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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 3 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 5 mg/day) and oral corticosteroids (prednisolone) Stable disease 3 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)
  1. Images from Case 2 are given in Fig. 2