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.

Neglect leads to extremes: maggots and malignancy in a case of discoid lupus erythematosus.

Discoid lupus erythematosus (DLE) is a chronic form of cutaneous lupus erythematosus that runs an indolent course. The rare complications of DLE include scarring, mutilation, non-healing ulceration, cicatricial alopecia and malignancy. DLE progresses to systemic lupus erythematosus (SLE) in around 5% of localized cases and 22% of generalized cases. We report a case of DLE, presenting with a six-month history of ulcerated fungating plaques and small crusted nodules superimposed on DLE plaques over both the forearms. Two weeks prior to the presentation, maggots were also noticed on these plaques. Skin biopsies from these lesions were suggestive of squamous cell carcinoma (SCC) and keratoacanthoma. A wide surgical excision of the tumor followed by partial split-thickness skin grafting was performed with complete healing of the lesions. No recurrence has been noted 18 months from follow-up.

Measurement properties of the Vitiligo Impact Scale-22 (VIS-22), a vitiligo-specific quality-of-life instrument.

BACKGROUND: Vitiligo has a significant psychological impact which needs to be evaluated separately from the extent of depigmentation. We have developed a vitiligo-specific quality-of-life (QoL) instrument, Vitiligo Impact Scale-22 (VIS-22) for this purpose. OBJECTIVES: To study the measurement properties of VIS-22 and compare it with the Dermatology Life Quality Index (DLQI) and Skindex-16. METHODS: Item-reduction analysis was used to reduce the number of items in the original VIS from 27 to 22. The 5-point Physician's Global Assessment (PGA) was used to evaluate the QoL followed by a Visual Analogue Scale (VAS) to assess patient-perceived severity. VIS-22, DLQI and Skindex-16 were self-administered. The validity of the VIS-22 was assessed in 161 patients, reliability in 69 patients and responsiveness in 72 patients and compared with DLQI and Skindex-16. RESULTS: Criterion validity was shown by strong correlation of VIS-22 with VAS (r = 0·7076). Convergent validity was evidenced by strong correlations with DLQI (r = 0·71) and Skindex-16 (r = 0·72). Known-groups validity was demonstrated by significantly higher scores in females, those with less education, patients with progressive disease and patients with vitiligo compared with controls (P < 0·001). Reliability was shown by excellent correlation of the scores between baseline and 2 weeks (r = 0·9053). VIS-22 was found to be responsive with scores at 12 weeks moving parallel to scores on VAS. Similar trends were noted with DLQI and Skindex-16. CONCLUSIONS: VIS-22 is a valid, reliable and responsive QoL instrument. It is comparable to DLQI and Skindex-16 in its measurement properties, while being specific to the needs of patients with vitiligo.

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.

A clinical study of the spectrum of photodermatoses in dark-skinned populations.

BACKGROUND: Photodermatoses are characterized by an abnormal cutaneous response to 'ordinary' light exposure. AIM: To study the spectrum of photodermatoses in populations with dark skin (skin types IV-VI) at a tertiary referral centre. METHODS: Consecutive patients with skin lesions confined to or predominantly located on photoexposed parts of the body and/or with photosensitivity were enrolled in the study, and their clinical details were recorded. Diagnosis was made on clinical grounds, and relevant investigations were carried out if required. Patch and photopatch testing were carried out in patients with chronic actinic dermatitis (CAD). Selected patients with CAD also underwent phototesting with UV (ultraviolet) A and broadband UVB light. RESULTS: We enrolled 362 patients (146 men, 216 women; mean age 35.6 ± 13.6 years), with mean disease duration of 3.4 years. The Fitzpatrick skin types were IV and V (52.8% and 47.2% of patients, respectively). Polymorphic light eruption (PMLE) was the commonest photodermatosis seen, affecting 59.7% of patients, followed by CAD (13.8%), collagen vascular disorders (7.7%), photoaggravated atopic dermatitis (6.1%), actinic lichen planus (ALP; 2.2%) and lichen planus pigmentosus (LPP; 1.6%). The majority (84.5%) of patients were involved in indoor work. Papular PMLE (37%) was the most common variant of PMLE, followed by pinpoint (31%), eczematous (22.2%), lichenoid (5.5%) and plaque-type (4.1%) PMLE. CONCLUSIONS: The spectrum of photodermatoses in Indian patients with dark skin phototypes (IV and V), is similar to that reported from other parts of the world. PMLE was the commonest photodermatosis seen, with the pinpoint and lichenoid variants accounting for over one-third of the PMLE cases. ALP and LPP were also not uncommon in our dark-skinned population.

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.

Vitamin D deficiency and rickets in children and adolescents with ichthyosiform erythroderma in type IV and V skin.

BACKGROUND: Ichthyosiform erythroderma due to keratinizing disorders may suppress cutaneous vitamin D synthesis, leading to vitamin D deficiency and rickets. OBJECTIVES: To determine the prevalence of vitamin D deficiency and rickets in children and adolescents with congenital ichthyosis and other keratinizing disorders with erythroderma and scaling. PATIENTS AND METHODS: In this cross-sectional study, 45 children and adolescents with ichthyosiform erythroderma due to keratinizing disorders, and 66 controls (group 1: age and sex matched, with skin diseases other than keratinizing disorders; group 2: age and sex matched, healthy volunteers) were included. Evidence of rickets was determined clinically (physical examination and radiographs) and biochemically {serum calcium, phosphorus, alkaline phosphatase, 25-hydroxy vitamin D [25(OH)D] and parathyroid hormone (PTH)}. RESULTS: All patients in the disease group had clinical, radiological or biochemical evidence of rickets [25(OH)D<20ngmL(-1) ], and analysis was done for all subjects with the available biochemical reports. The mean serum 25(OH)D levels of the disease group was 8·38±5·23ngmL(-1) and was significantly lower than in control group 1 (11·1±5·8ngmL(-1) ) (P<0·01) and control group 2 (13·5±6·9ngmL(-1) ) (P<0·001). The prevalence of vitamin D deficiency [25(OH)D<20ngmL(-1) ] was significantly higher in the disease group (n=38 of 39, 97·4%) than in control group 2 (n=12, 70·6%) (P<0·01), and total controls (n=56, 84·8%) (P=0·04). The frequency of hyperparathyroidism (PTH>65pgmL(-1) ) was also significantly higher in the disease group than in controls (P<0·01). CONCLUSIONS: Children and adolescents with various forms of ichthyosiform erythroderma, especially those with pigmented skin (types IV-VI), are at increased risk of developing vitamin D deficiency and clinical rickets.

Ichthyosiform erythroderma with rickets: report of five cases.

We describe five children with ichthyosis and rickets. The association of ichthyosis and rickets is very rare. Four children had lamellar ichthyosis and one child had nonbullous ichthyosiform erythroderma/psoriasis with atopy. All had biochemical and radiological evidence of rickets. Three had clinically evident rickets, of whom two had very severe skeletal deformities. Such a severe skeletal involvement due to rickets in association with ichthyosis is exceptionally rare. We suggest that children with severe ichthyosis, in particular those with pigmented skin, need to be evaluated for rickets, especially in developing countries where there is a background prevalence of vitamin D deficiency.

Familial pachydermoperiostosis in association with protein-losing enteropathy.

In this report we describe a rare association of pachydermoperiostosis with protein-losing enteropathy (PLE) in a family of three brothers. The first brother had the complete form of pachydermoperiostosis along with PLE. The second brother had the 'forme fruste' of pachydermoperiostosis, with minimal skin changes, bony abnormalities and PLE, which was due to intestinal lymphangiectasia. The third brother had an incomplete form of pachydermoperiostosis without evidence of PLE. To our knowledge, the association of pachydermoperiostosis with PLE due to intestinal lymphangiectasia has not been reported previously.

Osteoma cutis in pseudohypoparathyroidism.

Osteoma cutis is the formation of normal bone in the skin. Primary osteoma cutis occurs de novo, whereas the secondary type develops in association with the underlying inflammatory, tumorous or traumatic conditions. Primary osteoma cutis is also associated with Albright's hereditary osteodystrophy (AHO), which can include hypocalcaemic-type pseudohypoparathyroidism (also known as pseudohypoparathyroidism type Ia) or normocalcaemic-type pseudohypoparathyroidism (also known as pseudopseudohypoparathyroidism). We describe a case of osteoma cutis in a 7-year-old boy who had cutaneous, biochemical and phenotypic features of pseudohypoparathyroidism type Ia and AHO.

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.

Use of simple hematological, biochemical and clinical parameters to monitor response of multiple myeloma patients on high dose thalidomide therapy.

BACKGROUND: Evidence of increased bone marrow vascularity in multiple myeloma (MM) has led to the use of anti-angiogenic drugs especially thalidomide in relapsed or refractory patients. Currently, parameters such as serum/ urine electrophoresis for M (monoclonal) proteins, bone marrow biopsy with touch preparation and b2 microglobulin are routinely used to assess response to therapy. These investigations are expensive, invasive and require high technical setup. AIM: To correlate simple and routine hematological and biochemical parameters with the key marker of disease i.e. M proteins. SETTINGS AND DESIGN: This is an open label, uncontrolled, single-arm study. MATERIALS AND METHODS: Twenty nine refractory or relapsed multiple myeloma patients of both sexes (M=20, F=9) with age ranging between 35-72 years were initiated on 200 mg/day of thalidomide with fortnightly increments of 200 mg to a maximum tolerated dose not exceeding 800 mg/day. All hematological and biochemical parameters were monitored at monthly intervals for one year. STATISTICAL ANALYSIS: Correlation analysis was performed between hemoglobin (Hb), total leukocyte count (TLC), absolute neutrophil count (ANC), platelet count (PC), total proteins (TP), serum albumin and serum globulin on one hand and M protein levels on the other using Pearsons Correlation test by SPSS version 7.5. RESULT: Hb, TLC, ANC, PC and serum albumin levels showed a significant negative correlation with M proteins. A highly significant positive correlation existed between M proteins on one hand and TP and globulin levels on the other. Dryness of skin indicated positive response to therapy. These correlations were found to be significant at the end of one month of therapy in all the above-mentioned parameters except in TLC where it was significant after 2 months of thalidomide therapy. CONCLUSION: Results suggest that sustained efficacy of thalidomide therapy may be amenable to monitoring by these simple, inexpensive and easily available investigations after ascertaining an initial response by M protein and marrow plasmacytosis as these parameters closely follow M protein levels. However more studies are required to further substantiate these findings.

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.