Table 1 Clinical data of institutional case reports: For details, see Additional file 1
From: Systemic therapy of necrobiotic xanthogranuloma: a systematic review
Case | Age | Cutaneous involvement | Organ involvement (extracutaneous) | Prior treatments | Latest treatment | Response | Follow up |
|---|---|---|---|---|---|---|---|
1 | 59 | Extensive ulcerations on both sides of the lower legs with erythematous wound edges | Whitish discoloration of right cornea, left eye sunken back into the orbita, purulent discharge bilaterally | None | Oral corticosteroids (prednisolone) | Partial response | 3Â months later her condition had deteriorated |
2 | 61 | Ulceration with destruction of the auricular lobe on the left ear | Double vision and visual disturbances (due to infiltration of the eye muscles) | (a) Intravenous immunoglobulins (IVIG) (b) Cytoreductive therapy with melphalan | Chlorambucil and oral corticosteroids (prednisolone) | Partial response |  > 12 months progression free (survival) |
3 | 72 | Generalized cutaneous and subcutaneous lesions | Osseous involvement, mediastinal and hilar lymph node involvement on both sides, bilateral involvement of the lung | (a) Cytoreductive therapy with melphalan (b) lenalidomide in combination with dexamethasone | Dapsone and oral corticosteroids (prednisolone) | Partial repsonse |  > 12 months progression free (survival) |
4 | 69 | Ulceration of the chest, the neck and the perorbital region | Vestibular and cochlear involvement, olfactory system was also involved | Lenalidomide (stopped because of pronounced leukocytosis) | Lenalidomide (reduced dose of 5Â mg/day) and oral corticosteroids (prednisolone) | Stable disease | 3Â months later her condition had deteriorated |
5 | 67 | Bilateral yellowish swelling of both upper and lower eyelid | None | None | Oral corticosteroids (dexamethasone, tapered slowly to Cushing threshold) | Partial response |  > 12 months progression free (survival) |