Subcutaneous panniculitis-like T-cell lymphoma
SPTCL is considered a prognostically favorable cutaneous T-cell lymphoma with an indolent course. Average age of onset is at 36 years, but it can present in both adults and children. There is a slight female predilection. Survival rate is excellent (5-year survival rate above 90%). It is generally confined to the subcutaneous tissue.
While extracutaneous involvement by lymphoma cells is typically absent, hemophagocytic syndrome (HPS) is a serious complication seen in 15%-37% of patients. HPS is characterized by uncontrolled activation and proliferation of normal lymphocytes and macrophages with multisystem involvement, and is associated with poor survival.
SPTCL may be associated with an autoimmune disease in around one-fifth of cases. Reported associations include systemic lupus erythematosus, juvenile rheumatoid arthritis, Sjögren syndrome, rheumatoid arthritis, diabetes mellitus type 1, idiopathic thrombocytopenic purpura, multiple sclerosis, Raynaud disease, and Kikuchi-Fujimoto disease.
C86.3 – Subcutaneous panniculitis-like T-cell lymphoma
404133000 – Subcutaneous panniculitis-like T-cell lymphoma
Differential Diagnosis & Pitfalls
- Primary cutaneous γ/δ T-cell lymphoma – A more aggressive cutaneous lymphoma with similar clinical presentation to SPTCL, with a poor prognosis (5-year survival rate of 11%). It affects older patients (median age of 59 years). Neoplastic T-cells may infiltrate the epidermis, and ulceration may be observed clinically. There is a higher risk for development of HPS. The neoplastic T-cells are CD56+ with γ/δ T-cell receptors, while in SPTCL, they are universally CD56− with α/β T-cell receptors.
- Erythema nodosum
- Erythema induratum
- Infectious panniculitis
- Alpha-1 antitrypsin deficiency panniculitis
- Lupus panniculitis
- Natural killer (NK) T-cell lymphoma
- Subacute migratory panniculitis
- Pancreatic panniculitis
- Jessner lymphocytic infiltration of the skin
- Weber-Christian disease
- Polyarteritis nodosa
- Physical panniculitis
- Cold panniculitis
- Factitial panniculitis
- Cytophagic histiocytic panniculitis