Ectodermal dysplasias are defined as a group of congenital, nonprogressive, developmental syndromes with primary disorders in at least two ectoderm-derived structures namely eccrine glands, hair, nail and teeth. Pure hair and nail affecting ectodemal dysplasias are exceedingly rare in occurance and have been known to be associated with multiple abnormalities, such as keratoderma or ichthyosis, skeletal and hematological abnormalities, cardiac irregularities, mental or psychomotor retardation and cataracts. We herein report a case of hypotrichosis with trichorrhexis nodosa and twenty nail dystrophy- a hypohidrotic ectodermal dysplasia variant.

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Idiopathic inflammatory myopathies are rare group of systemic connective tissue diseases. The hallmark of these disorders is symmetrical chronic inflammation and weakness of proximal muscles. Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children. We describe a rare case of JDM in a 4-year-old female child who presented with characteristic cutaneous rash and proximal muscle weakness.

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Background: Lichen striatus is an idiopathic self-limiting inflammatory linear dermatosis, mostly affecting children. There is a definite paucity of studies on the clinical profile of lichen striatus in children from India. Objective: The aim of this study was to analyze the clinical profile of lichen striatus in children. Materials and Methods: The present observational study was conducted in the Department of Dermatology, Assam Medical College and Hospital, Dibrugarh, from June 2014 to May 2015. All children up to 13 years presenting with lichen striatus in the Dermatology Outpatient Department of Assam Medical College and Hospital, Dibrugarh, were taken as study participants. A detailed general, systemic, and cutaneous examination was done. Relevant investigations were carried out whenever necessary. The findings were recorded in a pro forma for analysis and interpretation of data. Results: The prevalence of lichen striatus in our outpatient pediatric population was 0.48%. Female cases outnumbered males with a ratio of 4:1. The mean age of onset was found to be 4.7 years. Upper extremities and trunk were the most common sites involved. An association with atopy was noted in 30% of the cases. Conclusion: Long-term studies with increased number of cases will help in better understanding of this rare disease in children and also its association with atopy.

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Scabies is commonly seen worldwide, in its usual classic form when afflicting older children and adults. However, neonatal scabies is described as its own entity in the literature. We present a case of a 4-week old infant with a generalized papulopustular, vesicular, and crusted rash who was diagnosed with scabies. We contrast the differing clinical features of neonatal and classic scabies, describe possible mimickers of this diagnostic dilemma, and review current treatment options available for scabies in this very young age group.

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A 5-year-old boy was referred because of urticarial flares with angioedema. Antihistamines and systemic steroids were not useful to prevent flares. At examination, at distance of a flare, chronic red-brownish plaques were found on the legs, trunk, and back, resembling granuloma annulare. The skin biopsy showed epithelioid and giant-cell granulomas, elastophagia, and “flames figures” surrounded with many eosinophils. A diagnosis of disseminated granuloma annulare-like variant of Wells' syndrome as described earlier by Caputo was made. A treatment with dapsone was initiated and enabled to stop flares. Wells' syndrome is a rare clinicopathologic entity with a chronic benign course and often misleading clinical presentation. Our case illustrates the clinical polymorphism and benefit of dapsone therapy.

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Olmsted syndrome (OS) is a rare congenital, sharply circumscribed transgredient palmoplantar keratoderma, first described by Olmsted in 1927, characterized by clinical features such as symmetrical involvement of keratoderma of the palms and soles and the symmetrical hyperkeratotic plaques around the body orifices. Other clinical findings include flexion deformities of the fingers, localized alopecia, leukokeratosis of the tongue, short stature, and laxity of the large joints. It starts in the neonatal period or in childhood. The disease has a slow but progressive and extremely disabling course. Treatment of OS is often based on topical therapy with retinoic acid, corticosteroid, emollients, and keratolytics. We present a case of OS in three siblings, two males and a female, born to nonconsanguineous parents with no family history. They were treated with topical corticosteroids and emollients and showed mild improvement in symptoms.

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Cutaneous leishmaniasis (CL) is a protozoal disease usually caused by Leishmania major and Leishmania tropica and transmitted by the bite of a sandfly. It is usually characterized by a single, polymorphous lesion located in an uncovered area. The eyelid is rarely involved and only makes up 2.5% of cases with CL. We report a case of CL localized on the left lower eyelid in a 6-year-old girl. The disease was characterized by a single, asymptomatic nodule, which was managed as sty or eczema by various practitioners previously. The diagnosis was confirmed by the presence of Leishman-Donovan bodies on slit skin smear, and the patient was treated with intramuscular sodium stibogluconate. Complete clearance of lesions with residual scarring was seen after 3 weeks of therapy.

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Bullous mastocytosis (BM) is a rare variant of diffuse cutaneous mastocytosis where there is disorderly infiltration of the skin by mast cells. We report a 4-month-old male infant with this rare disease who presented with wrinkled tough skin and generalized multiple bullous lesions. Diagnosis was done by serum tryptase level and histopathological examination. The patient responded well to oral corticosteroid and antihistaminic therapy. Although a congenital disorder, timely diagnosis and treatment can be potentially rewarding in patients with BM.

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Palisaded granulomatous neutrophilic dermatosis (PGND) is a cutaneous reaction pattern seen in association with some systemic diseases. It is mostly seen in adult patients with only three case reports of these lesions in children. We hereby report a 12-year-old female child with systemic lupus erythematosus flare having PGND, showing two different morphologies clinically which were also confirmed on histology depicting the much-speculated evolution of the disease. We are reporting the case for the rarity in pediatric patients, the presentation of different morphological variants in the same patient as well as the uncommon site of involvement.

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Mastocytosis represents a group of disorders characterized by abnormal accumulation of mast cells in the bone marrow, liver, spleen, lymph nodes, gastrointestinal tract, and skin. Skin involvement is seen in almost all the cases. A 1-year-old male child presented with complaints of multiple fluid-filled lesions all over the body since 2 months. Examination revealed multiple vesicles and tense bullae present on the face, neck and trunk along with surrounding erythema and multiple excoriation marks. Darier's sign was positive. Histopathology showed dense infiltrates of mast cells in the papillary dermis. Bone marrow aspiration showed no mast cells infiltrate. The patient was started on oral antihistamines, prednisolone, and narrowband ultraviolet B (NBUVB). The skin lesions showed dramatic improvement within a week and patient is on follow-up with NBUVB. Bullous mastocytosis (BM) is a rare variant of cutaneous mastocytosis manifested by diffuse infiltration of skin by mast cells, where cutaneous bullae are the predominant feature. This case is being presented for its rarity and highlights the effectivity of NBUVB for treating BM.

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Context: Hair disorders are a common complaint in dermatology clinics and can be caused by a number of conditions. Children have etiologies different from adults. Aims: There is a paucity of studies in evaluating pediatric hair disorders. The ultimate aim was to provide a guide for evaluating hair disorders commonly occurring in childhood in our environment. Materials and Methods: A prospective observational study was conducted from August 2014 to August 2015. Children suffering from various hair disorders were included in the study. Results: We report a clinico-epidemiological study of 503 children suffering from various scalp/hair disorders. The most common causes were folliculitis, furunculosis, pediculosis, alopecia areata, and tinea capitis. Conclusions: As has been observed in this study, hair problem may be due to various infections, and this is a very important problem to think of, especially in developing countries.

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Background: Acne is a common problem in adolescent children and has a considerable impact on their quality of life (QoL). Aims: The impact of acne on QoL in Indian adolescent patients remains undocumented. The study was undertaken to detect the impact of acne vulgaris in adolescents on the QoL using two questionnaires: the Children's Dermatology Life Quality Index (CDLQI) and the Cardiff Acne Disability Index (CADI). Materials and Methods: This was a hospital-based, prospective, cross-sectional, prestructured, questionnaire-based study done on 140 consenting individuals, who attended the Acne Clinic of our Dermatology Outpatient Department. Acne vulgaris was graded using simple grading system. QoL was measured using a combination of skin disease-specific (CDLQI) and acne-specific (CADI) questionnaires. Results: The study population included 140 cases with a female:male ratio of 1.5:1. Comedones (123, 87.9%) were the most common type of lesion. Grade I acne was the most common clinical type (76.4%). There was a statistically significant difference between acne severity and gender. The overall mean CDLQI score (7.21 of maximum 30) and the overall mean CADI score (4.8 of maximum 15) were low, indicating a mild impairment of QoL among adolescents. Statistically significant association was noted between CDLQI and CADI scores and grade of acne. There was no statistically significant association noted between CDLQI and CADI scores and gender. Conclusion: Although acne had an impact on patient's QoL, it was less severe in our study. The CDLQI and CADI questionnaires represent simple and reliable instruments for the assessment of QoL among adolescents and should be incorporated when managing acne patients to provide better and appropriate care.

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Superficial fungal infections are a common presentation in the pediatric age group and are a significant cause of morbidity in children. This review covers infections commonly seen in the pediatric age group like the dermatophytic infections,pityriasis versicolor and candidiasis including their management aspects.

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