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Dermatitis Herpetiformis In Childhood

muscle wastingWhat Is Muscle Wasting?

M uscle wasting, or atrophy, is a muscle disorder resulting from the loss of muscle tissue which is characterized by thin muscles that have reduced strength and endurance.

What Is Muscle Wasting In Celiac Disease and/or Gluten Sensitivity?

Bleeding: Fecal Occult Blood In Children 

Muscle fiber anatomy. Courtesy NIH.
Muscle Fiber Anatomy. Courtesy NIH.

What Is Muscle Weakness?

M uscle weakness is the impaired status of muscle function characterized by decreased or low muscle strength and inability to perform normal work such as lifting a pot off the stove.

Q: How do muscles work?

A: Muscles do their work by contracting or shortening. For example, to move the foot up and down at the ankle, muscles attached to the foot by tendons must contract to shorten or relax to return to their resting length. Calf muscles contract to point the foot down (flexion) while the shin muscles relax (extension).  For the foot to point up, calf muscles relax while the opposing shin muscles contract.

Each muscle is made up of individual muscle fibers. A muscle fiber is a long cylindrical cell that contains many nuclei, mitochondria, and sarcomeres. Each muscle fiber is surrounded by a thin layer of connective tissue called the endomysium.

Approximately 20–80 of these muscle fibers are grouped together in a parallel arrangement called a muscle fascicle or fiber bundle that is encapsulated by a perimysium. A distinct muscle is formed by enveloping a large number of muscle fascicles in a thick collagenous external sheath extending from the tendons called the epimysium.1

Muscles fall into three types:

  • Voluntary muscles.  These muscles, also called skeletal, we can control by will. Voluntary muscles function by contracting their fibers to draw one part of the body toward another in flexion while opposing muscles that extend or pull a body part away from another. They move our bones to perform activities such as walking to get somewhere, chewing to eat food, lifting to do work, and moving the eyeball to look at something.
  • Involuntary muscles. These muscles work independently of our conscious control. They are needed for internal organs, sphincters, and other parts to do their work, such as peristalsis in the gut that must function at all times to digest and move food, the squirting of bile juice into the duodenum by the Sphincter of Odi in the presence of fat eaten, and action of the pupil to see.
  • Cardiac muscles. These muscles are specialized to keep the heart functioning at all times.

  Muscle weakness can involve all types of muscles.

What Is Muscle Weakness In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. http://www.ncbi.nlm.nih.gov/books/NBK57140/ []

Anemia, Severe Iron Deficiency In Pregnancy 

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?

D 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. []

Penicillin V Impaired Absorption in Children 

osteomalacic myopathy in celiac diseaseWhat Is Osteomalacic Myopathy?

O steomalacic myopathy is a major feature of osteomalacia that is characterized by painful muscle weakness involving the thighs and upper arms and hyperreflexia.

Q: What is hyperreflexia?

A: Hyperreflexia means the action of reflexes is increased. Reflex action is an involuntary, or automatic, response to a stimulus. A reflex action is complicated but essentially depends on  a normal responding muscle which is altered in osteomalacia.

Osteomalacic myopathy is diagnosed on bloodwork that shows deficient levels of vitamin D and elevated levels of parathyroid hormone and alkaline phosphatase, a bone enzyme.

What Is Osteomalacic Myopathy In Celiac Disease and/or Gluten Sensitivity?

Celiac Disease After Childbirth (Puerperium)  

erythema elevatum diutinum of forearmWhat Is Erythema Elevatum Diutinum?

E rythema elevatum diutinum is a rare leucocytoclastic vasculitis thought to be caused by an immune response that is characterized by inflammation, a rash, and scarring of small blood vessels of the skin.

A characteristic poorly defined rash develops symmetically in the skin of extremities where the vessels are inflamed. Nodules and plaques are yellowish red and crust on healing, leaving darkened areas (hyperpigmentation).

Q: What is leucocytoclastic vasculitis?

A: Leucocytoclastic vasculitis is the most common form of vasculitis (inflammation of blood vessels) of the skin and usually results from deposition of immune complexes at the vessel wall. It presents in different forms and in association with different diseases. Diagnosis is made by skin biopsy with immunofluorescence.1

Erythema elevatum diutinum may be caused by inflammatory disease, certain infections such as HIV, immunological disease, malignancy, and drugs.

Corticosteroids are indicated when there are signs of incipient skin necrosis or when the disease is severe. Otherwise, treatment is with Colchicine or Dapsone medications.2

What Is Erythema Elevatum Diutinum In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Sunderkötter C, Bonsmann G, Sindrilaru A, Luger T. Management of leukocytoclastic vasculitis. J Dermatolog Treat. 2005;16(4):193-206. []
  2. Sunderkötter C, Bonsmann G, Sindrilaru A, Luger T. Management of leukocytoclastic vasculitis. J Dermatolog Treat. 2005;16(4):193-206. []

Juvenile Autoimmune Thyroid Disease

Trouseau Testing For Tetany.
Trouseau Testing For Tetany. Courtesy Digplanet.

What Is Tetany?

T etany is a painful feature of low blood calcium or low blood magnesium causing hyperexcitability of all nerves which in turn stimulates involuntary sudden, intermittent and repetitious tonic spasms of muscles. Tonic spasms are steady rather than jerky.

Causes include low function of the parathyroid glands which regulate calcium blood levels, vitamin D deficiency which inhibits calcium absorption from the gut that leads to calcium deficiency, and alkalosis.

In alkalosis, the chemical pH (acid/alkaline measure) of blood is too high, or alkaline, which inhibits adequate free calcium ions from being available for cell use.

Q: Why do low blood levels of calcium or magnesium cause muscles to spasm?

A: Calcium controls the readiness of muscle to respond to nerve stimulation. In muscle action, calcium and magnesium are minerals that oppose each other. Calcium allows a muscle to contract while magnesium allows it to relax. Inadequate levels of these opposing minerals affect the ability of muscles to properly function.

A simple test for tetany is called Trouseau’s named after the doctor who developed it. A blood pressure cuff is placed on an upper arm as usual and pumped up to a pressure sufficient to stop blood flow.  The pressure must be held for 3 minutes. A positive result is shown in the above photo of carpopedal spasm: the wrist and hand flexes inward while the fingers extend (opposite of wrist and hand) and the thumb goes against the palm.

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

Down Syndrome

Crohnie_sores_4[1]What Is Erythema Nodosum?

E rythema nodosum is an inflammatory disorder involving the deep dermis layer of skin and subcutaneous fat septa that underlies the skin. It is characterized by eruptions of recurrent or persistent multiple painful, red nodules under the skin that leave a bruised appearance when healing and do not scar.

The lower legs are most affected, but sores can appear anywhere there is subcutaneous fat.

Q: How do the nodules develop in erythema nodosum?

A: The edges of nodules are poorly defined, and the nodules vary from 2-6 cm.

During the first week of eruption, nodules become tense, hard, and painful. During the second week, they change color from bright red to bluish or livid and may become soft, but do not ulcerate. As absorption progresses, the color gradually fades to a yellowish hue, resembling a bruise. This disappears in 1 or 2 weeks as the overlying skin sloughs off and is replaced.1

The eruptive phase of erythema nodosum begins with flulike symptoms of fever and generalized aching followed by a painful rash within 1-2 days.  Aching legs and swelling ankles may occur and precede the eruption or appear during the eruptive phase and may persist for weeks.2

Currently, the most common cause of erythema nodosum is streptococcal infection in children and streptococcal infection and sarcoidosis in adults.3 Most sores in infection-induced erythema nodosum heal within 7 weeks, but active disease may last up to 18 weeks.

In contrast, 30% of idiopathic erythema nodosum cases may last more than 6 months. Idiopathic means that the cause is not known.

What Is Erythema Nodosum In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. http://emedicine.medscape.com/article/1081633-clinical#a0217 []
  2. http://emedicine.medscape.com/article/1081633-clinical []
  3. http://emedicine.medscape.com/article/1081633-overview#a0199 []

Congenital Anomalies 

Hangnail on lowest finger. Courtesy Wikipedia.org
Hangnail on lowest finger. Courtesy Wikipedia.org

What Is Hangnail?

H angnail is a broken strip of epidermis (piece of skin) at root or lateral (side) edge of fingernail or toenail that causes sharp pain.

A hangnail develops because the skin around the nail is unhealthy due to inadequate nutrition. Injury from trauma including biting the skin and pushing back the cuticles or exposure to excessive detergents and water that remove protective oils promote the development of hangnail.

All ages and both sexes can be affected.

Q: Can a hangnail become infected?

A: Infection, called paronychia, may develop from invasion of sore skin by any of these pathogens: bacteria, fungus, or yeast (Candida). Infected skin is red, swollen, and painful. Topical ointment is required to treat the infection.1

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

Sources:
  1. www.nlm.nih.gov/medlineplus/ency/article/001444.htm []

Cystic Fibrosis 

stages of melanoma
The dark brown coloring represents the progression of melanoma as it begins to grow in skin.

What Is Melanoma?

M elanoma is a cutaneous malignancy with rapid invasion and metastasis to other parts of the body.

Melanoma is characterized by growth from melanocytes appearing as a new mole or enlarging from an exisiting mole, changing shape, size or color.

Melanocytes are cells found in the lower epidermis layer that produce melanin, or pigment, that colors the skin.

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

Spina Bifida 

pityriasis rubraWhat Is Pityriasis Rubra Pilaris?

P ityriasis rubra pilaris (PRP) is a chronic generalized exfoliative dermatitis (sloughing skin) characterized by erythema (redness), scaling, dilated plugged hair follicles, and keratoderma (thickened skin) of the hands and feet that is often associated with anemia and low serum albumin.

It may manifest either as Type I classical adult onset PRP, Type II atypical adult (onset) PRP, or Type VI PRP (HIV-associated PRP pityriasis rubra pilaris) in contrast to classical juvenile (Type III) and circumscribed juvenile (Type IV) encountered among children.1

Q: Who is affected in the general population?

A: All ages are affected. Pityriasis rubra pilaris occurs all over the world but with racial variations – it is 1 in 5,000 in Great Britain and 1 in 50,000 in India.2

What Is Pityriasis Rubra Pilaris In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Sehgal VN, Srivastava G, Dogra S. Adult onset pityriasis rubra pilaris. Indian J Dermatol Venereol Leprol. 2008 Jul-Aug;74(4):311-21. []
  2. Sehgal VN, Srivastava G, Dogra S. Adult onset pityriasis rubra pilaris. Indian J Dermatol Venereol Leprol. 2008 Jul-Aug;74(4):311-21. []