Atopic Dermatitis, Eczema, and Noninfectious Immunodeficiency Disorders
Rick Lin, DO MPH
July 15, 2003
- Aka atopic eczema
- Aka infantile eczema
- Aka flexural eczema
- Aka disseminated neurodermatitis
- Aka prurigo diathsique
- High level of IgE antibodies to House dust mites
- IgE bound to Langerhans cells in atopic skin
- Food exacerbates symptoms in some patients: eggs, peanuts, cow’s milk represent up to 75% of positive test.
- Pruritis is the hallmark of AD
- Eczematous eruption leads to lichenified dermatitis
- Itching precedes the appearance of lesions
Infantile Atopic Dermatitis
- 60% of case AD present in the first year of life, after 2 months of age
- Begin as itchy erythema of the cheeks
- Distribution include scalp, neck, forehead, wrist, and extensors
- May become desquamate leading to erythroderma.
Infantile Atopic Dermatitis
- Most cases the symptoms will disappear toward the end of the second year.
- The role of food allergy in infantile and childhood atopic dermatitis has been clarified
- Egg, peanut, milk, wheat, fish, soy, and chicken may exacerbate infantile AD
Involvement of the cheeks is characteristic of the infantile pattern of AD.
Childhood Atopic Dermatitis
- Characterized by less acute lesions
- Distribution: antecubital and popliteal fossae, flexor wrist, eyelids, and face.
- Severe atopic dermatitis involving more than 50% of body surface area is associated with growth retardation.
Adult Atopic Dermatitis
- Distribution: antecubital and popliteal fossae, the front side of the neck, the forehead, and area around the eyes.
- Atopic individuals are at greater risk of developing hand dermatitis than are the rest of the population
- 70% develop hand dermatitis some times in their lives
- Dennie-Morgan fold
- Pityriasis alba
- Keratosis pilaris
- Hertoghe’s sign – thinning of the lateral eyebrows
- Keratosis punctata palmaris et plantaris
- Headlight sign – perinasal and periorbital pallor
- White dermographism – blanching of the skin at the site of stroking with a blunt instrument – cause edema and obscure color of underlying vessels.
- Staph aureus – 90% of chronic lesions
- Eczema herpeticum – generalized herpes simplex infection. Young children usually.
- Vaccination against smallpox is contraindicated in person with atopic dermatitis. Even when condition is in remission, widespread and even fatal vaccinia can occur.
- T helper cell type 2 (Th2) dominance
- Th2 produces IL-4, 5, and 10
- IL-4 and IL-5 produce elevated IgE and eosinophilia
- IL-10 inhibits delayed type hypersensitivity
- Th2 maybe sensitive to house mites or grass pollen
- Monocytes produces elevated amount of prostaglandin E2 (PGE2)
- PGE2 reduces gamma-interferon production, but not IL-4 from helper cells thereby enhancing the Th2 dominance
- PGE2 also directly enhances IgE production from B cells
- Langerhans cells of AD patient stimulate helper T cells into Th2 phenotype without the presence of antigen
- Langerhans cells have IgE bound to their suface receptors. These IgE are associated with atopic antigens, such as house dust mites
- Seb Derm
- Contact dermatitis
- Nummular eczema
- Spongiotic dermatitis
- Lichen simplex chronicus
- Eosinophiles may be seen
- Protect from scratching
- Adequate cleansing but not over bathing or rubbing
- Gentle cleanser
- Anti-histamines, especially at night
- Bathing protocol
- Food allergies concerns and dietary restrictions.
- Hydrate skin daily with moisturizers
- Topical steroid
- Wet compress of Burow’s solution such as Domeboro.
- Crude coal tar/liquor corbonis detergens (LCD)
- “Topical FK506 (Tacrolimus) is dramatically beneficial in SEVERE atopic dermatitis”
- 95% showed good improvement in Alaiti and Rusicka study in JAAD 1998, Archive 1999
- Ear eczema
- Eyelid dermatitis
- Nipple eczema
- Hand eczema
- Diaper dermatitis
- Infectious eczematoid dermatitis
- Juvenile plantar dermatosis
- Most frequently caused by seborrheic or atopic dermatitis
- Staph, Strep, or Psoeudomonas
- Earlobe is pathognomonic of nickel allergy
- When on one eye only, it is most frequently caused by nail polish
- When both eyelids are involved, consider mascara, eye shadow, eyelash cement, eyeline, etc
- Painful fissuring, seen especially in nursing mothers
- Maybe an isolated manifestation of atopic dermatitis
- If persist more than 3 month, and/or unilateral, biopsy is mandatory to rule out Paget’s
- Spongiosis histologically
- Irritant hand dermatitis- seen in homemakers, nurses. Resulting from excessive exposure to soaps
- Pompholyx- tapioca vesicles, on sides of fingers, palms, and soles
- Differentials – Bullous Tinea, id, allergic contact dermatitis
- Systemic Corticosteroids
- Phototherapy – UVA, PUVA, Radiotherapy (Grenz Ray)
- Jacquet’s erosive diaper dermatitis
- Pseudoverrucous papule and nodules
- Graduloma gluteal infantum
- Irritation caused by bacteria, change in the environment (wet, lower PH, feces)
- Candida albicans are secondary infection.
Infectious eczematoid dermatitis
- Vesicular, pustular, or cursted
- Ulceration and superficial infection may be present
- Treatment involve the removal of irritant and antibiotic treatment.
Juvenile plantar dermatosis
- Begins as a patchy symmetrical, smooth, red, glazed macules on the base of the great toes
- Affect age 3 to puberty.
- Symmetrical lesions on weight bearing area
- “toxic sock syndrome” – caused by repeated maceration of the feet by occlusive shoes and nonabsorbent synthetic socks
- Virtually always resolve after puberty
- Aka winter itch, nummular eczema, eczema craquele, and asteototic eczema.
- Anterior shins, extensor arms, and flank
- Elderly person predisposed.
- Use of bath oils in bath water is recommended to prevent water loss
- Moisturizers – urea or lactic acid.
Nutritional Deficiency Eczema
- Localized, thickened pattern with scaling patches.
- Exacerbated by nutritional deficiency
Hormone Induced Dermatoses
- Autoimmune progesterone dermatitis – urticaria, urticarial paplues, papulovesicular lesion, or eythema multiforme. Appear 5-10 days before menses
- Autoimmune estrogen dermatitis – a cyclic skin disorder with variable morphologies. Exacerbate premenstrually or occur only immediately before the menses. Treatment with tamoxifen maybe effective.
- X-Linked Agammaglobulinemia
- Isolated IgA Deficiency
- Common Variable Immunodificiency
- Isolated Primary IgM Deficiency
- Immunodificiency with Hyper-IgM
- Thymic Hypoplasia
- Thymic Dysplasia with Normal Immunoglobulins (Nezelof Syndrome)
- Purine Nucleoside Phosphorylase Deficiency
- Miscellaneous T-Cell Deficiencies
- Severe Combined Immunodeficiency Disease (SCID)
- Thymoma with Immunodeficiency
- Ataxia-Telangiectasia (Louis-Bar’s S.)
- Wiskott-Aldrich Syndrome
- X-Linked Lymphoproliferative Syndrome
- Chronic Granulomatous Disease
- Myeloperoxidase Deficiency
- Leukocyte Adhesion Molecule Deficiency
- Chediak-Higashi Syndrome
- Hyperimmunoglobulinemia E Syndrome
- Complement Deficiency
- Graft-Versus-Host Disease
- Aka Bruton’s syndrome, sex-linked agammaglobulinemia.
- Appear after 3-6 month of life
- Frequent Strep and staph infection. Viral resistance intact.
- IgA, IgM, IgD, and IgE are absent in the serum. IgG present with small amount
- Cell-mediated immunity intact. T lymphocytes are normal, B cells are completely lacking
- Defect lies in the maturation block in pre-B-cell to B-cell differentiation
- Protein tyrosine kinase (PTK) gene deletion and point mutation
- May develop leukemia, fatal encephalitis, resporatory
Isolated IgA Deficiency
- Absence or marked reduction of serum IgA
- 1:600 in white population, most are entirely well.
- Malignancy is increased in adult with IgA deficiency.
Common Variable Immunodificiency
- Aka acquired hypogammaglobulinemia
- HLA marker B8 and DR3 are affected
- Recurrent sinopulmonary infections
- B cells present but not terminally differentiated
- T cells dysfunction evident
Isolated Primary IgM Deficiency
- Eczematous dermatitis presents in 1/5 of patient with this condition
- Predisposition to bacterial infection
- Defect in maturation of IgM producing plasma cell.
Immunodificiency with Hyper-IgM
- Low or absent IgG, IgE, and IgA level. Normal or elevated IgM and IgD
- IVGG, and allogenic bone marrow transplant
- X-linked form caused by mutation or deletion of Xq26.3-27.1 region, which encodes a ligand of CD40, gp39
- Gp39-CD40 interaction signals for Ig isotype switching.
- DiGeorge anomaly, aka III and IV pharyngeal pouch syndrome
- Facies: notched, low-set ears, micrognathia, shorten philtrum, hypertelorism
- Congenital absence of the parathyroid, thymus, and abnormal aorta
- Hpocalcemia is the first sign
- Aorteic and cardiac defects are the cause of death
- Deletions within proximal long arm of chromosone 22
Thymic Dysplasia with Normal Immunoglobulins (Nezelof Syndrome)
- Faulty development of thymus gland
- Autosomal recessive
- Thymus is present but under developed, no cardiac abnormalities.
- Contrast to DiGeorge syndrome.
Purine Nucleoside Phosphorylase Deficiency
- Greatly reduced T-Cell counts, depressed cell mediated immunity
- B cells and antibody formation intact
- Mutation on 14q13
- Usually died of overwhelming viral infection
Miscellaneous T-Cell Deficiencies
- Cartilage-hair hypoplasia syndrome, AR, patient with short-limbed dwarfism, fine sparse, hypopigmented hair, defective cell mediated immunity.
- Omenn’s syndrome, AR, mimic GVHD, exfoliative erythroderma, eosinophilia, recurrent infection, hypogammaglobulinema, diarrhea, hepatosplenomegly, early death by 6 month. Inefficient and abnormal generation of T-Cell receptor.
SCID: Severe Combined Immunodeficiency Disease
- Severe impairment of humoral and cellular immunity
- Triad of Moniliasis of the oropharynx and skin, intractable diarrhea, and pneumonia.
- Overwhelming viral infection is the cause of death.
- Deficiency or total absence of circulating lymphocytes
Thymoma with Immunodeficiency
- Good’s syndrome
- Deficient in cell mediated immunity and benign thymoma occurring simultaneously
- Thymectomy does not affect the immunodeficiency
Ataxia-Telangiectasia (Louis-Bar’s S.)
- Distinctive telangiectasia in bulbar conjuctiva and flexural suraces of the arm developing during the 5th year of age
- Telangiectasia occurs on butterfly are of the face, palate, ear, and exposed skin. Café au lait patches, and Graying hair also present.
- Cerebellar ataxia is the first sign of this syndrome, beginning in the second year of life.
- Choreic and athetoid movement present.
- Persistent granulomatous plaques on the leg of child with ataxia–telangiectasia.
- Triad: chronic eczematous dermatitis resemble AD, increase suseptibility to infections (OM), and thrombocytopenia purpura/hepatoslpenomegly
- Death by age 6
- Accelerated IgA, IgM and IgE synthesis
- T-cell decline in numbers and activity
- Xp11 gene mutation. Codes for WASP protein which reorganize cytoskeleton
X-Linked Lymphoproliferative Syndrome
- Aka Duncan’s disease
- Inability to control Epstein-Barr virus infection.
- Pt normal until develop infectious Mono.
- Xq26 abnormailty
- B-cell lymphoproliferative disease with acquired hypoglobulinemia.
Chronic Granulomatous Disease
- Recurring purulent and granulomatous infections involving long bones, lymphatic tissue, liver, skin, and lung.
- Deficient in one of the component of NADPH-oxidase complex, which generates superoxide.
- Leads to inability to destroy bacteria per radical mechanism
Chronic Granulomatous Disease
- 65% of cases are the X-linked form, lacks the subunit of cytochrom b 558(gp91-phox)
- Female carrier has mixed and normal and abnormal cells thus shows an intermediate phenotype.
Leukocyte Adhesion Molecule Deficiency
- Autosomal recessive
- Recurrent bacteria and fungal infections and pus formations as a result of a block of leukocyte migration
- Faulty complexing of the CD11 and CD18 integrins
- Death occurs in first 4 years of life unless bone marrow transplant is undertaken.
- Progressively degenerative, fatal, familial disease of young children
- Partial oculocutaneous albinism, cutaneous and intestinal infections early in childhood
- Ocular albinism is accompanied by nystagmus and photophobia.
- Defect in the gene LYST, resulting in defective vascular transport to and from the lysosome
Hyperimmunoglobulinemia E Syndrome
- Consists of atopic-like eczematous dermatitis, recurrent pyogenic infection, high lever of IgE, elevated IgD, IgE antistaph antibodies, and eosinophilia.
- Face is consistently involved. Begin early in life (2 month to 2 years)
- Lesions resemble prurigo
- Keratoderma of the palms and soles
- Subset of HIE.
- Mainly affect girls with red hair, freckles, blue eyes, and hyperextensible joints. Cold abscesses occur.
- Immunocompetent cells are intor duced as graft or blood transfusion to host who is unable to reject the graft cell.
- Most commonly after bone marrow transplant.
- Begins between 4-5th weeks after transplant.
- Result in exfoliative erythroderma.
Early, chronic graft-versus-host reaction with widespread, almost confluent hyperpigmented lichenoid papules and toxic epidermal necrosis-like appearance on knee
Late, chronic graft-versus -host reaction with hyperpigmented sclerotic plaques on the back
Acute graft-versus-host reaction with vivid palmar erythema
Graft-versus-host reaction with early, chronic, diffuse, widespread lichenoid changes of lips
Acute erosions of the buccal mucosa in graft-versus-host reaction
Graft-versus-host reaction; acute basal cell hydropic degeneration with interepidermal necrotic keratinocytes
Graft-versus-host reaction; early chronic hyperkeratosis and hypergranulosis, irregular acanthosis, cytoid body and basal cell hydropic degeneration reminiscent of lichen planus
End of Lecture…