Faculty Bibliography

A 46-year-old man presented with a two-week history of fatigue, fevers, and multiple nonhealing ulcers on his abdomen and back. He also had several dermatomal plaques clinically consistent with multifocal herpes zoster. Biopsy revealed large atypical myeloid cells dissecting through the dermis as well as marked papillary edema reminiscent of Sweet's syndrome. Blood work revealed an elevated white count (35-10(9) cells/L) with 11 percent blasts. Fluorescence in situ hybridization demonstrated a t(15;17) rearrangement diagnostic of M3 acute nonlymphocytic leukemia/acute promyelocytic leukemia. Chemotherapy was initiated, but the patient became septic and expired within two weeks. Acute promyelocytic leukemia cutis is exceedingly rare with only 24 previously reported cases, all of which occurred following treatment with all-trans retinoic acid, which is thought to induce the dermal tropism. The authors believe this is the first reported case of acute promyelocytic leukemia initially presenting with cutaneous involvement. The case is also notable for the Sweet's-like features of the infiltrate.

OBJECTIVE: Membrane endothelial protein C receptor (mEPCR) is highly expressed in peritubular capillaries of kidneys from patients with active and poorly responsive lupus nephritis (LN). We investigated the hypothesis that changes in the microvasculature are widespread with extension to the dermal vasculature. METHODS: Skin biopsies from uninvolved skin (buttocks) were performed in 27 patients with LN and 5 healthy controls. Sections were stained with specific antibodies reactive with mEPCR, adiponectin, intercellular adhesion molecule-1 (ICAM-1), and CD31; then assessed by enumeration of stained blood vessels (percentage positive blood vessels) blinded to knowledge of clinical information. RESULTS: There was a significant increase in the prevalence of blood vessels that stained for mEPCR and ICAM-1 in patients compared to controls [94% vs 59% (p = 0.045) and 81% vs 67% (p = 0.037), respectively]. Adiponectin staining and CD31 staining were similar between the groups (45% vs 43% and 98% vs 92%). Dermal staining for mEPCR was greater in patients with proliferative glomerulonephritis than in those with membranous disease (96% vs 60%; p = 0.029). A composite of poor prognostic renal markers and death was significantly associated with greater expression of mEPCR staining. CONCLUSION: These data are consistent with the notion that in patients with LN, activation of the microvasculature extends beyond the clinically targeted organ. The insidious expression of this widespread vasculopathy may be a contributor to longterm comorbidities.

The authors report the first case of a Wilms tumor (WT) with diffuse anaplasia metastatic to the brain in a 13-year-old girl with a history of neurofibromatosis Type 1. At presentation, the metastatic tumor had radiological features that suggested a meningioma. Histologically it was characterized by striking anaplasia and features similar to the patient's previously resected WT with diffuse anaplasia.

Liquid silicon used for soft tissue augmentation can produce different histologic patterns when implanted into the dermis or subcutis, depending on the degree of purification and the injected volume. The pure medical-grade silicon implanted in miniscule amounts in a controlled manner results in minimal fibroplasia without significant inflammation. The industrial-grade silicon contaminated by additives may remain dormant for years and eventually cause formation of extensive granulomas and disfiguring nodules. We report a case of a 35-year-old transsexual woman who presented with bilateral buttock masses. Histologic evaluation revealed collections of cells with lipid-containing cytoplasmic vacuoles resembling lipoblasts. This pattern can be clinically and histologically confusing for a neoplastic process, particularly when this adverse effect of adulterated silicon develops years after the injection.

An increased incidence of neoplasia was recently reported in patients with familial dysautonomia. This suggests that, in addition to its role in neuronal development, the IKBKAP gene may also influence DNA repair. Here we report the case of a 28-year-old male with familial dysautonomia who was found to have neoplastic lesions detected post mortem as incidental findings. This case indicates that the prevalence of tumorgenesis within this population may be underestimated.

BACKGROUND: The Depleted Uranium Follow-Up Program is a clinical surveillance program run by the Baltimore Veterans Affairs Medical Center since 1993 for veterans of the Gulf and Iraqi wars who were exposed to depleted uranium (DU) as a result of 'friendly-fire' incidents. OBJECTIVES AND METHODS: In 2009, 40 veterans from this cohort were screened for skin reactivity to metals by patch-testing with extended metal series and uranyl acetate (0.25%, 2.5%, and 25%). A control arm comprised 46 patients without any known occupational exposures to DU who were seen at the University of Maryland Dermatology Clinic for evaluation of allergic contact dermatitis. RESULTS: Excluding irritant reactions, no patch-test reactions to uranyl acetate were observed in the participants. Irritant reactions to DU were more common in the clinic cohort, likely reflective of the demographic differences between the two arms of the study. Biologic monitoring of urine uranium concentrations in the DU program participants with 24-hour urine samples showed evidence of percutaneous uranium absorption from the skin patches. CONCLUSION: We conclude that dermatitis observed in a subset of the veterans was unrelated to their military DU exposure. Our data suggest that future studies of skin testing with uranyl acetate should utilize 0.25%, the least irritating concentration

Intracerebral hemorrhage (ICH) is a well-recognized complication of recreational cocaine use. The precise mechanism of the cocaine-induced hemorrhagic event is unclear, although multiple factors have been implicated. We report a case of a 62-year-old woman who suffered left parieto-occipital ICH with herniation and death, following a cocaine binge. Microscopic examination also revealed extensive cerebral amyloid angiopathy (CAA) in the vicinity of the hemorrhage. We additionally studied brain tissue in eight subjects between ages of 60 and 80 who were positive for cocaine metabolites at autopsy; of these, none had vascular amyloid-β deposits by immunohistochemistry. Whereas we found no evidence that chronic cocaine use is a risk factor for CAA, given the age-associated nature of CAA and the aging population using cocaine, CAA-induced hemorrhage in the setting of cocaine use may be more common than recognized. This is the first reported case of CAA-associated ICH precipitated by cocaine.

Pemetrexed is U.S. Food and Drug Administration-approved as a second line, single-agent treatment of recurrent metastatic non-small cell lung cancer. Gastrointestinal side effects, including stomatitis, diarrhea, and vomiting are reported to be less than 1% and rarely severe. In the premetrexed clinical trial database of 1327 patients, various types of colitis were reported by a total of nine patients (0.6%). Typhilitis is a gastrointestinal complication of chemotherapy, which presents as fever and abdominal pain. The diagnosis is supported by the findings of bowel wall thickening on computed tomographic imaging. Typhilitis is usually seen in the setting of severe chemotherapy-induced neutropenia for acute leukemia. Nevertheless, it is increasingly recognized as a complication of therapy in solid tumors. We present the first documented case of typhilitis after treatment with pemetrexed and successful therapy with supportive treatment.