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Lipophagic panniculitis of childhood
R K Winkelmann1, M T McEvoy, M S Peters
1Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
Insights
This study describes lipophagic granulomatous lipoatrophy in children, characterized by skin lesions and fat tissue loss. Corticosteroids may be an effective treatment for this condition.
Area of Science:
- Dermatology
- Pathology
Background:
- Lipophagic granulomatous lipoatrophy, previously termed Weber-Christian disease or Rothmann-Makai syndrome, is a rare condition affecting subcutaneous fat.
- Understanding its distinct clinical and histological features is crucial for accurate diagnosis and management.
Observation:
- Presents three pediatric cases of lipophagic granulomatous lipoatrophy.
- Observed characteristic erythematous lesions on limbs, followed by subcutaneous fat atrophy.
- Commonly associated symptoms include fever and edema.
Findings:
- Histological examination reveals lipophagic histiocytes and giant cells replacing fat lobules.
- Absence of vasculitis or phlebitis is a key diagnostic feature.
- The condition is best defined by its unique clinical presentation, disease course, and histopathology.
Implications:
- Highlights the importance of recognizing lipophagic granulomatous lipoatrophy as a distinct entity.
- Suggests corticosteroids as a potential therapeutic option for affected patients.
- Emphasizes the need for further research into the pathogenesis and optimal treatment strategies.
Abstract:
The cases of three patients with an atrophic lipophagic granulomatous lipoatrophy are described and compared with 14 similar cases in the literature. In affected children erythematous lesions of the arms and legs usually occur, after which subcutaneous atrophy develops. Fever and edema are common. The histologic feature is a replacement of the fat lobule by lipophagic histiocytes and giant cells. No vasculitis or phlebitis is present. Previously, such cases have been called Weber-Christian disease or Rothmann-Makai syndrome, but they are best characterized by the distinctive clinical lesions, the course, and histologic findings. Corticosteroids apparently have been effective in some patients.