Neutrophilic Panniculitis Associated With Myelodysplastic Syndrome/Myeloproliferative Neoplasm:A Case Report and Literature Review

Introduction:Neutrophilic panniculitis(NP)is a rare subtype of neutrophilic dermatosis,a group of neutrophilrich inflammatory skin disorders that can present in association with myeloid neoplasms.NP is defined by the presence of a neutrophilic infiltrate in the fat lobules of the subcutis in the absence of either infection or vasculitis.We herein describe a 65-year-old woman with a recent diagnosis of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndrome(MDS/MPN)who abruptly developed painful,pruritic nodules consistent with NP.Case presentation:A 65-year-old woman with MDS/MPN presented for evaluation of painful and pruritic nodules on her upper and lower extremities.A biopsy revealed a lobular neutrophilic infiltrate in the subcutis without evidence of microorganisms or vasculitis.The patient was diagnosed with NP and treated with oral prednisone.Within 1 month of treatment,she reported complete resolution of the nodules.Discussion:Similar to other neutrophilic dermatoses,NP may arise in association with hematologic malignancies of myeloid origin,such as MDS/MPN.A literature review revealed that most cases of NP associated with MDS occur after the onset of MDS and respond to systemic corticosteroids,not antibiotics.Infection should be ruled out before initiating treatment with systemic steroids.Conclusion:Although the mechanism is still unknown,it is important for clinicians to be aware that NP is associated with MDS;thus,hematological malignancies should be investigated on diagnosis of NP.Once diagnosed,NP is easily treated and has an excellent response to systemic corticosteroids.

Ets-2 deletion in myeloid cells attenuates IL-1α-mediated inflammatory disease caused by a Ptpn6 point mutation

The SHP-1 protein encoded by the Ptpn6 gene has been extensively studied in hematopoietic cells in the context of inflammation.A point mutation in this gene(Ptpn6spin)causes spontaneous inflammation in mice,which has a striking similarity to neutrophilic dermatoses in humans.Recent findings highlighted the role of signaling adapters and kinases in promoting inflammation in Ptpn6spin mice;however,the underlying transcriptional regulation is poorly understood.Here,we report that SYK is important for driving neutrophil infiltration and initiating wound healing responses in Ptpn6spin mice.Moreover,we found that deletion of the transcription factor Ets2 in myeloid cells ameliorates cutaneous inflammatory disease in Ptpn6spin mice through transcriptional regulation of its target inflammatory genes.Furthermore,Ets-2 drives IL-1α-mediated inflammatory signaling in neutrophils of Ptpn6spin mice.Overall,in addition to its well-known role in driving inflammation in cancer,Ets-2 plays a major role in regulating IL-1α-driven Ptpn6spin-mediated neutrophilic dermatoses.