Drug-induced subacute cutaneous lupus erythematosus associated with abatacept.

Numerous drugs have been linked to the induction or exacerbation of systemic cutaneous lupus erythematosus (SCLE). This report presents the third case of the biologic abatacept as an exacerbating medication for SCLE. A 73-year old woman with a remote history of subacute cutaneous lupus and rheumatoid arthritis, well controlled on hydroxychloroquine, presented with worsening annular erythematous, slightly scaly plaques on her forearms and hands. She had been started on abatacept a month prior. She was diagnosed with SCLE exacerbated by abatacept given the clinical findings, time course, and skin biopsy with interface dermatitis. Her skin eruption cleared completely several months later after discontinuing abatacept and switching to tociluzumab, while remaining on hydroxychloroquine. This case highlights the need to consider abatacept as a potential exacerbating medication for SCLE in any patient with a new photodistributed papulosquamous eruption.

Unilateral hyperhidrosis secondary to brainstem meningioma producing mass effect.

Unilateral hyperhidrosis of neurological origin has been associated with head trauma, cerebral palsy, spinal cord injury, peripheral neuropathy, lesions of the hypothalamus, and cerebral or brainstem strokes. In this report, we describe a 61-year-old man with isolated sweating on the left side of his entire body. A right-sided brainstem meningioma producing mass effect is suspected as the underlying etiology.

A survey study with assessment of esophageal screening and genetic counseling in patients with Howel-Evans syndrome.

BACKGROUND: It is important to better understand the role that environmental risk factors play on the development of esophageal cancer in Howel-Evans families. Additionally, there is little published about appropriate esophageal cancer screening practices in families genetically confirmed to have this condition. METHODS: Surveys were distributed to 47 addresses of an American family with Howel-Evans syndrome, of which 29 responded and met inclusion criteria. Data was collected about demographics, environmental risk factors, and medical history of participants. RESULTS: We report characteristics of family members with tylosis, rates of esophageal cancer, rates of genetic counseling, and levels of environmental risk factors. Of the survey respondents, 43% reported features of tylosis, 71.4% were male and 28.6% were female and 28.6% reported leukoplakia. Only 21.4% of tylotic family members smoked, 65% drank alcohol, and 28.6% drank well water. More than half (57.1%) of the tylotic individuals had never had an esophagogastroduodenoscopy (EGD) and no one had been diagnosed with esophageal carcinoma. Only 3.4% of respondents had ever received genetic testing for Howel-Evans syndrome, despite genetic confirmation of their relatives. CONCLUSIONS: We encourage dermatologists to discuss smoking-cessation, genetic counseling, and early EGD with affected families.

Erythematous nodule of the ear.

An elderly may with eccrine porocarcinoma of the ear is presented. This rare tumor in an unusual location has the potential for metastasis and local recurrence.

Pyoderma Gangrenosum: Diagnostic Criteria, Subtypes, Systemic Associations, and Workup.

Pyoderma gangrenosum (PG) is an inflammatory neutrophilic dermatosis with variable clinical features. The classic presentation is an ulceration with an erythematous to violaceous undermined border. Extracutaneous manifestations may occur. Associated systemic diseases include inflammatory bowel disease, inflammatory arthritides, and hematologic disorders. The pathophysiologic mechanism of disease is not completely known but likely related to the cumulative impact of inflammation, immune-mediated neutrophilic dysfunction, and genetic predisposition. Incidence is between 3 and 10 people per million but may be greater due to under recognition. In this article, we will discuss the diagnostic criteria, disease subtypes, systemic associations, and workup.

Topical and oral bexarotene.

Bexarotene is a retinoid that specifically binds retinoid X receptors and has numerous effects on cellular growth and differentiation. It is approved for the treatment of cutaneous T cell lymphoma both topically and systemically. Adverse effects include hyperlipidemia, central hypothyroidism, and neutropenia with bexarotene capsules, and an irritant dermatitis with bexarotene gel. With aggressive management of these potential side effects, bexarotene is an additional option in the armamentarium for management of cutaneous T cell lymphoma.

Management of neutrophilic dermatoses.

Neutrophilic dermatoses, including Sweet's syndrome, pyoderma gangrenosum, and rheumatoid neutrophilic dermatitis, are inflammatory conditions of the skin often associated with underlying systemic disease. These are characterized by the accumulation of neutrophils in the skin. The associated conditions, potential for systemic neutrophilic infiltration, and therapeutic management of these disorders can be similar. Sweet's syndrome can often be effectively treated with a brief course of systemic corticosteroids. Pyoderma gangrenosum, however, can be recurrent, and early initiation of a steroid-sparing agent is prudent. Second-line treatment for both of these conditions includes medications affecting neutrophil function, in addition to immunosuppressant medications.

Annular Erythematous Eruption in a Pediatric Patient.

An 11-year-old girl presented in autumn with an asymptomatic rash on her arms, face, and legs of a 3-week duration. What is your diagnosis?

Papular Mucinosis.

This case report describes erythematous patches on the face that involved the nasolabial folds, as well as numerous skin-colored homogenous waxy papules on the arms, neck, and trunk.

Evidence supports blind screening for internal malignancy in dermatomyositis: Data from 2 large US dermatology cohorts.

The association between dermatomyositis and internal malignancy is well established, but there is little consensus about the methods of cancer screening that should be utilized.We wished to analyze the prevalence and yield of selected cancer screening modalities in patients with dermatomyositis.We performed a retrospective analysis of 2 large US dermatomyositis cohorts comprising 400 patients.We measured the frequency of selected screening tests used to search for malignancy. Patients with a biopsy-confirmed malignancy were identified. Prespecified clinical and laboratory factors were tested for association with malignancy. For each malignancy we identified the screening test(s) that led to diagnosis and classified these tests as either blind (not guided by suspicious sign/symptom) or triggered (by a suspicious sign or symptom).Forty-eight patients (12.0% of total cohort) with 53 cancers were identified with dermatomyositis-associated malignancy. Twenty-one of these 53 cancers (40%) were diagnosed within 1 year of dermatomyositis symptom onset. In multivariate analysis, older age (P = .0005) was the only significant risk factor for internal malignancy. There was no significant difference in cancer incidence between classic and clinically amyopathic patients. Twenty-seven patients (6.8% of the total cohort) harbored an undiagnosed malignancy at the time of dermatomyositis diagnosis. The majority (59%) of these cancers were asymptomatic and computed tomography (CT) scans were the most common studies to reveal a cancer.This is the largest US cohort studied to examine malignancy prevalence and screening practices in dermatomyositis patients. Our results demonstrate that, while undiagnosed malignancy is present in <10% of US patients at the time of dermatomyositis onset, it is often not associated with a suspicious sign or symptom. Our data suggest that effective malignancy screening of dermatomyositis patients often requires evaluation beyond a history, physical examination, and "age-appropriate" cancer screening-these data may help to inform future guidelines for malignancy screening in this population.

Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts.

Importance: Pyoderma gangrenosum is a rare inflammatory skin condition that is difficult to diagnose. Currently, it is a "diagnosis of exclusion," a definition not compatible with clinical decision making or inclusion for clinical trials. Objective: To propose and validate diagnostic criteria for ulcerative pyoderma gangrenosum. Evidence Review: Diagnostic criteria were created following a Delphi consensus exercise using the RAND/UCLA Appropriateness Method. The criteria were validated against peer-reviewed established cases of pyoderma gangrenosum and mimickers using k-fold cross-validation with methods of multiple imputation. Findings: Delphi exercise yielded 1 major criterion-biopsy of ulcer edge demonstrating neutrophilic infiltrate-and 8 minor criteria: (1) exclusion of infection; (2) pathergy; (3) history of inflammatory bowel disease or inflammatory arthritis; (4) history of papule, pustule, or vesicle ulcerating within 4 days of appearing; (5) peripheral erythema, undermining border, and tenderness at ulceration site; (6) multiple ulcerations, at least 1 on an anterior lower leg; (7) cribriform or "wrinkled paper" scar(s) at healed ulcer sites; and (8) decreased ulcer size within 1 month of initiating immunosuppressive medication(s). Receiver operating characteristic analysis revealed that 4 of 8 minor criteria maximized discrimination, yielding sensitivity and specificity of 86% and 90%, respectively. Conclusions and Relevance: This Delphi exercise produced 1 major criterion and 8 minor criteria for the diagnosis of ulcerative pyoderma gangrenosum. The criteria may serve as a guideline for clinicians, allowing for fewer misdiagnoses and improved patient selection for clinical trials.

Eccrine syringofibroadenoma with co-existent squamous cell carcinoma.

Eccrine syringofibroadenoma (ESFA) is a rare, benign adnexal tumor arising most often on the extremities of elderly individuals. It is typically a slow-growing, flesh- to reddish-colored nodule or plaque. Histologically, the tumor consists of anastomosing cords of cuboidal epithelial cells surrounded by a fibrovascular stroma containing plasma cells. The cords contain scattered ductal structures lined with cuboidal cells resembling eccrine ducts. The co-existence of ESFA with squamous cell carcinoma has been described, eliciting the term eccrine syringofibroadenoma. The differential diagnosis includes poroma, porocarcinoma, fibroepithelioma of Pinkus and clear cell acanthoma. ESFA stain positively with epithelial membrane antigen and carcinoembryonic antigen. Cytokeratin studies have been inconsistent.

Thalamotomy, DBS-Vim, and DBS-GPi for generalized dystonia: a case report.

Generalized dystonia is a disabling disorder that can severely affect quality of life. Pharmacological treatment is unsatisfactory, and surgical therapy has been the focus for symptom improvement. We present the first case report of a patient with disabling generalized dystonia treated with a thalamotomy and deep brain stimulation of the thalamus and globus pallidus (DBS-Vim, DBS-GPi). His tremor and dystonic symptoms have dramatically improved through combining these surgical interventions.