Eczema

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