Loss of normal anagen hair in pemphigus vulgaris.

BACKGROUND: Pemphigus vulgaris (PV) is a known cause of loss of 'normal' anagen hair; that is, shedding of intact anagen hairs covered by root sheaths. However, studies on this subject are limited. AIM: To investigate anagen hair shedding in patients with PV, and ascertain its association with disease severity. METHODS: In total, 96 consecutive patients with PV (new patients or patients in relapse) who were admitted to the dermatology wards of a tertiary hospital were enrolled in this study. Demographic data, PV phenotype, disease severity and presence of scalp lesions were recorded. A group of 10-20 hairs were pulled gently from different areas of the scalp (lesional and nonlesional skin) in all patients, and anagen hairs were counted. Disease severity was graded according to Harman score. RESULTS: Anagen hair was obtained by pull test in 59 of the 96 patients (61.5%), of whom 2 had normal scalp. The mean ± SD anagen hair count was 5.9 ± 7.6 (range 0-31). In univariate analysis, anagen hair loss (P < 0.01) and the presence of scalp lesions (P = 0.01) were associated with severe disease. Mean anagen hair count was significantly higher in the severe (mean 6.83 ± 7.89) than the moderate (mean 1.06 ± 1.94) subgroup (P < 0.001). Multivariate analysis confirmed anagen hair loss (OR = 1.16, 95% CI = 1.05-1.28, P < 0.01), but not scalp lesions (P = 0.69) as an independent predictor of disease severity. CONCLUSIONS: According to our study, normal anagen effluvium is a frequent finding in patients with PV, and interestingly, this was observed in nonlesional as well as lesional scalp. In addition, severe anagen hair loss was an independent predictor of the disease severity.

[Open trial of mycophenolate mofetil in the treatment of resistant pemphigus vulgaris]. / Etude ouverte du mycophénolate mofetil dans le traitement du pemphigus résistant.

BACKGROUND: Pemphigus vulgaris is a severe autoimmune blistering disease of the skin and mucous membranes. In absence of treatment, mortality is high. In the past, prednisolone was the best treatment. Later, combination therapy with systemic prednisolone and other disease-modifying drugs was tried with better results. Unfortunately, some patients do not respond well to such treatment, or may exhibit multiple recurrences or complications. Some other patients may remain on high dose corticosteroids to maintain remission. OBJECTIVE: To evaluate the efficacy and safety of mycophenolate mofetil as a steroid sparing agent in the treatment of resistant pemphigus vulgaris. METHODS: We administered 2 g daily mycophenolate mofetil with systemic steroids to 10 patients with resistant and severe disease who did not respond to conventional therapy, or had multiple recurrences. RESULTS: Nine of the ten patients responded to treatment and showed complete clearance of lesions within 6 to 16 weeks of therapy. At the end of six months, the dose of prednisolone was significantly lower. Side effects were few and mild. After discontinuation of mycophenolate mofetil, 5 of the 9 patients relapsed. CONCLUSION: Mycophenolate mofetil is effective and safe as a disease-modifying drug combined with prednisolone in the treatment of patients with resistant pemphigus vulgaris. To induce long lasting remission has to be administrated for more than 6 months.

Pemphigus and associated environmental factors: a case-control study.

BACKGROUND: Recent reports have revealed the relatively high incidence of pemphigus in Iran. Occupational exposure and personal habits have been suggested to play a role in the aetiopathogenesis of this life-threatening disease. AIM: In order to analyse the association of environmental factors with pemphigus, we conducted a case-control study to evaluate the possible role of smoking, pesticide exposure and hormonal factors in Iran. METHODS: This study was conducted in Iran using a structured questionnaire. Questions included information on patients' smoking habits, occupational exposure to pesticides, use of oral contraception (OC) and number of pregnancies. RESULTS: We enrolled 210 patients with pemphigus and 205 control subjects. Fewer of patients with pemphigus (17.1%) reported a current or past history of smoking, which was statistically different from the control group (27.3% smokers). The duration of smoking and the number of cigarettes smoked daily was also significantly lower in patients. Although OC use was significantly higher in women with pemphigus, the mean number of pregnancies was not different between the two groups. Occupational exposure to pesticides was significantly higher in patients with pemphigus (14.8%) than in controls (5.4%); patients with pemphigus were exposed to pesticides three times more often than were healthy subjects. CONCLUSION: As a positive history of smoking was lower in patients with pemphigus compared with healthy subjects, it seems that smoking is a protective factor in pemphigus. This should encourage further investigations, searching for novel therapies. If pesticides and OC are confirmed as triggering factors, their cessation might reduce the need for pharmacological therapy.

Oculocutaneous tyrosinaemia or tyrosinaemia type 2: a case report.

Oculo-cutaneous tyrosinaemia type II is an autosomal recessive disease due to an abnormality of tyrosine metabolism, probably because of a deficiency of cytoplasmic tyrosine aminotransferase. It presents as a varying association of focal palmoplantar keratosis, bilateral keratitis and mental retardation. Herein, we report an 8-year-old boy with palmoplantar hyperkeratosis with peripheral oozing and dendritic keratitis appearing after the skin lesions. There was no mental deterioration despite the long delay in diagnosis of the disorder. The diagnosis was confirmed by the presence of hypertyrosinaemia and the absence of hepatorenal lesion. The child exhibited a remarkable degree of improvement in the hyperkeratotic lesions and keratitis after the dietary modifications were instituted. In conclusion, chronic focal bullous palmoplantar hyperkeratosis along with keratitis should alert the clinician to screen for abnormal serum and/or urine tyrosine level. Awareness of the presenting signs and symptoms may speed up the diagnosis and initiation of a tyrosine and phenylalanine-restricted diet that is most efficient in improving the symptoms and preventing visual and cognitive impairment.