Dermatitis Herpetiformis In Childhood

What Is Dermatitis Herpetiformis In Childhood?

[dropcap]D[/dropcap]ermatitis herpetiformis in childhood is an inflammatory skin manifestation of celiac disease in which immunoglobulin A (IgA) autoantibodies target components of the skin, leading to blister formation caused by ingestion of gluten in the diet.  

Dermatitis herpetiformis is characterized by multiple intensely itchy, red blisters appearing on the elbows which can extend down the forearm to the wrist and the back of the knees. Less usual areas involve the back, buttocks, scalp, and abdomen. 

Q: Do the blisters leave a mark when healed?

A: Crops of skin eruptions begin with itching or a burning sensation in reddened papules. There are grouped vesicles and tense blisters. The blister contents may be serous or bloody, with symmetrical distribution (eg, both knees or both elbows). Fluid filled elements rupture leaving denuded areas of sore skin and crust. Subsequently, there is residual hypopigmentation (a white area) or hyperpigmentation (dark area).¹

Most conditions in the spectrum of autoimmune blistering disorders are uncommonly seen in the pediatric population, even the most common ones, such as dermatitis herpetiformis.² The true incidence  is unknown. 

What Is Dermatitis Herpetiformis In Childhood In Celiac Disease and/or Gluten Sensitivity?

• Dermatitis herpetiformis is a skin manifestation of celiac disease and may be the presenting feature of untreated celiac disease.
• Diagnosis made by the finding of fluorescence in granular pattern in the basement membrane zone of a skin biopsy specimen is required to differentiate from other blistering skin disorders and hasten treatment with a gluten free diet.³
• The damage to the small intestine may be asymptomatic when the skin rash appears, but is indistinguishable from that seen in celiac disease.⁴
• Oral dapsone is usually needed in patients with newly detected dermatitis herpetiformis in order to alleviate extremely itchy symptoms.⁵
• Other associated autoimmune diseases can develop after treatment for dermatitis herpetiformis or precede it. High association autoimmune diseases include thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%), and collagen tissue disease.⁶

How Prevalent Is Dermatitis Herpetiformis In Childhood?

Dermatitis herpetiformis is an unusual presentation in a young child.⁷

A retrospective study of children diagnosed with non-diarrheal celiac disease observed a prevalence of 3.1% with dermatitis herpetiformis.⁸

What Are The Symptoms Of Dermatitis Herpetiformis In Childhood?

Dermatitis herpetiformis is marked by the following symptoms:

• Subepidermal blistering eruptions: intensely itchy, red skin eruptions appearing on the extensor surfaces of the elbows, knees, back, buttocks, or scalp mainly, but can appear other places including the face and belly.
• There may, or may not be, gastrointestinal symptoms such as diarrhea, constipation, dyspepsia or gastric reflux present.

How Does Dermatitis Herpetiformis In Childhood Develop In Celiac Disease and/or Gluten Sensitivity?

• Dermatitis herpetiformis results from etiology not fully understood involving exposure to gluten and the subsequent deposition of IgA in the basement membrane zone. An immune complex basis is most likely.⁹

Does Dermatitis Herpetiformis In Childhood Respond To-Gluten Free Diet?

Yes. Skin lesions in dermatitis herpetiformis resolve on a strict gluten free diet.¹⁰.⁷

6 Steps To Improve Dermatitis Herpetiformis In Childhood In Celiac Disease and/or Gluten Sensitivity:

• [dropcap]1[/dropcap]Remove the Trigger. Maintain a Strict, Nutritious Gluten Free Diet:

[box type=”shadow” ]Treatment. This condition responds to the complete elimination of gluten, which is the required treatment that improves both dermatitis herpetiformis and gut health.

• 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.¹¹
• The intestinal lining may take up to a year to heal.[/box]

• [dropcap]2[/dropcap] 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).

[box type=”shadow” ]Here Are Major Inflammatory Food Types That Reduce Healing:

• Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.¹²
• 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.¹²
• 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.¹².
• 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.¹²
• 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.¹²
• Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.¹²[/box]

[box type=”shadow” ]Here Are Important Anti-Inflammatory Food Types to Promote Health:

• 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.[/box]

• [dropcap]3[/dropcap] Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

• [dropcap]4[/dropcap] Manage Your Medications Safely:

[box type=”shadow” ]

Certain medications used to supress dermatitis herpetiformis 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.

[/box]

• [dropcap]5[/dropcap]Nutritional Supplements To Help Correct Deficiencies:

[box type=”shadow” ]

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.[/box]

• [dropcap]6[/dropcap]Manage Natural Remedies: 

[box type=”shadow” ]Hydration:

• 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.[/box]

[box type=”shadow” ]Carminatives. The following  anti-inflammatory plant sources called carminitives help heal the digestive tract. They also tone the digestive muscles which improves peristalsis, thus aiding in the expulsion of gas from the stomach and intestine to relieve digestive colic and gastric discomfort.

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.[/box]

[box type=”shadow” ]Exercise Helps:

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. [/box]

What Do Medical Research Studies Tell About Dermatitis Herpetiformis In Childhood In Celiac Disease and/or Gluten Sensitivity?

RESEARCH STUDY SUMMARIES

“Celiac disease presentation in a tertiary referral centre in India: current scenario.” This facility-based retrospective observational study compared the clinical spectrum of nondiarrheal celiac disease  (NDCD) with that of diarrheal/classical celiac disease (CCD) included consecutive patients diagnosed with celiac disease (CD) (as per modified ESPGHAN criteria) from October 2009 to August 2011. A total of 381 patients were diagnosed with CD during the study period. NDCD was present in 192 (51.8 %). NDCD had higher mean age at presentation (5.8 ± 2.8 years vs. 6.9 ± 2.9 years respectively) and longer duration of symptoms prior to diagnosis (2.9 ± 1.7 years vs. 3.6 ± 2.2 years) as compared to CCD.

In the NDCD group, the most frequent gastrointestinal (GI) symptoms were recurrent abdominal pain [122 (63.5 %)] and abdominal distension [102 (53.1 %)] followed by constipation [48 (25 %)], vomiting [76 (39.6 %)] and recurrent oral ulcers [89 (46.4 %)]. Vomiting and constipation were more frequently seen in NDCD as compared to CCD. Common extraintestinal manifestations in NDCD included failure to thrive [109 (56.8 %)], isolated short stature [36 (18.8 %)], persistent anemia [83 (43.2 %)] and hepatomegaly/splenomegaly or both [56 (29.2 %)].

Associated comorbidities included autoimmune thyroiditis [11 (5.7 %)], type 1 diabetes mellitus [8 (4.2 %)], bronchial asthma [23 (11.9 %)], idiopathic pulmonary hemosiderosis [4 (2.1 %)], Down’s syndrome [3 (1.6 %)], alopecia areata [6 (3.1 %)], polyarthritis [2 (1.0 %)], dermatitis herpetiformis [6 (3.1 %)] and chronic liver disease [6 (3.1 %)].¹³

CASE REPORT SUMMARIES

“Dermatitis herpetiformis presenting as chronic urticaria.” This case report of an 11-year-old boy describes an unusual presentation of dermatitis herpetiformis characterized by a 7-month history of chronic urticaria-like skin lesions. He had evanescent, largely asymptomatic, urticarial wheals on his trunk, face, and extremities that were unresponsive to conventional therapy for urticaria. Skin biopsy specimen findings were consistent with dermatitis herpetiformis and direct immunofluorescence of perilesional skin was diagnostic. The patient had no symptoms of gluten-sensitive enteropathy at the time of diagnosis, and his skin lesions rapidly cleared with dapsone therapy. This patient serves to highlight an unusual presentation of childhood dermatitis herpetiformis and the need to consider this diagnosis when evaluating chronic urticarial lesions in children.¹⁴

 “Dermatitis herpetiformis in a 30-month-old child.” This case report of dermatitis herpetiformis in a 30 month old child is one of the youngest cases so far. After clinical investigations, an asymptomatic gluten-sensitive enteropathy (small intestinal damage) was diagnosed. Skin lesions resolved on a Gluten Free Diet. Dermatitis herpetiformis should be considered in the differential diagnosis of chronic dermatitis in early childhood. Monitoring for complications is the greatest problem at this age.⁷

Sources:

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3. Powell GR1, Bruckner AL, Weston WL. Dermatitis herpetiformis presenting as chronic urticaria. Pediatr Dermatol. 2004 Sep-Oct;21(5):564-7. [↩]
4. Murray JA, The widening spectrum of celiac disease. American Journal of Clinical Nutrition. Mar 1999;69 (3):354-365. [↩]
5. Collin P, Reunala T. Recognition and management of the cutaneous manifestations of celiac disease: a guide for dermatologists. American Journal of Clinical Dermatology. 2003;4(1):13-20. [↩]
6. Mendes FB1, Hissa-Elian A, de Abreu MA, Gonçalves VS. Review: dermatitis herpetiformis. An Bras Dermatol. 2013 Jul-Aug;88(4):594-9. doi: 10.1590/abd1806-4841.20131775. [↩]
7. Medica I, Zmak M, Persic M. Dermatitis herpetiformis in a 30-month-old child. Minerva Pediatrica. Apr 2003;55(2):171-3. [↩] [↩] [↩]
8. Bhattacharya M, Kapoor S, Dubey AP. Celiac disease presentation in a tertiary referral centre in India: current scenario. Indian J Gastroenterol. 2013 Mar;32(2):98-102. doi: 10.1007/s12664-012-0240-y. Epub 2012 Aug 19. [↩]
9. Karpati S. Dermatitis herpetiformis: close to unraveling a disease. Journal of Dermatological Science. Apr 2004;34(2):83-90. [↩]
10. Collin P, Reunala T. Recognition and management of the cutaneous manifestations of celiac disease: a guide for dermatologists. American Journal of Clinical Dermatology. 2003;4(1):13-20. [↩]
11. 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. [↩]
12. 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. [↩] [↩] [↩] [↩] [↩] [↩]
13. Bhattacharya M, Kapoor S, Dubey AP. Celiac disease presentation in a tertiary referral centre in India: current scenario. Indian J Gastroenterol. 2013 Mar;32(2):98-102. doi: 10.1007/s12664-012-0240-y. [↩]
14. Powell GR1, Bruckner AL, Weston WL. Dermatitis herpetiformis presenting as chronic urticaria. Pediatr Dermatol. 2004 Sep-Oct;21(5):564-7. [↩]