Kim, Grace Y.

ABSTRACT Orofacial granulomatosis (OFG) is a rare syndrome that can occur in association with Crohn's disease (CD). The electronic medical record was searched for “OFG” and “CD.” A total of 297 patients were identified, and relevant data were abstracted. Five patients met inclusion criteria. Most (80%) had ileocolonic CD with nonstricturing/nonpenetrating phenotype. The most common treatments included intralesional corticosteroids (4 patients), oral corticosteroids (3 patients), and anti-TNF agents, specifically infliximab (3 patients). Intralesional corticosteroid administration was followed by partial response in 3 patients, but recurrences were common following initial injection. One patient had no response to topical or oral corticosteroids, antihistamines, or oral antibiotics, but partial response to intralesional corticosteroids and infliximab. None of the patients had complete response to any therapies directed at this condition. OFG may be characterized by partial response, often requiring long-term therapy. Additional investigations into novel treatments may improve future clinical outcomes.

BACKGROUND Extramammary Paget disease (EMPD) is a rare, slow growing neoplasm that presents most commonly in the anogenital region of older adults. OBJECTIVE To analyze the difference in local recurrence rates of EMPD in patients treated with wide local excision (WLE) versus Mohs micrographic surgery (MMS). MATERIALS AND METHODS A systematic review of the literature and meta-analysis were performed. Inclusion criteria were adults greater than 18 years of age with a diagnosis of EMPD who have undergone surgical intervention and had follow-up data. Studies were independently reviewed by 2 coinvestigators with discrepancies resolved by the principal investigator. RESULTS Twenty-seven studies met the inclusion criteria. Patients had a 2.67 times higher chance of local recurrence after WLE than MMS (95% confidence interval [CI]:1.47, 4.85; p = .001). Meta-analysis of single-arm studies revealed a 7.3% local recurrence rate after MMS (95% CI: 0.039, 0.107; p < .001) versus a 26.3% recurrence rate after WLE (95% CI: 0.149, 0.376; p < .001). After excluding recurrent tumors, the odds ratio for recurrence in WLE versus MMS was 2.3 (95% CI: 0.285, 18.43, p = .435). CONCLUSION There is a clinically and statistically increased risk of local recurrence of EMPD after WLE compared with MMS.

AbstractHidradenoma papilliferum (HP) is a benign adnexal neoplasm of the vulva that typically presents as a unilateral, flesh‐colored papule in the labium majus in middle‐aged Caucasian women. It is considered to be a close counterpart of the intraductal papilloma of the breast. Malignant transformation is rare with few reports in the literature. We present a case of vulvar mammary‐type apocrine hidradenocarcinoma arising in an HP.

Abstract Background Mobile applications allow health care providers to capture point-of-care medical photographs and transfer them to the electronic health record (EHR). It is unclear how providers use these photographs or how they affect clinical care. Objectives We aimed to understand the content, purpose, and outcomes of point-of-care medical photography performed in the pediatric emergency department (ED) at large academic medical center. Methods A retrospective chart review was conducted of patients <21 years of age who were seen in the ED and photographed between March 29, 2015 and July 1, 2017 using a secure smartphone application integrated with the EHR. Inter-rater agreement and reliability between the two reviewers was assessed for the first 50 charts, and any discrepancies in interpretation were resolved before proceeding with the remaining data abstraction. The documented rationale for photography, content of photographs, and outcomes were recorded. Results We identified 619 clinical encounters involving photographs of 605 patients who were eligible for inclusion. Skin was photographed in 499 (81%). The most common finding was rash (N = 177; 29%). Photos were of acceptable quality, with 569 (94%) achieving a score between 4 and 5 out of 5. The primary use of photography was documentation (N = 334; 54%), though teleconsultation was noted in 38 (6%). Nearly one-third (N = 187; 30%) of patients were seen in the ED or outpatient clinic for any reason within 2 weeks, and in 25 (13%), clinical notes explicitly referenced the initial photograph(s). In 53 (9%) cases, patients were photographed at a clinical visit in the subsequent 2 weeks, suggesting that photography was used to track changes over time. Conclusion Documentation of findings using mobile point-of-care photography allows for high-fidelity documentation and facilitates continuity of care.

BackgroundGraft‐versus‐host disease (GVHD) following solid organ transplantation (SOT) is extremely rare and infrequently described in the dermatologic literature.MethodsWe performed a retrospective clinicopathologic review of our institution's experience with patients diagnosed with SOT‐associated GVHD (SOT GVHD) (May 1, 1996 to September 1, 2017).ResultsOf nine patients with SOT GVHD, seven had undergone liver transplantation, while two had undergone lung transplantation. All presented initially with a skin eruption, which developed an average of 63 days (range: 11‐162 days) post transplant. The average time to diagnosis following the onset of the skin eruption was 12 days (range: 0‐54 days). Diagnosis was often delayed because of a competing diagnosis of drug reaction. Frequent skin findings included pruritic erythematous to violaceous macules and papules with desquamation. Histopathology showed vacuolar interface dermatitis in 12 of 15 cases (80.0%). Of the 11 specimens in which a hair follicle was present for evaluation, vacuolar interface changes around the hair follicle were present in eight (72.7%) cases. Seven patients (77.8%) died from complications during the follow‐up period.ConclusionsSOT GVHD presents initially with skin involvement, is associated with vacuolar interface changes on skin biopsy, and is associated with a high mortality rate. Clinicopathologic correlation is required for accurate diagnosis.