Efficacy and safety of repository corticotropin injection for refractory cutaneous dermatomyositis: a prospective, open-label study.

OBJECTIVES: Cutaneous dermatomyositis (DM) is often refractory to multiple medications. Repository corticotropin injection (RCI) is FDA-approved for DM, but little is known about its efficacy and safety for treating cutaneous DM. We conducted a prospective, open-label trial assessing efficacy and safety of RCI for treating refractory cutaneous DM. METHODS: DM patients with moderate-to-severe cutaneous activity [Cutaneous Dermatomyositis Disease Area and Severity Index activity (CDASI-A)] >14 despite prior treatment with ≥2 systemic agents were enrolled. Patients were initiated on 80 u RCI twice weekly for 6 months. Primary outcomes included significant decreases in CDASI-A and Physician's Global Assessment (PGA) scores at 6 months. RESULTS: Of nineteen patients enrolled, fifteen patients (11 females, 4 males) with DM (7 classic, 8 amyopathic) completed 6 months of RCI treatment. Patients were treated with a median 3.0 systemic medications prior to enrolment and were taking a median of 2.0 systemic medications at enrolment. Median baseline CDASI-A score was 19.0 and median PGA activity score was 2.5/10. For patient-reported outcomes, baseline median patient global skin score (PtGSS) was 3.0/10 and median dermatology life quality index (DLQI) score was 7.0/10. At 6 months, there were statistically significant improvements in CDASI-A scores (median= 10.0), PGA scores (median= 0.8/10), PtGSS scores (median= 7.0) and DLQI scores (median= 2.0), among others. Adverse effects were mild. CONCLUSIONS: RCI treatment resulted in statistically significant and clinically meaningful improvement in cutaneous DM activity and quality of life. Our results suggest RCI is an effective, safe, and well-tolerated treatment for patients with refractory cutaneous dermatomyositis. CLINICAL TRIAL REGISTRATION: This clinical trial was registered with ClinicalTrials.gov (ClinicalTrials.gov Identifier: NCT01906372).

Sweet Syndrome Associated with Active Inflammatory Bowel Disease: A Case Series of a Rare Extra-intestinal Manifestation.

BACKGROUND: Cutaneous extra-intestinal manifestations (EIM) occur in up to 20% of patients with IBD. Information about Sweet syndrome (SS)'s clinical course as a rare cutaneous EIM in IBD is limited to case reports. We present the largest retrospective cohort on the occurrence and management of SS in IBD. STUDY: Electronic medical records and paper charts since 1980 were retrospectively reviewed at a large quaternary medical center to identify all adult IBD patients with histopathology-proven SS. Patient characteristics and clinical outcomes were evaluated. RESULTS: 25 IBD patients with SS were identified; 3 patients were assessed to have AZA-induced SS. The majority of SS patients were female. Median age at diagnosis was 47 years (IQR 33-54 years) and SS appeared at a median of 6.4 years after IBD diagnosis. IBD patients with SS had a high rate of complicated IBD phenotypes (75% extensive colitis in UC and 73% stricturing or penetrating disease in CD, with 100% colonic involvement), as well as frequent co-occurring EIMs (60%). SS correlated with global IBD disease activity. Corticosteroids were an effective therapy for SS in IBD. Recurrence rate of SS was 36%. CONCLUSION: Contrary to previous case reports, SS was a cutaneous EIM occurring late after diagnosis of IBD in our cohort, with occurrences paralleling global IBD disease activity. Although AZA-induced and IBD-associated SS were both effectively treated with corticosteroids, distinguishing them is relevant for future IBD treatment strategies.

Quantitative Assessment of Eosinophils in Dermatomyositis Skin Biopsies With Correlation of Eosinophils to Pruritus and Other Clinical Features.

ABSTRACT: The objective of this retrospective study was to analyze dermatomyositis skin biopsies for the presence of eosinophils and correlate this finding with other histopathologic and clinical characteristics. Cases of dermatomyositis evaluated in a single dermatologist's adult autoimmunity practice over a 2.5-year period were identified via ICD-10 diagnosis code. Dermatopathology archives were then searched for any corresponding biopsies consistent with dermatomyositis, and those identified were assessed for eosinophils, adnexal involvement, epidermal atrophy, dermal mucin, and basement membrane thickening. Histopathologic findings were correlated with key clinical features, including itch. A total of 39 biopsies from 17 patients were included. Eosinophils were noted in 44% of biopsies (n = 17) from 12 patients. Dermal mucin deposition and adnexal interface dermatitis were noted in 72% (n = 28) and 44% (n = 17) of biopsy specimens, respectively. Of 12 patients with eosinophils present in at least 1 biopsy specimen, 11 (92%) patients had a clinical history of pruritus of their skin lesions (P = 0.052). Limitations of this study include retrospective design and small number of patients.

Family history of psoriasis, psychological stressors, and tobacco use are associated with the development of tumor necrosis factor-α inhibitor-induced psoriasis: A case-control study.

BACKGROUND: Tumor necrosis factor-α inhibitor-induced psoriasis (TNFI psoriasis) is a paradoxical reaction characterized by development of a psoriasiform rash that mimics psoriasis vulgaris. Temporal onset variability and low incidence rates suggest that underlying risk factors or outside triggers have a role in TNFI psoriasis initiation. OBJECTIVES: We aimed to identify underlying risk factors and outside triggers associated with TNFI psoriasis onset. METHODS: This case-control study included 97 patients at a tertiary care center between 2003 and 2013 who developed TNFI psoriasis. Ninety-seven control patients were matched to age, sex, disease, TNF-α inhibitor, and length of time on treatment before TNFI psoriasis onset. Patient medical records were reviewed ≥6 months immediately preceding TNFI psoriasis onset (similar equivalent time point for matched controls) for information about potential risk factors and outside factors categorized as: (1) serologic abnormalities, (2) acute events, and (3) social factors. RESULTS: Compared with those of matched controls, odds ratios (ORs) were significantly higher in the TNFI psoriasis group for psoriasis family history (OR, 16.0) and acute psychological stressors (OR, 3.14) and marginally associated with tobacco use (OR, 1.76). CONCLUSIONS: Our results suggest that psoriasis family history, psychological stressors, and tobacco use might be risk factors for developing TNFI psoriasis. Performing detailed patient histories when considering TNFI therapy may be useful in identifying patients at risk for TNFI-psoriasis.

Patch-type granuloma annulare: An institution-based study of 23 cases.

BACKGROUND: Granuloma annulare (GA) is a skin disorder of uncertain etiology. Patch (type) GA is an uncommon variant of GA with a paucity of data characterizing it. We describe the features of 23 cases of patch GA. METHODS: The archives of dermatopathology were searched for cases of patch GA. The clinical history and morphology for each patient were reviewed. Only cases with patch clinical morphology were included. The clinical and histopathologic features were assessed including the pattern of granulomatous inflammation and presence of other inflammatory cell types. RESULTS: Most patients were female (19/23) with erythematous patches on the trunk and proximal extremities. The most common clinical differential diagnosis included mycosis fungoides (MF), morphea and contact dermatitis. Dyslipidemia was the most common comorbidity (30%), followed by diabetes (15%) and hypertension (15%). Histopathologic features included interstitial lymphocytes and histiocytes with dermal mucin. Two cases showed focal palisaded granulomas. Eosinophils and plasma cells were present in 1/3 of cases. CONCLUSION: Patch GA is an uncommon GA variant with an interstitial granulomatous histopathologic pattern that predominantly affects women over 50. It can mimic interstitial MF and early morphea both clinically and histopathologically. Awareness of this GA variant can help prevent misdiagnosis and inappropriate treatment for these patients.

Diagnoses of hospitalized patients with skin abnormalities prompting biopsy by consulting dermatologists: A 3-year review from a tertiary care center.

BACKGROUND: Dermatologists play an important role in diagnosing and managing hospitalized patients with cutaneous abnormalities. Skin biopsies remain an indispensable tool for aiding dermatologists in accurate diagnosis and treatment. We aimed to determine the range of conditions, and the most common conditions, prompting skin biopsy by dermatology hospital consultation (HCON) services to aid in evaluation of hospitalized patients. METHODS: All hospitalized patients seen by a single tertiary care center dermatology HCON service between 2015 and 2018 who had associated skin biopsies were identified. Histologic features and clinical diagnoses of each patient were classified into 13 histologic reaction pattern categories. RESULTS: Eight hundred and thirty one inpatients evaluated by our dermatology HCON service had 914 skin biopsies. The most frequent diagnostic categories prompting biopsy were vasculopathic (17.6%), interface dermatitis (16.5%), infectious (12.6%), and spongiotic dermatitis (10.9%). The most frequent diagnostic categories included drug reaction (13.2%), leukocytoclastic vasculitis (8.5%), skin cancer (5.4%), graft-vs-host disease (3.5%), connective tissue disease (3.3%), and calciphylaxis (3.0%). CONCLUSION: Our study suggests a variety of serious diseases affecting inpatients prompts biopsy by dermatology consultation services. Educational curricula for dermatology and pathology residents, fellows, and staff designed with these data may enhance knowledge that improves the quality of inpatient dermatology care.