Fever, lipodystrophy and cutaneous lesions

Kieselova, Katarina; Santiago, Felicidade; Guiote, Victoria; Amado, Carolina; Henrique, Martinha

doi:10.1111/ced.13215

Cited by 2 publications

(3 citation statements)

References 6 publications

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“…Eleven studies reported the results of skin biopsy. 6,9,14,15,[18][19][20][22][23][24][25] The main findings of these biopsies were interstitial and perivascular infiltrate with one of these cells or mixed of them; histiocytic, lymphocytic, polymorphonuclear, atypical/typical mononuclear, or eosinophilic cells. Langerhans cells were also reported in some cases, but mature neutrophils were the most reported ones.…”

Section: Pathological Findingsmentioning

confidence: 99%

“…Nine studies mentioned the results of the bone marrow, and they were mostly normal. 1,2,9,15,19,20,[22][23][24] However, in a few cases, the specimen demonstrated one of these findings; trilineage hematopoiesis with normal cytogenetic evaluation, increased erythropoiesis, reactive changes, infiltration of immature neutrophils/myeloid precursors, and atypical mononuclear cells that were most likely activated macrophages (positive for CD68 and CD163).…”

Section: Pathological Findingsmentioning

confidence: 99%

“…Moreover, rare facial features were reported in the 5 patients. 1,2,14,18,20,21 Laboratories Laboratories were summarized in 2 tables according to the availability of the data (Table 5). Anemia (8.6 6 2.1 g/dL) was found in nearly half of the patients (44.1%) with high reticulocytes (8.8%).…”

Section: Symptoms and Signsmentioning

confidence: 99%

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

Altom,

Khader,

Gad

et al. 2023

The American Journal of Dermatopathology

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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.

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Section: Pathological Findingsmentioning

confidence: 99%

Section: Pathological Findingsmentioning

confidence: 99%

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

Altom,

Khader,

Gad

et al. 2023

The American Journal of Dermatopathology

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Insights into CANDLE Syndrome

Kieselova

Santiago

Henrique

2020

SPDV

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Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a recently described chronic autoinflammatory disease, pathophysiologically related to intracellular proteasome/immunoproteasome dysfunction. This review chronicles the recognition of CANDLE syndrome, the developments in the understanding of the pathomechanism, genetic background, and the emerging therapeutic strategies of this condition.

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Fever, lipodystrophy and cutaneous lesions

Abstract: Click https://www.wileyhealthlearning.com/ced.aspx for the corresponding questions to this CME article.

Cited by 2 publications

References 6 publications

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

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

Insights into CANDLE Syndrome

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