A case series of hydroxychloroquine exacerbating the dermatomyositis rash.

Hydroxychloroquine (HCQ) is an antimalarial agent that is commonly used in the management of rheumatic skin disease. Few reports exist documenting exacerbation of dermatomyositis (DM) related to HCQ. Herein, we describe three adult patients with worsening DM cutaneous disease after starting HCQ and resolution or improvement with cessation. The time to exacerbation ranged from two weeks to nine months after the initiation of HCQ 400mg/day. Two of the three patients had antibodies to transcription intermediary factor 1γ (TIF1γ) and the other had antibodies to anti-nuclear matrix protein 2 (NXP2). After discontinuation of HCQ, the time to improvement or resolution of cutaneous symptoms ranged from six weeks to six months. Hydroxychloroquine may be associated with worsening cutaneous features in DM. In patients who are not improving despite escalation of immunosuppressive medications, or are worsening, we recommend a trial of discontinuing HCQ.

Fluorescent Imaging as a Component of Diagnosing Pyoderma Gangrenosum: A Case Report.

ABSTRACT: A 64-year-old White woman was admitted to the hospital with complaint of progressive right hip ulceration at the wound site following a total right hip arthroplasty. Initial history and physical examination gave a leading differential diagnosis of pyoderma gangrenosum. Until recently, the exclusion of infection for pyoderma gangrenosum has been largely clinical and supported by cultures/biopsies demonstrating the absence of infection. The MolecuLight i:X (MolecuLight, Toronto, Ontario, Canada) is a novel bedside fluorescent imaging device capable of determining the bacterial burden within a wound in real time. Fluorescent imaging excluded infection at the initial visit, and debridement was avoided. Subsequently, pathergy was avoided as well. The patient was started on topical clobetasol with hypochlorous acid-soaked dressings. She also received 80 mg daily of prednisone and high-dose vitamin D3 (10,000 IU). Recovery was complicated by a deep tunnel along the incisional line at 3 months postdiagnosis, which required slowing of the prednisone taper and the addition of colchicine. Repeat cultures grew Parvimonas, Pseudomonas, and Streptococcus species. Appropriate antibiotics were given. The patient was transitioned from prednisone to adalimumab and started on negative-pressure wound therapy. Negative-pressure wound therapy was discontinued at 5 months, and the wound resolved at 6 months.

Programmed cell death protein 1 inhibitor-induced recalcitrant mixed small and medium vessel vasculitis.

Pembrolizumab, a programmed cell death protein 1 (PD1) inhibitor, has been known to be associated with several adverse reactions, including immune related adverse events. In less than one percent of patients, PD1 inhibitors have been linked to the development of connective tissue disease. Patients with previously known connective tissue disease are hypothesized to be at increased risk of flares in as many as 40% of cases. A 70-year-old man with a past medical history significant for rheumatoid arthritis in remission and stage IV lung adenocarcinoma presented to the dermatology clinic after one cycle of nivolumab and eight cycles of pembrolizumab exhibiting worsening, painful bilateral lower extremity ulcers for approximately one month. On the lower legs, three large black retiform eschars and bullous purpuric plaques were observed. Vasculitis is a rare complication of PD1 inhibitor therapy, with the majority of cases reported in literature either medium vessel or large vessel vasculitis. Only glucocorticoids have proven effective for PD1-induced vasculitis and these patients generally require multi-specialty management.

A perplexing case of superficial granulomatous pyoderma with sporotrichoid-like distribution.

Superficial granulomatous pyoderma (SGP) is a rare pyoderma gangrenosum (PG) variant that differs from classic PG in that the ulcers tend to be more superficial, lack a rapidly advancing border, and are not typically associated with an underlying systemic disease. The ulcers are most commonly painless and located on the trunk, with a clean granulating base. They generally do not show undermining but may have a vegetative border. Lesions usually respond well to either topical or intralesional corticosteroids with complete healing. The classic histopathologic finding is a "three-layer granuloma" in the superficial dermis consisting of central neutrophilic inflammation and necrosis, a surrounding layer of histiocytes and multinucleated giant cells, and an outer most layer of plasma cells and eosinophils. Herein, we present a unique case of SGP with sporotrichoid-like distribution on the lower extremity.

Hand-foot skin reaction with primarily dorsal involvement in a patient with metastatic renal cell carcinoma on cabozantinib.

A 61-year-old man with metastatic renal cell carcinoma on cabozantinib developed hand-foot skin reaction with predominantly dorsal involvement including painful violaceous plaques over the joints and keratotic yellow plaques on the palmar fingers. The medication was discontinued with resolution of the plaques and later reinitiated at a lower dose uneventfully.

Plexiform fibrohistiocytic tumor on the chest of a 5-year-old child and review of the literature.

Plexiform fibrohistiocytic tumor (PFT) is a rare neoplasm of mesenchymal origin that can be identified by its propensity for children and adolescents combined with a characteristic histologic arrangement of histiocytes and osteoclast-like giant cells whorled within tumor islands. A 5-year-old female presented with a raised, intermittently tender, and slowly enlarging tumor on her chest, which was histologically confirmed to be a PFT. We present this case along with a comprehensive review of PFT cases reported in the literature to describe the demographic, histologic, and rarely metastatic behavior of this entity. It is important to include PFT on the differential diagnosis of an enlarging tumor in the pediatric population.

Diversity and Career Goals of Graduating Allopathic Medical Students Pursuing Careers in Dermatology.

Importance: Dermatology is one of the least diverse specialties, while patients from minority racial and ethnic groups and other underserved populations continue to face numerous dermatology-specific health and health care access disparities in the US. Objectives: To examine the demographic characteristics and intended career goals of graduating US allopathic medical students pursuing careers in dermatology compared with those pursuing other specialties and whether these differ by sex, race and ethnicity, and/or sexual orientation. Design, Setting, and Participants: This secondary analysis of a repeated cross-sectional study included 58 077 graduating allopathic medical students using data from the 2016 to 2019 Association of American Medical Colleges Graduation Questionnaires. Main Outcomes and Measures: The proportion of female students, students from racial and ethnic groups underrepresented in medicine (URM), and sexual minority (SM) students pursuing dermatology vs pursuing other specialties. The proportions and multivariable-adjusted odds of intended career goals between students pursuing dermatology and those pursuing other specialties and by sex, race and ethnicity, and sexual orientation among students pursuing dermatology. Results: A total of 58 077 graduating students were included, with 28 489 (49.0%) female students, 8447 (14.5%) URM students, and 3641 (6.3%) SM students. Female students pursuing dermatology were less likely than female students pursuing other specialties to identify as URM (96 of 829 [11.6%] vs 4760 of 27 660 [17.2%]; P < .001) or SM (16 [1.9%] vs 1564 [5.7%]; P < .001). In multivariable-adjusted analyses, students pursuing dermatology compared with other specialties had decreased odds of intending to care for underserved populations (247 of 1350 [18.3%] vs 19 142 of 56 343 [34.0%]; adjusted odd ratio [aOR], 0.40; 95% CI, 0.35-0.47; P < .001), practice in underserved areas (172 [12.7%] vs 14 570 [25.9%]; aOR, 0.40; 95% CI, 0.34-0.47; P < .001), and practice public health (230 [17.0%] vs 17 028 [30.2%]; aOR, 0.44; 95% CI, 0.38-0.51; P < .001) but increased odds of pursuing research (874 [64.7%] vs 29 121 [51.7%]; aOR, 1.76; 95% CI, 1.57-1.97; P < .001) in their careers. Among students pursuing dermatology, female, URM, and SM identities were independently associated with increased odds of caring for underserved populations (eg, URM: aOR, 4.05; 95% CI, 2.83-5.80) and practicing public health (eg, SM: aOR, 2.55; 95% CI, 1.51-4.31). URM students compared with non-URM students pursuing dermatology had increased odds of intending to practice in underserved areas (aOR, 3.93; 95% CI, 2.66-5.80), and SM students compared with heterosexual students pursuing dermatology had increased odds of intending to become medical school faculty (aOR, 1.60; 95% CI, 1.01-2.57), to pursue administrative roles (aOR, 1.60; 95% CI, 1.01-2.59), and to conduct research (aOR, 1.73; 95% CI, 1.01-2.98). Conclusions and Relevance: The findings of this cross-sectional study suggest that diversity gaps continue to exist in the dermatology workforce pipeline. Efforts are needed to increase racial and ethnic and sexual orientation diversity and interest in careers focused on underserved care and public health among students pursuing dermatology.

An Intrapatient Concordance Study of Mismatch Repair Protein Immunohistochemical Staining Patterns in Patients With Muir-Torre Syndrome.

Importance: Appropriate use criteria for Muir-Torre syndrome (MTS) screening suggest that mismatch repair protein (MMRP) immunohistochemical (IHC) testing is usually appropriate in patients with 2 or more sebaceous neoplasms (SNs). While MTS is known to be caused by a germline mutation in mismatch repair genes, data are limited as to whether individual sebaceous tumors in these patients with multiple lesions show identical MMRP IHC staining patterns. Objective: To determine concordance of MMRP IHC staining patterns in lesions of patients with MTS who have multiple SNs. Design, Setting, and Participants: This retrospective single-center case series evaluated 38 SNs in 11 patients with MTS confirmed by genetic testing for MMRP IHC staining patterns. Tumor sites were classified as either facial or extrafacial. Data were collected between January 1, 2007, and January 1, 2018. Main Outcomes and Measures: In each patient, MMRP IHC staining patterns for SNs were compared with one another to evaluate intrapatient concordance between lesions, and to the patient's known germline mutation. Results: A total of 11 patients (7 women and 4 men) with MTS, with a mean (SD) age of 59.3 (10.6) years at time of SN biopsy, were identified. There was high concordance between MMRP IHC staining results (2-4 lesions per patient) and the patient's mutation status, with 36 of 38 total lesions (95%) matching (sensitivity, 94.7%; 95% CI, 82.3%-99.4%). Extrafacial site tumors represented 16 of 38 total lesions (42%) and demonstrated 100% concordance of IHC results to germline mutation. Only 1 of 11 patients (9%) demonstrated discordant results, with both lesions in this patient occurring on a facial site. Conclusions and Relevance: In patients with known MTS, SNs present with highly concordant MMRP IHC staining profiles across multiple lesions. There is also a strong association with underlying germline mutations. A diagnosis of MTS might be supported by MMRP IHC when the pretest probability is high.

Nail Unit Glomus Tumor with Myxoid and Symplastic Change Presenting with Longitudinal Erythronychia.

Glomus tumors are soft tissue neoplasms, which are most frequently encountered in the nail unit and generally straightforward to diagnose by histopathology. The typical clinical presentation is that of a circular violaceous or erythematous lesion within the nail bed. However, there are rare variants of glomus tumors which may pose diagnostic challenges because of the presence of unusual histologic features. Herein we report such a glomus tumor that demonstrates the rare combination of both myxoid and symplastic change. The clinical presentation of longitudinal erythronychia, as seen with this case, can occur with glomus tumors, but it is unusual, as longitudinal erythronychia on a single nail usually is caused by an onychopapilloma. The distinct nuclear atypia characteristic of symplastic change can raise alarm for a malignant process but the clinical course is benign. It is essential for dermatopathologists to be aware of this unusual variant of a glomus tumor in order to avoid overdiagnosis of atypia, which could result in unnecessary aggressive surgery. While unusual, there is good clinicopathologic correlation of the glomus tumor presenting with longitudinal erythronychia.