Autoimmune bullous diseases in non-HIV Kaposi's sarcoma: a retrospective study in a large cohort of patients.

BACKGROUND: Kaposi's sarcoma (KS) is a rare endothelial neoplasm caused by the human herpesvirus 8 (HHV-8). Its risk is increased in immunocompromised patients, including those undergoing immunosuppressive therapy for autoimmune bullous diseases. Conversely, HHV-8 infection has been hypothesized to be a triggering factor of bullous diseases, especially pemphigus. Given the fact that both KS and autoimmune bullous diseases have a low incidence in the general population, it could be expected that the association between these disorders would be exceptional. OBJECTIVES: To assess the frequency of bullous diseases in a large cohort of non-HIV KS patients and to describe our experience concerning the clinical features, natural history and treatment options in this setting. METHODS: We performed a retrospective review of all patients with non-HIV KS in association with bullous disease followed at our department between 1990 and 2016. Medical records were reviewed for demographics, medical history, clinical characteristics and treatment. RESULTS: Among 1362 patients with classic or iatrogenic KS, 14 (1.03%) also suffered from bullous disease. The mean age at diagnosis of both disorders was 85.8 years with a male/female ratio of 9 : 5. Among these 14 cases, nine (0.66%) were associated with bullous pemphigoid (BP), three (0.22%) with localized BP and two (0.15%) with pemphigus vulgaris. Seven had developed a bullous disease after being diagnosed with KS, while in the remaining seven cases, KS developed after the onset of bullous disease. As expected, KS worsened when corticosteroids were used. CONCLUSION: Bullous diseases seem to be more frequent among patients with KS, supporting the hypothesis that HHV-8 may be involved in their pathogenesis. Therapeutic management of these cases should take into account KS-inducing potential of corticosteroids.

Toe web intertrigo in Kaposi's sarcoma patients: a microbiological study in a large cohort of patients.

Kaposi 's sarcoma (KS) is a rare multifocal angioproliferative disease associated with human herpes virus 8 (HHV-8) infection, characterized by cutaneous nodules or plaques especially on the lower limbs. Some skin modifications, such as chronic lymphedema, plantar hyperkeratosis and interdigital desquamation, may be associated with consequent impairment of the local immunosurveillance and increased risk of some bacterial or mycotic infections. With the objective of evaluating if bacterial or mycotic infections in KS patients are supported by different microorganisms compared to control patients, we performed an observational retrospective study, comparing positive cultural swabs of interdigital intertrigo of KS patients with positive cultural swabs of interdigital intertrigo of patients admitted to our dermatologic unit during the last 10 years. One hundred KS patients and 84 control patients were admitted to this study. Some of the skin swabs from interdigital spaces were positive for more than one microorganism, and therefore we found 187 microorganisms among the KS group and 182 microorganisms in the control group. The most common microrganisms among KS patients were T. mentagrophytes (16%), S. aureus (14.9%), P. aeruginosa (13.9%), S. marcescens (5,9%), while among non-KS patients were S. aureus (26,9%), C. albicans (22%), S. agalactiae (7.7%) and E. coli (9.9%). These differences are statistically significant (p < 0.01). KS patients may be more affected by toe web intertrigo due to other bacteria and dermatophytes than the general population. During clinical examination, a careful inspection is necessary for an early diagnosis of toe web intertrigo, in order to prevent serious complications, such as cellulitis and sepsis. Consequently, a cultural examination with antibiogram is required to identify the causative agent of intertrigo and guide antimicrobial therapy.

Intralesional vincristine as first-line therapy for nodular lesions in classic Kaposi sarcoma: a prospective study in 151 patients.

BACKGROUND: Classic Kaposi sarcoma is a rare angioproliferative neoplasm with varying biological behaviour. Depending on the clinical stage, local or systemic therapy can be used. Vincristine has proven to be effective as systemic chemotherapy and in very few reports as intralesional treatment. OBJECTIVES: Our aim was to determine the efficacy and safety of intralesional vincristine in the treatment of classic Kaposi sarcoma nodular lesions. METHODS: We conducted a prospective, open-label, single-centre clinical trial in 151 patients with stage IB classic Kaposi sarcoma. Vincristine was injected in a single nodule (0.3-0.8 mm) on the lower limb. Another similar lesion on the same limb, at a distance of >or= 10 cm, or on the contralateral limb, was kept under clinical observation as control. Adverse effects were evaluated after 1 week, and efficacy after 4 and 12 weeks. RESULTS: One hundred and fifty-one patients were enrolled. At final evaluation, 115 patients presented complete response (76.1%), 28 had partial response (18.5%), six had improvement (4%), one had stable disease (0.7%) and only one patient had tumour progression (0.7%). Therefore the total response rate was 98.7% (149 patients). Therapy was generally well tolerated. The most frequently registered adverse events, observed in 21 patients (13.9%), were erythema and itching. CONCLUSIONS: Intralesional vincristine is an effective and safe treatment for nodular lesions in classic Kaposi sarcoma and can be recommended as first-line therapy in initial stages and as support therapy in advanced stages.

Treatment of classic Kaposi sarcoma with a nicotine dermal patch: a phase II clinical trial.

BACKGROUND: Kaposi sarcoma (KS), a malignancy of dermal endothelial cells that is caused by human herpesvirus 8 (HHV8) infection, is sensitive to perturbations of immunity. Nicotine might be effective against KS because of its immunologic and vascular effects and because smoking is associated with a low risk of KS. OBJECTIVE AND STUDY DESIGN: We conducted a masked, randomized phase 2 clinical trial of transdermal nicotine and placebo patches to assess the safety and efficacy of nicotine against classic KS (cKS). SUBJECTS AND METHODS: Three cKS lesions, predominantly nodules, in each of 24 non-smoking patients were randomly assigned to 15 weeks continuous treatment with nicotine patch (escalated to 7 mg), identical masked placebo patch or no patch. Changes in lesion area and elevation from baseline through six follow-up visits, by direct measurement and by two independent readers using digital photographs of the lesions, were compared using non-parametric and regression methods. Changes in longitudinal levels of HHV8 antibodies and DNA in blood cells were similarly assessed. RESULTS: There were no systemic or serious adverse events, and compliance was good. One patient resumed smoking and discontinued patches, and two patients withdrew at week 12 for unrelated indications. Six (29%) of the remaining 21 suspended use of patches to relieve local skin irritation; four of these six completed the trial at reduced dose. Treatment assignment was not associated with significant or consistent changes in cKS lesion area or elevation, HHV8 viral load or antibodies. CONCLUSION: Transdermal nicotine and placebo patches caused no serious toxicities but had no demonstrable effect on nodular cKS lesions or HHV8 levels.