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Panniculitis in Children
Isabelle Moulonguet1,2, Sylvie Fraitag3
1Cabinet de Dermatopathologie Mathurin Moreau, 75019 Paris, France.
Insights
Panniculitis, rare childhood inflammatory diseases of subcutaneous fat, present as tender nodules. Early screening for immune disorders is crucial for unexplained cases in young children.
Area of Science:
- Pediatric Dermatology
- Rheumatology
- Immunology
Background:
- Panniculitides are diverse inflammatory conditions affecting subcutaneous adipose tissue.
- While rare in children, they can be primary or secondary to other conditions.
- Several new pediatric panniculitis entities have emerged recently.
Purpose of the Study:
- To review childhood panniculitides, emphasizing newly described forms.
- To highlight the diagnostic approach and classification of these rare disorders.
- To underscore the importance of investigating immune disorders in children with unexplained panniculitis.
Main Methods:
- Literature review focusing on pediatric panniculitis.
- Analysis of clinical presentations, etiologies, and histopathology.
- Discussion of the link between panniculitis and immune system disorders.
Main Results:
- Panniculitis in children often presents as tender, erythematous subcutaneous nodules.
- Lobular panniculitis is common in pediatric cases.
- Panniculitis can be an early sign of autoimmune diseases and primary immunodeficiencies.
Conclusions:
- Histopathological assessment is often necessary for diagnosis and classification.
- Unexplained panniculitis in young children warrants screening for monogenic immune disorders.
- Understanding newly described entities is vital for pediatric rheumatologists and dermatologists.
Abstract:
Panniculitides form a heterogenous group of inflammatory diseases that involve the subcutaneous adipose tissue. These disorders are rare in children and have many aetiologies. As in adults, the panniculitis can be the primary process in a systemic disorder or a secondary process that results from infection, trauma or exposure to medication. Some types of panniculitis are seen more commonly or exclusively in children, and several new entities have been described in recent years. Most types of panniculitis have the same clinical presentation (regardless of the aetiology), with tender, erythematous subcutaneous nodules. Although the patient's age and the lesion site provide information, a histopathological assessment is sometimes required for a definitive diagnosis and classification of the disorder. In children, most panniculitides are lobular. At present, autoimmune inflammatory diseases and primary immunodeficiencies have been better characterised; panniculitis can be the presenting symptom in some of these settings. Unexplained panniculitis in a young child should prompt a detailed screen for monogenic immune disorders because the latter usually manifest themselves early in life. Here, we review forms of panniculitis that occur primarily in children, with a focus on newly described entities.
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