Courtney Schadt

Management of neutrophilic dermatoses.

Neutrophilic dermatoses, including Sweets 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. Sweets 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.

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.

Treatment of eccrine porocarcinoma with Mohs micrographic surgery: a cases series and literature review

Eccrine porocarcinoma (EPC) is a rare malignant tumor of the eccrine sweat gland. It is a potentially fatal neoplasm that is locally aggressive and commonly recurs. Wide surgical excision has traditionally been the treatment of choice and is curative in approximately 70–80% of cases. The disease is metastatic to lymph nodes and distant sites in 20% and 10% of cases, respectively. Metastatic EPC has not shown any great response to adjuvant chemotherapy or radiation.

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.

The cutaneous manifestations of gastrointestinal malignancy.

The skin is often the herald of an underlying systemic illness, and gastrointestinal malignancies can present in numerous ways in the skin. Paraneoplastic phenomenon, such as acanthosis nigricans and tripe palm, may be the first indicator of a gastrointestinal malignancy. In addition, gastrointestinal cancers can metastasize to the skin, as described in the well-known Sister Mary Josephs nodule. Inflammatory systemic conditions such as dermatomyositis and multicentric reticulohistiocytosis can be associated with underlying malignancy. Finally, in numerous genetic syndromes with underlying malignancies, such as Muir-Torre, recognition of the skin signs leads to early diagnosis and screening.

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

Abstract 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.

Hepatitis C mixed cryoglobulinemia with undetectable viral load: A case series

DAA: direct-acting antiretrovirals HCV: hepatitis C virus LCV: leukocytoclastic vasculitis INTRODUCTION Mixed cryoglobulinemic vasculitis is caused by circulating cold-precipitable immunoglobulins, or cryoglobulins, composed of monoclonal (type II) or polyclonal (type III) IgM directed against a polyclonal IgG. It causes palpable purpura and has long been associated with hepatitis C virus (HCV) infection. The initial theory regarding the new antiviral medications, which have been so successful in treating hepatitis C, was that once the HCV was treated, the mixed cryoglobulinemic vasculitis would also resolve. Although initial studies confirmed this theory, later studies found that the vasculitis is persistent in some patients despite successful HCV treatment. Vasculitis occurred within months after treatment of HCV infection in most cases in the literature, but we present 3 cases of mixed cryoglobulinemic vasculitis months to years after successful HCV treatment and negative viral load and a review of the current literature.

Erythematous Plaque to Lower Leg After Tropical Injury

A 70-year-old retired nurse presented with a 3-month history of an asymptomatic, nonhealing lesion of her left leg. It developed after she fell and scraped this specific location on her leg on a creek rock while hiking in the jungle during a vacation in Ecuador. She denies any other trauma at any time between the scraping and presentation. After the fall, she subsequently developed an enlarging erythematous plaque with an overlying hemorrhagic crust without drainage. This crust sloughed but soon thereafter reformed. She was treated with triple antibiotic ointment, an antifungal cream, and one week of trimethoprim-sulfamethoxazole prior to presenting to her dermatologist. She was otherwise asymptomatic with a negative review of systems. Past medical history included hypertension, hypercholesterolemia, and hypothyroidism, and her medications were levothyroxine, lovastatin, atenolol, and sodium bicarbonate. She was born in the United States and had not spent any significant time outside the country before this episode. Examination revealed a 2 cm erythematous crusted plaque with several smaller satellite papules on the left shin at the site of trauma (Figure ​(Figure1A).1A). A punch biopsy (Figure ​(Figure1B1B and C) and tissue culture were performed. What is the diagnosis? Figure 1. (A) Erythematous eroded plaque with satellite papules on left shin. (B) A 40× magnification demonstrating granulomatous infiltrate

Bruising in a man in his 60s.

A white man in his 60s with a history of hypertension presented to his dermatologist with a bruiselike rash of 2 months’ duration. He was referred by a hematologist-oncologist whom he had seen for mild erythrocytosis and ecchymoses. The rash was not symptomatic and appeared mostly on the left side of his face and trunk. He had not experienced any trauma in the area. Initially, the bruiselike rash would appear and then spontaneously regress, but it was becoming progressively more persistent. The patient had no history of arsenic or radiation exposure and did not report any accompanying symptoms, including night sweats, fevers, chills, or weight loss. His medications included amlodipine besylate, benazepril hydrochloride, metoprolol tartrate, lisinopril, and over-the-counter daily vitamins. Physical examination revealed brown irregular patches and slightly indurated plaques on the forehead, left temple, preauricular area, left neck, upper chest, and back (Figure, A and B). The plaque on the left clavicle had recently become raised and measured 2 × 3 cm. Punch biopsies of the left clavicle and left forehead were performed, and tissue was sent for pathologic analysis (Figure, C and D). Quiz at jamadermatology.com A B