SHORT COMMUNICATION
801
ActaDV ActaDV
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