Aquagenic Wrinkling of Skin: A Screening Test for Cystic Fibrosis

Objectives: To study the utility of aquagenic wrinkling asscreening test for children with cystic fibrosis.Design: Evaluation of diagnostic test.Setting: Pediatric Chest Clinic, and Pediatric Wards of a tertiarycare hospital in New Delhi.Participants: Three groups (children with cystic fibrosis,carriers of cystic fibrosis, and controls).Method: Time taken to develop aquagenic wrinkling wasmeasured. The test was performed by asking the enrolled subjectto put their one hand in water and was checked for development ofwrinkling every minute, and a photograph was also taken everyminute.Results: A total of 64 children with cystic fibrosis, 64 controls and64 carriers were enrolled in the study. Median (IQR) time todevelop aquagenic wrinkling in the three groups was 2 (1.5,3)minutes, 4 (3,5) minutes and 8 (5,11) minutes, respectively. Theoptimal cut-off was calculated as 3 minutes by Receiveroperating characteristic curve with a sensitivity and specificityfor identification of children with cystic fibrosis as 81% and 57%,respectively. The area under curve was 76.5%. The 3 minutecut-off for development of aquagenic wrinkling was applied to 54children referred for sweat test. 20 children had sweat chloridevalues of ≥60 mEq/l and diagnosed as cystic fibrosis. 15 of thesedeveloped aquagenic wrinkling at ≤3 minutes, giving a sensitivityof 75%.Conclusion: In places with no facility for sweat test, childrenwith phenotype compatible with cystic fibrosis who developaquagenic wrinkling in 3 minutes may be diagnosed as probablecystic fibrosis and referred for confirmation by sweat tes

Clinical effifi cacy of rituximab in the treatment of pemphigus: A retrospective study.

Background: Pulsed corticosteroids have been used successfully for the management of pemphigus. However, prolonged use of glucocorticoids may be associated with adverse effects and some patients show a poor response to conventional therapy. Biologics have shown a promising role in such cases; however, there is limited data from the Indian subcontinent. Objective: The primary objective was to assess the effi cacy and adverse effects of rituximab in pemphigus. The secondary objective was to measure the cumulative doses of corticosteroids required for these patients. Methods: We undertook a retrospective review of records of 25 pemphigus patients (pemphigus vulgaris: 21, pemphigus foliaceus: 4) who had received rituximab infusion (rheumatoid arthritis protocol in 21 patients, modifi ed in 4). Oral prednisolone was administered in dosages up to 0.5 mg/kg of body weight and tapered over the next 3–4 months according to the disease activity. However, other immunosuppressive agents such as cyclophosphamide and azathioprine were continued for one year after clinical remission was achieved. Results: Complete remission was observed in 22 (88%) patients. The mean time to disease control and complete remission was 1.10 and 4.36 months, respectively. Four (16%) patients experienced relapse after a mean duration of 11.75 months. The mean total dose of oral steroids administered was equivalent to 3535.64 mg of prednisolone. Exacerbation of disease was noted in two patients after the fi rst dose of rituximab and infectious complications, pneumonia and cellulitis, developed in one patient each. Limitations: A small sample size, the retrospective nature of the study and unavailability of follow-up anti-desmoglein autoantibodies levels were limitations. Conclusion: Rituximab is an effective agent in the treatment of pemphigus. The use of rituximab enabled use of a lower initial dose of oral prednisolone in pemphigus and hence reduced its total cumulative dose. Severe side effects were rare.

Cutaneous tuberculosis due to multidrug-resistant tubercle bacilli and difficulties in clinical diagnosis.

This report describes 6 HIV-negative patients including 5 children with scrofuloderma and an adult with lupus vulgaris, out of a total of 303 cases of cutaneous tuberculosis seen during a 4½-year period, who showed a positive tuberculin test and granulomatous histopathology, but failed to respond to fi rst-line antitubercular therapy. They were suspected to have multidrug-resistant infection as no other cause could be ascertained. Tissue aspirate or biopsy was sent for histopathology and culture. Mycobacterium tuberculosis was isolated from the aspirate in three patients and sputum in one with associated pulmonary tuberculosis. Drug susceptibility tests showed that all isolates were resistant to rifampicin and isoniazid, and one each additionally to streptomycin and ethambutol, respectively. In two, culture was unsuccessful. All were administered second-line antitubercular drugs. Clinical improvement was appreciable within 2 months as weight gain, and regression of ulcers, swellings and plaques. Two completed the recommended 24 months of therapy. Multidrug-resistant cutaneous tuberculosis should be suspected in patients with no response to fi rst-line drugs, with clinical deterioration, and where other causes of treatment failure are not forthcoming. Owing to poor isolation rates on culture and low sensitivity of molecular tests, in such cases, a trial of second-line anti-tubercular drugs may be justifi ed for a reasonable period of 2 months. Where facilities permit, culture and drug sensitivity tests should be done before starting treatment. Culture positivity is better from aspirated material.

Weekly azathioprine pulse versus daily azathioprinein the treatment of Parthenium dermatitis: A non-inferiority randomized controlled study.

Background: Azathioprine in daily doses has been shown to be effective and safe in the treatment of Parthenium dermatitis. Weekly pulses of azathioprine (WAP) are also effective, but there are no reports comparing the effectiveness and safety of these two regimens in this condition. Aims: To study the effi cacy and safety of WAP and daily azathioprine in Parthenium dermatitis. Methods: Sixty patients with Parthenium dermatitis were randomly assigned to treatment with azathioprine 300 mg weekly pulse or azathioprine 100 mg daily for 6 months. Patients were evaluated every month to assess the response to treatment and side effects. Results: The study included 32 patients in the weekly azathioprine group and 28 in the daily azathioprine group, of whom 25 and 22 patients respectively completed the study. Twenty-three (92%) patients on WAP and 21 (96%) on daily azathioprine had a good or excellent response. The mean pretreatment clinical severity score decreased from 26.4 ± 14.5 to 4.7 ± 5.1 in the WAP group, and from 36.1 ± 18.1 to 5.7 ± 6.0 in the daily azathioprine group, which was statistically signifi cant and comparable (P = 0.366). Patients on WAP had a higher incidence of adverse effects (P = 0.02). Limitations: The study had a small sample size and the amount of clobetasol propionate used in each patient was not determined, though it may not have affected the study outcome due to its comparable use in both groups. Conclusions: Azathioprine 300 mg weekly pulse and 100 mg daily dose are equally effective and safe in the treatment of Parthenium dermatitis.

Parthenium dermatitis treated with azathioprine weekly pulse doses.

BACKGROUND: Parthenium dermatitis is a serious problem in India. Corticosteroids are the mainstay of treatment but the prolonged use of corticosteroids can cause serious side effects. Azathioprine used in daily doses has been shown to be effective. AIM: We have evaluated the effectiveness of azathioprine weekly pulse doses for the treatment of parthenium dermatitis. METHODS: Twelve patients, ten males and two females, aged between 39 and 65 years (mean +/- SD = 53.5 +/- 8.7) having air-borne contact dermatitis to Parthenium hysterophorus for 3-19 years (mean = 6.33) were included in the study. The diagnosis in each patient was confirmed by patch-testing. The severity of the disease was determined by clinical severity score (CSS) on the basis of erythema, itching, type of lesions, and areas of body involved. RESULTS: The pretreatment CSS in these patients varied from 29.7 to 55.5 (mean +/- SD: 40.40 +/- 7.95). After clinical and laboratory evaluation, the patients were treated with 300-mg azathioprine once-weekly doses for 6 months. Clinical and laboratory evaluations were repeated at weeks 1, 2, and then every 4 weeks until the end of therapy to evaluate the therapeutic response and side effects. The response was excellent (80-100% clearance of disease) in seven (58.33%) patients and good (60% clearance) in five (41.66%) patients. The post-treatment CSS decreased from the mean +/- SD of 40.4 +/- 7.95 to 10.9 +/- 8.43 (P = 0.002). There were no significant side effects of the therapy. CONCLUSIONS: In this preliminary open study, azathioprine in weekly pulse doses has been found to be effective without any serious adverse effects in the treatment of parthenium dermatitis. The cost of therapy with this regimen is reduced by 60%.

KOH mount for superficial fungal infections using cellophane tape: comparison with standard technique.

Fifteen patients with superficial fungal infections (tinea versicolor--9, dermatophytic infections--6) were included in this study. Skin scrapings were collected directly on to the glass slide and also by using cellophane tape. Both the samples were mounted with KOH. Fungal elements were detected by both the methods in all cases and was better visualised by the tape method.

Cutaneous adverse drug reactions: clinical pattern and causative agents--a 6 year series from Chandigarh, India.

AIM: To study the different clinical spectrum of cutaneous adverse drug reactions (ADR) and to determine the causative drugs. MATERIALS & METHODS: A prospective, hospital based study was carried out over a period of 6 years recording various cutaneous ADR. RESULTS: A total of 500 patients with cutaneous ADR were enrolled in the study. The most common types of cutaneous ADR patterns were maculopapular rash (34.6%), fixed drug eruption (FDE) (30%) and urticaria (14%). The drugs most often incriminated for the various cutaneous ADR were antimicrobials (42.6%), anticonvulsants (22.2%) and NSAIDs (18%). Anticonvulsants were implicated in 41.6% of maculopapular rashes. Sulfonamides accounted for 43.3% and NSAIDs for 30.7% of FDE. Urticaria was caused mainly by NSAIDs(24.3%) and penicillins(20%). Anticonvulsants were responsible for 43.8% of life-threatening toxic epidermal necrolysis and Stevens Johnson syndrome. CONCLUSIONS: The clinical pattern and drugs causing cutaneous ADR are similar to those observed in other countries except for minor variations. Cutaneous ADR patterns and the drugs causing various reactions are changing every year, which may be due to the emergence of newer molecules and changing trends in the use of drugs.