|IMAGES IN RHEUMATOLOGY
|Year : 2017 | Volume
| Issue : 4 | Page : 228-229
Cutaneous T-cell lymphoma presenting as panniculitis with arthritis
Rasmi Ranjan Sahoo1, Anupam Wakhlu1, Kiranpreet Malhotra2, Puneet Kumar1
1 Department of Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India
2 Department of Pathology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
|Date of Web Publication||16-Nov-2017|
Department of Rheumatology, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Keywords: Leukopenia and panniculitis, lobular panniculitis, subcutaneous panniculitis-like T-cell lymphoma
|How to cite this article:|
Sahoo RR, Wakhlu A, Malhotra K, Kumar P. Cutaneous T-cell lymphoma presenting as panniculitis with arthritis. Indian J Rheumatol 2017;12:228-9
A 40-year-old female presented with a 5-month history of multiple painful nodules over bilateral thighs, abdomen, and back. She also had a painless, progressive swelling over the left cheek with right ankle arthritis. This was not associated with fever, fatigue, weight loss, or other systemic symptoms. Her vitals were stable. The nodules were erythematous, tender, of variable sizes with the largest being 8 cm × 5 cm over the abdomen [Figure 1]a. There was a nontender, firm swelling with ill-defined margins, and normal overlying skin, measuring approximately 10 cm × 7.5 cm over the left cheek [Figure 1]b. There was no lymphadenopathy. Systemic examination was unremarkable except for the right ankle arthritis. A diagnosis of panniculitis with arthritis was entertained. Investigations revealed hemoglobin 10.4 g/dl, total leukocyte count 1780/mm 3, differential count N71/L21/M6/E2, ESR 36 mm 1st h, and C-reactive protein 25.1 mg/L (0–6 mg/L). Peripheral smear revealed normocytic, normochromic red blood cells, reduced leukocyte count, adequate platelets, and no atypical cells. Serum calcium was 9 mg/dl (8.4–10), serum alkaline phosphatase 185.6 IU/L (44–147), serum protein: 7 g/dl (6–8 g/dl) with serum albumin 3.9 g/dl (3.5–5 g/dl), lactic dehydrogenase 340 IU/L (100–280). Serum ferritin was normal. Antinuclear antibody, antineutrophil cytoplasmic antibodies, and Mantoux test were negative. Urine examination, liver and renal function tests were normal. Bone marrow aspirate and biopsy revealed normocellular marrow with trilineage hematopoiesis, with no atypical cells. HIV, hepatitis B surface antigen, and antihepatitis C virus were negative. Chest radiograph and ultrasonography (USG) abdomen were normal. Radiograph of the ankle joint was normal, and USG revealed synovitis with minimal effusion (not aspirable). USG of the left cheek showed ill-defined, hyperechoic, homogenous lesion, measuring 10 cm × 7 cm × 3 cm with no vascularity. A deep excisional biopsy from nodule on the left thigh showed unremarkable epidermis and dermis with infiltration of atypical lymphocytes in subcutaneous fat lobules [Figure 2]a. Immunohistochemistry (IHC) revealed infiltrating cells with expression of CD3+, CD8+, CD20−, and CD56− markers [Figure 2]b.
|Figure 1: (a) Erythematous nodules (arrows) over the abdomen. (b) Swelling over the left cheek|
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|Figure 2: (a) Light microscopic appearance of the nodule (H and E, ×200) showing subcutaneous adipocytes (arrow) rimmed by an infiltrate of atypical large lymphocytes. (b) The section shows atypical cells positive for CD3 (arrowhead) (diaminobenzidine, ×400)|
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A final diagnosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) was made. The patient was referred to the Department of hematology for further management.
| Discussion|| |
SPTCL is a rare cutaneous Tcell lymphoma characterized by multiple, painless, subcutaneous nodules on the extremities, and trunk. The diagnosis is often challenging, as the clinical and systemic symptoms are nonspecific and lesions mimic panniculitis or cellulitis leading to a delay in diagnosis. Our patient presented with swelling over the cheek, arthritis, and leukopenia in addition to subcutaneous nodules. According to the WHO, European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas, SPTCL is restricted to cases with αβ T-cell receptor (TCR) whereas those with γδ TCR are categorized as primary cutaneous peripheral T-cell lymphomas. SPTCL's with αβ TCR are characterized by an indolent course and are usually CD3+, CD8+, CD4− and CD56− whereas γδ TCR is associated with rapid deterioration and coexisting hemophagocytosis and are usually CD3+, CD4−, CD8−, and CD56+. Therefore, a diagnosis of SPTCL is usually based on the examination of skin and subcutaneous tissue histology, IHC, and molecular analysis. Histologically, SPTCL is characterized by neoplastic lymphoid cells infiltrating lobules of the subcutaneous tissue with rimming of adipocytes, as is seen in our case. However, rimming of fat cells may also be seen in lobular panniculitis and cutaneous lymphomas. The common differential diagnosis of SPTCL includes benign panniculitis and lupus panniculitis. Benign panniculitis is aggregates of CD20+ B-cells mixed with CD3+ cells that are also CD4+ and CD8+. Lupus panniculitis is predominantly CD4+ T-cell infiltration with germinal center formation.
The management of SPTCL is challenging, and various modalities have been used. CHOP-regimen is the most commonly used initial chemotherapy with good response in αβ TCR SPTCL compared to γδ TCR cutaneous T-cell lymphoma. The overall 5-year survival rate for αβ TCR SPTCL is 82%, and γδ TCR primary cutaneous T-cell lymphomas is 11%.
SPTCL may be associated with autoimmune diseases in about 20% cases. These include systemic lupus erythematosus (SLE), Sjogren's syndrome, juvenile rheumatoid arthritis (RA), multiple sclerosis, Raynaud's, and Kikuchi disease.
Rheumatologists must remember that SPTCL is a rare and difficult to diagnose disease, mimicking nonspecific or lobular panniculitis and coexisting with other autoimmune diseases. The presence of arthritis, panniculitis, and leukopenia may point the clinician to other diagnoses such as sarcoidosis, Poncet's disease with bone marrow involvement, SLE, RA, Weber Christian disease, lymphomatoid granulomatosis, and infections such as HIV, Brucella More Details, and paraneoplastic syndromes. A deep biopsy including subcutaneous tissue is essential and IHC is the best way to differentiate between SPTCL and panniculitis of other etiologies.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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