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1. Atopic Dermatitis, Eczema, and Noninfectious Immunodeficiency Disorders Rick Lin, DO MPH
July 15, 2003
2. Atopic Dermatitis Aka atopic eczema
Aka infantile eczema
Aka flexural eczema
Aka disseminated neurodermatitis
Aka prurigo diathsique
3. Atopic Dermatitis 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, cows milk represent up to 75% of positive test.
4. Atopic Dermatitis Pruritis is the hallmark of AD
Eczematous eruption leads to lichenified dermatitis
Itching precedes the appearance of lesions
5. 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.
6. 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
8. 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.
10. 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
12. Adult with atopic dermatitis that favors the face and neckAdult with atopic dermatitis that favors the face and neck
13. Cutaneous stigmata Dennie-Morgan fold
Hertoghes sign thinning of the lateral eyebrows
Keratosis punctata palmaris et plantaris
14. Note the DennieMorgan lines and central facial pallor. Note the DennieMorgan lines and central facial pallor.
17. Vascular Stigmata 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.
18. Infection 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.
19. Immunology 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
21. Immunology 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
23. Immunology 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
25. Differential Diagnosis Seb Derm
27. Histology Spongiotic dermatitis
Lichen simplex chronicus
Eosinophiles may be seen
29. Management Protect from scratching
Adequate cleansing but not over bathing or rubbing
Anti-histamines, especially at night
Food allergies concerns and dietary restrictions.
Hydrate skin daily with moisturizers
30. Management Topical steroid
Wet compress of Burows solution such as Domeboro.
Crude coal tar/liquor corbonis detergens (LCD)
31. Management Topical FK506 (Tacrolimus) is dramatically beneficial in SEVERE atopic dermatitis
95% showed good improvement in Alaiti and Rusicka study in JAAD 1998, Archive 1999
32. Regional Eczema Ear eczema
Infectious eczematoid dermatitis
Juvenile plantar dermatosis
33. Ear Eczema Most frequently caused by seborrheic or atopic dermatitis
Staph, Strep, or Psoeudomonas
Earlobe is pathognomonic of nickel allergy
35. Eyelid dermatitis 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
36. Nipple eczema 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 Pagets
39. Hand eczema 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
41. Treatment Barrier
Phototherapy UVA, PUVA, Radiotherapy (Grenz Ray)
42. Diaper dermatitis Jacquets 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.
44. Infectious eczematoid dermatitis Vesicular, pustular, or cursted
Ulceration and superficial infection may be present
Treatment involve the removal of irritant and antibiotic treatment.
46. 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
47. Xerotic Eczema 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.
49. Nutritional Deficiency Eczema Localized, thickened pattern with scaling patches.
Exacerbated by nutritional deficiency
50. 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.
51. Immunodeficiency Syndromes X-Linked Agammaglobulinemia
Isolated IgA Deficiency
Common Variable Immunodificiency
Isolated Primary IgM Deficiency
Immunodificiency with Hyper-IgM
Thymic Dysplasia with Normal Immunoglobulins (Nezelof Syndrome)
52. Immunodeficiency Syndromes Purine Nucleoside Phosphorylase Deficiency
Miscellaneous T-Cell Deficiencies
Severe Combined Immunodeficiency Disease (SCID)
Thymoma with Immunodeficiency
Ataxia-Telangiectasia (Louis-Bars S.)
53. Immunodeficiency Syndromes X-Linked Lymphoproliferative Syndrome
Chronic Granulomatous Disease
Leukocyte Adhesion Molecule Deficiency
Hyperimmunoglobulinemia E Syndrome
54. X-Linked Agammaglobulinemia Aka Brutons 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
55. X-Linked Agammaglobulinemia 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
56. 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.
57. 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
58. Leukocytoclastic vasculitis on the legs of a girl with common variable immunodeficiency . Autoimmune disorders show a significant increase in frequency. Leukocytoclastic vasculitis on the legs of a girl with common variable immunodeficiency . Autoimmune disorders show a significant increase in frequency.
59. 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.
60. 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.
61. Extensive, recalcitrant warts in an adult with hyperimmunoglobulinemia M syndrome. Extensive, recalcitrant warts in an adult with hyperimmunoglobulinemia M syndrome.
62. Painful oral ulceration in a patient with hyperimmunoglobulinemia M syndrome Painful oral ulceration in a patient with hyperimmunoglobulinemia M syndrome
63. Thymic Hypoplasia 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
64. Thymic Dysplasia with Normal Immunoglobulins (Nezelof Syndrome) Faulty development of thymus gland
Thymus is present but under developed, no cardiac abnormalities.
Contrast to DiGeorge syndrome.
65. 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
66. Miscellaneous T-Cell Deficiencies Cartilage-hair hypoplasia syndrome, AR, patient with short-limbed dwarfism, fine sparse, hypopigmented hair, defective cell mediated immunity.
Omenns 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.
69. 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
70. Thymoma with Immunodeficiency Goods syndrome
Deficient in cell mediated immunity and benign thymoma occurring simultaneously
Thymectomy does not affect the immunodeficiency
71. Ataxia-Telangiectasia (Louis-Bars 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. Caf 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.
74. Wiskott-Aldrich Syndrome 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
75. The boy was successfully treated with bone marrow transplantation, and his dermatitis virtually cleared owing to the T-cell engraftment The boy was successfully treated with bone marrow transplantation, and his dermatitis virtually cleared owing to the T-cell engraftment
76. X-Linked Lymphoproliferative Syndrome Aka Duncans disease
Inability to control Epstein-Barr virus infection.
Pt normal until develop infectious Mono.
B-cell lymphoproliferative disease with acquired hypoglobulinemia.
77. 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
78. 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.
79. Swollen, erythematous draining inguinal lymphadenopathy related to Staphylococcus aureus infection in a boy with chronic granulomatous disorder Swollen, erythematous draining inguinal lymphadenopathy related to Staphylococcus aureus infection in a boy with chronic granulomatous disorder
80. 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.
81. Chediak-Higashi Syndrome 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
82. Light microscopic examination of hair shows giant melanosomes in patients with ChdiakHigashi syndrome. Light microscopic examination of hair shows giant melanosomes in patients with ChdiakHigashi syndrome.
83. 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
84. Jobs syndrome Subset of HIE.
Mainly affect girls with red hair, freckles, blue eyes, and hyperextensible joints. Cold abscesses occur.
85. Infant with hyperimmunoglobulin E syndrome shows several erythematous, slightly purulent cold abscesses on the forehead and scalp. Infant with hyperimmunoglobulin E syndrome shows several erythematous, slightly purulent cold abscesses on the forehead and scalp.
86. Coarse faces with scattered small staphylococcal pustules on a 9-year-old boy with hyperimmunoglobulin E syndrome. Coarse faces with scattered small staphylococcal pustules on a 9-year-old boy with hyperimmunoglobulin E syndrome.
87. Graft-Versus-Host Disease 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.
95. Screening laboratory tests for a patient with recurrent cutaneous infections (including staphylococcal) where there is suspicion of a primary immunodeficiency Screening laboratory tests for a patient with recurrent cutaneous infections (including staphylococcal) where there is suspicion of a primary immunodeficiency
96. End of Lecture