Family vitiligo impact scale: A scale to measure the quality-of-life of family members of patients with vitiligo.

BACKGROUND: Vitiligo places a significant psycho-social burden on caregivers and family members. AIMS: The aim of the study was to develop and preliminarily validate a scale to measure the psychosocial impact of vitiligo on adult family members. METHODS: Themes that emerged from qualitative interviews and a focus group discussion with family members were used to generate items for a preliminary scale, followed by pre-testing and scale development. The new scale was then tested with two comparator scales and a global question. RESULTS: A preliminary scale with 32 items was pilot tested on 30 participants. Following this, the scale was condensed to 16 items in 12 domains that were administered to 159 participants. Scale scores ranged from 0 to 48 with a mean of 19.75 ± 12.41. The scale had excellent internal consistency with Cronbach's alpha coefficient of 0.92 (0.70-0.95) and also showed good test-retest reliability at two weeks (r = 0.946). The scale showed criterion, convergent and known group validity. LIMITATIONS: It was conducted in a large teaching hospital which may have resulted in selection of patients with persistent or progressive disease and more worried family members. Vitiligo is highly stigmatized in our country and the performance of the scale may need to be evaluated in other communities and cultures as well where stigma is less oppressive. CONCLUSION: Family Vitiligo Impact Scale appears to be an easy-to-complete, reliable and valid instrument to measure the psychosocial impact of vitiligo in family members of patients. It may be useful as an outcome measure in both clinical and research settings.

The tangled web: a study of knowledge and attitude towards leprosy from a tertiary care hospital in India.

Leprosy stands tall among the oldest and most misunderstood diseases of man. Today leprosy is easily treated; unfortunately, persistent misconceptions result in unnecessary stigmatization. Thus the present study aims to assess the knowledge and attitudes regarding leprosy in people with and without leprosy, factors affecting the same; and to study their relationship with treatment status in leprosy patients. Detailed knowledge and attitude questionnaires were administered to 260 subjects (100 leprosy patients, 60 family members of leprosy patients and 100 people with non-leprosy skin diseases) at AIIMS, New Delhi. Crude scores based on subject responses were used for inter-group comparisons. Leprosy patients had significantly higher knowledge scores than family members who in turn scored significantly higher than people with other skin diseases. Leprosy patients had fair knowledge about common symptoms, but awareness about MDT was low. Delayed diagnosis and non-compliance were common. Attitudes did not differ between groups. Fear of the leprosy-affected and reluctance for physical contact, food sharing and marriage were prominent. Treated leprosy patients had the highest knowledge scores. Higher education and greater knowledge scores were positive predictors of attitude. Knowledge and attitude scores showed significant positive correlation. Knowledge and attitude towards leprosy are unsatisfactory. Improving knowledge may help to improve attitudes. In the post-elimination era, we must incorporate education about the disease into routine care of leprosy patients and focus on community education about leprosy.

Aggressive angiomyxoma of the vulva in a patient with systemic lupus erythematosus.

Aggressive angiomyxoma is a rare, slow-growing mesenchymal neoplasm with a tendency to recur. It mainly involves the pelvis, vulva, perineum, vagina, and urinary bladder in adult women of reproductive age group. We describe a 26-year-old female with large swellings of both labia majora which was histologically diagnosed as aggressive angiomyxoma. She also had systemic lupus erythematosus. The swelling was surgically removed and she had no recurrence at 1-year follow-up. Although it is a rare tumor, it must be considered as a differential diagnosis for any mass in the perineum or soft tissue of the pelvis. Long-term follow-up is necessary for early diagnosis of local recurrence.

Evaluation of a new fixed duration (12 weeks) multi-drug regimen of bactericidal drugs in multibacillary leprosy.

UNLABELLED: Leprosy still remains a public health problem mainly in Asia, Africa and South America. The WHO Expert Committee on Leprosy recommended, in 1997, the simplified treatment of leprosy for multibacillary (MB) cases, by reducing the duration of treatment from 24 to 12 months. From the operational point of view even this reduced duration is still long and monthly supervised drug administration may not always be practical in those areas where the accessibility is difficult and health infrastructure weak. The present study was carried out to compare the safety and efficacy of a new fixed duration regimen consisting of four bactericidal drugs with WHO/MDT (MB). METHODS: Thirty adult patients were randomly allocated to two groups. Group 1 (18 patients) received a new regimen of daily rifampicin 600 mg, sparfloxacin 200 mg, clarithromycin 500 mg and minocycline 100 mg for 12 weeks. Group 2 (12 patients) received WHO/MDT (MB) for 12 months. A detailed clinical evaluation and laboratory investigations, BI and MI were done at the baseline, every 4 weeks for 12 weeks, and thereafter every 8 weeks till 48 weeks. Skin biopsies were taken and chest X-rays were done at 0, 12 and 48 weeks. RESULTS: At 48 weeks, the net percentage clinical improvement in group 1 was 73.92% and in group 2 it was 66.66%. The net percentage reduction (NPR) in BI in group 1 was 19.17% and in group 2 it was 18.87% (p = 0.09). NPR in MI in both groups was 100% by 8 weeks, and somewhat faster in group 1. In group 1, 8 patients had mild gastrointestinal side-effect, and 16 had minocycline-induced hyperpigmentation. Three patients in group 1 developed type I reversal reactions. CONCLUSION: The regimen containing daily doses of rifampicin 600 mg, sparfloxacin 200 mg, minocycline 100 mg and clarithromycin 500 mg for 12 weeks was found to be an acceptable, effective and safe alternative regimen for MB leprosy with an additional operational advantage of shorter duration of treatment.

Randomised controlled trial of topical butenafine in tinea cruris and tinea corporis.

Butenafine is a new antifungal agent similar to allyl amine antifungals. A randomized controlled trial was conducted in 75 patients to compare its efficacy with clotrimazole in tinea cruris and corporis that was diagnosed on clinical features and demonstration of hyphae in a potassium hydroxide (KOH) preparation. Twenty patients treated with butenafine once daily for 2 weeks and 20 treated with clotrimazole twice daily for 4 weeks were analysed. At the end of treatment, 2 weeks and 4 weeks later, the KOH preparation was negative in 90.9%, 95.5% and 90.9% of patients respectively in the butenafine group and 100%, 96.4% and 92.85% respectively in the clotrimazole group. There was a reduction of 81.5% in the sign and symptom score at 4 weeks following treatment in the butenafine group and 85.93% in the clotrimazole group. There was no statistically significant difference between the two groups. Adverse effects were mild in both groups and did not require discontinuation of therapy except one patient treated with clotrimazole who developed dermatitis at the site of application. Butenafine appears to be as effective as clotrimazole in the treatment of tinea cruris and corporis while requiring a single daily application for a shorter of 2 weeks.

Chilblain lupus erythematosus mimicking acrofacial vitiligo.

Chilblain lupus erythematosus with depigmentation is a rare presentation of lupus erythematosus that may simulate vitiligo. A 52-year-old lady with such a manifestation is being reported.

Primary cutaneous amyloidosis involving the external ears along with the classical sites.

A 26-year-old woman had multiple itchy persistent gradually progressive papular lesions on the forearms and shins for 10 and 4 years respectively. She also noticed similar lesions on both the ears for 4 years. There were no systemic symptoms. Cutaneous examination revealed multiple 2-3 mm discrete firm hyperpigmented papules on the extensors of forearms, shins and earlobes. Skin biopsy from all sites demonstrated deposits of amyloid in the papillary dermis. The patient was treated with cyclophosphamide 50 mg daily orally. There was more than 50% improvement in her lesions.

Trachyonychia treated with oral mini pulse therapy.

A 12-year-old girl presented with dull, opalescent, nails with a roughened surface. She was successfully treated with oral mini-pulse therapy, which consisted of 4 mg betamethasone as a single oral dose with breakfast on two consecutive clays every week.

Multiple lichen striatus--an unusual presentation.

A 2-year-old mare child presented with multiple hypopigmented, flat topped papules with a bilateral distribution along the lines of Blaschko. A 7-year-old girl presented with multiple hypopigmented macules arranged linearly along the left upper and lower limb and also on the back along the lines of Blaschko. Three discrete lines in three distinct anatomical areas in a case of lichen striatus do not appear to have been described.

Syringocystadenoma papilliferum at unusual sites.

We describe two cases in which syringocystadenoma papilliferum was present in extremely unusual locations.

Differential representations of memory T cell subsets are characteristic of polarized immunity in leprosy and atopic diseases.

We identified functionally polarized subsets of CD4 memory T cells on the basis of the expression of CD11a, CD45RA and CD62L. Within the several phenotypically distinct subsets of CD4 memory cells are two that, upon stimulation, produce primarily IL-4 (MT(2), CD45RA(-)CD62L(+)CD11a(dim)) or primarily IFN-gamma (MT(1), CD45RA(-)CD62L(-)CD11a(bright)). In addition, four other phenotypically distinct subsets of CD4 cells have unique cytokine profiles. To determine the clinical relevance of the representation of these cell types, we analyzed blood from patients with the chronic diseases leprosy and atopy. These diseases are characterized as immunologically polarized, since T cell responses in affected individuals are often strongly biased towards T(h)1 (dominated by IFN-gamma production) or T(h)2 (IL-4 production). We show here that this polarization reflects homeostatic or differentiation mechanisms affecting the representation of the functionally distinct subsets of memory CD4 T cells, MT(1) and MT(2). Significantly, the representation of the MT(1) and MT(2) subsets differs dramatically between subjects with tuberculoid leprosy (a T(h)1 disease), or lepromatous leprosy or atopic disease (T(h)2 diseases). However, there was no difference in the cytokine profiles of these or any of the other finely resolved CD4 subsets, when compared between individuals across all disease states. Thus, it is the representation of these subsets in peripheral blood that is diagnostic of the polarized state of the immune system.

Olmsted syndrome.

A 20-year-old Sikh man had palmoplantar keratoderma, flexion deformity of digits, universal alopecia, keratotic plaques at the angles of mouth, gluteal cleft, knees and dorsal aspects of the metacarpophalangeal joints of the hand; features of Olmsted syndrome. He had normal nails, teeth, oral mucosa and normal joint movements. Treatment with acitretin, 25mg/day for three and a half months, followed by 25mg once daily alternating with 50mg once daily for 3 months resulted in significant improvement.