Lipodystrophia centrifugalis abdominalis infantilis accompanied by idiopathic encephalopathy

Kaibuchi-Noda, Kana; Sugiura, Kazumitsu; Akiyama, Masashi

doi:10.1111/jdv.13031

Cited by 4 publications

(2 citation statements)

References 6 publications

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“…Okita et al 13 found that CLD is related to the apoptosis of subcutis. Moreover, Kaibuchi‐Noda et al 14 first reported a case accompanied by idiopathic encephalopathy. Intriguingly, we first found a case of CLD complicated with Mucopolysaccharidosis type IIIC; the association requires exploration.…”

Section: Discussionmentioning

confidence: 99%

Centrifugal lipodystrophy on a spectrum with lupus erythematosus panniculitis in children and efficacy and safety of hydroxychloroquine: A clinicopathological study

Qiu

et al. 2022

Dermatologic Therapy

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This study aimed to investigate the relationship between centrifugal lipodystrophy (CLD) and lupus erythematosus panniculitis (LEP), and the efficacy and safety of hydroxychloroquine (HCQ) for treating CLD in children. A total of 29 cases clinically diagnosed as CLD (n = 24) and CLD/LEP overlap (n = 5) were enrolled and all were confirmed by skin biopsies of CLD and LEP. The clinicopathological findings, clinical outcomes, and prognosis with the treatment of HCQ between CLD and LEP were compared. All 29 cases (male: female = 1:1.6; median age at onset: 3 years) had cutaneous lesions of centrifugally expanding lipoatrophy, of which five cases overlapped with LEP lesions presented as erythematous indurated plaque (n = 2), subcutaneous nodules (n = 2) and alopecia along Blaschko's lines (n = 1). Antinuclear antibodies were found in six (25.0%) CLD and two (40.0%) overlapped patients (p = 0.597). Histopathologically, of the 24 cases of CLD, 14 (58.5%) exhibited subcutis loss or mild lobular inflammation. Ten (41.7%) cases displayed lobular panniculitis with moderate to dense lymphohistiocytic infiltrate and plasma cells, similar to the five cases of overlap. Small clusters of CD123 positive plasmacytoid dendritic cells were found in 62.5% (5/8) of CLD and 66.7% (2/3) of overlap cases (p > 0.99). HCQ (5 mg/kg/d) treatment showed improvement in 91.3% (21/23) of CLD and all overlap cases, including four cases unresponsive to previous oral glucocorticosteroid treatment. Our findings suggested that CLD and LEP represent a spectrum within the same disease. HCQ (5 mg/kg/d) was effective and safe for treating CLD (age >1.5years), and early treatment and a regular long-term follow-up are essential.

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Section: Discussionmentioning

confidence: 99%

Centrifugal lipodystrophy on a spectrum with lupus erythematosus panniculitis in children and efficacy and safety of hydroxychloroquine: A clinicopathological study

Qiu

et al. 2022

Dermatologic Therapy

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“…The main characteristics of all 7 cases (including ours) are summarized in Table 1. 2,[4][5][6][7] The clinical manifestations included depressed plaques (7/7), inguinal lymphadenopathy (3/7), and hair loss (2/7). Skin biopsy showed varying degrees of inflammatory infiltration in the degenerating adipose tissue.…”

Section: Therapy Lettersmentioning

confidence: 99%

Lipodystrophia centrifugalis abdominalis infantilis accompanied by immunoglobulin kappa chain gene rearrangement successfully treated by a low dose of prednisone

Huang¹,

Tang²,

Wang³

et al. 2020

Indian J Dermatol Venereol Leprol

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Skin diseases in Asian individuals that you do not want to miss: A selection of unique or relatively more common conditions in Asian populations

Tiao

Shin

Janahi

et al. 2021

Clinics in Dermatology

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Lipodystrophia centrifugalis abdominalis infantilis accompanied by idiopathic encephalopathy

Cited by 4 publications

References 6 publications

Centrifugal lipodystrophy on a spectrum with lupus erythematosus panniculitis in children and efficacy and safety of hydroxychloroquine: A clinicopathological study

Centrifugal lipodystrophy on a spectrum with lupus erythematosus panniculitis in children and efficacy and safety of hydroxychloroquine: A clinicopathological study

Lipodystrophia centrifugalis abdominalis infantilis accompanied by immunoglobulin kappa chain gene rearrangement successfully treated by a low dose of prednisone

Skin diseases in Asian individuals that you do not want to miss: A selection of unique or relatively more common conditions in Asian populations

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