Randomized double blind trial of prednisolone and azathioprine, vs. prednisolone and placebo, in the treatment of pemphigus vulgaris.

BACKGROUND: The classic treatment for pemphigus vulgaris is prednisolone. Immunosuppressive drugs can be used in association. OBJECTIVE: To compare the efficacy of Azathioprine in reducing the Disease Activity Index (DAI). PATIENTS AND METHODS: A double blind randomized controlled study was conducted on 56 new patients, assigned to two therapeutic groups: (i) prednisolone plus placebo; (ii) prednisolone plus Azathioprine. Patients were checked regularly for 1 year. 'Complete remission' was defined as healing of all lesions after 12 months, and prednisolone <7.5 mg daily, (DAI ≤ 1). Analysis was done by 'Intention To Treat' (ITT) and 'Treatment Completed Analysis' (TCA). RESULTS: Both groups were similar in age, gender, disease duration, and DAI. Primary endpoint: By ITT and TCA, the mean DAI improved in both groups with no significant difference between them. The difference became significant for the last trimester (3 months; ITT: P = 0.033, TCA: P = 0.045). Secondary endpoint: The total steroid dose decreased significantly in both groups, with no significant difference between them, except for the last trimester (ITT: P = 0.011, TCA: P = 0.035). The mean daily steroid dose decreased gradually in both groups becoming statistically significant in favour of azathioprine, in the last trimester, especially at 12th months (ITT: P = 0.002, TCA: P = 0.005). Complete remission was significant at 12 months only for TCA (AZA/Control: 53.6%/39.9%, P = 0.043). LIMITATIONS: Sample size was rather small to demonstrate all differences. Other limitations include the choice of primary and secondary endpoints and the unavailability to measure thiopurine methyltransferase activity. CONCLUSION: Azathioprine helps to reduce prednisolone dose in long-run.

Efficacy and safety of mycophenolate mofetil vs. methotrexate for the treatment of chronic plaque psoriasis.

BACKGROUND: Methotrexate (MTX) is a well-known systemic drug for moderate to severe chronic plaque psoriasis. Recently, mycophenolate mofetil (MMF) has been recommended for psoriasis. OBJECTIVE: To compare the efficacy and safety of MMF vs. MTX for the treatment of chronic plaque psoriasis. METHODS: Thirty-eight consecutive patients with Psoriasis Area and Severity Index (PASI)>10 were randomly assigned for 12 weeks of treatment with either MTX (18 patients; initial dose, 7.5 mg/week) or MMF (20 patients; dose; 2 g/day) and were followed for 12 weeks after discontinuing the treatment. The differences between the two groups were analysed at the end of treatment and follow-up comparing with baseline values. RESULTS: After 12 weeks of treatment, the mean ± SD score for the PASI decreased from 16.46 ± 5.29 at baseline to 3.17 ± 2.35 among 15 patients treated with MTX, whereas the score decreased from 17.43 ± 7.42 to 3.97 ± 5.95 among 17 patients treated with MMF (P>0.05). Twelve weeks after discontinuing the treatment, the scores were 4.77 ± 3.52 and 5.94 ± 4.27, respectively (P>0.05). PASI -75 were achieved in 58.8% of patients in MMF group and 73.3% in MTX group (P > 0.05). Three months after discontinuing the treatment, PASI-75 remained in 33.3% of patients in MMF and 53.3% of MTX group (P > 0.05). Both drugs were well tolerated and side-effects were minor and transient. CONCLUSIONS: No significant differences in efficacy were found between MTX and MMF groups. MMF may represent a good alternative for the treatment of psoriasis in patients who are unable to take MTX or other available drugs due to contraindication or toxicity.

A study on plucked hair as a substrate for direct immunofluorescence in pemphigus vulgaris.

BACKGROUND: It has recently been demonstrated in a study on 15 patients that plucked hair can be used as a substrate for direct immunofluorescence (DIF) in pemphigus. OBJECTIVE: Our aim was to assess the sensitivity of DIF on plucked hairs in pemphigus vulgaris (PV) patients with positive DIF of oral mucosa. METHODS: One hundred and ten new PV patients were enrolled in the study. They all showed the typical clinical and histological findings as well as positive DIF of the oral mucosa, diagnostic for PV. Approximately 30 hairs were obtained in the same way as for the trichogram. The hairs with their outer root sheaths (ORS) were processed for DIF in order to detect immunoglobulin G and C3. RESULTS: Immunodeposits favouring PV were demonstrated in the ORS of 100 cases showing a sensitivity of 91%. CONCLUSION: Regarding the relatively high sensitivity of DIF on plucked hair in PV patients with positive oral mucosal DIF in our study, it seems that hair plucking is a suitable alternative to the more invasive techniques of skin or mucosal biopsy for obtaining specimens for DIF in PV.

Cervicovaginal involvement in pemphigus vulgaris: a clinical study of 77 cases.

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune blistering disease which is known to involve the female genital tract, but the frequency at which this occurs is unknown. There are few reports in the literature of the cytological appearance of PV on cervicovaginal smears. OBJECTIVES: To evaluate involvement of the female genital tract and the appearance of cervicovaginal Papanicolaou (Pap) smears in PV. METHODS: The study included 77 patients with PV who attended between April 2005 and February 2007. Each patient was subjected to gynaecological examination and cervicovaginal Pap smear. RESULTS: Genital lesions were observed in 39 patients (51%). Of these, the labia minora were involved in 36 patients (92%), the labia majora in 11 (28%), the vagina in 14 (36%) and the cervix in six (15%). Cervicovaginal Pap smears of 20 of 77 patients (26%) showed PV. Of 72 satisfactory Pap smears, the cervical Pap smear was normal in 25 patients (35%), inflammatory in 43 patients (60%), and dysplastic (low-grade squamous intraepithelial neoplasia) in four patients (6%). CONCLUSIONS: Involvement of the female genital tract with PV might not be as infrequent as was previously thought; it is probably the second most common mucosal site of PV after the oral mucosa. Genital lesions may be missed and the need for thorough pelvic examination should not be overlooked.

The lipid profile in psoriasis: a controlled study.

BACKGROUND: Psoriasis is an inflammatory dermatosis that is characterized with hyperproliferation of keratinocytes and inflammatory infiltration in the epidermis and dermis. The high prevalence of atherosclerosis has been reported in psoriatic patients. High serum lipid level has been suggested in the pathogenesis of this phenomenon. In this study, our purpose was to compare the lipid profile in psoriatic patients with non-affected persons. METHODS: This study was designed and conducted as a case-control assay with 50 cases in the patient and control groups, respectively. The lipid profile, including serum level of triglyceride, cholesterol, low-density lipoprotein (LDL), and high-density lipoprotein (HDL), were assessed in both groups. RESULTS: The patient and control groups each consisted of 50 cases (39 male and 11 female). The serum triglyceride, cholesterol, and LDL was significantly higher in psoriatic patients (P < 0.05) but not for HDL (P = 0.29). CONCLUSION: This study, like previous assays, shows that high serum lipid level is significantly more common in psoriasis. This fact may be responsible for higher prevalence of cardiovascular accident in psoriatic patients. It may be useful to do early screening and treatment of hyperlipidaemia in psoriasis to prevent the atherosclerosis and its complications.

The association of pyoderma faciale and erythema nodosum.

Pyoderma facial (PF) is a sudden severe eruption of pustules and cystic swellings which may be interconnected by sinuses. It affects mainly adult women. We report the case of a 21-year-old woman presenting with sudden onset of coalescing nodules and abscesses on the face, with mild systemic disturbance. She also had erythema nodosum (EN)-like lesions on the legs. To the best of our knowledge, this is the first reported case of PF associated with EN.

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.

Diffuse plane xanthoma in an otherwise healthy woman.

We report a case of diffuse plane xanthoma in a 40-year-old otherwise healthy woman. Her disease began 18 years ago as xanthelasma and progressed to involve large areas of her face, neck and trunk. No associated diseases were detected on repeated laboratory testing.

Case report. An unusual case of cutaneous sporotrichosis and its response to weekly fluconazole.

Cutaneous sporotrichosis is clinically divided into two main types: lymphocutaneous and fixed plaque type. Our report represents an unusual case with both types simultaneously. Fluconazole 150 mg once weekly was used as the treatment. The fixed type lesions responded very well and were healed after 4 months, but the lymphocutaneous lesions were not controlled even after 6 months of the treatment.