Guidelines for clinical trials of frontal fibrosing alopecia: consensus recommendations from the International FFA Cooperative Group (IFFACG).

BACKGROUND: Frontal fibrosing alopecia (FFA) has become one of the most common causes of cicatricial alopecia worldwide. However, there is a lack of clear aetiology and robust clinical trial evidence for the efficacy and safety of agents currently used for treatment. OBJECTIVES: To enable data to be collected worldwide on FFA using common criteria and assessment methods. METHODS: A multicentre, international group of experts in hair loss was convened by email to create consensus recommendations for clinical trials. Consensus was defined at > 90% agreement on each recommended part of these guidelines. RESULTS: Standardized diagnostic criteria, severity rating, staging, and investigator and patient assessment of scalp hair loss and other clinical features of FFA were created. CONCLUSIONS: These guidelines should allow the collection of reliable aggregate data on FFA and advance efforts in both clinical and basic research to close knowledge gaps in this condition.

Partial congenital arrhinia: never seen before adult presentation.

BACKGROUND: Arrhinia is defined as the partial or complete absence of the nasal structures. It is a defect of embryonal origin and can be seen in association with other craniofacial anomalies, central nervous system anomalies, absence of paranasal sinuses, and other palatal and ocular abnormalities. Very few patients with arrhinia have been reported so far in the history of modern medicine. CASE REPORT: This study reports an adult patient with congenital partial arrhinia and reviews the literature along with the embryological basis of such a rare disease. CONCLUSION: Arrhinia is a medical condition with scarce documentation in the literature. This article presents the clinical as well as radiological features of this rare entity.

Inter-rater reliability of a paediatric outcome measure in Nepal.

The Hospital and Rehabilitation Centre for Disabled Children (HRDC) in Nepal identified the need to evaluate their in-hospital and community programmes. An instrument was developed to provide information regarding the functional level of the children treated by the HRDC as well as to provide information regarding care-givers' attitudes towards disability. Inter-rater reliability of the measure was tested in three regions of Nepal with 49 children. Six HRDC field workers travelled in pairs to the childrens' homes and alternated in roles as test administrator and observer. Correlations between the scores documented by the administrator and observer were used to estimate inter-rater reliability. Inter-rater reliability coefficients, calculated using a weighted kappa statistic, varied from 0.60 to 1.0. We conclude that the instrument demonstrated an acceptable level of inter-rater reliability in the field setting. Future studies to measure construct and concurrent validity, test-retest reliability and responsiveness of the instrument are recommended as well as testing the instrument in different cultures.

PIP: The Hospital and Rehabilitation Center for Disabled Children (HRDC) in Nepal identified the need to evaluate their in-hospital and community programs. An instrument was developed to provide information regarding care-givers' attitudes towards disability. Six HRDC field workers traveled in pairs to the childrens' homes and alternated in roles as test administrator and observer. Inter-rater reliability of the measure was tested in a sample of 49 children 3-19 years old (55% male and 45% female) who lived in the Kathmandu Valley, the Terai, or hilly regions. The most common disability types treated at the HRDC were congenital talipes equinovarus or club foot (31.5%), poliomyelitis (15.1%), burn contractures (14.2%), tuberculosis (11.1%), and osteomyelitis (5.6%). Inter-rater reliability was estimated for instrument domains of functional status (self-care, mobility, and social function), use of adaptive equipment, and knowledge and attitudes towards disability. The total scores for each domain were compared between raters using the weighted kappa statistic (Kw). Mean reliability coefficients varied from Kw = 0.60 to Kw = 1.0. All mean values demonstrated substantial to almost perfect correlations, except for the calculated value for self care equipment (Kw = 0.60). However, a Fisher Z transformation revealed no significant difference between the calculated value of 0.60 and the acceptable value of 0.61. Individual reliability coefficients for pairs of raters were generally acceptable, with the exception of one pair (E + F) on the self-care equipment domain. The mean weighted kappa value for younger children was 0.70 +or- 0.02 for the mobility level of assistance scale and 0.72 +or- 0.06 for the mobility functional activities scale. On application of the Fisher Z transformation formula, significant differences were found for this subgroup of younger children (or= 6 years) as compared with the sample population. The instrument demonstrated an acceptable level of inter-rater reliability.

Topical tretinoin in acanthosis nigricans.

Efficacy of topical tretinoin was assessed in 30 cases of idiopathic acanthosis nigricans which were recalcitrant to conventional modalities of treatment. Topical tretinoin once at night application was found to be very effective both clinically and histologically.

Crushing assisted shave excision and electrodesiccation: A novel approach to treat granuloma pyogenicum.

BACKGROUND: Granuloma pyogenicum (GP) is a common vascular lesion which often bleeds easily during most of the treatment modalities. A number of pyogenic granulomas often recur after such treatment. OBJECTIVE: The aim of this study was to establish a surgical procedure which is easy to operate, less invasive, minimal bleeding at the operation site and without any recurrence of the lesion. METHODS: Twenty-three patients (15males, and 8females) suffering from granuloma pyogenicum at different stages (ulcerated, reepithelialized and resolving) and at various sites of the body constituted the sample population. In all the patients, crushing of the base of the lesions followed by shave excision and electrodesiccation of the shaved base were carried out. The patients were followed up for six months to observe recurrence, scar formation and any other complications. RESULTS: Bleeding was practically nil during the surgical procedure in all the cases. There was no recurrence and cosmetic outcome was excellent. No complications were encountered. CONCLUSION: The technique is not only an alternative modality but can also be the treatment of choice in all stages of granuloma pyogenicum.

Ichthyosis linearis circumflexa.

A case of ichthyosis linearis circumflexa in a 9-year old boy is reported here for its rarity.

Schamberg's disease - an unusual presentation.

A 28-year-old man with typical lesions of Schamberg's progressive pigmented purpuric dermatosis involving his right forearm is reported here for its unusual localisation.

Treatment of stable and recalcitrant vitiligo by autologous miniature punch grafting: a prospective study of 1,000 patients.

BACKGROUND: Stable and refractory vitiligo may be unresponsive to medical therapy. Melanocyte transplantation by punch grafting (PG) can restore the normal pigmentation. OBJECTIVE: To evaluate the efficacy of PG on repigmentation of vitiligo patches. METHODS: Autologous miniature PG was undertaken in 1,000 patients with stable and recalcitrant vitiligo. Test grafting (TG) was done in all the patients. Those who showed negative TG results were excluded from the study. RESULTS: Of the 1,000 patients, 880 (88%) showed positive TG results. In 656 (74.55%) patients, 90-100% repigmentation was achieved. In 93 (10.57%) patients, there was no spread of pigment, while in 21 (2.39%) patients depigmentation of the graft(s) was notices. Of various complications, polka dot appearance (43.98%) and colour mismatch (34.32%) were most frequent. CONCLUSION: Partial to near-total repigmentation of a vitiligo patch can be achieved by PG.

Iontophoretic Treatment of Hyperkeratosis with Sodium Salicylate.

Ten patients having hyperkeratosis with moderate to severe fissures on palms and soles were treated with iontophoresis using 5-10% aqueous solution of sodium salicylate. The results were excellent within a period of 3-4 weeks (6-8 sittings each for a period of 10-15 minutes). The treatment was free from the problems of salicylism. Follow-up in some of the reclcitrant cases for 4 months after stop the treatment and with no local application, showed neither any relapse nor deterioration. Sodium salicylate iontophoresis in hyperkeratosis seems to be safe well tolerated and far more effective than the usual local applications of salicylic acid.