INTRODUCTION

Childhood Polyarteritis Nodosa (PAN) is a rare, systemic necrotizing vasculitis of the small to medium-sized vessels with an uncertain global distribution. The primary etiology is unknown. However, PAN is commonly associated with preceding Group A streptococcus infection in children. The most common cutaneous manifestations of PAN include tender subcutaneous nodules, livedo reticularis, digital ischemia and ulceration. To date, no reports have documented cutaneous PAN as a sequela of rheumatic fever.

CASE REPORT

This is a report of a 7-year-old Filipino male who presented with multiple, well-defined erythematous to hyperpigmented, firm, tender nodules, with some areas of lace or net-like macules and patches, some resolved leaving hyperpigmentation measuring 1x1 cm to 2x2 cm on the lower back, bilateral upper and lower extremities accompanied by fever, malaise, arthralgia and myalgia with a previous history of rheumatic fever. A 6mm skin punch biopsy revealed findings consistent with PAN. The patient was managed with prednisone. Due to the limited response to treatment, he was eventually given mycophenolate mofetil.

CONCLUSION

Childhood polyarteritis nodosa (PAN) is a rare form of necrotizing inflammation of the medium-sized blood vessels primarily linked to Group A streptococcal infection in children, with no known reported cases associated with rheumatic fever. However, in this case, we were able to observe that PAN could present as a probable rare sequela of rheumatic fever. This warrants close follow-up among such patients.

Human ; Male ; Child: 6-12 Yrs Old ; Polyarteritis Nodosa ; Rheumatic Fever ; Vasculitis

Netherton Syndrome (NS) is characterized by a triad of congenital ichthyosiform erythroderma, trichorrhexis invaginata, and atopic diathesis. Diagnosis of NS poses a challenge due to its variable presentation and overlap with other dermatological conditions. Herein, we report a case of NS in a sibling pair, underscoring the challenges in diagnosis and genetic implications of this condition.

We present a case of an 8-year-old female and a 7-year-old male sibling pair, with Netherton Syndrome, who initially presented with atopic dermatitis and erythroderma without hair shaft abnormalities. Further investigation and genetic testing revealed homogeneous SPINK5 gene mutations in both patients, leading to the diagnosis of NS.

Early recognition and diagnosis of Netherton Syndrome are essential for proper management. In patients with early-onset atopic dermatitis resistant to treatment and recurrent erythroderma, further investigation is needed to exclude other diagnoses like Netherton Syndrome.

Human ; Male ; Female ; Child: 6-12 Yrs Old ; Netherton Syndrome

Nevus comedonicus is a rare developmental anomaly of the pilosebaceous unit presenting as grouped dilated follicular orifices plugged with keratin. The comedones are usually arranged unilaterally along the lines of Blaschko. As of year 2000, only 200 cases have been reported.