|LETTER TO EDITOR
|Ahead of print publication
A patient with recurrent panniculitis and systemic inflammatory response
Anuj Shukla1, Jigna Padhiyar2, Nayankumar Harshadkumar Patel2, Amit Mistry3
1 Department of Clinical Immunology and Rheumatology, Niruj Rheumatology Clinic, Ahmedabad, India
2 Department of Dermatology, GCS Medical College, Hospital and Research Centre, Ahmedabad, India
3 Department of Dermatology, Dr. ND Desao Faculty of medical Sciences ans Research, Nadiad, Gujarat, India
|Date of Submission||31-Jul-2021|
|Date of Acceptance||30-Aug-2021|
|Date of Web Publication||27-Jan-2022|
Niruj Rheumatology Clinic, 209 Rajvi Complex, Rambaug, Ahmedabad - 380 008, Gujarat
Source of Support: None, Conflict of Interest: None
A 35-year-old female presented with intermittent fever, weight loss, subcutaneous nodules, myalgia, polyarthralgia, recurrent oral ulcers, diffuse hair fall, and fatigue from 2 years. On examination, there were multiple nonulcerating subcutaneous nodules, 1–3 cm in size, distributed over all the four limbs and trunk, pedal edema, pallor, icterus, mild hepatosplenomegaly, and no lymphadenopathy. The investigations showed pancytopenia, hyperbilirubinemia (total bilirubin 3 mg/dl), transaminitis (200-IU/L), mild proteinuria (1+), and no hematuria or pyuria. Anti-nuclear antibody (ANA) was positive by enzyme-linked immunosorbent assay at 24 IU/L. A diagnosis of systemic lupus erythematosus (SLE) was made and she was referred to us.
At our center ANA by indirect immunofluorescence was negative. Serum acute phase reactants were markedly raised with erythrocyte sedimentation rate of 125 mm at 1 h, C-reactive protein 3.5 mg/dl, and ferritin >2000 ng/ml. Bone marrow examination was normal with no evidence of hemophagocytosis. Histopathology of subcutaneous nodules showed moderate to dense infiltrate of subcutaneous adipose tissue by lymphoid cells. The cells had irregular nuclear membrane with scanty cytoplasm and showed rimming around the adipocytes [Figure 1]a. On immunohistochemistry, these cells were positive for CD3 [Figure 1]b, CD8, CD7, granzyme-B, and negative for CD20, CD4, CD56, and CD30. Ki67 positivity was seen in 50%–55% of cells (high proliferation index), thus confirming the diagnosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). The patient was treated with oral prednisolone 40 mg, has a good response and is planned for chemotherapy.
|Figure 1: (a) Skin and subcutaneous tissue histopathology showing atypical lymphocytes rimming the adipocytes (b) Immunohistochemistry showing CD3 positivity of the cells|
Click here to view
SPTCL is a rare primary cutaneous T-cell lymphoma presenting as panniculitis composed of mature cytotoxic αβ or γδ T-cells. The subcutaneous nodules are typically painless. Pancytopenia, hepatosplenomegaly, icterus, and transaminitis can be seen in 20%–40% cases and may be associated with hemophagocytosis. Patients have a good prognosis with a 5-year survival of 85%. Autoimmune disease can coexist in 20% and lupus-erythematous-panniculitis (LEP) is a close differential diagnosis. LEP clinically has lesions more common on the face, shoulder, breast, and buttocks. They can ulcerate (28%) and heal with lipoatrophy and scarring leaving behind the concavity of skin leading to cosmetic issues., LEP is seen in 1%–3% of patients with SLE, frequently can be present with no or minimal signs of systemic inflammation, and can rarely be a presenting manifestation.,, Histopathology lacks malignant cells with atypical features of the nucleus and cytoplasm described above in SPTCL and is characterized by lymphocytic lobular or mixed panniculitis with the frequent presence of germinal centers and plasma cells. Dermo-epidermal changes, lymphocytic vasculitis, and hyaline fat necrosis are other distinctive features. Many times, the features of both these conditions can overlap/evolve making the final diagnosis difficult. Panniculitis associated with the reaction to Mycobacterium leprae a nodosum leprosum) has a similar presentation with panniculitis, fever, polyarthralgia, hand-feet edema, and systemic inflammatory response., In the tropical areas where leprosy is more prevalent, it can be an important differential diagnosis clinically but can be easily differentiated by histopathology. Thus, rheumatologists evaluating the patients with recurrent panniculitis and systemic inflammatory response should consider a wide differential diagnosis including autoimmune, infectious, and malignant diseases.
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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
López-Lerma I, Peñate Y, Gallardo F, Martí RM, Mitxelena J, Bielsa I, et al.
Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients. J Am Acad Dermatol 2018;79:892-8.
Martens PB, Moder KG, Ahmed I. Lupus panniculitis: Clinical perspectives from a case series. J Rheumatol 1999;26:68-72.
Arai S, Katsuoka K. Clinical entity of Lupus erythematosus panniculitis/lupus erythematosus profundus. Autoimmun Rev 2009;8:449-52.
Zhao YK, Wang F, Chen WN, Xu R, Wang Z, Jiang YW, et al.
Lupus panniculitis as an initial manifestation of systemic lupus erythematosus: A case report. Medicine (Baltimore) 2016;95:e3429.
Massone C, Kodama K, Salmhofer W, Abe R, Shimizu H, Parodi A, et al.
Lupus erythematosus panniculitis (lupus profundus): Clinical, histopathological, and molecular analysis of nine cases. J Cutan Pathol 2005;32:396-404.
Pincus LB, LeBoit PE, McCalmont TH, Ricci R, Buzio C, Fox LP, et al.
Subcutaneous panniculitis-like T-cell lymphoma with overlapping clinicopathologic features of lupus erythematosus: Coexistence of 2 entities? Am J Dermatopathol 2009;31:520-6.
Vijendran P, Verma R, Vasudevan B, Mitra D, Badad A, Neema S. Rare atypical presentations in Type 2 lepra reaction: A case series. Int J Dermatol 2014;53:323-6.
Vinod KV, Chandramohan R, Dutta TK, Rajesh NG, Basu D. Type 2 lepra reaction as a cause of pyrexia of unknown origin. J Assoc Physicians India 2012;60:70-2.