Panniculitis in Children

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.

Fasciitis-panniculitis syndrome with autoantibodies reacting to collagen fibers: A case report

“…Raynaud's phenomenon, anti-Scl-70, anti-centromere, and anti-U1-RNP autoantibodies were all negative in the patient, which are atypical for systemic sclerosis along with the pediatric onset. DADA2 has been reported to be one of the causes of cutaneous polyarteritis nodosa (CPN) [5,6] and we did observe necrotizing arteritis in the patient's biopsy specimen. DADA2 is an autosomal recessive genetic disorder caused by a heterozygous missense mutation of ADA2, which decreases the enzymatic activity of ADA2, a protein necessary for the differentiation of vascular endothelial cells.…”

“…En bloc biopsy of the skin and fascia showed thickening of collagenous bers in the fat tissue, in ltration of in ammatory cells consisting mainly of neutrophils and lymphocytes from the fat tissue to the fascia, as well as necrotizing vasculitis, leading to the diagnosis of FPS. From a clinical point of view, systemic sclerosis should be a diagnosis of exclusion, and from a pathological point of view, adenosine deaminase 2 de ciency (DADA2) and otulipenia should be considered as possible causes [5]. Raynaud's phenomenon, anti-Scl-70, anti-centromere, and anti-U1-RNP autoantibodies were all negative in the patient, which are atypical for systemic sclerosis along with the pediatric onset.…”

“…Otulipenia is an autosomal recessive autoimmune disease caused by mutations in the gene encoding OTULIN, a ubiquitin kinase involved in the NF-κB signal transduction pathway [5]. This mutation causes constant activation of the NF-κB signalling in broblasts and mononuclear cells, resulting in symptoms such as fever, arthralgia, and lymphadenopathy due to a cytokine storm [12,13].…”

“…This mutation causes constant activation of the NF-κB signalling in broblasts and mononuclear cells, resulting in symptoms such as fever, arthralgia, and lymphadenopathy due to a cytokine storm [12,13]. The main pathological changes of oulipenia is observed in medium-sized vessels [5]. In the present case, the characteristic skin manifestations of CPN were clinically absent, collagen bers in the fat tissue were increased and thickened, and there was the in ltration of in ammatory cells from the fat tissue to the fascia, thus excluding CPN from the possible diagnoses.…”

New Insights in Paediatric Dermatopathology

“…The article from Moulonguet, I. [ 14 ] is the most recent and comprehensive review on this difficult subject.…”