Primary immunodeficiency diseases (PIDs) are a group of rare, chronic disorders with deficient or malfunctioning immune system. It commonly affects the hematopoietic system, with skin the second most affected organ. Skin involvement is observed in half of pediatric PID cases and often precedes the final diagnosis. Skin infections and eczemas are the two most common manifestations in PID.^[1] Skin manifestations associated with PIDs can be of infectious and noninfectious causes. Common noninfectious causes are eczema, erythroderma, cutaneous granulomas, dysplasia, vasculitis, and telangiectasia. It is important to be aware of skin manifestations in pediatric patients as early detection of PID may aid in the management of serious immunologic conditions and prevent associated morbidity and mortality.

Infantile hemangiomas (IHs) are the most common benign soft tissue tumor of infancy and childhood. Many patients seek early treatment to halt progression of tumor growth and accelerate regression to achieve quick resolution with good cosmetic outcomes. We reviewed literature through PubMed search on the treatment strategies for IH and share our experience in the field of laser treatment of IH. Treatment strategies for IH include both pharmacological, laser, and surgical interventions depending on the stage and severity of the lesion. Various laser beams have been attempted with varying effects and effectiveness. The 595-nm pulsed dye laser therapy has been most widely utilized owing to its great efficacy but minimal adverse effects. It works by targeting oxyhemoglobin chromophore in blood vessels located within the dermis, causing photothermal damage of these target vessels stimulating quick involution without damaging surrounding healthy skin. It is especially useful in treating ulcerated superficial facial hemangiomas that necessitate rapid healing to avoid unsightly scarring. It has a good safety profile but small risk of epidermal burn, blistering, postinflammatory pigment changes, and scarring remains in those with darker skin types treated with higher fluences and short-pulsed duration. Combination treatment with 1064 nm neodymium-doped yttrium aluminum garnet laser, oral propranolol, and even corticosteroids remains an option, especially in treatment of deep, large, and functionally threatening IH. Careful consideration in consultation with the child's parents given the complexities and potential complications surrounding treatment should always be considered. Laser treatment remains an appropriate treatment for rapidly growing IH in exposed locations at early presentation.

Atopic dermatitis is an immunologically mediated chronic inflammatory disease which cannot be controlled always with topical agents. Systemic drugs play important role in these difficult to manage cases. Systemic agents are also important in controlling the acute flare. Unfortunately these agents are very limited in number. Moreover none of them starting from corticosteroid to azathioprine can provide complete cure. Continuous research is unfolding different aspects of AD pathogenesis which was not known previously. Scientists are engaged in developing newer molecules targeting those novel pathological pathways thus adding in the armamentarium of existing drugs. This article is dedicated to current systemic treatment options available with their individual merit and demerit along with recent advances in this field.

Introduction: Palmoplantar keratodermas (PPKs) are a group of disorders in which there is marked thickening of the skin of palms and soles. The keratodermas can be diffuse, focal, or punctuate type. Aims: The aim of this study was to study various types of PPKs, both hereditary and acquired in children up to 17 years of age. Methods: A randomized controlled prospective study of twenty patients of PPK was taken up for the study. A detailed history, general physical examination, cutaneous examination with special examination of the oral mucosa, teeth, and nails along with routine investigations were done in all the patients. Results: Most common causes of PPK were secondary to psoriasis and ichthyosis (25% each), followed by 15% cases secondary to pityriasis rubra pilaris. PPK due to pachyonychia congenita was seen in 5% patients and secondary to erythrokeratodermia variabilis in 5% patients. Monogenic PPKs including Vorners syndrome and Unna-Thost PPK were seen in 5% patients each and focal PPK seen in 15% patients. Conclusion: It is important to note the age of onset of the keratodermas, severity of disease process, and the histopathological findings before reaching a diagnosis of PPKs.

Context: Psoriasis is a chronic immune-mediated disease. Although common in children, true incidence and prevalence are not exactly known. There is a paucity of data on childhood psoriasis in India. Aims and Objectives: To study the age and gender distribution, mean age of onset, family history, precipitating factors, seasonal variation, clinical pattern, and nail changes in childhood psoriasis. Study Design: This was a prospective, observational study. Materials and Methods: All children with psoriasis under the age of 18 years were enrolled in the study, and detailed evaluation was done. Statistical Analysis Used: SPSS 20 (IBM SPSS statistics for Windows, version 20.0: IBM Corp., Armonk, NY) and SIGMA STAT 3.5 (Sigma stat 3.5, Systat Software Inc., Richmond, CA.) by applying one-way ANOVA and Chi-square test. Observations and Results: Childhood psoriasis comprised 17.8% of total psoriatic patients. Male to female ratio was 1:1.4. Girls had higher mean age and delayed age of onset (P 0.028). Nearly one-third had positive family history of psoriasis. Trauma was the most common precipitating factor. Anti-streptolysin-O titer was positive in 10.18% of cases, out of which 72.72% belonged to guttate psoriasis. The most common clinical type was plaque psoriasis. Extremities were the most frequently involved site and also the most common site of onset. Pitting was the most common nail change. Conclusion: In our study, a considerable proportion of the psoriatic patients were children (17.8%). Infection was one of the common triggering factors in children; hence, early control of infection may help in reducing the severity and frequency of the disease. Further follow-up of these children is needed to know the outcome and prognosis of the disease.

Background: There is a recent rising trend of superficial dermatophytic fungal infections all over the globe. Apart from the causative organisms, there are many modifiable environmental factors contributing to this sudden pandemic. The prevalence of the disease in the pediatric age group needs to be studied more vigorously. Materials and Methods: All children in the age group of 2–15 years with dermatophytic infections were studied for the pattern of infection and various environmental associations. Results: Most (102 [51.51%]) of the patients belonged to 11–15 years age group with tinea cruris (99 [50%]) and tinea corporis (94 [47.47%]) type of pattern being the most common. The majority (175 [88.38%]) of the patients belonged to rural and semi-urban locality with improper sanitation system and poor quality of water source in use by the patients. Conclusion: This study highlights the prevalent pattern of dermatophytic infections in children in our locality.

Background: Acne is a common skin disorder that affects both adolescents and adults. Epidemiological data on acne are limited from developing countries. Objective: The objective of this study is to estimate the prevalence and pattern of acne vulgaris among adolescent students of Chandigarh (India), and to study the impact of acne on quality of life. Materials and Methods: Children from three schools were enrolled to investigate the demographic profile, severity and causative factors of acne and its impact on quality of life using a predesigned questionnaire and followed by examination for presence, site and severity of acne. Results: Acne was present in 72.3% of 1032 children included in this study. Mild acne was present in 81.9% students, moderate in 17.1%, and severe in 0.9%. There was a significant association of acne with stress (P = 0.001) and premenstrual flare (P = 0.000). No association was found between acne and diet, hygiene, weather, family history, and smoking. The quality of life was affected in 29% of children and was directly related to the severity of acne (P = 0.000). No difference of impact on quality of life was seen between boys and girls. Conclusions: This study presents the demographic features and clinical characteristics of acne in school children. This large-scale analysis reveals that acne is a very common dermatosis among Indian school children having a significant impact on their quality of life.

Introduction: Skin diseases are a major health problem in children, which is associated with significant morbidity. Its prevalence ranges from 4.3% to 49.1% in various parts of India in school-based surveys. Objective: The present study was conducted to know the prevalence, epidemiology of various dermatoses, and the factors contributing to those dermatoses among school children of rural areas of Salem. Materials and Methods: A cross-sectional prospective study was conducted in two schools of rural Salem between September 2014 and September 2015. A total of 1000 children between the age group of 5 and 14 years were examined for diseases of the skin and appendages. Data were coded and analyzed. Results: The overall prevalence of skin diseases was 68.2%. Almost 53 types of skin lesions were identified among the students; of these, 15 were infectious, 35 were noninfectious, and three were nutritional dermatoses. Infectious dermatoses were seen in 346 (50.73%), noninfectious in 253 (37.10%), and nutritional deficiency dermatoses in 83 (12.17%) children. The top three conditions, i.e.. pediculosis capitis (21%), scabies (11%), and bacterial infections (8.52%) contributed 40.52% of the total burden of skin diseases. Conclusion: The prevalence of skin disorders, especially infectious dermatoses, was high among the school children in our study area which is a reflection of the prevalence in the overall population of that area. It is mainly attributed to a lack of proper hygienic measures and health awareness which has to be addressed promptly for the improvement of that area.

Introduction: Lichen planus (LP) is a chronic inflammatory pruritic dermatosis occurring infrequently in children. Very few studies are available on childhood LP from South India. Aim: This study aims to analyze the clinical profile of LP among children. Settings and Design: This was a retrospective study. Materials and Methods: Records of the children <16 years with LP attending our tertiary care center from January 2011 to April 2016 were tabulated and analyzed. Results: Of the 76 children with childhood LP, examined over a period of 5.4 years, 42 were boys and 34 were girls with a male: female ratio of 1.2:1. Childhood LP accounted for 17.7% of total LP cases. The mean age of the children was 10.7 years, and the lesions appeared earlier in boys than girls. Limbs were the common site of onset, and classical LP was observed in 56.6% (43) of cases followed by, linear LP (11, 14.4%), hypertrophic LP (10, 13.2%), eruptive LP, and actinic LP in 5.3% each. Koebner's phenomenon was noted in 37.3% and a positive history of infective foci before the onset was noted in 14.4% of children. Palmoplantar, oral mucosal, and nail involvement were noted in 7.8%, 14.4%, and 15.7% of children, respectively. Multiple morphological types were noted in 14.4% of children. Conclusion: Hence, as per our study, LP in children is relatively common, and it can sometimes be triggered by infection. Apart from classical LP, linear LP, annular LP, and palmoplantar involvement were frequently observed in the present study.

Background: The objective of this study was to ascertain the pattern of dermatoses among adolescents attending the Dermatology Outpatient Department (OPD) at a tertiary care center in Manipur, Northeast India. Methodology: The retrospective study was conducted among 3850 patients belonging to adolescent age group (10–19 years) from January to December 2014. They constituted 20.18% of the total OPD attendance during the study. Results: Male to female ratio was 1:1. The study group was further divided into three categories based on age: early (10–13 years), middle (14–16 years), and late adolescence (17–19 years). Majority belonged to mid (36.3%) and late adolescence (36.64%) age groups. The dermatoses identified were classified into 13 broad categories. Infections were the most common dermatoses (25.5%), followed by sebaceous gland disorders (22.72%), eczemas (17.45%), infestations (17.03%), and urticaria (4.5%). Among infections, fungal infection (53.25%) was most common, followed by bacterial (24.59%) and viral infections (22.15%). Conclusion: It was concluded that infections, infestations such as scabies and pediculosis, eczemas, and sebaceous gland disorders such as acne vulgaris were common dermatoses seen in the adolescent age group.

Granulomatous cheilitis (GC) of Miescher is a chronic inflammatory disorder of unknown etiology affecting young adults, characterized by asymptomatic, unrelenting swelling of lips. An array of treatment modalities have been tried, only for the disease to recur again. We report a case of GC with significant remission after treatment with a combination of steroids, metronidazole, and doxycycline. There were no signs of relapse at 6 months of follow-up.

An extraordinary type of foreign body (FB) may be found in the female external genitalia, especially in children. Intravaginal FB, retained for long duration, can produce diagnostic dilemma. Vaginal bleeding as the result of a leech bite is extremely rare although reported. We report two cases of vaginal FB (intravaginal Scotch-Brite in a 5-year-old girl and leech in the vagina in a 7-year-old girl).

The acronym “PHACE” refers to the syndromic association of posterior fossa anomalies, hemangioma, arterial lesions, cardiac abnormalities/coarctation of the aorta, and eye anomalies. Till now, less than 400 cases of PHACE syndrome have been reported in the medical literature. Its etiopathogenesis is still unknown, and a great deal of genetic and molecular research is required. Moreover, there is always a high probability of misdiagnosis due to the certain factors such as mildly symptomatic patients, hasty diagnosis of relatively common simulating syndromic entities, and low awareness among medical fraternity about the standard diagnostic criteria for PHACE syndrome. Therefore, we report here a case of PHACE syndrome in a 2-year-old female child presenting with Dandy-Walker malformation, microphthalmia, leukocoria, hearing loss, and regressing lesions of large facial infantile hemangioma.

Berardinelli–Seip syndrome is a rare autosomal recessive disorder characterized by an almost total lack of subcutaneous fat with various metabolic abnormalities and complications such as diabetes mellitus and insulin resistance. Renal changes and complications are uncommon but reported. We present two such rare cases with renal involvement.

Reactive perforating collagenosis (RPC) is a rare disorder of transepidermal elimination in which genetically altered collagen is extruded out through the epidermis. This disease usually starts as asymptomatic or pruritic umbilicated papules on extensor aspects of extremities and face, and the lesions become more conspicuous with age. It has two forms: Acquired and inherited form, of which inherited form is extremely rare. Here, we document a case of inherited form of RPC in a 13-year-old boy having multiple asymptomatic hyperpigmented umbilicated papules with a central keratotic plug distributed chiefly over face and extensor aspect of upper and lower extremities since his infancy, with history of similar lesions in his 18-year-old elder brother.

The parameatal cyst (PMC) is a rare and benign lesion which occurs in newborn, children, or even in adults. We present five patients of PMC to discuss its embryology, clinical presentation, and the treatment along with a brief review of literature.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare variant of nevoid linear inflammatory dermatoses which appear most often in the first 6 months of life and persists for many years or even whole life. Clinically, it often shares many features with linear psoriasis, linear porokeratosis, and other linear dermatoses which can usually be differentiated by an established set of clinical and histopathological criteria. Herein, we are reporting an interesting and rare case fulfilling all the clinical and histopathological criteria of ILVEN in a 13-year-old boy which uniquely revealed cornoid lamella on histopathology so we termed it as “porokeratotic variant of inflammatory linear verrucous epidermal nevus.” To the best of our knowledge, only two such cases were reported previously in the literature and none from our country.

Skin is the largest organ of the body. Skin rash is not an uncommon manifestation, and sometimes, can give clue to the diagnosis of a systemic disease. Here we present two cases which presented with a typical skin rash and respiratory distress with extensive lung involvement, and ultimately diagnosed to be a case of Langerhans Cell histiocytosis.

Incontinentia pigmenti (IP) is a rare genodermatoses with multisystem involvement. Monochorionic diamniotic twins are presented with characteristic skin manifestation and ocular and neurological involvement. Identification of characteristic cutaneous manifestation can lead to rapid diagnosis of IP even in the absence of family history, resulting in prompt management of systemic manifestations.

Erythroderma in infants can be attributed to plenty of causes, the more common ones being nonbullous congenital ichthyosiform erythroderma, lamellar ichthyosis, bullous congenital ichthyosiform erythroderma, severe atopic dermatitis, etc., However, there can be circumstances where infantile psoriasis can present to us with erythroderma; this may be a diagnostic challenge to the clinician. Hereby, we present a case of a young boy with recurrent erythroderma.