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Transcript
Papulosquamous Disorders: Seb Derm,
Psoriasis, Palmoplantar, Pust Derm, Erythroderma
Chris Weyer D.O.
Dermatology Resident, PGY-1
Northeast Regional Medical Center
Seborrheic Dermatitis

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2-5 % of the population
Chronic, superficial, inflammatory disease caused by
the yeast, Malassezia globosa
Most commonly affects the scalp
Also, affects the eyebrows, ears, eyelids, nasolabial
creases, lips, sternal area, axillae, submammary folds,
umbilicus, groin, and gluteal crease
Characterized by itchy, pink, yellow or erythematous
patches that may be dry, moist, or greasy
Infantile Seborrheic Dermatitis



Presents around 1 week
after birth
AKA “Cradle cap”
Superimposed Candida
may occur
Adult Seborrheic Dermatitis

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Erythematous, greasy
yellow scale
Symmetrical
If extensive, think about
immunosuppression:
HIV, diabetes, and
Parkinson’s disease
Histology of Seb Derm

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Epidermis is acanthotic
Focal scale crust adjacent to
follicular ostia
Slight spongiosis
Adult seb derm has
superficial perivascular &
perifollicular lymphocytic
infiltrate composed mainly of
lymphocytes
Older lesions show irregular
acanthosis & focal
parakeratosis
Histo features are
combination of psoriasis &
spongiotic derm
Treatment-Adolescent & Adult

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Regular use of medicated shampoos -- over-thecounter: sulfide, tar, zinc, pyrithionate
Nizoral shampoo & cream
Loprox shampoo & solution
Elidel cream
Corticosteroids for quick control or flares
Key: leave shampoo on scalp for 3 minutes daily;
apply before going into shower, as patient will not
leave on shampoo for 3 minutes while in shower
Infantile seborrheic dermatitis

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Responds well to topical therapy
Hydrocortisone 1% on the face & skin folds
This may be combined with an antifungal agent
for skin folds
Remove scale after softening with an oil
preparation or a weak keratolytic agent
Reassurance
Psoriasis


A common, chronic, recurrent, inflammatory
disease of the skin characterized by round,
circumscribed, erythematous, dry, scaling
plaques of various sizes, covered by grayish
white or silvery white, imbricated and lamellar
scales
Predilection for the scalp, nails, extensor
surfaces, elbows, knees, umbilical, and sacral
region
Psoriasis nail
changes
onycholysis, nail
pitting, and oil spot
phenomenon
Psoriasis clinical features



Koebner’s phenomenon – the appearance of
typical lesions of psoriasis at sites of injury
Auspitz’s sign – pinpoint bleeding when the
psoriatic scale is forcibly removed, this occurs
because of severe thinning of the epidermis
over the tips of the dermal papilla
Woronoff ring – is concentric blanching of
the erythematous skin at or near the
periphery of the healing psoriatic plaque
Pathogenesis




Hyperproliferative disorder driven by
inflammatory mediators
TH1 cytokines- IL-2,6,8,12; IFN-g, TNF-a
IL-8: accumulation of neutrophils
IL-12: Primary TH1 signal, promotes IFN-g
production
Inheritence




Heredity has a high influence, incidence
increases with generations
Evidence that susceptibility to psoriasis is
linked to class I and II MHC chromo 6
Genetic loci: PSORS1 (6); PSORS2 (17q)
HLA asso: early onset -Cw6, -B57, and -DR7
late onset –Cw2
Inheritance cont
It is also believed that any individual that has
-B13 or –B17 has a fivefold risk of developing
psoriasis
 pustular psoriasis: HLA-B27
 guttate and erythrodermic psoriasis: -B13,-B17
 Palmarplantar pust: -B8, -Bw35, -Cw7, -DR3

Epidemiology of psoriasis
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Seen in 1-2% of US population
Equal frequency in both sexes
Mean age of onset is 27 years
Sunlight may improve psoriasis by decreasing
T-cells
Emotional stress, smoking, and alcohol
aggravates psoriasis
Increased incidence of celiacs, lymphoma
Drug-induced psoriasis

May be induced by many drugs: beta blockers,
lithium, and antimalarials, terbinafine, calcium
channel blockers, captopril, glyburide, and lipid
lowering agents, such as gemfibrozil
Pathology

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Regular epidermal
hyperplasia with long, testtube-shaped rete ridges
Thinning of dermal papillae
Granular layer is thin or
absent
Overlying parakeratosis
Small collections of
neutrophils (Munro
microabcesses) may be
present in the stratum
corneum
There is a perivascular
mononuclear cell infiltrate
Types of Psoriasis
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Seborrheic-Like, “sebopsoriasis”
Inverse
Napkin
Arthritis
Guttate
Generialized Pustular (von Zumbusch)
Acro dermatitis Continua of Hallopeau
Impetigo Herpetiformis
Keratoderma Blennorrhagica
Erythrodermic
Inverse Psoriasis

Flexural or
intertrigenous
location
Shiny erythematous plaques in the axilla that lack scale.
“Napkin” Psoriasis
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Infants 2-8 mo
Erythematous sharply demarcated in diaper area
Lesions typically clear w/ topical tx
Infants may be at risk for psoriasis in adulthood
Five clinical patterns of psoriatic
arthritis
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Asymmetrical DIP joint involvement with nail damage,
16%
Arthritis mutilans with osteolysis of phalanges and
metacarpals, 5%
Symmetrical polyarthritis-like rheumatoid arthritis, with
claw hands, 15%
Oligoarthritis with swelling and tenosynovitis of one or
a few hand joints, 70%
Ankylosing spondylitis alone or with peripheral
arthritis, 5%
Radiographic findings of psoriatic
arthritis


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Erosion of terminal phalangeal tufts; tapering of
phalanges or metacarpals; “cupping’ of proximal ends
of phalanges; bony ankylosis; osteolysis of metatarsals;
predilection for distal and proximal interphalangeal
joints; paravertebral ossification; asymmetrical
sacroiliitis; and “bamboo spine”
Nearly half the patients with psoriatic arthritis have
HLA-B27
Tx- biologics, MTX, cyclosporin- dz modifying prevent
deformity
Guttate Psoriasis


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Usually, occurs as an abrupt eruption following an
acute infection, such as streptococcal pharyngitis
Occurs mostly in patients under age 30
Recurrent episodes are likely
This type of psoriasis is usually rapidly responsive to
topical steroids or UVB
Tx strep infx
Guttate Psoriasis
A: Small papules and plaques of guttate psoriasis; note Koebner phenomenon
B: Numerous papules due to Koebner phenomenon after sunburn
Generalized pustular psoriasis
(von Zumbusch)

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
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Typical patients have plaque
psoriasis and often psoriatic
arthritis
The onset is sudden, with
formation of lakes of pus
periungally, on the palms,
and at the edge of psoriatic
plaques
Pruritus, pain, fever, and
malaise
Fetid odor develops
Generalized pustular psoriasis
(von Zumbusch)

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Etiology unclear
Iodides, coal tar, steroid withdrawal,
terbinafine, minocycline, hydroxychloroquine,
acetazolamide, and salicylates may trigger the
attacks
May occur in infants
Acitretin is drug of choice, with a rapid and
predictable response
Isotretinoin, cyclosporine, methotrexate,
dapsone
Acrodermatitis Continua of
Hallopeau
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Acral erythematious plaques with nail
involvment
Fingernails float away on lakes of pus: anonychia
Fingers taper to long keratotic points
Impetigo Herpetiformis
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Generalized pustular psoriasis of pregnancy
Retinoids not indicated
Prednisone: controversial, may induce pustular
flare, also matures fetal lung
Early delivery strongly encouraged
Treatment- Topicals
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Corticosteroids- class I- 2wks, pulse, occlusion; rapid return
when stopped
Calcipotriene- keratinocyte differentiation; plaque and scalp type
Tars- oils and shmp; stinks (literally)
Macrolactams (tacrolimus, pimicrolimus)- prevent steroid
atrophy/acne
Salicylic acid- keratolytic
Tazorac- modulate differentiation/proliferation
Anthralin- SCAT, supresses neut superoxide, inhib mono IL6,8/TNF-a
Treatment- Light


NB-UVB (311-313nm), MED, 70% response
Goeckerman Tech

Ingram

2-5% tar bath QD + UV; clear 18 d w/ long term
response
tar bath QD/carbonisdetergens/ UV/
anthalin/talcum/stocking dressing
PUVA- 2x/wk, clear 20-25 tx; risk cataracts,
SCC, MM
Methotrexate
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
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First effective systemic drug for psoriasis and is the
standard for systemic therapy
Blocks synthesis of deoxyribonucleic acid, which
decreases cell division
Indications- erythroderma, arthritis, pustular, lg BSA
MTX
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For patients with no risk factors for liver
disease, the first liver biopsy should be
obtained at approximately 1.5g of cumulative
methotrexate
Weekly dosing for oral or IM – divided in
three doses, 12 hours apart
IM injections have a much lower risk of
hepatotoxicity
Cyclosporine

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May down-modulate proinflammatory
epidermal cytokines
Rapid clearing and return
Low risk of renal tox w/ <6mo tx
Follow BP, Cr
Diet


Most recent trials have demonstrated the
antiinflammatory effects of fish oils rich in n-3
polyunsaturated fatty acids in rheumatoid
arthritis, inflammatory bowel disease, psoriasis
and asthma
Gluten-free diet

Koo J., Lee E., Lee C.S., Lebwohl M.. Psoriasis. J
Am Acad Dermatol 2004;50:613-22.
Oral antimicrobial therapy


Recent evidence suggest that Staphylococcus aureus
and streptococci secrete a large family of
exotoxins that are superantigens, producing
massive T-cell activation
Oral antibiotic for psoriasis patients infected
with these organisms is imperative
Retinoids
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Treatment with 13-cis-retinoic acid can produce
good results, especially in pustular psoriasis
It is a potent teratogen
Combinations of retinoic acids with
photochemotherapy for chronic plaque psoriasis
may also be very affective
Etretinate- long ½ life, EtOH changes acitretin
Acitretin – avoid pregnancy for up to 3 years
following cessation of therapy
Dapsone

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Use is limited largely to palmoplantar eruptions
2nd-3rd line therapy
Combination therapy
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Patient on methotrexate may have their dose
minimized with concomitant topical steroid
use
PUVA with acitretin, cyclosprine, or
methotrexate
Topical Dovonex with Acitretin,
cyclosporine, methotrexate, and
phototherapy
Biologic Agents
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Alefacept (Amevive)
Efalizumab (Raptiva)
Etanercept (Enbrel)
Infliximab (Remicaide)
Adalimumab (Humira)
These are indicated for moderate-to-severe
plaque psoriasis
Journal of the American Academy of Dermatology
Volume 53 • Number 2 • August 2005
Copyright © 2005 American Academy of Dermatology, Inc.
Biologics in psoriasis: A quick reference guide
Valencia D. Thomas, MD
F. Clarissa Yang, MD
Joseph C. Kvedar, MD
Reiter’s syndrome
Triad: urethritis, conjunctivitis, and arthritis

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Can’t see, can’t pee, can’t climb a tree!
May also be other features that involve the skin ,
mucous membranes, GI tract, and cardiovascular
system
American Rheumatism Association - criterion of
peripheral arthritis of more than 1 month duration,
in association with urethritis and/or cervicitis
Young men of HLA-B27 genotype
Clinical features

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May also have fever,
weakness, and weight loss
A nonbacterial urethritis may
develop with painful and
bloody urination and pyuria
About 1/3 of patients
develop conjunctivitis
Keratitis is usually superficial
and very painful. Iritis is
common.
Reiter’s syndrome

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An asymmetric arthritis
afflicts synovial joints,
especially those that are
weight bearing
Onset is sudden with
heat and tenderness and
swelling
Pain in one or both heels
is a frequent symptom
Cutaneous lesions of Reiter’s

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The skin lesions start as multiple, small,
yellowish vesicles that break, become confluent,
and form superficial erosions
Develop frequently on the genitals and palms
Eruption on the glans penis occurs in 25% of
patients
Cutaneous features


The eruption is known as
keratoderma
blennorrhagicum
Penile lesions are frequent



Balanitis circinata
Characterized by perimeatal
balanitis
Similar lesion are seen on the
vaginal mucosa of women
Etiology

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Syndrome has been attributed to many different
agents, such as: Shigella flexneri, Salmonella spp., yersinia
spp., Ureaplasma urealyticum, Borrelia burgdorferi,
Cryptosporidia, and Campylobacter fetus
May be responsible for the infectious enteritis that
precedes onset in a small percentage of patients
In cases that follow an infection of the genitourinary
tract, Chlamydia trachomatis may be associated
HLA-B27 positivity is present in 80%
Laboratory findings


Non-specific
A leukocytosis of 10, 000 to 20, 000/mm3 and
an elevated sed rate
Histopathology

Identical to psoriasis
Differential diagnosis

May be confused with rheumatoid arthritis,
ankylosing spondylitis, gout, psoriatic arthritis,
gonococcal arthritis, acute rheumatic fever,
chronic mucocutaneous candidiasis, and serum
sickness
Treatment
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Usually mucocutaneous lesions are selflimited and clear within a few months
Topical steroids
NSAIDs
Methotrexate
Cyclosporine
Acitretin
Subcorneal pustular dermatosis
(Sneddon-Wilkinson Disease)
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Chronic pustular disease, which
occurs chiefly in middle-aged
women
Pustules are superficial and
arranged in annular and serpiginous
patterns on the abdomen, axillae
and groin
Vesicles may be present
Cultures from pustules are sterile
Oral lesions are rare
Some cases occur in association
with an IgA monoclonal
gammopathy
Desmocollin 1 autoantigen for Iga
pemphigus
Subcorneal pustular dermatosis
(Sneddon-Wilkinson Disease)

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Dapsone appears to be
effective in most cases
Sulfapyridine
Acitretin
NB-UVB
Corticosteroids,
colchicine, and TCN
with niacinamide
Without treatment this is
a chronic condition with
remissions of variable
duration
Pathology of Sneddon-Wilkinson
Eosinophilic pustular folliculitis

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Also referred to as sterile eosinophilic pustulosis
AKA Ofuji’s disease
Males, Asia
HIV
Characterized by pruritic, follicular
papulopustules arranged in groups
Histo: eos in or around follicles
Eosinophilic pustular folliculitis

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Distribution is usually asymmetrical – affects
face, trunk, and upper extremities
Cause is unknown – numerous studies have
implicated chemotactic substances, ICAM-1,
and cyclooxygenase-generated metabolites
Typical course – spontaneous remissions and
exacerbations for a few months to several
years
Eosinophilic pustular folliculitis


Dapsone or systemic steroids are the treatment
of choice
Success with intralesional steroids, clofazimine,
minocycline, isotretinoin, UVB therapy,
indomethacin, colchicine, cyclosporine, and
cetirizine
Recalcitrant Palmoplantar
Eruptions


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Recalcitrant pustular eruptions of the hands and
feet are often examples of psoriasis
Need to then search for lesions elsewhere on the
body(e.g., scalp, ears, glans penis)
Search also for a family history to confirm your
suspicion
Dermatitis Repens




Aka- acrodermatitis continua and acrodermatits
perstans
It’s a chronic inflammatory disease of hands and feet
Rarely, can become generalized
Usually, as a pustule or paronychia
Dermatitis Repens


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
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Occasionally, mucous membranes are involved
Nails are often dystrophic or destroyed
Lesions cause skin atrophy
Crusted, eczematoid, and psoriasiform lesions may
occur, and there may be moderate itching
It is essentially unilateral in its beginning and
asymmetrical throughout its entire course
Dermatitis Repens

Histology




similar to those seen in psoriasis
the primary lesion is epidermal
An intraepithelial spongiform pustule is formed by infiltration
of pmn’s
Treatment


topical mechlorethamine, topical steroids, PUVA,
fluorouracil, and sulfapyridine
Acitretin, low dose cyclosporine, Acitretin plus calcipotriol
Palmoplantar Pustulosis



AKA pustular psoriasis
In contrast to dermatitis repens it is essentially
bilateral and symmetrical
Locations include: thenar/hypothenar
eminences or central portion of the palms and
soles
Palmoplantar Pustulosis


Patches begin as
erythematous areas in
which pustules form
Start as pinhead-sized,
enlarge and coalesce to
form small lakes of
pus



In the course of a week,
they tend to dry up, leaving
punctate brown scabs that
eventually exfoliate
Stages of quiescence and
exacerbation characterize
the condition
Meds, such as lithium, have
been reported to induce
Palmoplantar Pustulosis


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
Nails may become malformed, ridged, stippled, pitted and
discolored
May be associated with psoriasis vulgaris
Some regard palmoplantar pustulosis as a form of psoriasis,
while others consider it a separate entity
Female predominance; lack of seasonal variation; different
histopathologic features and
Associated with thyroid disorders and cigarette smoking
Palmoplantar Pustulosis


May be predisposed to joint
disease and possibly SAPHO
syndrome-Synovitis, Acne,
Pustulosis, Hyperostosis and
Osteoarthritis
It’s resistant to most
treatments



Acitretin is reportedly
effective(1mg/kg/day)
Low-dose cyclosporine
(1.25mg/kg/day3.75mg/kg/day)
Intramuscular Kenalog (4060mg)may be effective for
short-term relief
Palmoplantar Pustulosis
Pustular Bacterid


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Characterized by a symmetric, grouped, vesicular or
pustular eruption on palms and soles
Marked by exacerbations and remissions over long
periods
No involvement of webs of fingers or toes or
flexion creases of toes
WBC may be elevated
Scaling is usually present
Etiology is thought to be a remote focus of
infection; infection needs to be treated before
resolution will occur
Juvenile Plantar Dermatosis
•
•
•
•
•
•
Usually begins as a patchy, symmetrical, smooth, red, glazed
macule on great toes, sometimes with fissuring and
desquamation in children aged 3-13
Toe webs are rarely involved; fingers may be
Histologically, there is psoriasiform acanthosis and a sparse,
lymphocytic infiltrate in the upper dermis
Spongiosis is commonly present
Tx: bed rest, cotton socks and topical steroids
Spontaneous resolution within 4 yrs is the rule
Infantile Acropustulosis




Intensely itchy vesicopustular eruption of hands
and feet
Begins at any age up to 10 months, clearing in a
few weeks and recurring repeatedly until final
resolution at 6 – 36 months of age
Dapsone at 2mg/kg/day may help
Potent topical steroids aid in symptomatic relief
Infantile Acropustulosis


Should prompt an extensive workup to eliminate
serious infectious causes (i.e., Tzanck prep, gram
stain, KOH prep of pustule)
Some suspect that this condition may be a
persistent reaction to prior scabies

Acropustulosis of infancy
THE END