Faculty Bibliography

BACKGROUND:Cutaneous (or "Metastatic") Crohn disease (CCD) is a rare and underrecognized disease characterized by cutaneous granulomatous inflammation. We describe patient demographics, clinical characteristics, histology, and treatment of 89 pediatric cases of CCD, including 78 previously reported and 11 new cases seen at four academic institutions. We emphasize the efficacy of biologic mono- and dual therapy. METHODS:PubMed identified cases using keywords including "metastatic Crohn disease" and "cutaneous Crohn disease". Patients were identified by retrospective review of the electronic health record including histopathologic diagnosis consistent with CCD. Chart review collected demographic, clinical, and histologic data. RESULTS:Most pediatric patients with CCD are male 55% (49/89), present with edema (73/89, 82%) and erythema (47/89, 53%) of the genitals (33/49, 67%), and have intestinal Crohn disease (69/89, 78%). Oral corticosteroids (53/75, 71%) and metronidazole (29/75, 39%) are the most frequently prescribed medications. Of the 17 patients treated with tumor necrosis factor (TNF)-blockade, 94% (16/17) had partial or total clearance. Ustekinumab resulted in clearance of cutaneous disease in two patients (2/3, 67%) and partial clearance in one patient (1/3, 33%). Two cases achieved total clearance with the use of dual biologic therapy defined as the use of two biologic therapies with differing mechanisms of action or the use of a biologic therapy and small molecule inhibitor. CONCLUSIONS:TNF blockade is an effective treatment for pediatric CCD, and interleukin-12/23 inhibitors may be similarly effective. Consideration of dual biologic therapy may be useful in pediatric patients requiring discordant therapies for their intestinal and cutaneous CD.

BACKGROUND/UNASSIGNED:Alopecic sarcoidosis is an uncommon cutaneous manifestation of sarcoidosis. Scarring and nonscarring alopecic sarcoidosis have been reported; however, information on the epidemiology, systemic disease associations, and treatment efficacy is limited. OBJECTIVE/UNASSIGNED:To address these gaps, we conducted a retrospective chart review and systematic literature review of alopecic sarcoidosis cases. METHODS/UNASSIGNED:Full-text English publications from PubMed, Scopus, and Google Scholar from inception to August 2023 were analyzed. Treatment evidence quality was assessed using the modified Oxford Centre for Evidence-Based Medicine rating scale. Three patients with biopsy-proven alopecic sarcoidosis were included as a case series, all demonstrating systemic sarcoidosis and 2 requiring multiple therapies. Among 1778 search results, 60 articles representing 77 cases of alopecic and scalp sarcoidosis were included. Patients were categorized into 4 distinct alopecic subgroups. Black patients constituted the majority of all subgroups. RESULTS/UNASSIGNED:Extracutaneous sarcoidosis burden was high across all alopecic subgroups, with ocular disease appearing overrepresented. Topical and oral corticosteroids were the main treatments. Though scarring alopecia patients had poor outcomes despite receiving immunomodulators/cx, limited data suggest potential efficacy of tumor necrosis factor-alpha inhibitors. LIMITATIONS/UNASSIGNED:This study has a small sample size. CONCLUSION/UNASSIGNED:Our findings underscore the importance of evidence-based strategies for improving alopecic sarcoidosis management. Prompt diagnosis and systemic evaluation, especially for scarring alopecia, are essential for timely intervention to optimize patient outcomes.

Low dose oral minoxidil (LDOM) is an efficacious and safe treatment for alopecia, however, a notable side effect is hypertrichosis. Spironolactone, known for treating hirsutism, is also used off-label for the treatment of certain forms of alopecia and may reduce LDOM-induced hypertrichosis. We performed a retrospective review of 54 patients seen at NYU Langone Health and compared hypertrichosis rates in female alopecia patients on LDOM monotherapy versus those on combination therapy with spironolactone. Among 54 patients, 37 received LDOM alone and 17 received the combination. Hypertrichosis developed in 33.3% of patients, with lower rates in the combination group (17.6% vs. 40.5% for monotherapy). Although not statistically significant, the trend suggests spironolactone may mitigate hypertrichosis. The study highlights the potential of combination therapy to address hypertrichosis and calls for larger studies to confirm these findings.

Sarcoidosis is a multisystem disorder characterised by granulomatous inflammation affecting various organs. The skin is commonly involved and can serve as an initial indicator of disease. While the precise aetiology of sarcoidosis remains elusive, evidence suggests involvement of T-helper type (TH)-1 and TH-17 pathways. Psoriasis shares common inflammatory pathways with sarcoidosis, prompting the repurposing of biologic therapies approved for psoriasis for off-label treatment of cutaneous sarcoidosis. However, this approach has raised concerns due to the development of granulomatous eruptions in some patients. We present a case of a patient with psoriasis who was diagnosed with systemic sarcoidosis while on ustekinumab. We review previous cases of ustekinumab-associated sarcoidosis and discuss the challenges associated with utilising biologic agents originally intended for psoriasis treatment for sarcoidosis.