Homozygous CARD14 variant presenting as infantile erythroderma.

A wide range of inherited and acquired conditions can manifest as infantile erythroderma, among which CARD14-associated papulosquamous eruption (CAPE) is a rare cause. An infant boy presented with a psoriasiform rash that progressed to erythroderma and was unresponsive to topical steroids and cyclosporine. The early onset of the disease, its severity and resistance to conventional treatment were suggestive of a genetic cause. Genetic evaluation revealed a homozygous CARD14 variant of uncertain significance establishing the diagnosis of CAPE, and his parents were heterozygous carriers. There was only minimal improvement in the condition with supportive management and treatment with acitretin. Unfortunately, the child succumbed to sepsis and metabolic complications following a sudden worsening of skin disease. This case highlights the significance of genetic studies in diagnosing treatment-refractory cases of infantile erythroderma and emphasises the importance of early recognition of this rare condition.

Surgical Matricectomy Versus Phenolization in the Treatment of Ingrown Toenails: A Randomized Controlled Trial.

BACKGROUND: Treatment of ingrown toenail includes various nonsurgical and surgical treatments. However, there is no consensus on the ideal first-choice treatment. OBJECTIVE: To compare phenolization versus surgical matricectomy (SM) after lateral nail plate avulsion in terms of efficacy, recurrence rates, postoperative outcomes, and cosmesis. METHODS: The authors enrolled 45 consenting patients and randomized them into 2 groups (Group 1 = phenolization and Group 2 = SM) using stratified block randomization and followed them up at 1 week, 1 month, and 6 months. RESULTS: The median percentage improvement in pain visual analog scale (VAS) score was comparable between the 2 groups ( p = 0.793). The mean photo VAS showed significant improvement in Group 1 at 1 week ( p = 0.00) and 1 month ( p = 0.02) but not at 6 months ( p = 0.44). The median number of days for pain relief ( p = 0.169), for healing ( p = 0.192), and for resuming work ( p = 0.136) were not significantly different between the 2 groups. The time required to regain normal morphology was significantly longer in Group 2 ( p = 0.006). None of the patients in either group presented with recurrence at 6 months and 1 year. The authors observed failure of treatment in 1 patient in Group 2. CONCLUSION: Both procedures were equally efficacious, had minimal complications, and showed no recurrence at 6 months and 1 year.

An unusual case of keratinopathic icthyosis: a diagnostic conundrum.

Epidermolytic ichthyosis (EI) is a rare inherited ichthyosis related to heterozygous mutations in the Keratin 1 or Keratin 10 genes. Because of the broad phenotypic spectrum, it is sometimes difficult to differentiate it from other keratinopathic ichthyoses (KI) in clinical practice. We report an intriguing case of KI presenting as generalized ichthyosis in a reticulate pattern surrounding islands of normal skin, epidermolytic hyperkeratosis and binucleate cells on histopathology, and heterozygous mutation in KRT10. Through this case, we would like to demonstrate the importance of genetic studies and genotype-phenotype correlation in diagnosing such challenging cases.

A Novel Desensitization Protocol for Infliximab Hypersensitivity in a 13-Year Old Patient with Pustular Psoriasis.

BACKGROUND: Drug hypersensitivity reactions to infliximab have been reported in pediatric patients. At times, these patients may need infliximab administration in spite of hypersensitivity. However, only a few reports of desensitization protocols are available in the literature in pediatric patients. CASE REPORT: We report a case of immediate hypersensitivity reaction to intravenous infliximab in a 13-year-old child suffering from pustular psoriasis who eventually underwent a 14 step desensitization protocol for the administration of infliximab in a pediatric intensive care unit. RESULTS AND CONCLUSION: Although our desensitization protocol was safe and effective, we recommend the entire desensitization procedure to be performed under the supervision of experienced personnel in a pediatric intensive care unit. Future studies with larger sample size are needed to confirm our findings.