An aggressive cutaneous T-cell lymphoma masquerading as non-healing ulcers

Victor Desmond Mandel1, Giulia Odorici1, Francesca Di Tullio1, Marco Manfredini1, Giovanni Pellacani1, Francesca Farnetani1

1Department of Surgical, Medical, Dental and Morphological Sciences with Interest transplant, Oncological and Regenerative Medicine, Dermatology Unit; University of Modena and Reggio Emilia, Modena, Italy.

 

Case presentation. A 71-year-old Caucasian woman was referred to our department for evaluation and treatment of asymptomatic plaques and ulcers of the body. Physical examination revealed multiple erythematous plaques, erosions and ulcers located on the right mammary region, upper and lower extremities (Figures A-D). No mucosal lesions were present.

She reported the onset of lesions on the right mammary region 4 months before our observation and then she noticed the progressive spread to her extremities. During the last 4 months she had lost nearly 8 Kg in weight and since few days she presented a low-grade fever.

The patient was a smoker and the medical history revealed only hypertension treated with ramipril. We performed a complete routine laboratory investigation including immunologic tests (immunoglobulins, rheumatoid factor, antinuclear factor and organ-specific antibodies) and we detected thrombocytopenia, mild lymphopenia, hypokalaemia, hypocalcemia, increase of C-reactive protein and beta-2 microglobulin. We performed also the neoplastic markers and we found an increase of CA 125 (39 U/ml) and CA 15.3 (159 U/ml) serum levels.

Diagnostic work-up and treatment: Histologic examination of a 6 mm punch biopsy on a lesion of the left thigh revealed an ulcerated skin with a mild lymph granulocyte perivascular infiltrate in the dermis and presence of isolated, medium size, atypical lymphocytes. In the low adipose tissue there was an atypical lymphoid infiltrate composed of elements of small and medium size. Immunophenotype was CD3+, CD4+, CD56+, granzyme B+, TIA-1+ focally, CD3-, CD8- and CD30-. Moreover, the citoproliferative activity was rather high with aspects of peri-adipocyte rimming. Furthermore, the Epstein-Barr virus was absent and the clonal rearrangement of TCR gamma gene was monoclonal.

Based on all these findings the diagnosis of aggressive cutaneous T-cell lymphoma was established.

A total body computed tomography scan was performed showing multiple millimetre pulmonary nodules (maximum diameter of 5 mm) in the mantle and parenchymal center. Moreover, this exam revealed focal areas of subcutaneous edema of the abdomen, hips and buttocks, which was very marked in the right breast. Then, we decided to perform a positron emission tomography that revealed diffuse cutaneous and subcutaneous localization of the underlying disease and suspected osteomedullary involvement.

A multiagent chemotherapy was started but the patient died only one month later.

Key message: This is a case of aggressive primary cutaneous T-cell lymphoma CD3+, CD4+ and CD56+. Cutaneous lymphomas expressing CD56 are characterised in most cases by a highly aggressive clinical course1, regardless of their histologic presentation and the extent of skin involvement. Currently, no effective treatment is available.2

References

  1. Assaf C, Gellrich S, Whittaker S, et al. CD56-positive haematological neoplasms of the skin: a multicentre study of the Cutaneous Lymphoma Project Group of the European Organisation for Research and Treatment of Cancer. J Clin Pathol 2007;60(9):981-9.
  2. Gniadecki R, Rossen K, Ralfkier E, Thomsen K, Skovgaard GL, Jønsson V. CD56+ lymphoma with skin involvement: clinicopathologic features and classification. Arch Dermatol 2004;140(4):427-36.

 

Figures A-C:

Clinical pictures of the multiple erythematous plaques, erosions and ulcers of the trunk and the arms.

Figure A

Figure B


Figure C


Figure D:

Clinical picture of the legs involved by multiple erythematous plaques, erosions and ulcers.