Primary cutaneous marginal zone B-cell lymphoma

Definition

This type of extranodal lymphoma is classified among the extra-nodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. It is probably the most common type of cutaneous B-cell lymphoma¹.

Aetiology

Infection with Borrelia burgdorferi, the spirochete responsible for Lyme disease, appears to be associated with European cases^2,3, with penicillin therapy curing some cases of B. burgdorferi-associated cutaneous B-cell lymphomas^4,5. B. burgdorferi infection in cutaneous lymphomas appears rare in the USA⁶ and Asia¹.

Clinical features

This lymphoma occurs in adults with a wide age-range. The head and neck regions are commonly involved.

Macroscopic appearances

The lesions are erythematous and may form plaques, papules or nodules.

Histopathology

The dermis is the principal site of involvement, with frequent involvement of the upper subcutis. The epidermis is not infiltrated. As with other marginal zone lymphomas, there is a proliferation of marginal cells with centrocyte-like morphology or monocytoid B-cells with plasmacytic differentiation. Reactive follicles are common. Occasionally, there may be infiltration of skin adnexa to form lymphoepithelial lesions. An infiltrate of eosinophils may be present.

Immunohistochemistry

• Nuclear expression of bcl-10 was seen in 11 of 24 cases¹. Nuclear expression of bcl-10 correlated with a nodular macroscopic morphology¹.

Molecular genetics

The API2-MALT1 fusion gene was not detected in any of 24 cases¹. (The frequency of the API2-MALT1 fusion gene seems to vary with the anatomic site of MALT lymphomas.)

Management

Localised lesions have been successfully excised with complete remission¹.

Prognosis

The clinical course is usually favorable. Spontaneous regression has been reported¹.

References

¹ Li, C., H. Inagaki, et al. (2003). "Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathologic study of 24 asian cases." Am J Surg Pathol 27(8): 1061-9.

² Cerroni, L., N. Zochling, et al. (1997). "Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma." J Cutan Pathol 24(8): 457-61.

³ Goodlad, J. R., M. M. Davidson, et al. (2000). "Primary cutaneous B-cell lymphoma and Borrelia burgdorferi infection in patients from the Highlands of Scotland." Am J Surg Pathol 24(9): 1279-85.

⁴ Roggero, E., E. Zucca, et al. (2000). "Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin." Hum Pathol 31(2): 263-8.

⁵ Slater, D. N. (2001). "Borrelia burgdorferi-associated primary cutaneous B-cell lymphoma." Histopathology 38(1): 73-7.

⁶ Wood, G. S., N. V. Kamath, et al. (2001). "Absence of Borrelia burgdorferi DNA in cutaneous B-cell lymphomas from the United States." J Cutan Pathol 28(10): 502-7.

⁷ Campo E, Chott A, Kinney MC, et al. Update on extranodal lymphomas. Conclusions of the Workshop held by the EAHP and the SH in Thessaloniki, Greece. Histopathology 2006; 48:481-504 FULL TEXT

This page last revised 28.4.2006.

©SMUHT/PW Bishop