Some parents often encounter this situation when taking care of their children, their children’s eczema is not good, and sometimes accompanied by the formation of pustules, have seen many hospitals, a variety of Chinese medicine, Western medicine, creams have been used, but eczema is still no improvement, and children often get bronchitis, pneumonia, thrush and other kinds of infections. Finally, I can only lament: why is eczema so well controlled in other children, but so stubborn in our children? It’s so hard to bring up a child!
Why is it so hard to bring up a child? Is there any other reason?
As a professional doctor, our advice is this.
Make sure to distinguish what is different between your child and other children. Is your child really suffering from eczema? Is it really eczema? Or is it an eczema-like manifestation of another disease?
If your child has recurrent eczema-like dermatitis with abscess formation soon after birth; if your child’s milk teeth are late in falling out; if your child has recurrent bronchitis and pneumonia infections, it is time to alert parents and ask a question in your mind, what is wrong with my child? Because this may not be a simple eczema, but may be a primary immunodeficiency disease – hyper-IgEemia.
Hyper IgEemia: a primary immunodeficiency disease characterized by chronic dermatitis, abscesses and pneumonia caused by recurrent Staphylococcus aureus infections. Laboratory tests reveal increased serum IgE levels, increased eosinophils and other non-specific manifestations.
More than half of the cases occur in infants under the age of 1 year, and both sexes can develop the disease. The initial skin symptoms resemble atopic dermatitis or chronic eczema, with intense itching. Staphylococcal purulent infections occur at the site of scratching, manifesting as boils, carbuncles, and so-called recurrent “cold” staphylococcal pustules. Folliculitis may occur on the head, and pustules, crusting, and flaking may occur around the ears, head, mouth, and groin. Blepharitis may occur in the eyes. Recurrent upper respiratory tract infections and pneumonia are likely to occur, and in severe cases, abscess chests or lung abscesses may occur. In addition, hyperextension of joints and nail dystrophy (such as gray nails) are common, and fractures may occur in older children.
Skin manifestations: chronic eczema-like dermatitis, but the distribution and nature of the eczema is not like atopic eczema, the first symptoms appear soon after birth (about 80% of patients rash occurs within 35 days after birth), papular or papular blistering rash, clear borders, pruritus. The lesions are mostly located on the head and face, behind the ears and on the extensor surfaces of the trunk, and their severity is not seasonal. Skin biopsy shows a large eosinophilic infiltrate with epidermal white herpes.
Infection characteristics: All children have a history of recurrent severe infections, often occurring within the first 3 months of life. The most common symptom of hyper-IgEemia is recurrent infection with S. aureus as the causative agent. It often manifests as cold skin abscesses (so named because of the mild inflammatory response such as redness, swelling, heat and pain), recurrent bronchitis and pneumonia. It can lead to complications such as pulmonary maculopathy, lung abscess, abscess chest, and bronchiectasis. Other common infections include otitis media, chronic sinusitis, septic arthritis, and osteomyelitis. Infections of mucosal areas caused by Candida albicans such as thrush, toenail atrophy or gray toenails caused by Candida infection of the fingernails can also be seen. Other non-bacterial infections include Pneumocystis carinii infection, herpes zoster, cutaneous herpes, and herpetic keratoconjunctivitis.
3. Musculoskeletal manifestations: Patients often have peculiar facial features. Early closure of cranial suture and delayed or no loss of milk teeth are the characteristics of this disease. According to statistics, about 72% of hyper-IgEemia still have non-dislodgment of milk teeth at the age of 8 years, permanent teeth have not erupted or both milk teeth and permanent teeth exist, thus forming a double row of teeth. 70% to 90% of patients have coarse and ugly face, wide nasal bridge, protruding nose and disproportion between cheek and jaw (hemifacial hypertrophy). Fractures are also more common in older patients and less common in patients less than 1 year of age and may be related to weight bearing. Fractures are often caused by unconscious or minor trauma, often involving the long bones, ribs, and pelvis, and are more notable in patients with high IgE because they do not heal easily after fracture. Scoliosis worsens with age, and scoliosis is basically not visible in small children.
4. Other manifestations: growth retardation, osteoporosis, hyperextension of joints and enlarged pores may be present.
Any of the above clinical manifestations such as chronic eczema, recurrent staphylococcal infections with onset in infancy, persistently high serum IgE values, and low leukocyte chemotaxis should be considered as a possible cause of the disease. Increased serum IgE levels and eosinophilia are the strongest laboratory evidence of hyper-IgEemia. Of course, increased serum IgE can also be seen in other diseases, such as atopic dermatitis and chronic active EBV infection.
Laboratory tests.
The vast majority of patients have elevated serum IgE, generally >2000u/mL and up to 50,000u/mL in individual patients. but the levels of IgG, IgA, IgM are mostly normal.
Patients have a long-term increase in blood sedimentation, which may be related to chronic inflammation.
Other non-specific manifestations such as anemia, normal or elevated total leukocyte count, and increased eosinophil ratio are seen.
Neutrophil function often shows various impairments, especially poor chemotaxis. The ability to respond to antigenic stimuli is low, and the delayed skin test is mostly negative.
Treatment
The treatment of this disease is still at the level of empirical therapy. Since patients with hyper-IgEemia are prone to recurrent bacterial infections, especially Staphylococcus aureus infections, anti-Staphylococcus aureus infections are the mainstay of treatment.
IFN-γ can reduce IgE production by B cells, so that IgE levels are reduced, while other immunoglobulin levels are not affected, and IFN-γ can also improve the chemotactic function of neutrophils, which is currently used more often, but some patients have developed autoimmune thrombocytopenia after applying IFN-γ, so it should still be applied with caution.
In case of severe infection, intravenous infusion of gammaglobulin (IVIG) or plasma exchange can also lead to better control of the infection, and these two methods are also effective for severe eczema.
The use of bone marrow transplantation to treat high IgE has also been reported, but it is important to note that bone marrow transplantation can normalize the patient’s immune system and thus improve the patient’s symptoms of infection, but it does not prevent fractures from occurring.
Prognosis
The long-term prognosis of this syndrome is unknown. Those who are diagnosed early and treated aggressively have a low chance of infection and a relatively good prognosis; otherwise, death can occur due to severe infection. The disease also has the potential to develop into a lymphatic malignancy.