Background VEXAS (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease with frequent cutaneous manifestations. Methods We conducted a retrospective study of all patients with genetically confirmed VEXAS syndrome seen at our institution. Available clinical photographs and skin biopsy slides were reviewed. Results Cutaneous manifestations developed in 22/25 (88%) patients with VEXAS syndrome. From this group, 10/22 (45%) developed skin involvement before or at the time of other clinical features of VEXAS. Twenty distinct dermatologic presentations of VEXAS from 14 patients were reviewed, and histopathologic patterns were classified as follows: neutrophilic urticarial dermatosis (n = 5, 25%), leukocytoclastic/urticarial vasculitis (n = 4, 20%), urticarial tissue reaction (n = 4, 20%), neutrophilic dermatosis (n = 3, 15%), neutrophilic panniculitis (n = 2, 10%), and nonspecific chronic septal panniculitis (n = 2, 10%). Common systemic findings included macrocytic anemia (96%), fever (88%), thrombocytopenia (76%), weight loss (76%), ocular inflammation (64%), pulmonary infiltrates (56%), deep venous thrombosis or pulmonary embolism (52%), and inflammatory arthritis (52%). Conclusions Cutaneous involvement is a common feature of VEXAS syndrome, and histopathologic findings exist on a spectrum of neutrophilic inflammatory dermatoses.

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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.

Background/ObjectiveThere are few studies examining pediatric scarring alopecia. The objective of this study is to characterize the clinicopathologic findings, comorbidities, and treatment outcomes of pediatric patients with scarring alopecia.MethodsRetrospective review of patients under age 18 diagnosed with scarring alopecia at Mayo Clinic from 01/01/1992 through 02/05/2019.Results27 patients met inclusion criteria with a mean age of 11.2 years and a racial breakdown of 85.2% (23) White, 11.1% (3) Black, and 3.7% (1) Multiracial. Clinical scarring was noted in most (23, 85.2%). Biopsy confirmed the diagnosis in most (24, 88.9%). The most common diagnoses were folliculitis decalvans (6, 22.2%), lichen planopilaris (6, 22.2%), aplasia cutis congenita (4, 14.8%), tinea capitis (4, 14.8%), and morphea (3, 11.1%). Comorbid depression (6, 22.2%) and anxiety (6, 22.2%) were prevalent. Of the patients who received follow‐up, most who pursued treatment achieved stabilization (55.5%) or slowing of progression (27.8%), with 44.4% of those treated experiencing regrowth. Mean time to stabilization in the treated population was 19.6 months. Two patients did not pursue treatment, but received follow‐up and these untreated patients did not experience hair regrowth.ConclusionsMost patients presented with clinically evident primary scarring alopecia. Biopsy may confirm the diagnosis. Active treatment should be pursued, and successful treatment often requires combination therapies. Time to stabilization often takes years. Screening for depression and anxiety should be pursued.

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SDRIFE), palmoplantar erythrodysesthesia, toxic epidermal necrolysis, and bullous dermatosis, mainly pemphigus-like lesions. 2 BP and EV-induced bullae can be clinically indistinguishable.However, histology may allow the distinction. A lichenoid pattern with basal vacuolization and interface dermatitis is observed in EV-induced bullous reactions. IFI studies in EV dermatosis are negative for BP180, BP230, Dsg1, and Dsg3 autoantibodies.EV-induced bullous dermatosis is thought to be caused by the disruption of nectin-4 at the suprabasal layers of the epidermis, where it acts as a cell-to-cell adhesion protein. 2,3 Differential diagnosis included bullosis diabeticorum and pseudoporphyria, among others. The former is a specific bullous disease occurring in patients with diabetes mellitus usually associated with peripheral neuropathy. It is usually manifested by acral asymptomatic blisters which heal slowly after 2-6 weeks. Although histology was initially described as epidermal blisters, recent publications have also reported subepidermal bullae. DIF and IFI studies are negative. 4 Pseudoporphyria is a photodistributed bullous disorder with clinical and histological features that resemble porphyria cutanea tarda. It was originally described in patients using nonsteroidal anti-inflammatory drugs, antibiotics, diuretics, and suffering from chronic renal failure among others. Clinically, it may present as vesicles, bullae, skin fragility, milia, and scarring occurring on sun-exposed skin. Histology shows subepidermal bullae with or without festooning of dermal papillae and scant to mild lymphocytic perivascular infiltrate. DIF demonstrates a granular deposit of C3 and IgG at the dermoepidermal junction. IFI studies are negative. 5 We would like to highlight the importance of considering EV in the differential diagnosis of drug-induced bullous dermatosis.EV is a novel medication recently introduced for urothelial cancer treatment, which will gain importance in the near future; hence, it is important to be aware of possible new side effects.

Background Lichen planopilaris (LPP) is a scarring alopecia rarely described in men. Objective To investigate the clinical and histopathologic features of LPP in men. Methods We performed a retrospective cohort study of male patients with LPP seen at Mayo Clinic between 1992 and 2016. Results Nineteen men with biopsy‐confirmed LPP were included. The disease most commonly presented with diffuse (42.1%) or vertex scalp (42.1%) involvement. None of the patients had eyebrow or body hair involvement. Perifollicular erythema (94.7%) and pruritus (57.9%) were the most frequent clinical findings. Androgenetic alopecia (AGA) co‐occurred in 26.3% of patients. Mucosal lichen planus was found in four patients (21.1%). Thyroid disease occurred in three patients (15.8%). Disease improvement (47.3%) occurred with combination topical and systemic therapy, topical clobetasol monotherapy, and minocycline monotherapy. Conclusions LPP in men has similar clinical and histologic presentations as reported in women. Nonscalp hair loss appears less likely in men with classic LPP than reported in men with frontal fibrosing alopecia, while mucosal lichen planus and thyroid disease appear to be more common in classic LPP. Men with AGA can present with new‐onset concomitant LPP. Limitations included small study size, variable follow‐up, and lack of standardized clinical assessment due the study’s retrospective nature.