Faculty Bibliography

Kaposi sarcoma (KS) is a vascular neoplasm associated with human herpesvirus 8 (HHV-8) infection that can be confused with the clinical and histological findings of chronic venous insufficiency. Definitive diagnosis of KS can only be achieved by performing a polymerase chain reaction for HHV-8 or by immunostaining for the HHV-8 antigen. We describe 2 unusual clinical presentations of KS in the setting of chronic venous insufficiency with clinical and histologic features consistent with stasis dermatitis but positive HHV-8 immunostaining. Both patients had no known risk factors for KS. We propose the possibility that these cases may represent a new clinical variant of KS that may become more prevalent over time. Further studies are needed to identify the risk factors involved. Meanwhile, skin biopsy with HHV-8 testing may be warranted for violaceous patches and plaques arising on the legs in the setting of chronic venous insufficiency, especially in patients who are unresponsive to treatment.

Generalized essential telangiectasia, which is a rare condition that is characterized by the progressive development of telangiectases on the skin, is a clinical diagnosis of exclusion. We present a 65-year-old man with a ten-month history of an asymptomatic eruption of the trunk and proximal aspects of the arms and hands that was comprised of macules and patches of telangiectases. The clinical presentation, associated diseases, hypotheses regarding pathogenesis, differential diagnoses, and reports on treatment modalities are reviewed. The relatively new association of this entity with systemic signs that include hemorrhage as well as the occurrence of generalized essential telangiectasia in patients with a history of hepatitis is discussed.

We report a 52-year-old man with a one-year history of multiple, firm, skin-colored nodules on the vertex of the scalp. Histopathologic examination was consistent with a spiradenoma, which is a rare, benign adnexal tumor of controversial histogenesis. Multiple spiradenomas may arise in association with Brooke-Spiegler syndrome, which is an autosomal dominant condition of multiple cyclindromas, trichoeptheliomas, and cyclindromas.

We present a case of cutaneous sarcoidosis in a 77-year-old woman without systemic disease, who presented with two subtypes of lesions: erythematous and hyperpigmented macules on the arms and anterior upper legs and erythematous, hyperkeratotic nodules, some crateriform, on the lower legs and feet. Whereas cutaneous sarcoidosis is present in up to one-third of cases and may present with a wide variety of lesions, verrucous sarcoidosis is uncommon. Diagnosis is by clinical and histopathologic exclusion of other entities, which include other causes of granulomatous inflammation such as infections and foreign-body reactions. Management options include topical, intralesional, or systemic glucocorticoids, antimalarials, tetracycline derivatives, and methotrexate.

We present a case of acrokeratosis verruciformis of Hopf in a 58-year-old woman with multiple, skin-colored, flat-topped, hyperkeratotic papules on the dorsal aspects of the hands and feet. Her lesions appeared at age 13 and her son developed similar lesions at about the same age. Acrokeratosis verruciformis of Hopf is a rare genodermatosis, which is distinct from keratosis follicularis; sporadic cases have also been described. Management options include cryotherapy, keratolytics, superficial ablation with CO2 or Nd:YAG lasers, topical retinoids, and acitretin.

We report the case of a 63-year-old obese man with a rapid-onset of widespread acanthosis nigricans (AN) in the setting of having recently initiated treatment with niacin for dyslipidemia. Although obesity and insulin-resistance are risk factors for AN, AN associated with endocrine dysfunction tends to have a more gradual onset and limited involvement. Owing to our patient's age, the rapid onset, and extensive distribution of his eruption, we initially were concerned about paraneoplastic AN. However, an evaluation for a malignant condition was negative. The timing of the onset of our patient's eruption within several months of starting niacin therapy is consistent with niacin-induced AN. Niacin is known to cause rapidly progressive, widespread AN that is reversible upon discontinuation of the medication. We discuss the pathogenesis of AN, which is thought to be the final common manifestation of stimulation of different subtypes of tyrosine kinase receptors by various epidermal growth factors.

We present a 40-year-old man with erythematous-to-violaceous, broken, reticulated patches on the upper chest, back, and extremities, which is consistent with livedo racemosa. The cutaneous findings appeared after an increase in dilantin dose and subsequently improved after a reduction in dilantin dose. Furthermore, antinuclear antibodies and antihistone antibodies were detected. We therefore believe that the livedo racemosa is a cutaneous manifestation of a drug-induced systemic lupus erythematosus. We review the distinctive features of livedo racemosa as well as its associations with several disorders. Although there are no effective treatments for livedo racemosa, patients often are placed on low-dose aspirin and counseled to avoid smoking in an effort to protect against their increased risk of stroke and arterial thrombosis

BACKGROUND: Esophageal carcinoma is a particularly interesting tumor because of the dramatic difference in its incidence and geographic distribution among populations of similar ethnic origin. Epidemiologic data have suggested that many environmental exposures may be associated with an increased risk of its formation. METHODS: In this study, 92 samples of esophageal squamous cell carcinoma (ESCC) were collected from patients who resided in 2 geographic areas in China with different incidences of ESCC: Linxian and Zhejiang. Overexpression and mutations of the p53 tumor-suppressor gene were examined by using immunohistochemistry, single-strand conformation polymorphism analysis, and direct sequencing. RESULTS: The rates of point mutation and overexpression of p53 in the ESCC specimens studied were 30.4% (29 of 92 specimens) and 51.1% (47 of 92 specimens), respectively. The overexpression of p53 was associated with tumor metastasis and with 5-year case fatality. Significant differences were found in the rates of overexpression and mutations in patients with clinical T2 tumors between the specimens from Linxian, which is a high-incidence geographic area, and the specimens from Zhejiang, which is a low-incidence area. Furthermore, different mutational spectra were found in the tumor samples from these two geographic areas: In tumor samples from Linxian, the most common substitution mutation was a transversion in exon 5, whereas the most common mutations in tumor samples from Zhejiang were transitions in exon 7. CONCLUSIONS: The data suggest that the mutation and overexpression of p53 may play important roles in the development of ESCC. The changes in p53 may reflect environmental exposure to the different combinations of mutagenic factors and genetic instability demonstrated by the populations in Linxian and Zhejiang. The overexpression of p53 protein may have significance as a prognostic factor for patients with esophageal carcinoma.