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Histologic Patterns and Clues to Autoinflammatory Diseases in Children: What a Cutaneous Biopsy Can Tell Us

1
Department of Dermatology and Dermatopathology, Saint-Pierre, Brugmann and Queen Fabiola Children’s University Hospitals, Université Libre de Bruxelles, 1000 Brussels, Belgium
2
Department of Pathology, Laboratoire Luc Olivier, 5380 Fernelmont, Belgium
3
Department of Dermatology, Oregon Health and Sciences University, Portland, OR 97239, USA
4
Department of Pathology, Oregon Health and Sciences University, Portland, OR 97239, USA
5
CTA Pathology, Portland, OR 97223, USA
*
Author to whom correspondence should be addressed.
Academic Editors: Sylvie Fraitag and Gürkan Kaya
Received: 22 April 2021 / Revised: 30 May 2021 / Accepted: 31 May 2021 / Published: 8 June 2021
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
Autoinflammation is defined by aberrant, antigen-independent activation of the innate immune signaling pathways. This leads to increased, pro-inflammatory cytokine expression and subsequent inflammation. In contrast, autoimmune and allergic diseases are antigen-directed immune responses from activation of the adaptive immune system. The innate and adaptive immune signaling pathways are closely interconnected. The group of ‘complex multigenic diseases’ are a result of mutual dysregulation of both the autoinflammatory and autoimmune physiologic components. In contrast, monogenic autoinflammatory syndromes (MAIS) result from single mutations and are exclusively autoinflammatory in their pathogenesis. Studying the clinical and histopathological findings for the various MAIS explains the phenotypical correlates of their specific mutations. This review aims to group the histopathologic clues for autoinflammation into three recognizable patterns. The presence of these histologic patterns in a pediatric patient with recurrent fevers and systemic inflammation should raise suspicion of an autoinflammatory component in MAIS, or, more frequently, in a complex multigenic disease. The three major histopathological patterns seen in autoinflammation are as follows: (i) the ‘neutrophilic’ pattern, seen in urticarial neutrophilic dermatosis, pustular psoriasis, aseptic neutrophilic folliculitis, and Sweet’s syndrome; (ii) the ‘vasculitic’ pattern seen in small vessel-vasculitis (including hypersensitivity/leukocytoclastic vasculitis, thrombosing microangiopathy and lymphocytic vasculitis), and intermediate-sized vessel vasculitis, mimicking polyarteritis nodosa; and (iii) the ‘granulomatous’ pattern. Beyond these three patterns, there are additional histopathologic clues, which are detailed below. It is important for a dermatopathologist to recognize the patterns of autoinflammation, so that a diagnosis of MAIS or complex multigenic diseases may be obtained. Finally, careful histopathologic analyses could contribute to a better understanding of the various clinical manifestations of autoinflammation. View Full-Text
Keywords: autoinflammation; cryopyrin; inflammasome; interferonopathies; pustular psoriasis; lupus erythematosus; neutrophilic urticarial dermatitis; pyoderma gangrenosum; suppurative hidradenitis autoinflammation; cryopyrin; inflammasome; interferonopathies; pustular psoriasis; lupus erythematosus; neutrophilic urticarial dermatitis; pyoderma gangrenosum; suppurative hidradenitis
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MDPI and ACS Style

Kolivras, A.; Meiers, I.; Sass, U.; Thompson, C.T. Histologic Patterns and Clues to Autoinflammatory Diseases in Children: What a Cutaneous Biopsy Can Tell Us. Dermatopathology 2021, 8, 202-220. https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020026

AMA Style

Kolivras A, Meiers I, Sass U, Thompson CT. Histologic Patterns and Clues to Autoinflammatory Diseases in Children: What a Cutaneous Biopsy Can Tell Us. Dermatopathology. 2021; 8(2):202-220. https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020026

Chicago/Turabian Style

Kolivras, Athanassios, Isabelle Meiers, Ursula Sass, and Curtis T. Thompson 2021. "Histologic Patterns and Clues to Autoinflammatory Diseases in Children: What a Cutaneous Biopsy Can Tell Us" Dermatopathology 8, no. 2: 202-220. https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020026

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