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Advances in dermatology and venereology Acta Dermato-Venereologica
Remission of Cutaneous Involvement in Splenic Marginal Zone Lymphoma after Splenectomy
Shoko MAI 1 , Ken NATSUGA 1 *, Souichi SHIRATORI 2 , Yoshimasa TAKAHASHI 3 and Hiroshi SHIMIZU 1
1
Department of Dermatology and 2 Department of Hematology , Hokkaido University Graduate School of Medicine , North 15 West 7 , Sapporo 060-8638 , and 3 Kitago Dermatology Clinic , Sapporo , Japan . * E-mail : natsuga @ med . hokudai . ac . jp Accepted May 29 , 2018 ; Epub ahead of print Jun 1 , 2018
Marginal zone lymphoma ( MZL ) is an indolent neoplasm that originates from lymphocytes of the marginal zone . MZL is classified into 3 subtypes : extranodal MZL of mucosa-associated lymphatic tissue ( MALT ), splenic MZL ( SMZL ) and nodal MZL ( 1 ). Although skin involvement often occurs in MALT lymphoma , infiltration of SMZL to the skin is quite rare ( 2 ). We describe here a 70-year-old man with cutaneous involvement in SMZL , which disappeared after splenectomy .
CASE REPORT
A 70-year-old man presented with numerous plaques and papules on his trunk and limbs , which had developed over an approximately 4-month period ( Fig . 1A ). He reported having had no fever , night sweats , weight-loss or bleeding . Physical examination revealed erythematous to violaceous plaques and papules on his chest , abdomen , back , buttocks and extremities . Some of the eruptions were accompanied by ulceration or crust formation . The initial clinical diagnosis was pityriasis lichenoides et varioliformis acuta ( PLEVA ). Histopathological examination of biopsy specimens revealed dense , bandlike infiltrates of small monomorphic lymphocytes in the papillary dermis and reticular dermis , and patchy perivascular infiltration in the deep dermis ( Fig . 1B ) to the subcutaneous fat tissue ( not shown ). The dermoepidermal junction was blurred , and the monomorphic lymphocytes infiltrated the mid-epidermis ( epidermotropism ), resembling mycosis fungoides . The atypical cells were positive for CD20 and bcl-2 , but negative for CD3 , CD10 and cyclinD1 . Ki-67 positivity was approximately 30 %. The kappa ; lambda light chain ratio could not be evaluated due to poor labelling of either kappa or lambda . Eosinophils were not observed in the infiltrates in the specimens . The pathological diagnosis of the skin specimens was low-grade B-cell lymphoma . PCR analysis of immunoglobulin heavy-chain ( IgH ) gene rearrangement confirmed the monoclonality of the tumour cells ( VH-FR1 , VH-FR2 and VH-FR3 ). A complete blood count revealed thrombocytopaenia ( 62,000 / mm 3 ). Bone marrow biopsy specimens showed lymphoid aggregates , suggesting the involvement of lymphoma . Positron emission tomography – computed tomography ( PET-CT ) revealed massive splenomegaly ( Fig . 1C ). Due to the thrombocytopaenia and the possibility of splenic
Fig . 1 . ( A ) Scattered erythematous to violaceous plaques and papules on the chest and abdomen . ( B ) A skin biopsy specimen from the abdomen shows dense , band-like lymphocytic infiltrates in the epidermis and superficial dermis , as well as patchy perivascular infiltration in the deep dermis . ( Haematoxylin and eosin ( H & E ) × 40 ). ( C ) An axial fused positron emission tomography – computed tomography ( PET / CT ) image of the abdomen reveals marked splenomegaly . ( D , E ) Histological findings of spleen specimens . Predominant white pulp involvement by atypical cells ( H & E × 100 ) ( D ). ( E ) Tumour cells positive for CD20 . ( F ) The skin lesions have disappeared after the splenectomy , leaving pigmentation ( 45 days after the procedure ).
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2981 Acta Derm Venereol 2018 ; 98 : 801 – 802