enlarged lymph nodes or extranodal tumors in multiple regions of the body, such as the skin, bone, intestine, muscle, liver, or spleen.…”

“…In addition, systemic ALCL presenting with skin lesion is rare. 3 In conclusion, in patients with cellulitis-like lesions, making a conclusive diagnosis of infection could be dangerous, especially when the conventional treatment does not work. Though rare, the possibility of malignancy should be considered.…”