Vitiligo prevalence and quality of life among adults in Europe, Japan and the USA.

BACKGROUND: Vitiligo, an autoimmune disorder characterised by skin depigmentation, is associated with reduced quality of life (QoL). Vitiligo may be under-reported, in part because of misconceptions that it is a cosmetic disease. OBJECTIVES: This survey sought to characterise vitiligo prevalence and explore the relationship between sociodemographic and clinical characteristics with QoL in a population-based, multinational study. METHODS: Participants aged ≥18 years were recruited via an online panel in Europe, Japan and the USA to answer questions regarding skin disorders they may have experienced. Those reporting vitiligo (diagnosed or undiagnosed) or vitiligo signs (experiencing loss of skin colour but unaware of vitiligo and not diagnosed) were included in the analyses of vitiligo prevalence. Participants who self-reported physician-diagnosed vitiligo were given a broader survey to characterise disease progression, management and QoL (as measured with the Vitiligo-specific QoL [VitiQoL] instrument). RESULTS: The total estimated vitiligo prevalence among 35 694 survey participants (Europe, n = 18 785; USA, n = 8517; Japan, n = 8392) was 1.3% (diagnosed, 0.6%; undiagnosed, 0.4%; vitiligo signs, 0.3%). Among 219 patients formally diagnosed with vitiligo (Europe, n = 150; USA, n = 48; Japan, n = 21), total VitiQoL scores were associated with age (P = 0.00017), disease extent (P < 0.0001), disease progression (P < 0.0001), disease management (P < 0.0001) and time since diagnosis (P = 0.0015). Behaviour scores varied based on skin phototype (P = 0.024) and ethnicity (P = 0.048). Higher total VitiQoL scores were reported in patients with head lesions (P = 0.027) and those with head and hand and/or wrist lesions (P = 0.018). Substantial high concern (rated 8-10 on an 11-point Likert scale) for lesions was found across all body areas and varied with geographical region. CONCLUSIONS: The vitiligo prevalence rate may be higher than previously reported, with a substantial proportion attributed to people who have not received a formal diagnosis. Among formally diagnosed patients with vitiligo, QoL was most severely impacted by more progressive and higher extent of disease.

International Initiative for Outcomes (INFO) for vitiligo: workshops with patients with vitiligo on repigmentation.

BACKGROUND: There is no cure or firm clinical recommendations for the treatment of vitiligo. One of the main issues is the heterogeneity of outcome measures used in randomized controlled trials for vitiligo. OBJECTIVES: To define successful repigmentation from the patients' point of view and to propose how and when repigmentation should be evaluated in clinical trials in vitiligo. METHODS: We conducted three workshops with patients with vitiligo and their parents or caregivers. Workshop 1 was held at World Vitiligo Day (Detroit, MI), workshop 2 at the University of Texas Southwestern Medical Center and workshop 3 at the Vitiligo and Pigmentation Institute of Southern California, University of California. RESULTS: Seventy-three participants were recruited. Consensus on the following questions was achieved unanimously: (i) the definition of 'successful repigmentation' was 80-100% of repigmentation of a target lesion and (ii) both an objective and a subjective scale to measure repigmentation should be used. CONCLUSIONS: This was the largest patients' outcomes workshop. We followed the guidance from the CSG-COUSIN and the Vitiligo Global Issues Consensus Group. Our recommendations to use percentage of repigmentation quartiles (0-25%, 26-50%, 51-79%, 80-100%) and the Vitiligo Noticeability Scale are based on the best available current evidence. A limitation of the research is that the workshops were conducted only in the U.S.A., due to pre-existing organisational support and the availability of funding.

The effect of melasma on self-esteem: A pilot study.

Melasma is a common disorder of hyperpigmentation characterized by tan or brown macules and patches affecting sun-exposed areas, particularly the face. Melasma has been shown to have a significant impact on the quality of life and self-esteem of those affected. We interviewed six patients who were diagnosed with moderate-to-severe melasma with regard to the effect of their disorder on their self-esteem. All patients reported a significant negative effect on their quality of life and self-esteem. With successful therapy using a triple combination of cream and oral tranexamic acid to treat their melasma, all reported a marked improvement in self-esteem. Physicians who treat patients with melasma should be aware of its profound psychosocial effects and the improvement that successful melasma treatment can have on self-esteem.

Treatment of pemphigus with gold.

BACKGROUND: Although gold has been reported to be useful in treating pemphigus vulgaris, its use has waned in recent years because of concerns regarding efficacy and toxicity. OBJECTIVE: To review 26 patients with pemphigus who were treated with intramuscular gold over a 10-year period. RESULTS: Gold was effective in 62% of patients as a primary treatment for pemphigus or as a steroid-sparing agent. An average of 3 months of therapy was required before the daily prednisone dosage could be halved. Four patients were free of disease and stopped receiving all therapy at the conclusion of the study. Toxic effects due to gold therapy developed in 42% of patients and all adverse effects resolved with its cessation. CONCLUSIONS: While toxic effects limit the use of gold in many patients with pemphigus, it may be effective in treating a large percentage of patients who otherwise are unable to reduce their steroid requirement. Because of its delayed onset of action, patients treated with gold usually require systemic steroids when therapy is initiated. Controlled, prospective trials are needed to further evaluate the efficacy of gold and its potential steroid-sparing effects.

Treatment of pemphigus vulgaris with pulse intravenous cyclophosphamide.

BACKGROUND: Although corticosteroids have dramatically altered the prognosis in pemphigus vulgaris, morbidity and mortality from systemic corticosteroid side effects remains high. While immunosuppressive agents have been successfully used in pemphigus vulgaris, there is a high incidence of side effects with these agents as well. Particularly bothersome are reports of increased risk of malignancy with long-term use of immunosuppressive agents. For these reasons, we used a protocol that includes low-dose oral cyclophosphamide coupled with pulse intravenous cyclophosphamide in two patients with recalcitrant pemphigus vulgaris. OBSERVATIONS: Both patients responded well to monthly doses of intravenous cyclophosphamide with rapid decrease in the frequency and severity of blistering, resulting in resolution of their disease after 7 and 10 months, respectively. CONCLUSIONS: Pulse doses of immunosuppressive agents appear to be successful in the treatment of pemphigus vulgaris. High-dose steroid therapy can be tapered with the use of this treatment. Because monthly intravenous doses of cyclophosphamide lead to a substantially reduced cumulative dose, when compared with standard oral regimens, the risk of developing malignancy may also be reduced. Further studies using larger groups of patients are needed to evaluate the efficacy of pulse intravenous cyclophosphamide in pemphigus vulgaris. Long-term follow up will be necessary to compare the incidence of malignancy in patients receiving pulse doses of immunosuppressive agents with that in patients receiving continuous oral treatment.

Pulse intravenous cyclophosphamide therapy in pemphigus.

BACKGROUND: Adjuvant therapy is commonly used in pemphigus to mitigate the high morbidity and mortality associated with the use of corticosteroids and improve disease control. However, these adjuvant agents are not without adverse effects of their own, including an increased risk of malignancy with the use of oral immunosuppressives. Intravenous pulse cyclophosphamide, which may be more efficacious and less toxic than oral immunosuppressives, has been used successfully in the treatment of pemphigus. OBJECTIVE: To review 9 patients with severe or previously recalcitrant pemphigus who were treated with intravenous pulse cyclophosphamide therapy. RESULTS: Six of the 9 patients responded to therapy, with 2 patients achieving remission from skin lesions. Five patients were able to decrease their daily dose of prednisone, and 1 was able to discontinue the use of prednisone completely. Most patients experienced minimal or no adverse effects. CONCLUSIONS: Intravenous pulse cyclophosphamide may be an alternative treatment option in patients with pemphigus recalcitrant to standard therapy. The decreased cumulative dose of cyclophosphamide observed with monthly pulse doses may reduce the incidence of secondary malignancies when compared with continuous oral therapy. Controlled trials are needed to further evaluate the efficacy of this mode of therapy.

Pigmentary diseases.

Abnormalities of pigmentation are common and frequently produce great concern in patients presenting to primary care physicians. Although many pigmentary changes arise as a consequence of inflammatory skin disorders, it is important to differentiate a primary skin disease from postinflammatory changes. Early recognition of a pigmentary disease allows the clinician to begin appropriate therapy at a stage when medical intervention may be more effective. Although many skin disorders are mainly of cosmetic concern, the condition may be devastating psychologically, requiring the clinician to be sensitive to the overall impact of the disorder and treat it accordingly.

Disorders of hyperpigmentation.

Hyperpigmentation is a common disorder of the skin, particularly in brown-skinned patients. Melasma is a common cause of facial hyperpigmentation and can be resistant to treatment. A combination of topical creams and gels, chemical peels, and sunscreens may be necessary for significant improvement. Erythema dyschromicum perstans is a dermal pigmentation seen on the trunk and proximal extremities, most commonly presenting in dark-skinned Hispanics. Drug-induced and postinflammatory hyperpigmentation may last for many months after the offending drug or dermatitis has been eliminated. These disorders, including their management, is reviewed in this article.

Pulse intravenous cyclophosphamide therapy for dermatologic disorders.

Cyclophosphamide is a potent immunosuppressive agent that has an important role in the treatment of autoimmune, neoplastic, granulomatous, and neutrophilic disorders. Pulse intravenous cyclophosphamide has been shown to be efficacious for several dermatologic disorders, particularly pemphigus vulgaris, with a low incidence of toxicity reported. As reported earlier, studies performed on the use of pulse intravenous cyclophosphamide in the treatment of a variety of dermatology-related diseases strongly suggest that the toxicities frequently noted with the use of oral cyclophosphamide therapy may be significantly less common with pulse intravenous administration of cyclophosphamide. The short follow-up period of patients treated with this modality so far, however, requires constant vigilance for the development of side effects, particularly secondary malignancy. At this time, pulse intravenous cyclophosphamide is a promising treatment modality with an acceptable risk profile for moderate-to-severe dermatologic diseases recalcitrant to standard therapy. Prospective comparative trials are needed to assess further the efficacy and toxicity of this therapy.

Nontuberculous mycobacterial infections of the skin.

Nontuberculous mycobacteria are playing an increasingly important role in human disease owing to higher prevalence of antibiotic resistance and immunodeficiency. These organisms cause a variety of cutaneous findings which are often misdiagnosed by the clinician. Compounding this problem is the fact that most mycobacteria require special culture conditions, which if not specifically requested, are frequently not used. Recognition of susceptible patients is imperative and is not limited to the immunocompromised. Successful treatment of mycobacterial infections requires knowledge of currently available and recommended antibiotics followed by tailoring of the antimicrobial regimen after sensitivity testing is performed.