Nicolau syndrome (embolia cutis medicamentosa) is a rare cutaneous adverse reaction occurring at the site of intramuscular, intra-articular or, rarely, subcutaneous injection of particular drugs. We hereby report a fatal case of Nicolau syndrome in a 13-year-old young male patient.

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Congenital alopecia has broad differential diagnosis and poses diagnostic and therapeutic challenges. It is a rare form of irreversible alopecia inherited autosomal recessively. Atrichia congenita with papular lesions represents a complex and heterogeneous group of genodermatoses characterized by irreversible complete hair loss soon after birth, and associated with the development of keratin-filled cysts over the body. We report a case of 4-year-old boy presenting with complete loss of hair over scalp, eye brows, eye lashes, and body since birth. Patient also had papular lesions over body.

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Introduction: With increasing knowledge about limitations of systemic steroids, the use of steroid sparing agents is now being accepted by practicing dermatologists. Methotrexate (MTX) is a well accepted steroid sparing drug in adults with dermatological conditions. However, its use in pediatric dermatoses has not been the subject of many investigations. Materials and Methods: We decided to do a computerized search for the use of MTX in pediatric dermatoses using terms like "pediatric,'' "MTX," "psoriasis,'' "pityriasis rubra pilaris (PRP)," "atopic dermatitis," "pityriasis lichenoides," "lichen planus," "juvenile dermatomyositis (JDMS)," "localized morphea," "systemic lupus erythematosus (SLE)," "vesiculo-bullous diseases," "alopecia areata," "vasculitis," "eosinophilic fasciitis," "urticaria," "sarcoidosis," "pompholyx" and "vitiligo vulgaris." All the articles were retrieved and classified into review articles, studies, double-blinded trials and case reports. The final data were then analyzed. Results: It has been found that MTX has been increasingly used in psoriasis, PRP, pityriasis lichenoides, JDMS, SLE and sarcoidosis. However, its use in several other dermatoses is restricted to isolated case reports. Conclusion: Children tolerate MTX better than adults. However, proper clinical and laboratory surveillance has to be maintained in patients on long term MTX.

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The hereditary sensory and autonomic neuropathies (HSAN) are a group of very rare genetic disorders, characterized by prominent sensory as well as autonomic neuropathy without any motor involvement. Five main groups (type-I through type-V) of HSAN do exist. We report here a case of a chronic nonhealing leg ulcer in a young boy associated with anhidrosis, diagnosed as HSAN type-IV with osteomyelitis, for its rarity, and to emphasize the importance of considering this entity in the differential diagnoses of chronic nonhealing ulcers in the pediatric population.

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Background: Vitiligo is a chronic, polygenic dyschromia that presents with multiple depigmented macules and patches. Among the various therapeutic modalities for treatment of vitiligo in children, phototherapy as one of the mainstay treatments has varying therapeutic efficacy. There is limited data regarding the use of combination of tacrolimus and 308 nm excimer lamp for treatment of vitiligo in children. Aim: The aim of the present study is to retrospectively assess the efficacy and tolerability of 308 nm Excimer lamp in combination with topical tacrolimus to treat vitiligo in children below 17 years with skin type III, IV and V. Methods: Case sheets and photographs of 47 vitiligo patches from 24 children (male = 9, female = 15), <17 years were evaluated in this study. Data of patients with active disease and stable vitiligo not treated previously were chosen for this study. The patches were treated with topical tacrolimus daily and excimer lamp thrice weekly, for a maximum of 20 sittings. Results: Of the 47 patches, 35 patches achieved repigmentation accounting to 74.4% of the total patches. The repigmentation response was good in ultraviolet (UV) sensitive areas but minimal in UV resistant areas. All the patches retained pigmentation during the follow-up period of 12-18 months. Conclusion: The degree of repigmentation achieved with topical tacrolimus and excimer lamp in a period of 4-6 weeks is much higher than other contemporary vitiligo therapies in children. This dual therapy of topical tacrolimus and 308 nm Excimer lamp is a safe and effective therapy showing an early response, for localized vitiligo in children.

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The use of oral and topical beta blockers has revolutionized the management of infantile hemangiomas. Oral propranolol at 2 mg/kg/d in divided doses not only prevents further proliferation, but actually shrinks IH. Propranolol should be administered for at least 6 months or until the baby reaches one year of age. Side effects to be watched out for include hypoglycaemia, bradycardia and hypotension. Oral propranolol should be prescribed only in those IH where clearly indicated. More cardioselective beta blockers like atenolol are also being explored for treating IH. Topical timolol has been prescribed for smaller and superficial IH or when propranolol is contraindicated.

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Epilepsy is common childhood problem and advent of newer anticonvulsant drugs has led to injudicious uses. Anticonvulsant hypersensitivity syndrome (AHS) has been previously described developing secondary to aromatic anticonvulsants such as phenytoin and carbamazepine. AHS developing secondary to non-aromatic anticonvulsant agents such as lamotrigine (LTG) and valproic acid (VPA) is rare. We present a case of an 11-year-old girl with known epilepsy on VPA, LTG and carbamazepine therapy that developed fever with the clinical pattern of Steven-Johnson syndrome/toxic epidermal necrolysis requiring pediatric intensive care. Judicious use of anticonvulsant should be done in appropriate dose especially in pediatric population and when used in combination to prevent AHS like life-threatening event.

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Nail bed pigmentation or melanonychia is common in Blacks. Sometimes it is seen in Whites and Japanese, and may be associated with pigmentation of lips and oral mucosa, termed as Laugier-Hunziker (LH) syndrome. Nail pigmentation is usually in the form of longitudinal bands, sometimes with nail dystrophy, mostly acquired, and majority of the times associated with a family history. Here, we are reporting two cases of Indian origin with benign hyperpigmented nails, in which no possible cause was identified and there was no family history with such lesions.

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Netherton syndrome is a rare symptom complex characterized by an ichthyotic skin disorder, greatly elevated IgE levels with atopic manifestations, and characteristic hair shaft abnormalities. We report a 2-year-old male child, who presented with erythroderma, increased serum IgE levels, and trichorrhexis nodosa which can be easily misdiagnosed as psoriatic erythroderma.

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HIV-infected patients have a higher risk of developing cutaneous reactions than the general population, which has a significant impact on patients' current and future care options. The severity of cutaneous adverse reactions varies greatly, and some may be difficult to manage. HIV-infected patients just at the beginning of antiretroviral therapy can frequently show a wide variety of adverse drug effects such as erythema multiforme, urticarial reaction, drug rashes, Stevens-Johnson syndrome, and toxic epidermal necrolysis.

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We report a case of pseudoainhum with underlying vascular malformation in a 4΍-year-old male patient over distal part of left lower extremity with a constriction involving the fifth toe.

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Pediatric vasculitis is the presence of inflammation of blood vessels. It accounts for 2 - 10% of rheumatology clinic attendance. Henoch-Schonlein purpura is the commonest followed by Kawasaki disease. The diagnosis of vasculitis is challenging as it may manifest to it with myraid of symptom and sign including fever, myalgia, arthritis, nephropathy, skin rashes and skin ulceration. Elevation acute phase reactants with identification of some specific markers are necessary to made the diagnosis.

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