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What Is Chronic Bullous Dermatosis Of Childhood?
Chronic bullous dermatosis of childhood, also termed linear IgA dermatosis, is the most common acquired autoimmune blistering disorder of childhood and is characterized by itchy, urticated papules and plaques as well as polycyclic lesions (merged circles) with blisters at the edge, located on normal looking skin around the mouth and perineum in young children. In children over 7 years, other parts of the body may rather be affected.
Q: What tissue is targeted in chronic bullous dermatosis of childhood?
A: In chronic bullous dermatosis of childhood, there is an autoimmune attack on structural proteins, usually proteolytic fragments of collagen XVII, which renders the dermal-epidermal junction prone to blistering.
The dermal-epidermal junction is where the surface skin layer, or epidermis, meets the lower layer, or dermis. Diagnosis is confirmed by characteristic histology and direct immunofluorescence showing linear IgA (immunoglobulin A antibody) staining of the basement membrane zone.1
The incidence of chronic bullous disease of childhood is rare. Age of onset is typically before 5 years of age and is seen in all ethnic groups.
What Is Chronic Bullous Dermatosis Of Childhood In Celiac Disease and/or Gluten Sensitivity?
- Chronic bullous dermatosis is a rare skin disease associated with celiac disease and may be a presenting feature of untreated celiac disease.
- Patients with chronic bullous dermatosis may have an atypical form of celiac disease with an immunopathogenesis that differs from current concepts in celiac disease and serological screening for celiac disease may need to be repeated.2
- The association between celiac disease and other immune disorders may be due to the sharing of a common genetic background, such as HLA antigens. However, in a very large study, involving 909 patients with celiac disease, Ventura and his associates found that the development of immune disorders in celiac disease was clearly related to the duration of exposure to gluten.3
How Prevalent Is Chronic Bullous Dermatosis Of Childhood In Celiac Disease and/or Gluten Sensitivity?
Chronic bullous dermatosis is described as an association with biopsy verified celiac disease.2
What Are The Symptoms Of Chronic Bullous Dermatosis Of Childhood?
Chronic bullous dermatosis is marked by these symptoms:
- Long-standing or relapsing itchy wheals and tense blisters erupt on the face and perineal area.
- Legs and other areas of the body are uncommonly affected.
- Blisters may occur in a rosette pattern called a “cluster of jewels.”
How Does Chronic Bullous Dermatosis Of Childhood Develop In Celiac Disease and/or Gluten Sensitivity?
- Chronic bullous dermatosis results from an unclear autoimmune mechanism involving gluten exposure.
Does Chronic Bullous Dermatosis Of Childhood Respond To Gluten-Free Diet?
Yes. Chronic bullous dermatosis resolves on a gluten free diet.2
6 Steps To Improve Chronic Bullous Dermatosis Of Childhood In Celiac Disease and/or Gluten Sensitivity:
- 1Remove the Trigger. Maintain a Strict, Nutritious Gluten Free Diet:
- Gut health is the foundation to restore ALL health. Restored health will enable you to maintain a strict gluten free diet, just as other life tasks will be easier.
- A strict gluten free diet means removing 100% of wheat, barley, rye and oats from the diet.
- Cutting out bread and other obvious sources of gluten is not good enough for recovery. Even 1/8th teaspoon of flour or bread crumb is enough to sustain the inflammation that is damaging your small intestine, causing increased permeability (leaky gut) and allowing undigested gluten to enter your body where it can damage structures and function, and instigate immune inflammatory responses.
Correct Your Individual Nutritional Needs.
- Eat foods that can replenish missing nutrients. Find them under NUTRIENT DEFICIENCIES.
- Take nutritional supplements as needed. Find them under NUTRIENT DEFICIENCIES.
Recovery. You should begin to feel better within a week and notice more energy as inflammation subsides and the absorbing cells that make up the surface lining of your small intestine are better able to function.
- Intestinal lining cells are replaced every 5 days. The healing process is like sunburn where the damaged surface layer of skin sloughs off and is replaced with new normal cells.
- Leaky gut normally resolves in two month after starting a gluten free diet and brings about a big improvement in health. Improvement in intestinal permeability precedes morphometric recovery (cell appearance and structure) of the small intestine in celiac disease.4
- The intestinal lining may take up to a year to heal.
- 2 Reduce Inflammation. Foods to Eat and Foods Not to Eat:
Because gluten is inflammatory, eliminate OTHER inflammatory foods from your diet to reduce an additive effect to gluten. At the same time, try to eat foods that reduce inflammation (anti-inflammatory).
- Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.5
- Allergenic Foods. Includes foods that trigger the immune sytem to produce IgE antibodies. Allergy testing is the usual way to discover these offending foods.
- Shelf Stable Processed Foods. Includes any that contain additives and preservatives. Look for them on the nutrition label of the box or package. Additives and preservatives also disrupt intestinal permeability causing leaky gut.5
- Fats. Limit deep fried foods, trans-fats, saturated fats (animal fat/butter), and EXCESSIVE omega-6 fatty acid oils like corn oil. Rancid fats, sodium caprate (a medium chain fat), and sucrose monester fatty acid (a food grade surfactant) induce significant disruption of the intestinal barrier that causes leaky gut.5.
- Excessive Refined White Flours (bran layer removed). Includes products made from them such as cookies, bread, cakes, pies. Bran contains the vitamins and minerals that metabolize grains and slows the otherwise rapid entry of sugar from their digestion into the bloodstream. Also disrupt intestinal permeability causing leaky gut.5
- Refined Sugars. Includes white sugar, corn fructose and high fructose corn syrup.
- Certain Spices. Includes paprika and cayenne pepper which disrupt intestinal permeability causing leaky gut.5
- Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.5
- Fruits. Contain ample amounts of vitamins, minerals and phytochemicals which are naturally occuring components in plants that detoxify toxins, carcinogens (reducing the risk by 50%) and mutagens.
- Non-Starchy Vegetables. Support intestinal integrity and provide ample amounts of vitamins, minerals and phytochemicals. Includes green leafy vegetables such as lettuce and kale, also onion, broccoli, garlic, and others.
- High Quality Complex Carbohydrates. Provide vitamins, minerals, and fiber while boosting serotonin levels to help you relax and feel calm. Includes whole grains, legumes, and root vegetables such as carrots, parsnips, sweet potatoes, turnips, red beets, and others.
- Antioxidants. Protect the body from inflammatory oxidant molecules that continually occur and help us handle stress and reduce irritability. Includes vitamin C-containing foods such as lemon, grapefruit, apricot, Brussels sprouts and strawberries, and others. Also, includes vitamin E-containing foods such as nuts, seeds, avocado, olive oil, and others. Cocoa is good, too.
- Omega-3 Fatty Acids. Balance opposing omega-6 fatty acids and bad fats. Fish sources includes tuna, salmon, cod, and others. Plants sources include flax, chia seeds, canola oil, and others.
- Probiotics. Supply normal microbes needed for colon health and health of the body such as these fermented foods: yogurt, kefir, and unpasteurized apple cider vinegar.
- Prebiotics/ High Fiber Foods. Food with fiber keeps our population of colonic microbes healthy.
- Protective Herbs and Spices. See below #6 below for examples.
- 3 Information Sheet You Can Take to Your Doctor or Other Health Professional:
- 4 Manage Your Medications Safely:
Certain medications used to supress chronic bullous dermatosis cause nutritional deficiencies that promote complications in celiac disease. Ask your doctor or pharmacist about this possible adverse effect if you are taking any of the drugs listed below. Do not stop prescribed medications without supervision.
This is not a complete listing.
ANTIBIOTICS disrupt intestinal permeability which complicates celiac disease.
- Dapsone depletes Vitamin K.
- 5Nutritional Supplements To Help Correct Deficiencies:
The type and quantity of nutritional supplements that may be needed depend on which nutrients are deficient.
- Multivitamin/mineral combination that provides 100% once a day is useful to improve overall nutrient levels. This is a safe dose, but always check with your doctor to avoid interactions with medications.
- Vitamin K as prescribed following blood test for status.
Storage Note: Store container tightly sealed, away from heat, moisture and direct light to avoid loss of potency. That is, in a safe kitchen cabinet – not in the bathroom or on the kitchen table.
- 6Manage Natural Remedies:
- Eight glasses of water are recommended per day unless there is a contraindication such as kidney or heart disease. The Institute of Medicine recommends approximately 2.7 liters (91 ounces) of total water, from all beverages and foods, each day for women and 3.7 liters (125 ounces) daily of total water for men.
- If you are thirsty, drink water. Add fresh, squeezed lemon to water. Lemon is anti-inflammatory, alkalizing and provides vitamin C.
- Hydration Test: Urine should be pale yellow. Fingertips should be plump, without pruning but this may not be reliable when fingers are swollen with edema. Lips should be plump, without puckering. The feeling of thirst can be unreliable.
- What is wrong with soda, coffee, tea, and alcohol? These drinks are dehydrating, increase acid, and deplete nutrients.
Carminative Food Remedies:
- Raspberry.
- Carrot is also a cleansing digestive tonic.
- Grape is also bile stimulating and a cleansing remedy for sluggish digestion and laxative.
- Redbeets also stimulate and improve digestion and are easily digested.
- Cabbage also stimulates and improves digestion and is also a liver decongestant.
- Lettuce also stimulates and improves digestion and is also an alterative, meaning it improves the function of organs involved with the digestion and excretion of waste products to bring about a gradual change.
- Potatoes are antispasmodic (due to atropine like properties) and a liver remedy.
Carminative Herb Remedies:
- Sage is also a digestive, astringent, bile stimulant and energy tonic that heals the mucosa. Drink as tea or use in cooking.
- Chamomile, lemon balm, and fennel, (as a tea) also help relieve nervous tension.
- Parsley also relieves indigestion.
- Rosemary as a tea and in cooking also is a nervous system tonic for stress and fatigue, bile stimulant, and can relieve headaches and indigestion.
- Thyme is also soothing remedy useful for stimulating digestion of rich, fatty foods.
Carminative Spice Remedies:
- Cloves are also antispasmodic.
- Nutmeg is also useful for indigestion.
- Ginger.
Exercise improves circulation and rids the body of toxins.
- Walking is aerobic exercise that reconditions the whole body to improve stamina. Read more about Exercise and Fitness.
- Weight training builds muscle. Read more about Exercise and Fitness.
- Stretching improves flexibilty. Read more about Exercise and Fitness.
Note: Exercise is important, but the amount and type of exercise undertaken depends on your health. Your first priority is to heal.
What Do Medical Research Studies Tell About Chronic Bullous Dermatosis Of Childhood In Celiac Disease and/or Gluten Sensitivity?
“Chronic bullous dermatosis of childhood associated with coeliac disease in a 6-year-old boy.” This case report of a 4 year old boy with chronic bullous dermatosis of childhood (CBDC) contradicts the supposed non-association with gluten sensitive enteropathy. At 3.5 year of age the child was diagnosed with CBDC with fairly good response to Dapsone. Bouts of new lesions kept appearing due to a low dose (conceding to parents’ fear of side effects) and topical steroids were added.
At 4 years of age, subnormal levels of iron and zinc were found. S-anti-gliadin antibodies (AGA) were normal but S-IgA anti-endomysium antibodies (EMA) were positive at 1/20 (ref value >1/10). A peroral small bowel capsule biopsy from the distal duodenum showed a light microscopically normal mucosa without an increased number of intraepithelial lymphocytes. He was kept on a normal diet including gluten. At 6.1 years of age the AGA was still negative but the EMA was higher at, 1/640 and 6 months later the EMA was even higher at 1/1,280 without obvious GI symptoms. A re-biopsy of the small bowel showed hyperplastic villous atrophy of the crypts, severe inflammatory activity and increased numbers of intraepitheleal lymphocytes consistent with celiac disease. A gluten free diet was instituted and Dapsone dose decreased to half. At 4 month follow-up, the boy had only slight perioral changes. EMA and tTG antibodies were negative and Dapsone treatment was stopped. A small bowel biopsy after one year on a gluten free diet showed a normal mucosa.6
- Mintz EM, Morel KD.Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhood. Dermatol Clin. 2011 Jul;29(3):459-62, ix. doi: 10.1016/j.det.2011.03.022. [↩]
- Hogberg L, Sokolski J, Stenhammar L. Chronic bullous dermatosis of childhood associated with coeliac disease in a 6-year-old boy. Acta Dermato-Venereologica. 2004;84(2):158-9. [↩] [↩] [↩]
- La Villa G, Pantaleo P, Tarquini R, Cirami L, Perfetto F, Mancuso F, Laffi G. Multiple immune disorders in unrecognized celiac disease: a case report. World J Gastroenterol. 2003;9(6):1377-1380, Available at: http://www.wjgnet.com/1007-9327/9/1377.asp. Accessed Jan 3, 2005. [↩]
- Cummins AG, Thompson FM, Butler RN, et al. Improvement in intestinal permeability precedes morphometric recovery of the small intestine in coeliac disease. Clinical Science. Apr 2001;100(4):379-86. [↩]
- Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [↩] [↩] [↩] [↩] [↩] [↩]
- Hogberg L, Sokolski J, Stenhammar L. Chronic bullous dermatosis of childhood associated with coeliac disease in a 6-year-old boy. Acta Dermato-Venereologica. 2004;84(2):158-9. [↩]