This document discusses atopic dermatitis (AD), also known as eczema. It describes the characteristic features of AD including pruritis, eczematous lesions, and association with other atopic conditions. It outlines the three stages of AD - infantile, childhood, and adult. It discusses immunological factors, common triggers, differential diagnoses, management strategies, and regional variants. AD results from a T helper 2 cell dominant immune response and is characterized by IgE elevation and eosinophilia. Proper management focuses on protection from scratching, cleansing, moisturizing, and topical corticosteroids.

1. Atopic Dermatitis, Eczema,
and Noninfectious
Immunodeficiency Disorders

2. Atopic Dermatitis
 “atopic eczema”
 “infantile eczema”
 “flexural eczema”
 “disseminated neurodermatitis”
 “prurigo diathsique”

3. Atopic Dermatitis
 Pruritis is the hallmark of AD
 “Itch that rashes” -itching commonly
precedes the appearance of lesions
 Eczematous eruption often leads to
lichenified dermatitis

4. Atopic Dermatitis
 Commonly associated with xerosis and susceptibility to
irritants and proteins, as well as an atopic diathesis
(tendency towards asthma, allergic rhinitis, IgE mediated
systemic manisfestations)
 IgE bound to Langerhans cells in atopic skin
 Food exacerbates symptoms in some patients: soy,
eggs, peanuts, cow’s milk represent up to 75% of
positive tests for food allergies- most commonly seen in
infants & kids w/ mod-severe AD (up to 40% of pts.)
 Most frequent allergens leading to respiratory allergies
are dust mites, pollen, animal fur, & molds- (seen in up
to 70% of pts and associated w/ adult AD)

5. Atopic Dermatitis
 Triggering Factors
 Temperature change
 Sweating
 Excessive washing
 Contact with irritating
substances (wool, soaps,
detergents, cigarette
smoke)
 Contact allergy
 Aeroallergens
 Microbial agents
 Food
 Stress

8. AD – 3 Stages
 Infantile
2 months to 2 years
 Childhood
2 years to 10 years
 Adult
adolescence to adulthood

9. Infantile Atopic Dermatitis
 60% of 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 desquamate leading to erythroderma
 Buttocks and diaper area frequently
spared

10. Infantile Atopic Dermatitis
 Partial or even
complete remission in
summer and relapse
in winter are the rule
 Worsening observed
after immunizations
and viral infections

11. Infantile Atopic Dermatitis
 In most cases the symptoms will
disappear toward the end of the second
year.
 Egg, peanut, milk, wheat, fish, soy, and
chicken may exacerbate infantile AD

12. Involvement of the cheeks is characteristic of the infantile pattern of AD.

13. 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.

15. 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 sometime in their
lives
 Common in women after birth of their 1st child, when
increased exposure to soaps and water trigger
disease

18. Cutaneous stigmata
 Dennie-Morgan folds
 Pityriasis alba
 Keratosis pilaris
 Hertoghe’s sign – thinning of the lateral eyebrows
 Keratosis punctata palmaris et plantaris
 Xerosis
 Icthyosis vulgaris
 Palmarplantar hyperlinearity
 Cheilitis
 LSC
 Prurigo Nodularis

22. AD and Ichthysosis vulgaris
 Up to 50% of AD will
have Ichthysosis vulgaris
 Autosomal dominant
 Excessive scaling most
prominent on shins
 White, translucent,
quadrangular scales on
the extensor aspects of
the arms and legs
common with atopy

23. 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.

24. Infection
 Pts w/ AD more prone to certain cutaneous infx, may
have more widespread infx, & may have exacerbations
of their AD provoked by skin infx
 Staph aureus – 90% of chronic lesions
 Eczema herpeticum – generalized herpes simplex
infection. Young children usually.
 Secondary to reduced cell-mediated immunity
 Vaccination against smallpox is contraindicated in person
with atopic dermatitis. Even when condition is in
remission, widespread and even fatal vaccinia can occur.
 Also more prone to other cutaneous viral diseases such
as flat warts and molluscum contagiosum

25. Eczema herpeticum:
typical vesicular
lesions on the hand,
around the eye, and
on the face

26. 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 may be sensitive to house mites or
grass pollen

28. 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

30. 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

31. Differential Diagnosis
 Seborrheic Dermatitis
 Contact dermatitis
 Nummular eczema
 Scabies
 Psoriasis
 LSC
 Asteatotic eczema
 Dermatophytosis
 HIV or HTLV-1 Associated
dermatoses
 Chronic mucocutaneous
candidiasis
 Impetigo
 Congenital syphilis
 Dermatitis herpetiformis
 GVHD
 Dermatomyositis
 Pemphigus foliaceus
 Lupus erythematosis
 Wiskott- Aldrich
 Hyperimmunoglobulin-E syndrome
 SCID
 DiGeorge Syndrome
 CTCL
 Langerhans Cell histiocytosis
 Netherton’s syndrome
 Ectodermal dysplasias
 Familial KP
 Ataxia telangiectasia
 Hartnuo disease
 PKU
 Zinc deficiency
 Drug eruption
 Photoallergic dermatitis
 Chronic actinic damage

32. Histology
 Spongiotic dermatitis
 Lichen simplex chronicus
 Eosinophils may be seen

34. Management
 Protect from scratching
 Adequate cleansing but not over bathing or
rubbing
 Gentle cleansers- mild, non-alkali soaps
 Anti-histamines, especially at night
 Bathing protocol
 Food allergy identification and dietary
restrictions.
 Hydrate skin daily with moisturizers

35. Management
 Topical steroids
 Wet compress of Burow’s solution such as
Domeboro.
 Crude coal tar/liquor corbonis detergens
(LCD)
 PUVA
 Cyclosporine

36. Management
 “Topical FK506 (Tacrolimus) is
dramatically beneficial in SEVERE atopic
dermatitis”
 95% showed good improvement in Alaiti
and Rusicka study in JAAD 1998, Archives
1999

38. Regional Eczema
 Ear eczema
 Eyelid dermatitis
 Nipple eczema
 Hand eczema
 Diaper dermatitis
 Infectious eczematoid dermatitis
 Juvenile plantar dermatosis

39. Ear Eczema
 Most frequently caused by seborrheic or
atopic dermatitis
 Staph, Strep, or Pseudomonas
 Earlobe is pathognomonic of nickel allergy

41. Eyelid dermatitis
 When on one eye only, it is most
frequently caused by nail polish, and
usually affects the upper eyelid
 When both eyes are involved, consider
mascara, eye shadow, eyelash cement,
eyeliner, etc
 In contrast, atopic dermatitis affects both
upper and lower eyelid.

42. Nipple eczema
 Painful fissuring, seen especially in nursing
mothers
 Maybe an isolated manifestation of atopic
dermatitis
 If persist more than 3 months, and/or
unilateral, biopsy is mandatory to rule out
Paget’s

44. 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

47. Treatment
 Barrier
 Moisturizer
 Systemic Corticosteroids
 Phototherapy – UVA, PUVA, Radiotherapy
(Grenz Ray)
 New research suggests use of oral
retinoids for severe recalcitrant hand
eczema

48. Diaper (Napkin) Dermatitis
 Erythematous, papulovesicular dermatitis
distributed over the lower abdomen,
genitals, thighs, and the convex surfaces
of the buttocks
 Irritation caused by bacteria, change in
the environment (moisture, lower PH,
feces)
 Candida albicans secondary infection.

51. Diaper dermatitis -
complications
 Jacquet’s erosive diaper dermatitis
 Punched out ulcers/erosions with elevated
borders
 Pseudoverrucous papule and nodules
 Granuloma gluteal infantum

52. Jacquet’s diaper dermatitis with eroded nodules on the labia

53. Granuloma gluteale infantum

54. Diaper Dermatitis
 Differential diagnosis: Napkin psoriasis,
seborrheic dermatitis, atopic dermatitis,
langerhans cell histiocytosis, tinea cruris,
allergic contact dermatitis, acrodermatitis
enteropathica, biotin deficiency, congenital
syphillis
 Treatment: prevention

55. 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

56. Juvenile plantar
dermatosis

58. 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.

59. Xerotic eczema

60. Hormone Induced Dermatoses
 Autoimmune progesterone dermatitis – Appear 5-10 days before
menses. Oophorectomy, danazol, and tamoxifen are treatment
modalities
- Urticaria
- Angioedema
- Eczema
- Erythema multiforme
- Stomatitis
- Folliculitis
- Papulopustular/papulovesicular lesions
- Stephens-Johnson syndrome
- Vesiculobullous reactions
- Dermatitis herpetiformis-like rash
- Mucosal lesions
 Autoimmune estrogen dermatitis – a cyclic skin disorder with
variable morphologies. Exacerbate premenstrually or occur only
immediately before the menses. Treatment with tamoxifen maybe
effective.

61. 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)

62. 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

63. 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

64. X-Linked Agammaglobulinemia
 Aka Bruton’s syndrome, sex-linked agammaglobulinemia.
 Patients develop recurrent bacterial infections in the first year of
life. The skin is the most common site of infection, usually manifest
as furuncles and cellulitis, and occasionally ecthyma gangrenosum.
 Staphylococcus, Streptococcus, Haemophilus and Pneumococcus are
the most common organisms.
 Increased susceptibility to hepatitis B and enteroviral infections.
 Increased risk of eczema.
 Associated papular dermatitis may result from extensive
lymphohistiocytic infiltration of the skin.
 Non-infectious cutaneous granulomas have been described.
 Small percentage of patients develop a dermatomyositis-like
disorder with slowly progressive neurologic involvement, usually
related to echoviral meningoencephalitis.
 May develop leukemia, lymphoma, fatal encephalitis, pulmonary fibrosis

65. X-Linked Agammaglobulinemia
 Defect lies in the maturation block in pre-B-cell
to B-cell differentiation
 IgA, IgM, IgD, and IgE are absent in the serum.
IgG present in small amount
 Cell-mediated immunity intact. T lymphocytes
are normal, B cells are completely lacking
 Protein tyrosine kinase (PTK) gene deletion and
point mutation
 Tx: gamma globulin

66. Selective IgA Deficiency
 Most common immunoglobulin deficiency
 Usually asymptomatic
 Clinical manifestations 10-15%
 Sinopulmonary bacterial infections
 Giardia gastroenteritis
 1/3 with clinical disease develop autoimmune disorders
 SLE, Vitiligo, chronic mucocutaneous candidiasis, lipodystrophia
centrifugalis abdominalis, ITP
 No sexual predilection
 A number of patients have severe atopic-like dermatitis, asthma,
cow's milk allergy, and/or allergic rhinoconjunctivitis.
 An extremely rare selective immunoglobulin deficiency, IgM
deficiency, is apparently caused by an inability of T-helper cell
function to stimulate IgM production. In addition to recurrent
bacterial infections, severe eczema and extensive large warts have
been described.

67. Hyperimmunoglobulinemia M
syndrome

68. 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.

69. Common Variable
Immunodificiency
 Aka acquired hypogammaglobulinemia
 HLA marker B8 and DR3 are affected
 B cells present but not terminally differentiated
 T cell dysfunction evident
 Recurrent sinopulmonary infections- patients are
especially predisposed to pyogenic upper and
lower respiratory tract infections

70. Common Variable
Immunodeficiency
 Increased risk of autoimmune disorders
 Vitiligo, alopecia areata, vasculitis
 8- to 13-fold increased risk of cancer overall
 Increased incidence of lymphoma
 400 fold increase risk in female patients
 Giardia infections are more common in CVID than in the X-linked
form. Patients frequently have cutaneous pyodermas and eczema.
 Abnormalities of cell-mediated immunity may occur in addition to
the Ig deficiency and may manifest in the skin as widespread warts
and extensive dermatophyte infections.
 Pts may develop non-caseating granulomas of the lungs, liver,
spleen, and/or skin that are not due to microorganisms.
 Death in patients with CVID usually results from infection,
respiratory insufficiency, or neoplasia.

72. Isolated Primary IgM Deficiency
 Eczematous dermatitis presents in 1/5 of
patients with this condition
 Predisposition to bacterial infection
 Defect in maturation of IgM producing
plasma cell.

73. Immunodificiency with
Hyper-IgM
 Low or absent IgG, IgE, and IgA level. Normal
or elevated IgM and IgD
 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.
 Tx: IVGG, and allogenic bone marrow transplant

74. Thymic Hypoplasia
 DiGeorge anomaly, aka III and IV pharyngeal pouch
syndrome
 Facies: notched and low-set ears, micrognathia, shorten
philtrum, hypertelorism
 Congenital absence of the parathyroid, thymus, and
abnormal aorta
 Hypocalcemia is the first sign
 Aortic and cardiac defects are the most common cause
of death
 Deletions within proximal long arm of chromosone 22

75. Noninfectious, persistant cutaneous granulomas in a patient with
DiGeorge Syndrome. The granulomas are indistinguishable
clinically from cutaneous granulomas associated with other
immunodeficiencies.

76. Thymic Dysplasia with Normal
Immunoglobulins
(Nezelof Syndrome)
 Faulty development of thymus gland
 Autosomal recessive
 Thymus is present but underdeveloped;
no cardiac abnormalities
 Contrast to DiGeorge syndrome

77. Purine Nucleoside
Phosphorylase Deficiency
 Greatly reduced T-Cell counts, depressed
cell mediated immunity
 B cells and antibody formation intact
 Mutation on 14q13
 Usually die of overwhelming viral infection

78. Miscellaneous T-Cell Deficiencies
 Cartilage-hair hypoplasia syndrome
 AR, patient with short-limbed dwarfism, fine sparse,
hypopigmented hair, defective cell mediated
immunity. Some may also have deecreased humoral
immunity.
 Most common in Amish and Finns
 May have “doughy” skin secondary to degenerated
elastic tissue
 Increased risk of non-Hodgkin’s lymphoma and basal
cell carcinomas
 Patients are highly susceptible to severe disseminated
varicella

79. Miscellaneous T-Cell Deficiencies
 Omenn’s syndrome
 AR
 Mimics GVHD
 exfoliative erythroderma, eosinophilia,
recurrent infection, hypogammaglobulinema,
diarrhea, hepatosplenomegly, alopecia
 Early death by 6 months
 Inefficient and abnormal generation of T-Cell
receptors.

80. SCID: Severe Combined
Immunodeficiency Disease
 Severe impairment of humoral and cellular immunity
 75% of pts are males
 Recurrent infections, diarrhea, and failure to thrive are apparent by
3-6 months of age
 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
 Infants can present with diffuse skin involvement: seborrheic-like
dermatitis or morbilliform eruptions.
 Common early infections are mucocutaneous candidiasis, virus-
induced chronic diarrhea with malabsorption, and pneumonia due to
bacteria, viruses, or Pneumocystis carinii.
 Cutaneous infections are most often caused by C. albicans, S.
aureus, and S. pyogenes.

81. Ataxia-Telangiectasia
(Louis-Bar Syndrome)
 Distinctive telangiectasia in bulbar conjuctiva
and flexural suraces of the arm developing
during the 5th year of age
 Telangiectasia occurs on butterfly area 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.

83. Ataxia-Telangiectasia
 Progeric changes seen in 90%
 Subcutaneous fat is lost
 Facial skin becomes atrophic and sclerotic early on
 Poikiloderma
 Sinopulmonary infections in 80%
 Defects in cell mediated immunity
 Most common cause of death is bronchiectasis with
respiratory failure

84.  Persistent granulomatous
plaques on the leg of child
with ataxia–telangiectasia.

85. Wiskott-Aldrich Syndrome
 Exclusively in boys
 Triad: chronic eczematous dermatitis resemble AD,
increase suseptibility to infections (OM), and
thrombocytopenic purpura/hepatosplenomegly
 Thrombocytopenia, petechiae and hemorrhagic episodes
 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
 Bone marrow transplant is tx of choice

87. Petechiae and ecchymoses in a young boy with WAS

88. X-Linked Lymphoproliferative
Syndrome
 Aka Duncan’s disease
 Inability to control Epstein-Barr virus infection.
 Pt normal until develop infectious Mono.
 Necrotic hepatitis and exanthem are common
 Xq26 abnormailty
 B-cell lymphoproliferative disease with acquired
hypoglobulinemia.

89. 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
 Patients develop granulomas as a compensatory
effort to confine organisms

90. Chronic Granulomatous Disease
 65% of cases are the X-linked form, lacks
the subunit of cytochrome b 558(gp91-
phox)
 Female carrier has mixed, normal and
abnormal cells thus shows an intermediate
phenotype.

91. Chronic Granulomatous Disease
 Myeloperoxidase producing bacteria
characteristically cause infections because
their destruction requires generation of
oxygen free radicals
 Staph. Aureus
 Serratia

92. Chronic Granulomatous Disease
 Screening test: Nitroblue tetrazolium
(NBT) reduction assay
 NBT is normally yellow
 80-90% of normal leukocytes reduce NBT
during phagocytosis to insoluble precipitate,
turning it blue
 Only 5-10% of leukocytes from patients with
CGD are able to reduce NBT during
phagocytosis

94. Leukocyte Adhesion Molecule
Deficiency
 Autosomal recessive
 Affects the adherence of neutrophils, cytolytic T
lymphocytes, and monocytes
 Faulty complexing of the CD11 and CD18 integrins
 Necrotic ulcerations resembling pyoderma gangrenosum
 Frequent skin infections, mucositis, and otitis
 Poor wound healing
 Delayed separation of the umbilical cord
 Death usually occurs by 5 years of life unless bone
marrow transplant is undertaken.

95. A minor scratch from his sister evolved during the subsequent weeks
into a large ulcer on the arm of a boy with leukocyte adhesion
disorder.

96. Chediak-Higashi Syndrome
 Autosomal recessive
 Abnormal pigmentation with silvery hair
 Photophobia
 Partial oculocutaneous albinism,
cutaneous and intestinal infections early in
childhood
 Ocular albinism is accompanied by
nystagmus and photophobia
 Parental consanguinity common

97. Chediak-Higashi Syndrome
 Defect in the gene LYST, resulting in
defective vesicular transport to and from
the lysosome and melanosome
 Causes the “giant” intracytoplasmic granules
found within leukocytes, melanocytes, hair
shafts, renal tubular cells, CNS neurons, and
other tissues

99. Hyperimmunoglobulinemia E
Syndrome
 Autosomal dominant with variable expressivity
 Consists of atopic-like eczematous dermatitis,
recurrent pyogenic infection, high level of IgE,
elevated IgD, IgE anti-staphlococcal 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

101. Job’s syndrome
 AKA Buckley Syndrome
 Subset of HIE.
 Mainly affect girls with red hair, freckles,
and blue eyes.
 Hyperextensible joints
 Cold abscesses occur.

103. Graft-Versus-Host Disease
 Immunocompetent cells are introduced 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.

104. Early, chronic graft-versus-host reaction with widespread, almost
confluent hyperpigmented lichenoid papules and toxic epidermal
necrosis-like appearance on knee

105. Late, chronic graft-versus -host reaction
with hyperpigmented sclerotic plaques
on the back

106. Acute graft-versus-host reaction with vivid palmar erythema

107. Graft-versus-host reaction with early, chronic, diffuse,
widespread lichenoid changes of lips

108. Acute erosions of the buccal mucosa in graft-versus-
host reaction

109. Graft-versus-host reaction; acute basal cell hydropic
degeneration with interepidermal necrotic keratinocytes

110. Graft-versus-host reaction; early chronic hyperkeratosis
and hypergranulosis, irregular acanthosis, cytoid body
and basal cell hydropic degeneration reminiscent of
lichen planus

112. THE END