Neutrophilic panniculitis M79.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Neutrophilic lobular panniculitis; Neutrophilic panniculitis; Neutrophil paniculitis; Panniculitis neutrophilic lobular

Definition
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Polyätiological panniculitis in existing myelodysplastic syndrome or rheumatoid arthritis; also occurring as ADR in therapy with BRAF inhibitors, e.g. vemurafenib.

Furthermore, the syndrome has been observed in autoinflammatory syndromes such as Sweet's syndrome and Crohn's disease as well as in hereditary alpha-1-antitrypsin deficiency.

Manifestation
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Mainly occurring in middle-aged women.

Localization
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The lower leg.

Clinical features
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Painful nodules and plates in the subcutis with red, overlying skin.

Laboratory
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Signs of inflammation with BSG and CRP elevation, leukocytosis and neutrophilia.

Histology
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Diffuse lobular fat tissue necrosis with basophilic necrosis, dense infiltrates with neutrophilic granulocytes, foam cells. Perivascularly, some lymphocyte infiltrates are also visible. Lipomembranous fat cell necrosis and micropseudofattysts are also present in the lobular periphery.

Therapy
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Treatment of the underlying disease. Rapid response to systemic glucocorticoids. Symptomatic local therapy. Reduction of therapy with BRAF inhibitors.

Literature
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  1. Chan MP et al (2013) Subcutaneous Sweet syndrome in the setting of myeloid disorders: a case series and review of the literature. J Am Acad Dermatol 68:1006-1015
  2. Choy B et al (2014) Panniculitis in patients treated with BRAF inhibitors: a case series. At J Dermatopathol 36:493-497
  3. Fernández-Torres RM et al (2014) Subcutaneous histiocytoid sweet syndrome associated with crohn disease in an adolescent. Case Rep Dermatol Med doi: 10.1155/2014/954254
  4. Leiva-Salinas M et al (2014) Neutrophilic lobular panniculitis as an expression of a widened spectrum of familial mediterranean fever. JAMA Dermatol 150:213-214
  5. Maldonado-Seral C et al (2014) Vemurafenib-associated neutrophilic panniculitis: An emergent adverse effect of variable severity. Dermatol Online J 19:16
  6. Nobeyama Y et al (2014) Subcutaneous Sweet's syndrome and neutrophilic panniculitis J Dermatol 41:861-862
  7. Srisuttiyakorn C et al (2014) Subcutaneous histiocytoid Sweet's syndrome in a patient with myelodysplastic syndrome and acute myeloblastic leukemia. J Cutan catheter 41:475-479
  8. Zhang M et al (2014)Ponatinib-induced neutrophilic panniculitis. J Cutan catheter 41:597-601
  9. Stubbs E et al(2014) Radiology-pathology conference: neutrophilic fasciitis and panniculitis of the feet (Sweet's syndrome). Clin Imaging 37:608-612
  10. Kim GH et al (2014) Neutrophilic panniculitis developing after treatment of metastatic melanoma with vemurafenib. J Cutan catheter 40:667-669
  11. Kim J et al (2010) A case of Sweet's Panniculitis Associated with Spinal Metastasis from Prostate Cancer. Ann Dermatol 22:478-481
  12. Llamas-Velasco M et al (2013) Sweet's syndrome with subcutaneous involvement associated with pegfilgrastim treatment: first reported case. J Cutan Pathol 40:46-49
  13. Monfort JB et al (2012) Vemurafenib-induced neutrophilic panniculitis. Melanoma Res 22:399-401

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020