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Dermatitis Herpetiformis or Duhring’s Disease

Dermatitis Herpetiformis. Blisters Opened Giving Relief From Pain and Itching.
Dermatitis Herpetiformis On Forearm. Skin Is Darkened Where Old Blisters Healed.

What Is Dermatitis Herpetiformis?

[dropcap]D[/dropcap]ermatitis herpetiformis (DH) is an autoimmune extremely itchy, painful bullous skin rash (blistering eruptions) arising from the underlying dermis layer of skin as a consequence of gluten sensitivity.

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 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

Rupture of blisters begins relief from intense burning and itching.

Dermatitis Herpetiformis Eruptions On Knees.
Dermatitis Herpetiformis Eruptions On Knees. Notice White Areas Showing Loss of Pigmentation From Healed Blisters.

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

Primary care providers should be aware of this skin condition, as they are more likely than a gastroenterologist to be confronted with this type of presentation of celiac disease.2

Sources:
  1. Mendes FB, Hissa-Elian A, de Abreu MA, Gonçalves VS. Review: dermatitis herpetiformis. An Bras Dermatol. 2013 Jul-Aug;88(4):594-9. [↩]
  2. Robinson BL, Davis SC, Vess J, Lebel, J. Primary care management of celiac disease. Autoimmune Disorders. Nurse Practitioner. February 2015: Vol 40 – Issue 2; 28–34. [↩]

Dermatomyositis

dermatomyositisWhat Is Dermatomyositis?

[dropcap]D[/dropcap]ermatomyositis is a rare autoimmune systemic disease of the connective tissue that is characterized by inflammatory and debilitating degenerative changes in the muscles and in the skin. 

Dermatomyositis results in symmetric, proximal muscle weakness of limbs (upper arms and legs), and skin manifestations. 50-70% of patients have circulating myositis-specific auto-antibodies.

The course of dermatomyositis is unpredictable being marked by spontaneous flare-ups and remissions. It can begin slowly or abruptly according to the factor that is triggering the onset such as infection, medications like phenytoin, and autoimmune disease.

Q: What are the skin manifestations of dermatomyositis?

A: Classic skin manifestations of dermatomyositis include these features:

  • The heliotrope rash (lilac color) on upper eyelids.
  • Rash on face, neck, shoulders, upper chest, elbows, knees, knuckles, and back.
  • Gottron’s papules (scaly, red eruptions or  patches over the knuckles, elbows, and knees).
  • The V-sign (rash front of neck and chest).
  • The shawl sign (rash distribution on shoulders and back).1

Additional cutaneous manifestations are described below under symptoms.

Dermatomyositis is associated with an increased risk of  cancer, other autoimmune diseases, such as lupus and psoriasis, and it can be a complication of interferon-α therapy. About 1 person in 100,000 are affected according to various studies. While it affects all ages, women have twice the occurence of men.

There is no cure for dermatomyositis, but the symptoms can be treated.  Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest.  The standard treatment for dermatomyositis is a corticosteroid drug, given either in pill form or intravenously.  Immunosuppressant drugs, such as azathioprine and methotrexate, may reduce inflammation in people who do not respond well to prednisone.2

What Is Dermatomyositis In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Marvi U, Chung L, Fiorentino DF. Clinical presentation and evaluation of dermatomyositis. Indian J Dermatol. 2012 Sep;57(5):375-81. doi: 10.4103/0019-5154.100486. [↩]
  2. National Institute of Neurological Disorders and Stroke. [↩]

Eczema (Atopic Dermatitis)

eczemaWhat Is Eczema?

[dropcap]E[/dropcap]czema, or atopic dermatitis, is a chronic recurring inflammatory skin disorder characterized by itching, eczematous plaques and a defective epidermal barrier (surface layer of skin cells) that fails to hold moisture, with affected areas becoming dry then inflamed.

Q: Why does eczema develop?

A: Patients with atopic dermatitis have genetically determined risk factors that affect the barrier function of the skin and immune responses that interact with environmental factors.1

Atopic eczema is associated both with an abnormal skin matrix and impaired systemic immune response. Therefore, isolated topical treatment may have suboptimal effect.2 

Atopic eczema is a common presentation of  immune response towards food proteins in infancy.3

Global evidences reflect a marked increase in prevalence, which has tripled since 1960. In the United States, the current prevalence rates range from 10% to 20% in children and 1 to 3% in adults.4

What Is Eczema In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Mesquita Kde C, Igreja AC, Costa IM. Atopic dermatitis and vitamin D: facts and controversies. An Bras Dermatol. 2013 Nov-Dec;88(6):945-53. doi: 10.1590/abd1806-4841.20132660. [↩]
  2. Caffarelli C, Santamaria F, Vottero A, Bernasconi S. Progress in Pediatrics in 2012: choices in allergy, endocrinology, gastroenterology, hematology, infectious diseases, neurology, nutrition and respiratory tract illnesses. Ital J Pediatr. 2013 May 8;39(1):26. [↩]
  3. Ho MH, Wong WH, Chang C. Clinical Spectrum of Food Allergies: a Comprehensive Review. Clin Rev Allergy Immunol. 2012 Nov 16. [↩]
  4. Mesquita Kde C, Igreja AC, Costa IM. Atopic dermatitis and vitamin D: facts and controversies. An Bras Dermatol. 2013 Nov-Dec;88(6):945-53. doi: 10.1590/abd1806-4841.20132660. [↩]

Psoriasis

Psoriasis_on_back[1]

 What Is Psoriasis?

[dropcap]P[/dropcap]soriasis is a chronic, autommune relapsing skin disorder characterized by scaling, erythema (redness), and less commonly, pustulation.1 

The body surface area affected and the degree to which psoriasis is a problem varies considerably among patients and over time.2 Often there are additional manifestations in the nails and in joints.3

Q: Are there different forms of psoriasis?

A: There are five forms of psoriasis. The lesions in all forms are itchy and red but vary in appearance and severity. Plaque psoriasis is the most common form observed in more than 80% of patients. Atypical forms include guttate, inverse, pustular, and erythrodermic psoriasis.4

  • Plaque psoriasis features thickened or raised red areas that have a distinct edge and are covered with silvery white buildup of flaky skin typically on elbows, knees, scalp and buttocks.
  • Gutate psoriasis appears as small, flat red patches with shiny buildup that are not usually painful, just itchy. There may be a few or many patches and they can group together.
  • Inverse psoriasis affects folds of skin, armpits and the groin area. Lesions are deep red with shiny buildup. It can be a thin red area along a crease line or involve, for example, the whole armpit.
  • Pustular psoriasis features an itchy, red base followed by blisters of white, non-infectious pus that appears glossy after a day or two and then sloughs in cycles. These areas may be limited to certain areas such as the hands and feet or be more widespread.
  • Erythrodermic psoriasis involves large areas of the body’s surface, inflaming normal skin and changing it into very red, raw looking flesh that is painful, swollen and itchy. This form requires extensive treatment, and complications can be life-threatening. Fortunately, this form of psoriasis is the least common.

Psoriasis in children has been reported to differ from that among adults being more frequently itchy and plaque lesions are relatively thinner, softer, and less scaly, face and flexural involvement is common and guttate type is the characteristic presentation.5

In children, psoriasis is a common skin disorder with about one third of all patients having onset of disease in the first or second decade of life. A chronic disfiguring skin disease, such as psoriasis, in childhood is likely to have profound emotional and psychological effects, and hence requires special attention.6

What Is Psoriasis In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Addolorato G, Parente A, de Lorenzi G, et al. Rapid regression of psoriasis in a coeliac patient after gluten-free diet. A case report and review of the literature. Digestion. 2003;68(1):9-12. [↩]
  2. Stern, R. S., Nijsten, T., Feldman, S. R., Margolis, D. J. and Rolstad, T.

    Psoriasis is common, carries a substantial burden even when not extensive, and is associated with widespread treatment dissatisfaction. J. Invest. Dermatol. Symp.. 2004 Mar;9(2):136-9.. [↩]

  3. Weigle N, McBane S. Psoriasis. Am Fam Physician. 2013 May 1;87(9):626-33. [↩]
  4. Weigle N, McBane S. Psoriasis. Am Fam Physician. 2013 May 1;87(9):626-33. [↩]
  5. Dogra S, Kaur I. Childhood psoriasis. Indian J Dermatol Venereol Leprol. 2010 Jul-Aug;76(4):357-65. doi: 10.4103/0378-6323.66580. [↩]
  6. Dogra S, Kaur I. Childhood psoriasis. Indian J Dermatol Venereol Leprol. 2010 Jul-Aug;76(4):357-65. doi: 10.4103/0378-6323.66580. [↩]

Vitiligo

What Is Vitiligo? [dropcap]V[/dropcap]itiligo is a pigmentation disorder of the skin characterized by permanent loss of melanocytes in defined areas and, in some patients, antibodies to melanin. Vitiligo has significant psychological impact if occurring before adulthood.1… 

Seborrhea Dermatitis

Seborrhea patches at the inner eyebrows. GFW
Seborrhea patches at the inner eyebrows. GFW

What Is Seborrhea Dermatitis?

[dropcap]S[/dropcap]eborrhea dermatitis is a recurring inflammatory disorder of sebaceous glands characterized by scaly patches of skin, often with bumps.

Seborrhea dermatitis results from the body’s inflammatory reaction to invasion by pityrosporum yeast that naturally inhabits the scalp and skin.1 Inflammation is the normal response to tissue injury and germ invasion.

Pityrosporum is a yeast that is commonly present worldwide. Its development depends on various factors that predispose to pityriasis versicolor, a chronic and mild superficial yeast infection. These infections usually are asymptomatic without itching or pain and without cellular and/or antibody responses.2

Q: Why are the sebaceous glands particularly affected by this yeast?

A: Pityrosporum yeast is an organism that needs oil produced by sebaceous glands to grow. If conditions permit, this yeast invades the superficial layer of skin and hair shafts to reproduce, causing infection. Such conditions include weakened skin due to nutritional deficiencies, excessive build-up of oil on skin, and altered immunity due to systemic disease such as psoriasis.

In adults, areas of skin that are the most affected have the greatest number of sebaceous glands especially the scalp, back, underarms, and the face including the eyelids, eyebrows and side folds of the nose.

All ages are subject to seborrhea dermatitis, and males have a higher occurence than females.

What Is Seborrhea In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Krause’s Food, Nutrition, & Diet Therapy. 10th Edition. Kathleen Mahan, Sylvia Escott-Stump. 2000. W.B. Saunders Company. [↩]
  2. Zarei-Mahmoudabadi A, Zarrin.  M, Mehdinezhad F. Seborrheic dermatitis due to Malassezia species in Ahvaz, Iran. Iran J Microbiol. 2013 Sep;5(3):268-71. [↩]

Scleroderma (Systemic Sclerosis)

sclerodermaWhat Is Scleroderma?

[dropcap]S[/dropcap]cleroderma is a chronic skin manifestation of progressive systemic sclerosis characterized by generalized thickened, edematous skin firmly bound to subcutaneous tissue which causes limited movement.

Systemic sclerosis a connective tissue disease that involves destructive changes in the skin, blood vessels, muscles, and internal organs. The course can be mild or it can be fatal. Cardiopulmonary complications from fibrosis are the most common cause of death.

Gastrointestinal problems mainly due to fibrosis affect 50 to 90% of patients.1

Q: Is there a cure for scleroderma?

A: There is no cure for scleroderma. Treatment is aimed at improving symptoms.

  • Heartburn (acid reflux) can be treated with antacid drugs.
  • Scleroderma kidney disease can be treated with blood pressure medications called “angiotensin converting enzyme inhibitors” (ACE inhibitors). These can often effectively control kidney damage if started early and use of these drugs has been a major advance for treating scleroderma.
  • Muscle pain and weakness can be treated with anti-inflammatory drugs such as prednisone, intravenous immunoglobin (IVIg), and/or immunosuppressive medications. Physical therapy may be useful to maintain joint and skin flexibility.2

 

What Is Scleroderma In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii36-9. doi: 10.1093/rheumatology/ ken485. [↩]
  2. http://www.rheumatology.org [↩]

Prurigo Nodularis (Hyde’s Prurigo)

Hyde's Prurigo. Courtesy quizlet.com
Hyde’s Prurigo. Courtesy quizlet.com

What Is Prurigo Nodularis (Hyde’s Prurigo)?

[dropcap]P[/dropcap]rurigo nodularis is a chronic dermatitis characterized by hard, dry, deep seated, intensely itchy papules (small bumps like pimples) and/or nodules (large bumps) that erupt most commonly on the arms, legs, and back.

Papules and nodules vary in number and may become infected after picking or scratching.

Q: Does the itching go away?

A: New nodules develop from time to time, and existing nodules may remain itchy indefinitely, although some may regress spontaneously and leave scars. In most cases, the disease runs a very protracted course with exacerbations and remissions.1

Prurigo nodularis is an unusual disorder of unknown etiology, which is notoriously resistant to therapy. A variety of systemic conditions have been reported to be associated with prurigo nodularis. However, the mechanism by which these disorders may trigger prurigo nodularis is unknown.2

It has been shown to be associated with malnutriton and infection such as tonsillitis, which resolved after removal of tonsils.3

What Is Prurigo Nodularis In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Katotomichelakis M, Balatsouras DG, Bassioukas K, Kontogiannis N, Simopoulos K, Danielides V. Recurrent prurigo nodularis related to infected tonsils: a case report. J Med Case Rep. 2008 Jul 24;2:243. doi: 10.1186/1752-1947-2-243. [↩]
  2. Lee MR, Shumack S. Prurigo nodularis: a review. Australas J Dermatol. 2005 Nov;46(4):211-18; quiz 219-20. [↩]
  3. Katotomichelakis M, Balatsouras DG, Bassioukas K, Kontogiannis N, Simopoulos K, Danielides V. Recurrent prurigo nodularis related to infected tonsils: a case report. J Med Case Rep. 2008 Jul 24;2:243. doi: 10.1186/1752-1947-2-243. [↩]

Chronic Bullous Dermatosis of Childhood or Linear IgA Dermatosis

chronic bullous dermatosis of childhoodWhat Is Chronic Bullous Dermatosis Of Childhood?

[dropcap]C[/dropcap]hronic 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?

Sources:
  1. 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. [↩]

Edema

Pitting edema. Right photo shows that indent remains from pressing. Courtesy wikimedia.
Pitting edema. Right photo shows that indent remains from pressing. Courtesy wikimedia.

What Is Edema?

[dropcap]E[/dropcap]dema is an abnormal swollen condition of the skin characterized by excess extracellular fluid volume, meaning there is an increase of the fluid that normally surrounds cells. Edema may be hardly noticeable or it can become extensive.

Edema can have various appearances and can develop from various causes.

Q: What are the appearances and causes of edema?

A: Here are the appearances of edema:

  • Pitting edema. It is called pitting edema if when the skin is pressed with a finger, the indent remains. This edema results from fluid leaking out of the bloodstream into the surrounding tissues. Pitting edema can be a feature of many disorders including heart disease, kidney disease, vascular disease, cancer, and malnutrition.
  • Myxedema is a firm swelling or thickening of subcutaneous skin that does not pit, as seen in thyroid disease.

Here are causes of edema:

  • Local edema as a response to trauma or infection.
  • Lower extremity edema from poor circulation and malnutrition.
  • Edema in the lungs from right sided heart disease.
  • Abdominal edema from liver disease, intestinal disease.
  • Body wide edema from heart disease, kidney disease, thyroid disease.

What Is Edema In Celiac Disease and/or Gluten Sensitivity?