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Background: Reactive granulomatous dermatitis (RGD) is an umbrella term used to describe interstitial granulomatous dermatitis (IGD), palisaded neutrophilic and granulomatous dermatitis (PNGD), and interstitial granulomatous drug eruption (IGDR)., Objective: The aim of this study was to describe systemic associations of RGD, explore possible associations between histopathologic findings and systemic RGD associations and determine clinical relevance of RGD subtypes., Methods: We retrospectively studied clinical and histopathologic characteristics of patients with RGD from 1990 through 2020., Results: Of 65 patients with RGD (41 women, 24 men; median age at diagnosis, 62 years), 37 had IGD, 26 had PNGD, and 2 had IGDR. Fifty patients (76.9%) had an associated systemic condition; rheumatologic conditions were identified for 34 (52.3%) patients. The associated systemic condition occurred before RGD in approximately 75% of patients. Statistical analyses did not show significant associations between specific subtypes of RGD and systemic diseases or treatment response, and specific histopathologic findings were not predictive of an associated systemic disease., Conclusions: Although most patients with RGD had an associated systemic condition, subtypes of RGD did not correlate with systemic associations, lending support to the use of the umbrella term RGD., (© 2022 European Academy of Dermatology and Venereology.)

Importance: Vaccine-derived and wild-type rubella virus (RuV) has been identified within granulomas in patients with inborn errors of immunity, but has not been described in granulomas of healthy adults., Objective: To determine the association between RuV and atypical granulomatous inflammation in immune-competent adults., Design, Setting, and Participants: This case series, conducted in US academic dermatology clinics from January 2019 to January 2021, investigated the presence of RuV in skin specimens using RuV immunofluorescent staining of paraffin-embedded tissue sections, real-time reverse-transcription polymerase chain reaction, whole-genome sequencing with phylogenetic analyses, and cell culture by the US Centers for Disease Control and Prevention. Rubella immunoglobulin G, immunoglobulin M enzyme-linked immunoassay, and viral neutralization assays were performed for the sera of immunocompetent individuals with treatment refractory cutaneous granulomas and histopathology demonstrating atypical palisaded and necrotizing granulomas. Clinical immune evaluation was performed., Main Outcomes and Measures: Identification, genotyping, and culture of vaccine-derived and wild-type RuV within granulomatous dermatitis of otherwise clinically immune competent adults., Results: Of the 4 total immunocompetent participants, 3 (75%) were women, and the mean (range) age was 61.5 (49.0-73.0) years. The RuV capsid protein was detected by immunohistochemistry in cutaneous granulomas. The presence of RuV RNA was confirmed by real-time reverse-transcription polymerase chain reaction in fresh-frozen skin biopsies and whole-genome sequencing. Phylogenetic analysis of the RuV sequences showed vaccine-derived RuV in 3 cases and wild-type RuV in 1. Live RuV was recovered from the affected skin in 2 participants. Immunology workup results demonstrated no primary immune deficiencies., Conclusions and Relevance: The case series study results suggest that RuV (vaccine derived and wild type) can persist for years in cutaneous granulomas in clinically immunocompetent adults and is associated with atypical (palisaded and necrotizing type) chronic cutaneous granulomas. These findings represent a potential paradigm shift in the evaluation, workup, and management of atypical granulomatous dermatitis and raises questions regarding the potential transmissibility of persistent live RuV.

Common variable immunodeficiency disorder is a primary immunodeficiency disorder characterized by reduced levels of serum immunoglobulins and impaired antibody response. This condition may be associated with development of noninfectious granulomatous dermatitis of the skin which may be disfiguring and destructive. There are no published guidelines for the treatment of cutaneous granulomas in this patient population. In recent studies, rubella virus-positive cells in granulomas were localized to M2 macrophages which have an important role in wound healing and the secretion of immunoregulatory cytokines. We present a case of treatment-refractory, disfiguring common variable immunodeficiency disorder-associated granulomatous dermatitis. Immunofluorescence microscopy of the biopsy specimen confirmed the presence of rubella vaccine capsid proteins in M2 macrophages within the granuloma, a newly recognized phenomenon in this patient population. This knowledge may serve to identify future therapeutic targets or preventative strategies for granulomatous dermatitis in patients with primary immunodeficiency disorder.

Objective: To characterize the clinical and histologic presentation of reactive granulomatous dermatitis (RGD) in the pediatric population., Methods: In this multicenter retrospective chart review, 7 pediatric patients with biopsy-proven RGD were identified. Photographs, histology reports, and clinical course were reviewed to discover patterns in demographics, comorbid conditions, autoimmune sequelae, drug exposures, infections, morphology, and histologic features., Results: Overall, 7 patients were included and analyzed. Most were female and Hispanic. All presented with a similar dermatologic phenotype previously described in the adult literature including macular erythema and annular, pink to violaceous, edematous papules and plaques, often involving proximal extremities and extensor joints. All biopsies demonstrated variable collagen alteration and a perivascular interstitial infiltrate of histiocytes with or without mucin. Neutrophils or karyorrhexic debris were present in 4/7 of the biopsies, and eosinophils were occasionally seen (2/7 cases). In all cases, RGD was associated with active SLE or led to a new diagnosis, and initiation of systemic treatment improved cutaneous disease., Conclusions: Pediatric RGD was more common in female patients and ethnic minorities, and strongly associated with SLE. Clinical and histologic presentations were consistent across all cases with only minor variations, suggesting that recognition and confirmation might be expedited by familiarity with these dominant patterns. Diagnosis of RGD in pediatric patients should prompt screening for SLE., (© 2020 Wiley Periodicals, Inc.)

Background: Lichenoid granulomatous dermatitis (LGD) is an uncommon reaction pattern for which clinical correlates can be difficult to establish. LGD combines vacuolar degeneration with variable types of granulomas., Objective: To determine clinical correlates of LGD., Methods: The laboratory information systems at the University of Florida, the Medical College of Wisconsin, and Inform Diagnostics Research Institute were queried to identify 56 cases of LGD. Cases were reviewed for information regarding eosinophils, plasma cells, deep perivascular infiltrates, granuloma subtype, parakeratosis, epidermal atrophy, psoriasiform epidermal changes, pseudoepitheliomatous hyperplasia, periadnexal inflammation, vasculitis, and red blood cell extravasation., Results: The most common clinical correlates were drug eruption (39.3%, n = 22) and lichenoid keratosis (19.6%, n = 11). Tattoo reaction, postherpetic dermatitis, and scabies or postscabietic dermatitis each accounted for 7.1% (n = 4) of cases. Pigmented purpuric dermatosis and lichen striatus each accounted for 5.4% (n = 3) of cases. Dermal eosinophils (P = .005) and psoriasiform epidermal changes (P = .055) were associated with drug hypersensitivity. Perineural (P = .049) and perifollicular (P = .003) inflammation were associated with tattoo reaction and postherpetic dermatitis. Red blood cell extravasation was helpful in cases of pigmented purpuric dermatosis (P = .049)., Limitations: This study is limited by its retrospective nature and statistical power., Conclusion: Dermal eosinophilia, psoriasiform epidermal changes, periadnexal inflammation, and red blood cell extravasation might aid in the clinical diagnosis of patients with LGD., (Copyright © 2019 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)