An unusual case of recurrent infection and eczema
Case 1: TD, a weeks-old boy, was referred to me. He had a vesicular (butlig na may tubig) lesion and episodes of bloody stool. The rash initially started on the head and progressively moved to all parts of the body. The patient was given broad-spectrum antibiotics as prophylactic treatment and the symptoms subsided over the next few days.
At the time of referral, there was no recent immunization done yet and the patient was apyrexial (without fever). Prenatal and postnatal history was uneventful at that time. Possible autoimmune reactions leading to the symptoms were suggested.
Months later, the same patient had several bruises all over the body, including the lips and buccal cavity (mouth), together with epistaxis (nose bleed), melena (bloody stools) and fever. Platelet count was very low on admission (22,000/cm). The patient started having coffee brown vomitus (meaning there is blood in the vomit). The patient’s uncle died with similar symptoms at the age of six. Bone marrow biopsy was done and it was normal. All the other tests were normal except for his immunoglobulin (antibody) profile which showed high Immunoglobulin A and E, low Immunoglobulin M and normal Immunoglobulin G.
Case 2: GA, a four-year-old boy with sparse hair, had recurrent eczematoid pruritic rash on the scalp, neck, skinfolds of upper and lower limbs described as red, itchy, dry, flaky rough skin. At first, his problem seemed to be just a simple case of skin asthma. Noticeable, too, was the presence of multiple abscesses all over his scalp, body, arms, and legs which are common among atopic children. Sometimes, GA would have pinpoint bleeding on the skin, on top of red, flaky, itchy skin that enlarges and becomes more obvious as a bruise. It was like this for several weeks until GA started having bloody diarrhea. So I referred him to a pediatrician who treated him as a case of dysentery but there was no improvement in the bleeding. He was referred to another hospital where he was diagnosed as a case of hemolytic uremic syndrome. Patient then was hospitalized for days and managed with fresh whole blood transfusion, broad-spectrum antibiotics and was discharged when he was without symptoms after five days. Fifteen days later, patient again developed multiple smaller abscesses with ecchymosis (pasa) and seven days after I started him on antibiotics, he had another episode of bloody diarrhea.
Our immune system is our body’s defense against infections and other harmful invaders. Without it, illnesses from bacteria or viruses would be constant. Our immune system is made up of special cells, tissues, and other organs that work together to protect us. The lymphatic system is a major part of the immune system. It is a network of lymph nodes (kulani) and vessels. Lymphatic vessels are thin tubes that branch out, like blood vessels, throughout the body. They carry a clear fluid called lymph. Lymph contains tissue fluid, waste products, and the immune system cells. Lymph nodes are small, bean-shaped clumps of immune system cells that are connected by lymphatic vessels. They contain white blood cells that trap viruses, bacteria, and other invaders, including cancer cells.
White blood cells are the cells of the immune system. They are made in one of our lymph organs, the bone marrow. Other lymph organs include the spleen and thymus.
What can go wrong with one’s immune system?
When one’s immune system doesn’t work the way it should, it is called an immune system or immunodeficiency disorder. There are four kinds:
1. One may be born with a weak immune system called a primary immune deficiency. Severe combined immunodeficiency (SCID) is an example. Children are in danger of infections from bacteria, viruses, and fungi. It is referred to as the“bubble boy disease” (in the 1970s, a boy had to live in a sterile environment inside a plastic bubble) because those with SCID have little or no white blood cells (B- and T-lymphocytes) that defend them from infection by viruses, bacteria, and fungi.
2. One may get a disease that weakens one’s immune system, the so-called acquired immune deficiency. Drugs can weaken the immune system. This happens to people on chemotherapy or other drugs to treat cancer or to people who, following organ transplants, take medication to prevent organ rejection. Also, infections like the flu virus, mononucleosis, measles, and HIV can weaken the immune system for a brief time. Smoking, alcohol, and poor nutrition can also weaken the immune system.
3. One may have an immune system that is too active, like asthmatics, wherein response in one’s lungs can cause coughing, wheezing, and trouble in breathing. Asthma can be triggered by a common allergen like dust or pollen or by an irritant like tobacco smoke.
4. One may have an immune system that turns against that same person called autoimmune disease. Like in Type 1 diabetes where the immune system attacks the cells in the pancreas that make insulin. Insulin removes sugar from the blood to use as energy. Here the tissues in the body attack normal healthy tissues. The cause is unknown and can probably be due to a combination of a person’s genes and something in the environment that triggers those genes.
Our two cases above are examples of a person born with a weak immune system. Both patients have problems with their infection-fighting white blood cells (B & T cells and clot-forming platelets) called Wiskott Aldrich Syndrome. This can result in prolonged episodes of bleeding, recurrent bacterial and fungal infections, and an increased risk of cancers and autoimmune diseases.
An in-depth investigation can help a great deal patients who depend on their dermatologists for correct diagnosis, a more thorough management, exceptional care, and a better outcome.
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