Faculty Bibliography

Hemophagocytosis may be encountered in association with a variety of underlying conditions, including primary or familial disorders, and secondary forms induced by infections, malignancy, and metabolic disorders. It is usually observed in organs such as the spleen, lymph nodes, bone marrow, and central nervous system, but rarely in the skin. When hemophagocytosis coexists with a sepsis-like systemic disorder it is termed hemophagocytic syndrome or hemophagocytic lymphohistiocytosis. Recently, 2 cases with evidence of perivascular hemophagocytosis in skin biopsy specimens of patients without additional findings of hemophagocytic lymphohistiocytosis have been reported. We report 2 additional cases of patients with cutaneous lesions suggestive of leukocytoclastic vasculitis whose skin biopsies showed a perivascular and interstitial infiltrate of neutrophils with nuclear dust and extravasated erythrocytes, and the presence of histiocytes with phagocytized red blood cells and nuclear fragments. There was also evidence of fibrin in the walls of the venules. One patient presented with a sepsis-like clinical scenario, but an extensive work-up failed to demonstrate any underlying disease or infection. The second patient was asymptomatic at the time of presentation, but further work-up revealed an underlying B-cell lymphoma. Whether these histologic findings represent late lesions of leukocytoclastic vasculitis or an incomplete presentation as part of a hemophagocytic syndrome is debatable, because both are associated with activated immunity and cytokine release, which could account for the presence of hemophagocytosis. Although the histopathologic finding alone of hemophagocytosis is insufficient to label as a syndrome, it should incite the clinician for further systemic evaluation.

BACKGROUND:The association between baseline seropositivity to human adenovirus (HAdV) type 5 and increased HIV acquisition in the Step HIV Vaccine Study has raised questions concerning frequency of acquired and/or persistent Adenovirus infections among adults at high risk of HIV-1 infection. METHODOLOGY/METHODS:To evaluate the frequency and pattern of HAdV shedding from the lower GI tract, we retrospectively tested rectal swabs for HAdVs in a cohort of 20 HSV-2 positive HIV-positive Peruvian men who have sex with men (MSM) undergoing rectal swabbing three times/week for 18 consecutive weeks, in a prospective study of HSV-2 suppression in HIV infection. Viral DNA was extracted and amplified using a sensitive multiplex PCR assay that detects all currently recognized HAdV types. Molecular typing of viruses was performed on selected samples by hexon gene sequencing. Baseline neutralizing antibody titers to HAdVs -5, -26, -35 and -48 were also assessed. PRINCIPAL FINDINGS/RESULTS:15/20 individuals had HAdV detected during follow up. The median frequency of HAdV detection was 30% of samples (range 2.0% to 64.7%). HAdV shedding typically occurred on consecutive days in clustered episodes lasting a median of 4 days (range 1 to 9 days) separated by periods without shedding, suggesting frequent new infections or reactivation of latent infections over time. 8 of the 15 shedders had more than one type detected in follow-up. 20 HAdV types from species B, C, and D were identified, including HAdV-5, -26 and -48, HAdV types under development as potential vaccine candidates. 14/20 subjects were seropositive for HAdV-5; 15/20 for HAdV-26; 3/20 for HAdV-35; and 2/20 for HAdV-48. HAdV shedding did not correlate with CD4 count, plasma HIV-1 viral load, or titers to HAdV-5 or HAdV-35. The sole individual with HAdV-5 shedding was HAdV-5 seropositive. CONCLUSIONS:HAdV shedding was highly prevalent and diverse, including types presently under consideration as HIV vaccine vectors. Subclinical HAdV infection of the GI tract is common among MSM in Peru; the prevalence of HAdV in the enteric tract should be evaluated in other populations. The association between ongoing recent enteric HAdV and the immune response to recombinant HAdV vaccines should be evaluated.

Spitz nevus is an uncommon melanocytic nevus distinctive by its epithelioid and spindled melanocytes. Many studies have attempted to characterize Spitz nevus, but none of them in a Hispanic population. Our aim is to characterize the clinical and histopathological presentation of the Spitz nevus in a Hispanic population. A retrospective study was carried out from our files that included those cases histopathologically diagnosed as Spitz nevus. A blinded examination was performed to evaluate the histopathological characteristics of 130 lesions. The demographics of the patients, the anatomic location, and the accuracy of the clinical diagnosis were analyzed. Eighty-one females and 49 males (ratio of 1.7:1) were included in the study. The mean age was 18.8 years. Overall, the most common location was the lower extremities (41%), followed by the upper extremities (27%), trunk (16%), and head and neck (16%). The nevi followed a similar anatomic distribution in females and males. The lesions were clinically diagnosed with accuracy in 20% of the cases and characterized as a pigmented papule in 42% of the cases. Upon histopathological evaluation, most nevi exhibited symmetry (84%), were well circumscribed (91%), and exhibited epidermal hyperplasia (69%). The junctional type was seen in 42% of the cases, the compound type in 38%, and the dermal type in 20%. Sixty-eight percent of nevi were mostly composed of epithelioid melanocytes, the spindled-shaped melanocytes predominated in 17% of cases, and 12% were composed of both epithelioid and spindled-shaped melanocytes. Multinucleated melanocytes were seen in 7% of nevi, mostly in the epithelioid Spitz nevus (67%). Abundant melanin was observed in 51 cases, from which the most common variant was the classic Spitz nevi. The typical dull eosinophilic globules (Kamino bodies) were observed in a minority of the cases (11%), mostly in the classic Spitz nevus. The most common variant was the classic Spitz nevus (65%), followed by the dermal Spitz nevus (15%). In conclusion, Spitz nevus in a Hispanic population most commonly presents as a pigmented papule on the lower extremities irrespective of sex and age. It is characterized by a melanocytic proliferation most commonly composed of nested epithelioid melanocytes in a junctional or compound arrangement, with the presence of abundant melanin.

Lupus erythematosus has a wide spectrum of cutaneous manifestations, including periorbital mucinosis. We report 3 cases of periorbital mucinosis occurring in association with other cutaneous signs of lupus erythematosus. Based on a review of the literature, periorbital mucinosis is a rare and not widely recognized clinical manifestation of the disease. Although unusual, familiarity with periorbital mucinosis as a manifestation of lupus erythematosus broadens our understanding of these entities and expands the spectrum of cutaneous lupus erythematosus.

BACKGROUND: Clinical experience has shown that, in patients with psoriasis, suspending treatment with etanercept at week 24, as indicated in the prescribing information, may lead to a rebound effect. Several clinical trials support long-term use of etanercept, which was shown to have a good safety and efficacy profile. MATERIAL AND METHODS: This was a retrospective, observational study of 43 patients with moderate to severe plaque psoriasis, with and without joint involvement, who received continuous treatment with etanercept for more than 24 weeks. RESULTS: Etanercept was administered for a mean of 57 weeks. Overall, the Psoriasis Area and Severity Index (PASI) score decreased from a baseline value of 22.5 to 4.3 after treatment. In addition, with continuous treatment, most patients maintained decreases in PASI scores of 50% and even of 75%. Some patients without significant improvement in their PASI score in the first 24 weeks did manage to achieve significant results after prolonged treatment. These outcomes were achieved with a low incidence of adverse effects (reported in 13 patients [30.2%]), which were generally mild. CONCLUSION: We present our clinical experience with long-term etanercept treatment in patients with moderate to severe psoriasis, with and without associated joint involvement. The efficacy and safety profiles were found to be favorable.

Rosacea is a common, chronic, skin condition characterized by recurrent episodes of facial flushing, transient or persistent erythema, papules, pustules, and telangiectasias, in a symmetrical facial distribution. The etiology of rosacea remains unknown and this condition represents a therapeutic challenge because of its chronic nature, progression, potential for disfigurement and psychological impact. Although there is no curative therapy for rosacea, the most widely used systemic agents are oral tetracycline derivatives, including tetracycline, doxycycline, and minocycline. This article reviews the available evidence for the use of doxycycline, a second-generation tetracycline, in the treatment or rosacea.

Persistent facial swelling may occur as a rare complication of rosacea. This finding has been referred to as lymphedematous rosacea, Morbihan's disease or persistent solid facial edema. A literature review for cases of lymphedematous rosacea revealed that the chronic inflammatory process that accompanies the disease contributes to an increase in the permeability of blood vessels. With time, the lymphatic drainage system becomes permanently impaired, leading to fluid accumulation in the affected skin. Herein, we report the case of a 58-year-old female with history of rosacea who developed bilateral periorbital swelling with associated erythema of the conjunctiva (ocular rosacea) over a 4 month period that only responded to oral corticosteroids. Biopsy revealed changes of lymphedematous rosacea.