Small lymphocytic lymphoma with florid perniosis-like features: a case report

Abstract

Background: Small lymphocytic lymphoma is a relatively rare B-cell non-Hodgkin lymphoma that is considered to be the tissue equivalent of the much more common entity chronic lymphocytic leukemia. Cutaneous manifestations of small lymphocytic lymphoma are infrequent and the literature regarding them sparse. We describe here a case of a patient with a history of small lymphocytic lymphoma who developed perniosis-like features of the digits.

Case presentation: An 86-year old male patient with previously diagnosed small lymphocytic lymphoma developed painful erythematous swelling of the periungual area of his fingers and toes. This was associated with a dense dermal infiltration of CD5-positive B-lymphoid cells consistent with his low-grade B-cell lymphoma. Although partially refractory to local radiotherapy, the painful swelling of the fingers and toes resolved fully following systemic therapy with chlorambucil and rituximab.

Conclusions: This unusual cutaneous manifestation of a lymphoma and the favourable response to systemic therapy may be instructive for the management of other patients who develop similar perniosis-like features.

Fig. 1

Upper panel: Bilateral periungual swelling of the fingers. Lower panel: Images of the fingers after six cycles of chlorambucil and rituximab

Fig. 2

Micrograph prepared from periungual biopsy of right second finger. a, H&E x 40 - Low power view of micrograph prepared from periungual biopsy of right second finger, showing diffuse dermal infiltration by monomorphous lymphoid infiltrate, typical of SLL/CLL. b, H&E x 400 - Detail of uniform small lymphocytes with round nuclei, condensed chromatin and scanty cytoplasm. c, CD20 - The lesional cells are uniformly positive for CD20. d, CD5 - The CD20 + cells show aberrant weak positivity for CD5. Note the scattered T-cells that stain more strongly for CD5