Nonsegmental vitiligo follows Blaschko's lines and embryonic pigmentary segments.

BACKGROUND: Pathogenic mechanism that determines the localization of vitiligo patches and thus a patterned distribution in patients with nonsegmental vitiligo has remained poorly elucidated. A distributional similarity of the vitiligo patches with Blaschko's lines has been documented in patients with segmental vitiligo, both isolated segmental vitiligo and mixed vitiligo but never in cases of nonsegmental vitiligo. METHODS: Distribution of nonsegmental vitiligo patches on face and neck regions was assessed and compared with Blaschko's lines and also with embryonic pigmentary segments on the face. RESULTS: This study has documented distributional similarity of the nonsegmental vitiligo patches on face and neck with Blaschko's lines and the "embryonic pigmentary segments" among 154 (58.6%) cases. Patches around the palpebral and other fissures like periorbital, perinasal, perioral, and periaural were more common. In addition to the vitiligo patches, the spared areas were also found to respect the embryonic segmental outlines and follow the Blaschko's lines. CONCLUSION: Distributional pattern of the individual nonsegmental vitiligo patches along the Blaschko's lines and embryonic pigmentary segments suggests that mosaicism might control the susceptibility to the disease process in a patterned manner. LIMITATION: No genetic testing could be performed to confirm the hypothesis. Evaluation of nonsegmental vitiligo was done only on the face and neck areas.

Hand, foot, and mouth disease: current scenario and Indian perspective.

Hand, foot, and mouth disease (HFMD), first reported in New Zealand in 1957 is caused by Coxsackievirus A16 (CVA16) and human enterovirus 71 (HEV71) and occasionally by Coxsackievirus A4-A7, A9, A10, B1-B3, and B5. This is characterized by erythematous papulo vesicular eruptions over hand, feet, perioral area, knees, buttocks and also intraorally mostly in the children. HFMD has been known for its self limiting course. Only small scale outbreaks have been reported from United States, Europe, Australia, Japan and Brazil for the first few decades. However, since 1997 the disease has conspicuously changed its behavior as noted in different Southeast Asian countries. There was sharp rise in incidence, severity, complications and even fatal outcomes that were almost unseen before that period. Following the near complete eradication of poliovirus, HEV71, the non-polio enterovirus, may become the greatest threat to cause significant neurological complications. This adds to the fact that effective therapy or vaccine is still a far reaching goal. There are reports of disease activity in different corners of India since 2004. Although of milder degree, continuous progress to affect larger parts of the country may indicate vulnerability of India from possible future fatal outbreaks. Low level of awareness among the health care providers may prove critical.

Peeling skin syndrome in eight cases of four different families from India and Bangladesh.

Peeling skin syndrome (PSS) is a rare recessively inherited ichthyosiform genodermatoses characterized by superficial skin peeling. This has 2 subtypes, acral (APSS; OMIM 609796) and generalized form (OMIM 270300). The later has been subdivided into type A (non-inflammatory) and type B (inflammatory). Eight cases of peeling skin syndrome in 4 families were recorded over a period of 5 years. They were diagnosed clinically and confirmed histopathologically. Disease onset ranged from birth to childhood age (mean 5.25 ± 4.528 years) and age at presentation ranged from 7-35 years (mean 23.25 ± 10.471 years). Males outnumbered females (M:F - 5:3). All had non-inflammatory generalized disease of type-A PSS variety, except one who had type-B PSS. Two Muslim families (1 st and 2 nd family, total 5 patients) came from nearby country Bangladesh, and the 2 Hindu families were Indian. Higher severity over acral areas in generalized type, possible autosomal dominant pattern of inheritance and improvement with age as found in this series were new manifestations and possibly unreported previously. The disease was found to be poorly responsive to oral retinoids. Prevalence of the disease may be higher than expected. Importance of mutational analysis was also highlighted.

Clinico-allergological pattern of allergic contact dermatitis among 70 Indian children.

BACKGROUND: Rapid urbanization, westernization of lifestyles, poor quality of objects available and extremely relaxed vigilance on adherence to 'product safety guidelines' make any developing country like India highly susceptible to allergic contact dermatitis (ACD) even in children. There has been no previous attempts to assess the magnitude of childhood ACD in India. AIMS: To assess the clinico-allergological profile of ACD in Indian children. METHODS: All consecutive children up to 15 completed years of age who were patch tested over the last 3 years were analyzed from the records. RESULTS: A total of 70 children were studied (average age of disease onset 8.39+/-3.59 years [SD], range 1-15 years and average age of presentation 10.8 +/- 2.99 years [SD], range 5-15 years). Relevant allergy was noted in 48.6% of the patients. Age and sex had no significant role on the prevalence of ACD. Common allergens were paraben (43%), potassium dichromate (27%) and fragrance mix (26%). Most relevant allergens were potassium dichromate, paraben and fragrance. Foot was the most commonly involved site (25.7% of patients). Atopy was present in 18 patients (25.7%). A total of 22 irritant reactions were noted in 13 patients. CONCLUSION: This study reflects the current status of childhood ACD of this region.

Familial disseminated plaque type porokeratosis with multiple horns and squamous cell carcinoma involving anal skin.

Porokeratosis is a disorder of keratinization showing a well-defined lesion with a hyperkeratotic ridge on the border that contains the coronoid lamella. We report familial (autosomal dominant with reduced penetrance) disseminated plaque type (Mibelli's type) porokeratosis in a father and son. In the father, there were multiple horns and a large squamous cell carcinoma in a large lesion over the perianal region that reached up to the squamo-columnar junction of the anal mucosa and even invaded the anal sphincteric muscles. Disseminated lesions of the Mibelli's type, development of horns, and malignancy in this unusual location have not been previously reported.

Mycophenolate mofetil as adjuvant in pemphigus vulgaris.

Pemphigus vulgaris (PV) is a life threatening autoimmune blistering disease of skin and mucous membranes. Advent of systemic steroids has greatly reduced the mortality rate. However, steroids and adjuvant immunosuppressive therapy are nowadays frequent contributory agents of morbidity and mortality of PV. Mycophenolate mofetil (MMF) has been reported to be an effective adjuvant to systemic steroids. It helps in increasing the immunosuppressive effect and minimizing the toxicities by steroid sparing effect. However, its efficacy in refractory cases of PV is not well documented. The lowest possible dose with satisfactory therapeutic efficacy and least side effects is known. We used MMF 1 g/day and systemic steroids in 3 Indian patients with pemphigus vulgaris who were resistant to systemic steroid monotherapy or combination treatment with azathioprine. In our experience, MMF offers an effective adjuvant with minimal side-effects in the treatment of resistant PV.

Unilateral proteus syndrome.

Proteus syndrome is a complex developmental abnormality. It is characterized by both hypertrophic and hypoplastic changes. Deformities have been occasionally found to be localized in one half of the body in head or digit but presence of all signs in one half of the body in a wide spread manner is not reported in the literature. We report the case for its unusual presentation of unilateral localization of signs.

Inducing repigmentation by regrafting and phototherapy (311 nm) in punch grafting failure cases of lip vitiligo: a pilot study.

BACKGROUND: Punch grafting followed by PUVA/PUVASOL is an established mode of therapy in vitiligo including that on the lips. AIMS: To assess the efficacy of NB-UVB along with regrafting in patients in whom punch grafting had failed to evoke any response. METHODS: Five patients with stable and isolated lip vitiligo in whom punch grafting and PUVA had failed to produce pigmentation were treated by regrafting and subsequent NB-UVB (311 nm) phototherapy. RESULTS: Complete repigmentation was observed in three of the five cases after 16 weeks. In the remaining two, the results were mixed. CONCLUSIONS: Stable lip vitiligo usually responds very well to punch grafting and PUVA/PUVASOL. In patients who fail to respond to this treatment, complete repigmentation following regrafting and NB-UVB is an observation that needs to be explained.