SHORT COMMUNICATION 665
ActaDV ActaDV
Advances in dermatology and venereology Acta Dermato-Venereologica
Cutaneous Gamma-delta T-cell Lymphoma with an Initially Indolent Course Mimicking Lupus Panniculitis
Johannes BENECKE 1 , Cyrill GÉRAUD 1 and Jan P . NICOLAY 1 , 2 *
1
Department of Dermatology , Venereology and Allergy , University Medical Center Mannheim , Ruprecht-Karls-University of Heidelberg , Theodor-Kutzer-Ufer 1-3 , DE-68167 Mannheim , and 2 Division of Immunogenetics , German Cancer Research Center , Heidelberg , Germany . E-mail : j . nicolay @ umm . de Accepted Feb 7 , 2017 ; Epub ahead of print Feb 8 , 2017
Primary cutaneous γ-δ T-cell lymphoma ( CGD-TCL ) is a rare disease characterized by a monoclonal proliferation of mature activated γ-δ T cells and an unfavourable prognosis . CGD-TCL represents approximately 1 % of CTCLs as well as an entity of its own in the WHO classification ( 1 ). Thus far , there is sparse literature on this entity . We report here an atypical case of CGD-TCL , which showed an indolent course for almost 2 years , thus mimicking lupus panniculitis , prior to a rapid progression to the typical clinical presentation .
CASE REPORT
A 68-year-old woman presented to our clinic with multiple indurated erythematous plaques and nodes on the right upper leg as well as one painful , immobile subcutaneous node on the left lower leg ( Fig . 1A ). These nodular lesions had developed over 9 months and had been pretreated with topical steroids with no success .
Haematoxylin and eosin ( H & E ) histology of a biopsy taken from the lesions revealed a dermal and subcutaneous infiltrate of atypical lymphocytes with mitoses , plasma cells and neutrophils as well as a vacuolar degeneration of basal cells . The pattern of panniculitis was predominantly lobular without rimming of adipocytes by atypical lymphocytes ( Fig . 2A ). Furthermore , histopathology showed a distinct dermal accumulation of mucin ( Fig . 2B ). Immunohistochemistry revealed CD3 expression of the pleomorphic lymphocytes , whereas CD79a and CD8 were negative ( Fig . 2C ). T-cell receptor ( TCR ) -γ , as well as TIA-1 , granzyme-B and perforin expression , was significant in the pleomorphic cells ( Fig . 2D ). A monoclonal TCR-γ gene rearrangement was identified . Anti-nuclear antibodies showed a titre of 1:320 , lactate dehydrogenase was slightly elevated with 318 U / l .
Based on these results it was initially difficult to distinguish between CGD-TCL and lupus panniculitis , whereas γ-δ-derived mycosis fungoides or lymphomatoid papulosis could be excluded .
Staging via computed tomography ( CT ) and magnetic resonance imaging ( MRI ) showed no suspect lesions within the organs and lymph nodes .
Given the discrepancy between the rather aggressive histological and largely indolent clinical course we favoured the diagnosis of lupus panniculitis and started treatment with topical corticosteroids and hydroxychloroquine . With this therapy , the lesions showed a stable clinical course with no improvement , and with no aggravation for more than one year . When the patient developed further lesions of the abdomen and the legs with rapid growth and central ulceration ( Fig . 1B ) more than one year later , however , the clinical picture matched the threatening histological findings . Thus we could clearly confirm the diagnosis of CGD-TCL . We subsequently started polychemotherapy following the CHOP regimen ( cyclophosphamide , doxorubicin , vincristine , and prednisone ). Before obtaining an allogeneic stem cell transplantation complete remission was achieved after 5 months of polychemotherapy .
The patient developed opportunistic infections and a graftversus-host disease of the gastrointestinal tract during the following months and died due to cardiac and renal failure following a haemolytic-uraemic syndrome .
DISCUSSION
In most cases CGD-TCL has a poor prognosis with a median survival time of 15 months ( 1 ). In contrast , benign lupus erythematosus panniculitis ( LEP ) has a prolonged clinical course with a favourable prognosis . In addition it is important to distinguish CGD-TCL from subcutaneous panniculitis-like T-cell lymphoma ( SPTCL ), which bears an alpha-beta phenotype and displays a favourable prognosis ( 2 ).
Most patients with primary CGD-TCL present deeply infiltrated plaques and early ulcerations , especially on the legs ( 3 ). Lupus panniculitis , however , commonly shows erythematous nodes and plaques on the trunk , face , shoulders and proximal extremities .
Histopathology in CGD-TCL usually presents with epidermotropism as well as dermal and subcutaneous infiltrates of atypical lymphocytes . Rimming of adipocytes
Fig . 1 . Initial and late clinical characteristics . ( A ) Initial clinical presentation of the patient ’ s right leg at first visit to our hospital . Two of the nodular lesions are marked with violet paint for excision . ( B ) Clinical presentation of the growing ulceration at the patient ’ s abdomen upon disease progression .
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2630 Acta Derm Venereol 2017 ; 97 : 665 – 666