Cutaneous T-Cell Lymphomas:Sézary Syndrome; Mycosis Fungoides(Nejm 2004;350:1978); and Reticulum Cell Lymphoma(Ann IM 1988;109:372)
Cause:Malignancies of T-cell lymphocytes (Ann IM 1974;80:685)
Pathophys:
"Helper" T 4 lymphocyte lymphomas
Sézary syndrome has erythrodermic variants plus circulating malignant T cells
Mostly in older (age >40 yr) patients; associated with industrial solvent exposure in older studies, also chronic contact dermatitis
Sx:Erythematous, scaly plaques, nodules, and tumors
Si:
Stage I: polymorphic indurated papulosquamous rash; polycyclic red-brown scaly plaques (r/o sarcoid, Behçet's syndrome)
Stage II: generalized erythroderm and cutaneous nodules
Stage III: organ invasions lead to "-megalys"
Stage I: 12+-yr average survival (Arch Derm 1996;132:1309)
Stage II: 5-yr average survival; after ulcerates, 3-5 yr no matter what rx
Stage III: 2.5-yr average survival
Meningeal involvement occasionally occurs with CNS symptoms, even when skin changes are in remission (Ann IM 1975;82:499)
Rx: When disease limited to skin: topical nitrogen mustard, steroids, bexarotine (Targeretin) gel
PUVA in early stages (Arch Derm 1996;76:475)
Systemic rx w chemotherapy drugs and interferon is effective palliation, not curative (Ann IM 1994;121:592)
Radiation
Extracorporeal photochemotherapy with UVA (Med Let 1988;30:96)
of Sézary syndrome: etretinate and electron beam rx (J Am Acad Derm 1992;26:960)