Atopic Dermatitis, Eczema, and Noninfectious Immunodeficiency Disorders
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Atopic Dermatitis, Eczema, and Noninfectious Immunodeficiency Disorders

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Atopic Dermatitis. Aka atopic eczemaAka infantile eczemaAka flexural eczemaAka disseminated neurodermatitisAka prurigo diathsique . Atopic Dermatitis. High level of IgE antibodies to House dust mitesIgE bound to Langerhans cells in atopic skinFood exacerbates symptoms in some patients: eggs, peanuts, cow\'s milk represent up to 75% of positive test..
Atopic Dermatitis, Eczema, and Noninfectious Immunodeficienc...

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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, cow?s 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 Pityriasis alba Keratosis pilaris Hertoghe?s sign ? thinning of the lateral eyebrows Keratosis punctata palmaris et plantaris

14. Note the Dennie?Morgan lines and central facial pallor. Note the Dennie?Morgan 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 Contact dermatitis Nummular eczema Scabies Psoriasis

27. Histology Spongiotic dermatitis Lichen simplex chronicus Eosinophiles may be seen

29. Management 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

30. Management Topical steroid Wet compress of Burow?s 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 Eyelid dermatitis Nipple eczema Hand eczema Diaper dermatitis 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 Paget?s

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 Moisturizer Systemic Corticosteroids Phototherapy ? UVA, PUVA, Radiotherapy (Grenz Ray)

42. Diaper dermatitis 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.

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 Hypoplasia 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-Bar?s S.) Wiskott-Aldrich Syndrome

53. Immunodeficiency Syndromes 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

54. X-Linked Agammaglobulinemia 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

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 Autosomal recessive 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. 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.

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 Good?s syndrome Deficient in cell mediated immunity and benign thymoma occurring simultaneously Thymectomy does not affect the immunodeficiency

71. 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. 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 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.

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 Ch?diak?Higashi syndrome. Light microscopic examination of hair shows giant melanosomes in patients with Ch?diak?Higashi 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. Job?s 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?


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