Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature Syndrome: A Systemic Review

Am J Dermatopathol. 2023 Jun 1;45(6):355-370. doi: 10.1097/DAD.0000000000002345. Epub 2023 Feb 17.


Background: Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome is a rare, hereditary, autoinflammatory disease. However, there are few cases reported in the literature. Therefore, we conduct this systematic review to summarize current evidence.

Methods: We conducted a systematic search in July 2021 using 11 different electronic databases. The included articles were screened according to our inclusion and exclusion criteria and assessed using an appropriate quality assessment tool. Then, the relevant data were extracted and summarized in tables accordingly. Each step of the previous one was done by 3 independent reviewers, and the conflicts were resolved by discussion and sometimes by counseling a senior member.

Results: The final included studies were 18 articles with 34 cases (mean age = 8 years, male/female = 19/15). The most reported symptoms and signs were fever 97.1%, erythematous plaques 76.5%, arthralgia 67.6%, hepatomegaly 61.8%, violaceous hue 61.8%, lipodystrophy in extremities 53.1% in addition to low weight and height. Rare features were reported too. The laboratories were not specific, which may be explained by a systemic inflammatory response. Vasculitis was the dominant feature in the skin biopsy, whereas the calcification in the basal ganglia was a prominent sign in many cases.

Conclusions: Fever, skin lesions, and systemic inflammatory response were the prominent features of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome. The clinical picture is the main guide in addition to the pathological findings. Mutation detection is the confirmatory test. Prednisolone is the most effective reported treatment for acute presentations in the literature.

Publication types

  • Systematic Review

MeSH terms

  • Child
  • Chronic Disease
  • Dermatitis*
  • Female
  • Fever / diagnosis
  • Humans
  • Lipodystrophy* / diagnosis
  • Lipodystrophy* / genetics
  • Lipodystrophy* / pathology
  • Male
  • Skin Diseases* / diagnosis
  • Skin Diseases* / drug therapy
  • Skin Diseases* / pathology
  • Sweet Syndrome* / diagnosis
  • Sweet Syndrome* / drug therapy
  • Sweet Syndrome* / pathology
  • Systemic Inflammatory Response Syndrome

Supplementary concepts

  • chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature