Table 1.

Treatment modalities in 25 patients with SM [54]

Treatments	Patients	Complete response	Partial response	Failure
Tried 1	14
IVIG alone		3	3
Steroids alone (PS/dexamethasone)				2
Thalidomide			1
Acitretin			1
Melphalan				1
Mycophenolate mofetil				1
PS + thalidomide			1
PS + hydroxychloroquine			1
Tried 2	3
PS, followed by IVIG			2*
Hydroxychloroquine, followed by phototherapy				1
Tried 3	5
PSL, followed by photopheresis, followed by IVIG			1*
MTX, followed by PS, followed by IVIG			1*
Phototherapy, followed by radiotherapy, followed by isotretinoin				1
Chloroquine, followed by etretinate, followed by interferon alfa-2b				1
Melphalan + PS, followed by photopheresis + PS + MTX, followed by radiotherapy				1
Tried 4	2
Hydroxychloroquine, followed by PS, followed by thalidomide, followed by IVIG + lenalidomide		1†
Thalidomide, followed by bath PUVA, followed by photopheresis, followed by PSL + IVIG			1++
Tried 5
Cyclosporine, followed by azathioprine, followed by cyclophosphamide, followed by MTX + PS, followed by IVIG	1		1*

MTX = Methotrexate; PS = prednisone; PSL = prednisolone.

^*Caused by IVIG.

^†Caused by IVIG + lenalidomide.

⁺⁺Caused by IVIG + steroids.

With a better understanding of the exact pathogenesis of SM and the mechanisms of newly emerged, more specific and directed therapies, especially in the era of biological treatment, we can help these patients receive more effective treatment with fewer side effects. In this context, we would like to encourage more studies on the pathogenesis of SM, along with multicenter studies to assess the efficacy of different treatment modalities, taking into consideration the rarity of this disease entity and its possible fatal outcome.