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Byline: Henrietta. Albela, Wai. Lee, Siti. Nordin, Lalitha. Gopinathan, Muhammad. A. Ramli, Kin. Leong Sir, A 4-year-old girl, of Chinese descent, presented to us with a history of progressive skin [...]

This article discusses the use of secukinumab therapy for pediatric patients with various phenotypes of congenital ichthyosis (CI), a group of skin disorders characterized by scaling, hyperkeratosis, and inflammation. The study included six patients who were treated with secukinumab and assessed using the Ichthyosis Area and Severity Index (IASI) and the Peak Pruritus Numerical Rating Scale (PP NRS). Three patients showed a good treatment response, while the remaining three had a minor response. The study suggests that secukinumab therapy is safe and effective for some patients with CI, but further research is needed to understand the factors that influence treatment outcomes. [Extracted from the article]

Generalized Pustular Psoriasis (GPP) is a rare, severe, life-threatening form of psoriasis and accounts for up to 13.1% of all childhood psoriasis. Common first-line systemic treatment for pediatric patients with GPP include oral acitretin, cyclosporin and methotrexate which have varying efficacy and side effects but multiple interventions are often needed to induce remission and maintain long term control. Recently, the anti IL 17 A monoclonal antibody secukinumab have been shown to be effective in adult patients with GPP; however, there is lack of evidence of its usage in the pediatric population. We describe a case series of 4 pediatric patients with GPP who were treated with off-label use of secukinumab. All four patients had marked clearance and reduction in Generalized Pustular Psoriasis Area & Severity Score (GPPASI) within first 48 h of first injection with subsequent almost complete to complete clearance of skin lesions by 1 month follow up. In conclusion, secukinumab was found to be successful in inducing remission, with rapid clearance and maintaining remission, with or without combination with other systemic agents for pediatric GPP. [ABSTRACT FROM AUTHOR]

Due to the discovery of this similar inflammatory pathway, the IL-17A inhibitor secukinumab, which has been efficacious in the treatment of psoriasis, may also be beneficial in the treatment of ichthyoses. Her baseline score was 36 prior to commencement of therapy and reduced to 24 within the first 4 weeks of treatment and almost 50% from baseline score to 20.3 after 12 weeks of therapy. Recently, Th17/IL-17 skewing has been demonstrated in autosomal recessive congenital ichthyoses (ARCI), which is similar to the pathogenesis of psoriasis. [Extracted from the article]

PHACES syndrome (posterior fossa – haemangioma – arterial – cardiac – eye – sternal) is a multisystem disorder which often presents first with a facial, segmental infantile haemangioma (IH). Prompt recognition of this subtype of IH is key to enable early detection of neurological/ophthalmological/cardiac anomalies. As per international consensus guidelines, the presence of a facial segmental haemangioma > 5 cm triggers screening for associated extracutaneous anomalies. We present three infants with absent/minimal facial haemangioma, but who fulfilled extracutaneous criteria of PHACES syndrome. Patient 1 was a 3-week-old female who presented with a 1–2-cm linear superficial left post-auricular IH. PHACES screening revealed an atrial septal defect, intracranial haemangiomas and hypoplastic cerebrovasculature. Patient 2, a 9-month-old girl, was admitted to intensive care following acute respiratory deterioration. Examination revealed a segmental IH of the occipital scalp. PHACES screening revealed a vascular ring, right aortic arch, aberrant subclavian artery, subglottic and mediastinal IH and narrowing/tortuosity of the left internal carotid artery. Patient 3, a 4-month-old male, was referred for evaluation of three superficial IH, all < 0.5 cm, one on the right post-auricular skin and two on the right forehead. He suffered acute collapse at 2 weeks of life leading to a diagnosis of obstructed total anomalous pulmonary–venous connection requiring emergency surgical correction. PHACES screening revealed bilateral dysplastic/torturous internal carotid arteries. We present three cases of PHACES syndrome without the classic facial segmental IH. Clinicians should be aware of the spectrum of IH observed in PHACES syndrome to enable prompt screening of those presenting with atypical scalp and post-auricular lesions. [ABSTRACT FROM AUTHOR]