Lipodystrophia Centrifugalis Abdominalis Infantilis: A Case Report

Llistosella, E; Puig, L.; Pérez, Ferràn

doi:10.1111/j.1525-1470.1997.tb00241.x

Cited by 10 publications

(12 citation statements)

References 9 publications

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“…They described five young Japanese girls with circumscribed depressed skin lesions affecting the groin and lower abdomen. Subsequent reports originate almost exclusively from Japan and Asia, 2,3 but a handful of cases involving Caucasian children do exist 4,5 . Most cases present below age of three, 6 although three cases have been reported in adults 6 .…”

Section: Discussionmentioning

confidence: 99%

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Lipodystrophia centrifugalis abdominalis infantilis in a Caucasian girl: report of a case with perieccrine inflammation

2008

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Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a rare, self-limiting disease typically affecting Asian children around the age of three. In classic cases, patients present with a hypopigmented patch with central atrophy and an erythematous border in the groin area. We present a case of LCAI affecting a female Caucasian toddler that shows striking perieccrine inflammation, a finding not previously reported in this entity. LCAI has been rarely reported in Caucasian children; as a result, there is sparse literature on the histopathological findings in this subset. This case illustrates how significant deep dermal and perieccrine inflammation can be seen in this condition, thus broadening the histopathological spectrum of this disorder.

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Section: Discussionmentioning

confidence: 99%

“…Subsequent reports originate almost exclusively from Japan and Asia, 2,3 but a handful of cases involving Caucasian children do exist. 4,5 Most cases present below age of three, 6 although three cases have been reported in adults. 6 Girls outnumber boys in the ratio of 2 : 1.…”

Section: Discussionmentioning

confidence: 99%

Lipodystrophia centrifugalis abdominalis infantilis in a Caucasian girl: report of a case with perieccrine inflammation

2008

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“…Treatment of LCA is ineffective (5–18). In previous studies, oral penicillamine, systemic prednisolone, topical corticosteroids, and topical dimethyl sulfoxide were used without benefit (5–18). In one of our patients, photochemotherapy resulted in softening and cessation of the enlargement without notable histologic change.…”

Section: Discussionmentioning

confidence: 99%

“…Treatment of LCA is ineffective (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). In previous studies, oral penicillamine, systemic prednisolone, topical corticosteroids, and topical dimethyl sulfoxide were used without benefit (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”

Section: Discussionmentioning

confidence: 99%

Lipodystrophia Centrifugalis Abdominalis in Korea

Chang

Bae

Jeong

et al. 2004

Pediatric Dermatology

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Lipodystrophia centrifugalis abdominalis infantilis is a rare disorder described exclusively in Asians. Thirty Korean patients (4 women, 20 girls, and 6 boys) with a clinical and/or histopathologic diagnosis of this disorder were evaluated. The mean age of onset was 6.2 years. Infantile or congenital onset was seen in only three girls and two boys. In 50% of females, onset was after 5 years of age. All patients had involvement of the lower abdomen and groin both as the site of onset and as the main site. Histopathologic analysis showed loss of fat with or without an infiltrate of lymphocytes and histiocytes in the subcutis. Only one patient responded to treatment, an adult female who received photochemotherapy with softening of the lesions and symptomatic improvement. In previous reports, the age of onset of this disorder was at an age younger than 5 years but older than infants, making the term lipodystrophia centrifugalis abdominalis juvenilis more appropriate. In our series, 50% of females had onset after 5 years of age and 12.5% after 18 years of age. In one woman, the lesion that developed at 4 years of age progressed until adulthood. Our study of affected Korean patients showed a fourfold female predilection and later onset in female patients.

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“…The differential diagnosis of LCAI includes progressive lipodystrophy, insulin lipoatrophy, morphea, and atrophoderma of Pasini and Pierini (13). The characteristic clinical features of LCAI and the ethnic background of the patient will usually point to the correct diagnosis.…”

Section: Discussionmentioning

confidence: 99%