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Background: Linear scleroderma (LS) is a variant of localized scleroderma, which is termed as “en coup de sabre” (ECDS) when it involves the head and/or face. Although mainly a dermatological condition, extracutaneous manifestations have been infrequently reported. Clinical Description: A 13-year-old boy presented with fever, altered sensorium with seizures, right hemiparesis, and right-sided upper motor neuron type of facial palsy. He was noticed to have a depression in the left frontoparietal area of the scalp with skin changes. Management and Outcome: The child was initially managed along the lines of acute encephalitis supported with mechanical ventilation. Brain imaging revealed tiny calcification in the left temporal and parietal lobes with altered signal intensities ipsilateral to the scalp lesion. Suspecting an association with the overlying scalp skin lesion, biopsy of the skin lesion was done which confirmed LS. The erythrocyte sedimentation rate was elevated and the antinuclear antibody was positive. The child was started on pulse methylprednisolone following which encephalopathy improved facilitating extubation. Later, he was started on subcutaneous methotrexate and he improved on follow-up. Conclusion: This case creates awareness regarding the association between neurological manifestations and LS EDCS, which responds favorably to immunosuppressives and methotrexate.

Alopecia areata (AA) is an autoimmune disorder that results from the targeted attack of hair follicles by multiple T-cell subsets leading to hair loss. Childhood-onset AA is characterized by a severe clinical course with a poorer treatment outcome. However, it does carry a significant detrimental impact on the quality of life and the self-confidence of the child, besides impairing the emotional health of parents in severely affected cases. The data on pediatric AA is limited. Despite numerous advances made in recent years for the management of AA in children, Food and Drug Administration approved systemic treatment required for severe AA cases is still elusive. In this review, we review the recent literature on clinical manifestations, comorbidities, investigations, and the treatment modalities of AA in children.

A novel observation of chronic eczema developing over the skin of adequately treated lesions of tinea cruris/tinea corporis in six atopic individuals is being shared. We propose that the skin of atopic individuals affected by dermatophytosis be observed for secondary changes even after the complete resolution of the lesions. The use of bland emollients with topical antifungal agents should be encouraged in atopic patients. The extent and duration of the compromised epidermal barrier function of the skin of atopic individuals with dermatophytosis need to be studied further.

SJS and TEN are diseases characterised by epidermal detachment and necrolysis predominantly of drug induced etiology. SJS/TEN begins with a prodrome of fever, malaise, anorexia, pharyngitis, and headache lasting for 2-3 days, at times, extending to 10-11 days. Mucosal lesions usually precede skin lesion. Usually, two mucosal membranes are involved, most commonly conjunctiva and oral mucosa. Oral mucosal involvement is seen in 90% of SJS and almost all patients diagnosed with TEN. In SJS, mucosal involvement is widespread and confl uent in contrast to erythema multiforme where it is focal and seen in only 25%-60% of cases. The exact pathogenesis of SJS and TEN is not fully elucidated. In view of the paucity of T-cell infiltrate,keratinocyte apoptosis could be the result of autocrine or paracrine interaction between Fas, a death receptoron keratinocyte and Fas Ligand (FasL) produced by the keratinocytes along with the substantial contributionof soluble FasL from peripheral mononuclear cells. FasL upregulation in keratinocytes is nitric oxide-dependent anddriven by T-cell derived tumor necrosis factor (TNF) alpha and Interferon-gamma.The management revplves around immediate stoppage of drug and.,supportive care .IVIG,corticosteroids and cyclosporine are all effective drugs but no RCT is available for any of them

Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis which occurs either idiopathically or associated with various systemic diseases and malignancies. Although the association of PG with myeloid malignancies is well known, its association with lymphoid malignancy especially in children is extremely rare. We describe PG in association with acute lymphoblastic leukemia in a 4-year-old child, an occurrence which has been reported just once previously to the best of our knowledge.

Kaposiform hemangioendothelioma involving whole of a leg in a neonate with Kasabach-Merritt phenomenon causing limb and life-threatening situation has not been reported. One such case and its successful management is presented in this case report. Literature review is made.