Vibhu Mendiratta

The Spectrum of Skin Disease Among Indian Children

Abstract:  Skin diseases in children are encountered frequently and their characterization is essential for the preparation of academic, research and health plans. A retrospective study was designed to evaluate the epidemiologic features of pediatric dermatoses in India. The setting was a tertiary care referral center in India (Kalawati Saran Children’s Hospital, New Delhi) during January 1997 to December 2003. A total of 30,078 children less than 12 years of age with 32,341 new dermatoses were recorded, with a male to female ratio of 1.07:1. Most of the disease was seen in the 1‐ to 5‐year age group (44.94%). The most common skin diseases were infections and infestations (47.15%) consisting of bacterial infections (58.09%) and scabies (21.54%), followed by eczemas (26.95%), infantile seborrheic dermatitis, scabies, and pityriasis alba. Other unique dermatoses in our settings were papular uticaria (3.59%), miliaria (5.46%), postinflammatory pigmentary abnormalities (1.68%), and nutritional deficiency dermatoses (0.45%). A majority of patients were diagnosed clinically and special diagnostic tests were conducted in 2.6% of patients. The most common diagnostic test used was KOH mount (59.2%), followed by skin biopsy (39%). Nearly 90% of patients were seen without any referral and in the remaining, a majority were referred by pediatricians (75%). A majority of patients were diagnosed to have infection followed by dermatitis in our setting.

Infantile hemangioma: An update

Infantile hemangiomas (IH) are neoplastic proliferations of endothelial cells, characterized by a period of growth after birth, and eventual spontaneous involution. The course can be uneventful, culminating in spontaneous resolution; or it may be marked by complications such as bleeding; ulceration; infection; visual, feeding and breathing compromise; cosmetic and life-threatening complications such as congestive heart failure. Recognition of associated syndromes and impending complications of hemangiomas is of utmost importance. Great advances have taken place in the nomenclature, pathogenesis, immunohistochemistry, diagnostic workup and management of hemangiomas in the recent years. This article reviews current advances in the understanding of the pathogenesis, diagnostic tools, medical and surgical modalities of treatment for infantile hemangiomas.

Ellis–van Creveld Syndrome: A Report of Two Cases

Ellis–van Creveld syndrome (EVC) or chondroectodermal dysplasia, a rare autosomal recessive disorder, is a tetrad of chondrodysplasia, ectodermal dysplasia, polydactyly, and congenital heart disease, of which chondrodystrophy of the tubular bones is the most common feature, while central nervous system (CNS) and urinary tract anomalies are some of its rarer associations. This report describes EVC syndrome in two sisters of Indian origin, ages 8 and 6 years, the products of nonrelated, unaffected parents. The patients had chondrodysplasia of tubular bones resulting in disproportionate dwarfism, polydactyly, severely dystrophic nails, partially absent teeth, and short and bound‐down upper lips with multiple frenulae. Other features noted in the girls were syndactyly and mild mitral regurgitation. All four of the classic features of EVC syndrome were present in patient 1 and three in patient 2. Additional findings were ichthyosis and plantar keratoderma in the former and absent clavicles in the latter, which have not been reported previously. The importance of prenatal diagnosis of EVC is stressed and a multidisciplinary approach for the management of these patients is highlighted.

Spontaneously Improving Michelin Tire Baby Syndrome

Abstract: Michelin tire syndrome is described in a 22‐month‐old girl of Indian origin who had generalized folding of the skin, esotropia, epicanthic folds, and delayed speech. Histopathology showed diffuse lipomatous hypertrophy. The skin changes showed partial improvement on follow‐up. We report this case because of its rarity. Furthermore, the spontaneous improvement is significant, unlike the persistent course of other congenital nevi.

A proposal for classification of entities combining vascular malformations and deregulated growth.

BACKGROUND Agreement on terminology and nomenclature is fundamental and essential for effective exchange of information between clinicians and researchers. An adequate terminology to describe all patients showing vascular malformations combined with deregulated growth is at present not available. OBJECTIVES To propose a classification of patients with vascular malformations, not restricted to the face, and growth disturbances based on simple, clinically visible characteristics, on which clinicians and researchers can comment and which should eventually lead to an internationally accepted classification. METHODS Rooted in our joint experience we established a classification of vascular malformation not limited to the face, with growth disturbances. It is based on the nature and localization of the vascular malformations; the nature, localization and timing of growth disturbances; the nature of co-localization of the vascular malformations and growth disturbances; the presence or absence of other features. Subsequently a mixed (experienced and non-experienced) group of observers evaluated 146 patients (106 from the Netherlands; 40 from the UK) with vascular malformations and disturbed growth, using the classification. Inter-observer variability was assessed by estimating the Intra-Class Correlation (ICC) coefficient and its 95% confidence interval. RESULTS We defined 6 subgroups within the group of entities with vascular malformation-deregulated growth. Scoring the patients using the proposed classification yielded a high inter-observer reproducibility (ICC varying between 0.747 and 0.895 for all levels of flow). CONCLUSIONS The presently proposed classification was found to be reliable and easy to use for patients with vascular malformations with growth disturbances. We invite both clinicians and researchers to comment on the classification, in order to improve it further. This way we may obtain our final aim of an internationally accepted classification of patients, which should facilitate both clinical treatment and care of, as well as research into the molecular background of entities combining vascular malformation and deregulated growth.

A suspected case of primary cutaneous actinomycosis on the buttock.

Primary cutaneous actinomycosis is rare and can mimic mycetoma, tuberculosis and nocardiosis. The results of cultures (aerobic and anaerobic) are not always positive. A high degree of clinical suspicion should be entertained and a combination regimen of penicillins with sulphonamides should be used for treatment. We report a patient with primary cutaneous actinomycosis over the buttock, who was successfully treated with amoxycillin and sulphonamides.

A Prospective Study of Cutaneous Manifestations of Spinal Dysraphism from India

Abstract:  Recognition of cutaneous markers of spinal dysraphism is important to prevent the morbidity associated with underlying spinal anomalies. To investigate the frequency and type of cutaneous stigmata in different forms of spinal dysraphism and to assess the role of ultrasonography and/or magnetic resonance imaging in diagnosing spinal dysraphism at two pediatric dermatology tertiary care centers. Over a 4‐year period, all pediatric patients presenting to the dermatology clinic with dorsal midline cutaneous stigmata were evaluated clinically and with imaging studies (radiography, ultrasonographic examination and magnetic resonance imaging/Doppler). Surgical interventions were planned in conjunction with neurosurgery and orthopedic specialists. On examination, 245 (4.2%) had 285 cutaneous stigmata. Of the 180 patients evaluated with radiography, ultrasonographic examination and magnetic resonance imaging, 50 patients (28%) had spinal dysraphism (with 64 cutaneous stigmata). The most common stigmata associated with occult spinal dysraphism were lipoma (10) and dimples (12) and in open spinal dysraphism lipomeningomyelocoele (10) and meningomyelocoele (10). Statistically, lipomeningomyelocoele/myelomeningocoele, atypical dimples and port‐wine stains were most associated with spinal dysraphism (p < 0.001). In 80 children less than 6 months of age, radiography with ultrasonographic examination revealed an SD in 16, while magnetic resonance imaging was diagnostic in four cases. Ultrasonographic examination performed fairly well in children less than 6 months and in cases of flat cutaneous stigmata it missed only 5% of cases, but in cases with bulky overlying masses (lipoma, hemangioma) it missed 15% of cases.

Perforating folliculitis with jaundice in an Indian male: a rare case with sclerosing cholangitis.

our experience with six patients does suggest several conclusions. Firstly, there does not appear to be an increased frequency of adverse reactions to high-dose tetracycline in patients with localized EBS. Secondly, two-thirds of our patients did indeed experience reduced disease activity while on prolonged tetracycline therapy, based on objective, standardized, lesional counts. Thirdly, the response lasted during at least 4 months of treatment, rather than representing a transient effect seen only at 2 but not at 4 months of treatment. Fourthly, no decreases in lesional counts occurred following treatment with placebo. Although the limited number of patients completing therapy precludes statistical validation of this intervention, based on our encouraging experience in four of our six wellstudied patients, coupled with the lack of any other known effective treatment for this disease, we would recommend empirical treatment with tetracycline of any EBS patient who is willing to risk the known adverse side-effects of this drug and who is sufficiently symptomatic to desire an attempt at systemic therapy. It could even be used in children at risk for tetracycline-induced dental discoloration if the severity and chronicity of their symptoms justified such treatment, given how easily permanent crowns could be placed in these patients if cosmetic dental intervention were necessary and desired at a later age.

A study of leprosy reactions in a tertiary hospital in Delhi.

A retrospective study of 531 leprosy patients was undertaken to study the profile of reactions in the post Multi‐Drug‐Therapy period in a tertiary hospital in Delhi. BT was the most common group. The prevalence of reactions was found to be 8.09% for the Type 1 and 4.70% for the Type 2 reactions for a male:female ratio of 2.2. The Type 1 reaction was most frequently observed in the BB group followed by BL, BT and LL groups respectively. More than half of the patients had reactions at the time of presentation. In only 39.8% of the patients did reaction follow Multi‐Drug‐Therapy. In 4.5% of the patients with Type 1 reactions (T1R), concomitant infections were noted. The most common presentation of T1R was cutaneous lesions (74.41%) followed by cutaneous lesions and neuritis (53.6%), neuritis alone (12.1%), and only edema of hands and feet (7.31%) respectively. The Type 2 reactions (T2R) presented chiefly as papulo‐nodular (92%) lesions followed by pustulonecrotic (8%) lesions. Associated neuritis was found in 40% and periosteitis and iritis in 8% and 4%, respectively. In 8.6% of the patients with T2R, precipitating factors could be observed. The prevalence of deformities in patients with reaction was 25%, and was more common in females. Deformities were observed in 23.25% of the T1R patients and 28% of the T2R patients.

Three unusual siblings with Harlequin icthyosis in an Indian family.

Harlequin fetuses occurring as three siblings in an Indian family are described here. All three were preterm, low birth weight, and did not survive. There was no history of consanguinous marriage in the parents or in the family. Thus autosomal recessive inheritence appears to be a remote possibility, although not impossible or, as recently described, these recurrent harlequin fetuses could be the result of new dominant mutations with parental mosaicism.