RESUMO
Hyper-immunoglobulin E (IgE) syndrome (HIES) is an immunodeficiency syndrome characterized by atopic dermatitis, recurrent skin abscesses, and sinopulmonary infections with elevated serum IgE. In addition, patients also present with other skeletal and non-immune symptoms. We present a six-year-old boy with severe atopic dermatitis, multiple food allergies, mild asthma, and recurrent sinopulmonary infections, who presented to the ER with left ankle pain, fever, and inability to bear weight. Physical examination showed generalized eczematous lesions, significant left ankle ecchymosis, swelling, and tenderness. Investigations were pertinent for leukocytosis with neutrophilia and markedly elevated IgE levels with normal IgM, IgG, and IgA levels. HIES genetic panel was negative. MRI with contrast of the affected limb was consistent with osteomyelitis that responded to antimicrobial therapy. This case highlights a diagnostic challenge for allergists and clinicians when evaluating patients with severe atopic dermatitis, recurrent infections, and markedly elevated serum IgE without positive genetic results.
RESUMO
Gianotti-Crosti syndrome (GCS) is a benign acral dermatitis commonly seen in children younger than five years of age with no gender predilection. Clinical features are often vague, including but not limited to fever, lymphadenopathy, and erythematous papular rash that commonly spares the trunk, palms, and soles of the feet. It is presumably underdiagnosed as most children presenting with a widespread papular rash are diagnosed with non-specific viral exanthem. This benign condition has been linked to multiple viruses, and treatment is mainly supportive. We present a previously healthy 18-month-old girl who presented to the emergency room with a progressive skin rash and low-grade fever 10 days after receiving routine immunizations. GCS was diagnosed, and she received supportive care with spontaneous resolution of symptoms in four weeks.
