Title:Panniculitis: A Cardinal Sign of Autoinflammation
Volume: 20
Issue: 4
Author(s): Tiago Borges*Sérgio Silva
Affiliation:
- Trofa Saúde Gaia, Rua Fernão de Magalhães nº 2 Fr E, 4404-501 Vila Nova de Gaia, Portugal
Keywords:
Autoinflammatory diseases, glucocorticoids, inflammasomes, interleukin-1, innate immunity, Janus kinase inhibitors, panniculitis, proteasome-associated autoinflammatory syndromes.
Abstract:
Panniculitis was first described in the nineteenth century and is characterized by inflammation
of the subcutaneous fat. It may be categorized in septal or lobular subtypes, but other histopathological
features (e.g., presence of vasculitis, nature of inflammatory infiltrates, characteristics
of fat necrosis) are also important for diagnostic purposes. Clinically, panniculitis is characterized
by the presence of subcutaneous nodules, and both ulcerative and nonulcerative clinical subtypes
have been proposed. In this review, we aimed to describe the occurrence of panniculitis in
autoinflammatory disorders (AIDs) and related diseases.
Among monogenic AIDs, panniculitis is common in IFN-mediated disorders. Panniculitis is a distinctive
feature in proteasome-associated autoinflammatory syndromes (PRAAS), including chronic
atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome
and Nakajo-Nishimura syndrome. On the other hand, erythema nodosum corresponds to
the most common clinical form of panniculitis and is common in polygenic AIDs, such as Behçet’s
syndrome, inflammatory bowel disease, and sarcoidosis. Cytophagic histiocytic panniculitis,
lipoatrophic panniculitis of children, and otulipenia are rare disorders that may also present
with inflammation of the subcutaneous fat. Therefore, panniculitis can identify a specific subgroup
of patients with AIDs and may potentially be regarded as a cardinal sign of autoinflammation.
