The following is a summary of “Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature Syndrome: A Systemic Review,” published in the June 2023 issue of Dermatopathology by Altom et al.
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is an uncommon inherited autoinflammatory disorder. However, only some cases have been documented in the literature. Researchers execute this systematic review to summarize the available evidence. They conducted a systematic investigation using 11 electronic databases in July 2021. The included articles were screened based on their inclusion and exclusion criteria and evaluated using a suitable quality assessment instrument.
The relevant data were then extracted and summarized accordingly in tables. Three independent reviewers performed each stage, and conflicts were resolved through discussion and, on occasion, consultation with a senior member. About 18 articles with 34 cases (mean age = 8 years, male/female = 19/15) were included in the final set of studies (mean age = 8 years, male/female = 19/15). In addition to low weight and height, the most frequently reported symptoms and indications were fever (97.1%), erythematous plaques (76.5%), arthralgia (67.6%), hepatomegaly (61.8%), violaceous hue (61.8%), and lipodystrophy in extremities (53.1%).
Also reported were rare characteristics. A systemic inflammatory response may explain the lack of specificity in the laboratories. In most cases, calcification in the basal ganglia was the most prominent feature of the skin biopsy. The most prominent features were chronic atypical neutrophilic dermatosis with lipodystrophy, elevated temperature syndrome, fever, cutaneous lesions, and systemic inflammatory response. In addition to the pathological findings, the clinical picture is the principal guide. Detecting mutations is the confirmation test. Prednisolone is the most efficacious treatment reported in the literature for acute presentations.
