Table 1.
Reported cases of necrobiotic xanthogranuloma.
| Author/yr | Age, y/sex | Location | Paraprotein | BM | Other organ involvement | Treatments received / outcome |
|---|---|---|---|---|---|---|
| Venencie12 1995 | 65/F | Periorbital, presternal, shoulders | IgG kappa | <10% plasma cells | none | Initial Melphalan+ prednisone, then Interferon alfa-2b+prednisone. Still on prednisone 1.5 yrs later. Lesions flattened with residual pigmentation |
| Barzilai 13 1996 | 85/F | Face, trunk extremities | IgG kappa | Not done | Possibly cardiac | None. Patient refused |
| Johnston14 1997 | 69/F | Thighs, arms, back | IgG kappa | Normal | None | Intralesional corticosteroid injection of symptomatic lesions. Outcome not noted |
| Georgiou15 1999 | 82/M | Chest, back | Not indicated | Not done | None | Interferon alpha -2a. Resolution after 4 months therapy. No recurrence 22 months after therapy |
| Nestle16 1999 | 77/F | Neck, trunk, arms, legs | IgG lambda | Normal | None | Initial PUVA and radiotherapy. Then melphalan x 6 cycles with resolution. No long-term follow up noted. |
| Randell17 1999 | 51/F | Periorbital | IgG lambda | Normal | None | Treatment not discussed. |
| Chave18 2001 | 66/F | Periorbital, neck, chest, abdomen | IgG kappa | Normal | None | Chlorambucil, cytoxan, melphalan, etoposide plasmapheresis and IFN-alph2b all ineffective. high-dose dexamethasone x 2 cycles with continued prednisolone with encouraging response |
| Chave 19 2001 | 75/M | Periorbital, Upper back, shoulders, arms, thigh | IgG kappa IgG lambda |
2% plasma cells | None | No systemic treatment at 1 year since presentation |
| Machado20 2001 | 51/M | Neck, trunk | IgG lambda | 5–10% plasma cells | Cryoglobulinemia, spleenomegaly | Chlorambucil x 7 mos with eventual disappearance of all lesions. No recurrence at 9 mos follow up |
| Vasconcellos21 2002 | 40/F | Periorbital, legs, thighs, back | IgG lambda | Normal | None | Melphalan +prednisone. Stopped treatment after 2 mos- reason not given |
| Burdick22 2003 | 57/F | Periorbital, upper body | IgG lambda | Xanthogranuloma within medullar No malignancy | Hepatomegaly | No treatment initiated |
| Fernandez23 2004 | 73/M | Periorbital | IgG kappa | 14% plasma cells | Multiple myeloma | Surgical removal of eyelid skin. Melphalan ongoing at 9 mos |
| Meyer24 2005 | 53/F | Periorbital | IgG kappa | 5% plasma cells | None | Numerous nonresponsive therapies. Stable therapeutic response after 9 cycles of intravenous cytoxan+dexamethasone (mesna given for bladder protection. Treatment was ongoing. |
| Viera25 2005 | 68/F | Periorbital, buttocks | IgG lambda | Not done | Prior history of lymphoplasmacytic lymphoma | Carbon dioxide laser for 3 sessions with residual lesions of NXG present. No relapse at 12 months follow-up. |
| Langlois26 2006 | 75/F | Periorbital, neck, arms, torso, neck, umbilical | IgG lambda | Not reported | Multiple myeloma | Melphalan+prednisone. Skin lesions faded after 2 courses. Length of treatment not noted |
| Oumeish27 2006 | 56/F | Periorbital, face, chest, back, | IgG kappa | NHL, CLL | NHL, CLL | Cytoxan, endoxan, leukeran, melphalan and prednisolone. Excellent response of the NHL and CLL. Unsatisfactory response of skin lesions despite cyclosporine and systemic steroids |
| Torabian28 2006 | 55/M | Periorbital, extremities, trunk | IgG lambda | 2% plasma cells | None | Chlorambucil. Flattening and disappearance of skin lesions but persistent Paraprotein spike at 6 mos of treatment |
| Ito29 2007 | 86/F | Limbs | IgG lambda | Patient refused | None | Patient died of myocardial infarction shortly after diagnosis |
| Silapunt 2010 | 81/M | Extremeties, trunk, face | IgG Kappa | 10% plasma cells | None | Lenalidomide and dexamethosone x 3 months with resolution of skin lesions.. no recurrence at 12 months follow-up |
CLL means chronic lymphocytic leukemia, NHL non hodgkins lymphoma