Department of Pharmacy Practice, Ezhuthachan College of Pharmaceutical Sciences, Marayamuttom, Neyyattinkara, Thiruvananthapuram, Kerala, India
Sézary syndrome (SS) is a rare and aggressive leukemic variant of cutaneous T-cell lymphoma (CTCL) characterized by the triad of erythroderma, generalized lymphadenopathy, and the presence of malignant T-cells (Sézary cells) in peripheral blood. Due to its overlapping features with chronic inflammatory skin conditions such as psoriasis or eczema, the diagnosis is frequently delayed or misinterpreted. Here presenting a case of a 71-year-old male with a known history of type 2 diabetes mellitus and presumed psoriasis who developed fever, abdominal discomfort, and generalized lymphadenopathy. Initial imaging studies and ultrasonography suggested reactive or granulomatous lymphadenopathy; however, cytological and histological evaluation revealed a lymphoproliferative lesion. Dermatological assessment and skin biopsy later confirmed the diagnosis of Sézary syndrome. The patient was started on systemic chemotherapy with chlorambucil and prednisolone along with local radiotherapy. His condition progressed to pulmonary involvement with lymphomatous infiltration as evident in chest CT. This case emphasizes the need for high clinical suspicion and early biopsy in patients with refractory skin lesions and generalized lymphadenopathy to avoid misdiagnosis and ensure timely intervention. The complexity of such presentations and the importance of an interdisciplinary approach are discussed in light of existing literature.
Sézary syndrome (SS) is an aggressive leukemic form of cutaneous T-cell lymphoma (CTCL), accounting for less than 5% of all CTCL cases. It was first described by Albert Sézary in 1938 and is primarily characterized by erythroderma, lymphadenopathy, and the presence of Sézary cells—abnormal CD4+ T lymphocytes—in peripheral blood and is associated with poor survival due to systemic dissemination and treatment resistance¹–³. The disease predominantly affects elderly males and carries a poor prognosis due to its rapid systemic spread and resistance to conventional therapies.
The diagnosis of SS is often delayed due to its insidious onset and resemblance to more common dermatological conditions like eczema or psoriasis. Erythroderma, one of the hallmark features, may be mistaken for psoriatic flare or drug reaction, particularly in individuals with a pre-existing history of skin disorders. Accurate diagnosis therefore requires integration of clinical morphology with histopathology, immunophenotyping, and hematologic findings?–?.In many cases, patients with SS present with vague systemic symptoms such as fever, weight loss, and lymphadenopathy, making the differential diagnosis extensive. Persistent or atypical cutaneous disease in individuals with presumed chronic dermatoses should prompt reconsideration of malignant etiologies?. In our patient, the initial suspicion was pyrexia of unknown origin, with imaging revealing renal and prostatic abnormalities and lymphadenopathy, while the dermatological symptoms were thought to be related to pre-existing psoriasis. This underscores the importance of re-evaluating persistent or worsening cutaneous symptoms in chronic disease contexts.Histological confirmation through fine-needle aspiration cytology (FNAC) and skin biopsy, alongside immunophenotyping and peripheral smear evaluation, are pivotal in differentiating SS from benign dermatoses. Additionally, elevated tumor markers such as CEA and CA-125, and eosinophilia, though non-specific, can provide supporting evidence for systemic malignancy?.
This report aims to shed light on the clinical complexity of Sézary syndrome through a case presentation. We also compare the diagnostic and therapeutic approaches of similar published cases and discuss the current best practices in managing this rare yet aggressive disease.
CASE REPORT
A 71-year-old male presented with intermittent fever for three days duration, abdominal discomfort, and painful bilateral cervical swelling. His medical history included type 2 diabetes mellitus, systemic hypertension, and was under treatment for presumed psoriasis. His BMI was within normal limits. Physical examination demonstrated generalized lymphadenopathy involving cervical, axillary, and inguinal regions, along with diffuse erythema, scaling of the scalp, palms, and soles, and erythematous nodules over the face and trunk.Initial investigations were aimed at evaluating pyrexia of unknown origin. A plain CT scan of the kidneys, ureters, and bladder (KUB) revealed left renal calculi, left renal cortical cyst, and prostatomegaly. Ultrasonography of the left axilla showed multiple enlarged lymph nodes with significant cortical thickening, central homogenous vascularity, largest of maximum short axis diameter 33 mm and periadenitis, suggestive of reactive lymphadenopathy or granulomatous pathology. Histopathological examination advised excisional biopsy to rule out non-Hodgkin lymphoma. Fine-needle aspiration cytology of the left axillary lymph node showed a polymorphous population of lymphoid cells with a predominance of medium-sized lymphocytes possessing immature chromatin and scant cytoplasm, suggestive of a lymphoproliferative disease.Peripheral smear revealed eosinophilia, thrombocytopenia with scattered large platelets. Serum LDH (574 U/L), Tumor markers including CA-125, and CEA (6.73 ng/mL) were elevated. Dermatologic evaluation followed by skin biopsy confirmed CTCL consistent with Sézary syndrome, and circulating Sézary cells were identified in peripheral blood. The patient was allergic to clindamycin and vancomycin and had been on dermatological medications including Mupirocin, Clobetasol (0.05% w/w), Fusidic Acid (2% w/w), Tab.Desloratadine (5mg) and emollients for symptom relief.
The patient was treated with oral chlorambucil and prednisolone in combination with localized radiotherapy and supportive dermatologic care. Treatment for other comorbidities was continued. However, follow-up chest imaging later demonstrated bilateral ground-glass opacities, consolidation and bilateral peribronchovascular interstitial thickening suggestive of pulmonary lymphomatous infiltration.
Table 1. Investigations
|
Investigation |
Findings |
Interpretation |
|
CT KUB |
Left renal calculi, cortical cyst, prostatomegaly |
Incidental findings |
|
USG Left Axilla |
Multiple enlarged nodes, cortical thickening |
Reactive/Granulomatous Lymphadenopathy |
|
FNAC (Axilla & Skin) |
Lymphoproliferative lesion |
Suggestive of CTCL |
|
Peripheral Smear |
Eosinophilia, large platelets |
Reactive or malignant process |
|
CT Chest |
Bilateral GGO, consolidation |
Suspected lymphomatous infiltration |
Table 2. Laboratory values
|
Parameter |
Value |
|
CA-125 |
125 U/mL |
|
CEA |
6.73 ng/mL |
|
Serum LDH |
574 U/L |
|
Eosinophil Count |
2250 cells/µL |
|
Platelet Count |
90,000 cells/mm³ |
Table 3. Treatment Chart
|
Sl no |
Drug |
Dose |
|
1 |
T. CHLORAMBUCIL |
2 mg |
|
2 |
T. PREDNISOLONE |
40 mg |
|
3 |
CLOBETASOL CREAM |
0.05% w/w |
|
4 |
T. METFORMIN |
500 mg |
|
5 |
T. DESLORATADINE |
5 mg |
|
6 |
T. CILNIDIPINE |
10 mg |
|
7 |
INJ.(PIPERACILLIN+TAZOBACTAM) |
4 g+500 mg |
|
8 |
INJ.ESOMEPRAZOLE |
40 mg |
|
9 |
TOTAL SKIN ELECTRON BEAM THERAPY |
10 Gy |
DISCUSSION
Sézary syndrome represents the leukemic and most aggressive end of the CTCL spectrum and carries significantly worse prognosis than early-stage mycosis fungoides. Diagnostic confirmation requires fulfilment of clinical, histologic, and hematologic criteria, including erythroderma, lymphadenopathy, and circulating malignant T cells with characteristic immunophenotype???. Sézary syndrome is often a diagnostic challenge due to its variable presentation and resemblance to benign dermatological conditions. In the present case, the patient had a long-standing diagnosis of psoriasis, which likely masked the early dermatological manifestations of SS. Similar cases have been documented where erythrodermic CTCL was initially misdiagnosed as psoriasis or dermatitis, delaying definitive therapy and worsening prognosis¹??.
The diagnostic criteria for SS, as per the WHO-EORTC classification, include erythroderma, generalized lymphadenopathy, and the presence of ≥1000 Sézary cells/µL in peripheral blood, along with immunophenotypic abnormalities (loss of CD7 or CD26, high CD4/CD8 ratio) ???. Our patient met these criteria and further exhibited systemic manifestations including fever, lymphadenopathy, and lung involvement, making this a disseminated presentation.
In terms of treatment, there is no curative therapy for SS, and management typically includes skin-directed therapies, systemic immunosuppressants, photopheresis, monoclonal antibodies, and chemotherapy ??¹°. Our patient was treated with oral chlorambucil and prednisolone, a regimen known for its tolerability in elderly patients¹¹ and electron beam therapy. Other studies have reported use of extracorporeal photopheresis and agents like bexarotene, alemtuzumab, or mogamulizumab in relapsed or advanced cases¹²?¹?.
The involvement of internal organs, as seen in our case with probable pulmonary infiltration, has been described in literature but remains uncommon and poorly understood¹??¹?. Early systemic therapy may help delay such complications. Our case emphasizes the significance of a multidisciplinary approach involving dermatologists, pathologists, oncologists, and internists to promptly diagnose and manage SS.
Comparison with Other Case Reports
The present case shares several clinicopathological similarities with previously reported cases of Sézary syndrome (SS), particularly in terms of delayed diagnosis and initial misinterpretation as benign dermatological conditions. Multiple case reports have documented SS presenting as chronic psoriasis or eczema, often leading to prolonged periods of inappropriate treatment before definitive diagnosis is established¹–?. In our case, the patient had a prior history of psoriasis, which masked the early manifestations of SS, consistent with findings reported in earlier literature.
A study by Pinter-Brown et al.¹? described similar hematologic profiles and cutaneous findings in elderly males with SS, including eosinophilia and circulating atypical lymphocytes, highlighting the importance of integrating peripheral smear findings with histopathology and immunophenotyping for early detection.
Studies have shown that the average delay in diagnosis of cutaneous T-cell lymphomas, including SS, can exceed two years due to their indolent onset and clinical resemblance to inflammatory dermatoses¹?.
In contrast to many reported cases where disease remains primarily cutaneous for extended periods, our patient demonstrated early systemic involvement, including suspected pulmonary lymphomatous infiltration. Although extracutaneous spread has been described in the literature, it is relatively uncommon and typically associated with advanced disease stages¹?,¹?. This makes the present case more aggressive in comparison to standard presentations.
Therapeutically, while several recent case reports emphasize the use of targeted therapies such as extracorporeal photopheresis, bexarotene, alemtuzumab, and mogamulizumab, our patient was managed with chlorambucil and prednisolone a regimen often preferred in elderly patients due to better tolerability¹¹–¹? and electron beam therapy. This reflects a pragmatic approach tailored to patient-specific factors such as age and comorbidities.
Overall, this case aligns with existing literature in terms of diagnostic challenges but differs in its rapid progression and early visceral involvement, underscoring the heterogeneity of SS presentations.
Prognosis
Sézary syndrome is associated with a poor overall prognosis, primarily due to its aggressive nature, systemic dissemination, and resistance to conventional therapies. The median survival ranges between 2 to 4 years, depending on factors such as age, tumor burden, extent of blood involvement, and response to treatment¹?.
In the present case, several factors indicate an unfavourable prognosis:
Visceral involvement, particularly pulmonary infiltration as suspected in this patient, is considered a marker of advanced disease and poor survival outcomes¹?. Additionally, delayed diagnosis due to initial misclassification as psoriasis may have contributed to disease progression before initiation of appropriate therapy.
Modern therapeutic options, including biologics and immunomodulatory therapies, may improve outcomes but are often limited by accessibility, cost, and patient tolerance¹²–¹?.
Therefore, the prognosis in this case remains guarded to poor, emphasizing the need for early diagnosis, timely intervention, and multidisciplinary management to potentially improve survival and quality of life.
CONCLUSION
Sézary syndrome remains an underdiagnosed yet aggressive malignancy, particularly in elderly individuals with pre-existing dermatological conditions. This case underscores the need for high clinical suspicion, early skin and lymph node biopsy, and the use of comprehensive diagnostic criteria to avoid misclassification. Treatment with oral chemotherapy was initiated in accordance with the patient’s age and performance status, but the disease course progressed systemically. Clinicians must consider cutaneous lymphoma in cases of generalized lymphadenopathy and atypical skin changes unresponsive to conventional therapy. Early recognition and multidisciplinary management are crucial despite the generally guarded prognosis
PATIENT CONSENT
Written informed consent was obtained from the patient for publication of this case report and accompanying details.
REFERENCES
Neethu Krishna B. R., Reshma Babu, Shaiju S. Dharan, A Rare Case of Sézary Syndrome Mimicking Psoriasis: A Diagnostic Challenge, Int. J. of Pharm. Sci., 2026, Vol 4, Issue 3, 2965-2970 https://doi.org/10.5281/zenodo.19200995
10.5281/zenodo.19200995
