Download Seborrheic Dermatitis, Psoriasis, Recalcitrant Palmoplantar Eruptions, Pustular Dermatitis, and

Document related concepts

Acute lymphoblastic leukemia wikipedia , lookup

Transcript
Seborrheic Dermatitis,
Psoriasis, Recalcitrant
Palmoplantar Eruptions,
Pustular Dermatitis, and
Erythroderma
P214-239
ANDREWS’
DISEASES OF THE SKIN
Seborrheic dermatitis
2- 5 % of the population
 Chronic, superficial, inflammatory disease of
the skin
 Predilection for the scalp, eyebrows, eyelids,
nasolabial creases, lips, ears, sternal area,
axillae, submammary folds, umbilicus, groin,
and gluteal crease
 Characterized by scanty, loose, dry, moist, or
greasy scales, and by crusted pink or
yellowish patches of various shapes and sizes

Clinical features
Remissions and exacerbations
 No to mild itching
 On the scalp is the most common
 Pityriasis steatoides – oily type,
accompanied by erythema and
accumulation of thick crust
 Frequently spreads beyond the scalp






Extreme cases involve the entire scalp and
may have an offensive odor
Cradle cap
Marginal blepharitis – edges of the lids
becoming erythematous and granular.
Conjunctiva may be injected
On and in the ears may be mistaken for otitis
externa caused by a fungal infection
KOH –
The presternal area is a favored site on the
trunk
 Common in the groin and the gluteal crease
 In acute stages the inflammation may be
intense, with moist exudation from the scalp
and ears and papulovesicles on the palms
and soles. Secondary infections,
impetiginization, or furunculosis may ensue

May progress to a generalized
exfoliative state
 In the newborn this type of severe and
generalized seborrheic dermatitis is
known as erythroderma desquamativum
 May be associated with or accentuated
by several internal diseases
 Parkinson’s, HIV, DM,

Etiology and pathogenesis
Remains unresolved
 Presence of lipophilic yeast Pityrosporum
ovale in large numbers in scalp lesion
 Also demonstrated in those without
seborrheic dermatitis
 Healthy individuals have been found to have
higher IgG antibodies to the organism

The significance of P ovale in infantile
seborrheic dermatitis has not been fully
evaluated
 Familial tendency toward infantile and
adult seborrheic dermatitis

histology
Epidermis is acanthotic
 There is overlying focal scale crust often
adjacent to follicular ostia
 Slight spongiosis
 Dermis contains a perivascular mixed
cell inflammatory infiltrate

Differential diagnosis
Psoriasis, heavier scale and erythema,
Auspitz’s sign, resistance to treatment,
nail involvement
 Crusted scabies of the scalp in
immunodeficiency syndromes
 Otitis externa, blepharitis, tinea
corporis, pityriasis rosea, keratosis
lichenoides chronica

Treatment
Selenium sulfide, tar, zinc, pyrithionate,
resorcin shampoos
 Nizoral
 Corticosteroid solution
 Antimicrobial preparations may be
necessary
 Bifonazole shampoo has been shown
effective in treating infants and small
children

Cortisporin otic usually brings about
prompt clearing
 Desonide Otic Lotion, 0.05% Desonide
and 2% acidic acid is also effective
 Topical steroids should not be used for
blepharitis, since steroid preparations
used in area may induce glaucoma and
cataracts

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

Typically symmetrical
 May be a solitary macule to more than 100
 Usually develops slowly but may be
exanthematous, with a sudden onset of
numerous guttate lesions
 Subjective symptoms such as itching or
burning may be present and cause extreme
discomfort

Scales are micaceous
 Auspitz’s sign may be observed
 Annular, lobulated, and gyrate figures
may be produced
 Old patches may be thickened and
tough, and covered with lamellar scales
like the outside of an oyster shell –
psoriasis ostracea


Various other descriptive terms
Psoriasis guttata
 Psoriasis follicularis
 Psoriasis figurata, psoriasis annulata,
psoriasis gyrata
 Psoriasis discoidea
 Psoriasis rupioides
 Plaque psoriasis

Seborrheic-like psoriasis
In some cases of psoriasis prominent
features of seborrheic dermatitis may
occur not only if the typical sites of
psoriasis vulgaris but also in the flexural
areas such as the antecubital areas,
axillae, under the breasts, groins,
umbilicus, and intergluteal areas
 Sebopsoriasis or seborrheic psoriasis

Inverse psoriasis
Flexural psoriasis or volar psoriasis
 Selectively and almost exclusively
involves folds, recesses, and flexural
surfaces
 Scalp quite often participates as well
 Onycholysis, “Oil spots”, and nail pitting
may be seen

“Napkin” psoriasis
Diaper dermatitis caused by the irritative
effects of urine in the wet diaper area, may
imitate a psoriasiform eruption
 In addition there is commonly an infection
with Candida albicans
 Lesions typically clear
 Infants may be at risk for psoriasis in
adulthood

Psoriatic arthritis

The incidence of psoriasis is 10 times
greater in persons with seronegative
arthritis than in persons without
arthritis
Five clinical patterns





Asymmetrical distal interphalangeal 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 suggestive of psoriatic
arthritis include: 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
rarity of “bamboo spine”when the spine is
involved
 Nearly half the patients with psoriatic arthritis
have HLA-B27

Rest, splinting, passive motion, and aspirin or
NSAIDs are appropriate
 Methotrexate, cyclosporine, oral retinoids,
sulfasalazine, tacrolimus, and PUVA are all
likely to help both the psoriasis and arthritis
 Systemic steroids however, the long term
complications and potential for rebound in
cutaneous disease restricts their use

Guttate psoriasis





This distinctive form of psoriasis typical
lesions are the size of water drops
Usually occurs as an abrupt eruption
following some acute infection, such as
streptococcal pharyngitis
Occurs mostly in patients under age 30
Recurrent episodes are likely, because of
pharyngeal carriage of the responsible
streptococcus
This type of psoriasis is usually rapidly
responsive to topical steroids or UVB
Generalized pustular psoriasis
(von Zumbusch)





Typical patients have had 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
Pruritis and intense burning cause extreme
discomfort
There is a fever, and a fetid odor develops
The pustules dry up to form yellow-brown
crusts over a reddish-brown shiny surface
Generalized pustular psoriasis
(von Zumbusch)





Mucous membrane lesions are common on
the tongue and in the mouth
The lips are red and scaly, and superficial
ulcerations of the tongue and mouth occur
May go through several stages
A number of cases of acute respiratory
distress syndrome associated with pustular
and erythrodermic psoriasis have been
reported
Systemic complications include pneumonia,
CHF and hepatitis
Generalized pustular psoriasis
(von Zumbusch)
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

course
The course of psoriasis is unpredictable
 Usually begins on the scalp or elbows
 May first be seen over the sacrum
 Onset may be sudden and widespread
 First lesions may be limited to the
fingernails
 Two of the chief features of psoriasis
are its tendency to recur and persist

However, patients may remain completely
free of lesions for years
 Koebner’s phenomenon – the appearance of
typical lesions of psoriasis at sites of eve
trivial 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

On the scalp absence of itching or hair loss,
marked predilection for frontal scalp margin,
deep erythema, and resistance to effective
therapy for seborrheic dermatitis all suggest
psoriasis
 The palms and soles are often, sometimes
exclusively, affected
 “flexural” or inverse psoriasis shows salmonred, demarcated plaques that frequently
become eczematized, moist and fissured

Numerous cases of psoriasis of the mucous
membranes have been reported
 Fingernails and toenails may be involved.
Characteristic changes include pits,
onycholysis and cracking of the free edges
 Many studies report an association of
hepatitis C and psoriasis
 Hepatitis C has also been implicated in
psoriatic arthritis

etiology
The cause of psoriasis is still unknown
 Heredity is of significance in some cases
 Evidence that susceptibility to psoriasis
is linked to class I and II major
histocompatibility complex on human
chromosome 6 (17q)
 HLA associations include -Cw6, -B57,
and -DR7 for early onset and -Cw2 for
late onset psoriasis

It is believed also that any individual
that has –B13 or –B17 has a fivefold
risk of developing psoriasis
 In pustular psoriasis HLA-B27 may be
seen
 -B13 and -B17 are increased in guttate
and erythrodermic psoriasis

epidemiology
Equal frequency in both sexes
 Mean age of onset is 27 years, range is
wide
 Sunlight improves
 Emotional stress aggravates
 39% of patients experience complete
periodic disappearance of psoriasis
during its course
 Seen in 1-2% of US population. Not
seen in Native Americans

Distinct tendency to improvement with
pregnancy
 Exacerbation is common after childbirth

pathogenesis
Characterized by three main pathogenic
features: abnormal differentiation,
keratinocyte hyperproliferation and
inflammation
 Accelerated epidermopoiesis has been
considered to be the fundamental pathologic
event in psoriasis
 The transit rate of psoriatic keratinocytes is
increased, and the deoxyribonucleic acid
synthesis time is decreased

Early lesion are infiltrated
predominantly by lymphocytes in the
papillary dermis
 The cause of T-lymphocyte activation
and the role of these cell populations
remains unclear

stress

Studies have shown a positive
correlation between stress and the
severity of disease
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






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
Differential diagnosis
Seborrheic dermatitis, pityriasis rosea,
lichen planus, eczema, psoriasiform
syphilid, and lupus erythematosis
 Distribution, quality of the scale and
presence of Auspitz’s sign, symptoms,
serologic testing

treatment
Topical
 Systemic
 Combination
 Rotating therapeutic approaches,
especially with systemic agents that
have varying toxicities

Topical treatment

In many patients topical applications
alone will suffice to keep psoriasis
under control. Numerous local
medications are available
Corticosteroids





Most frequent therapy
Ointments are more effective
Creams are preferred in the intertriginous
areas and in exposed areas
Effectiveness may be augmented by an
occlusive dressing, prompt response is usually
noted
Side effects include miliaria, pyoderma,
epidermal atrophy
Intralesional corticosteroid injections
 Good results when treating psoriatic
nails

tars
The staining property and the odor of
the tars may hinder their use
 Coal tar or liquor carbonis detergents is
applied to the lesions before ultraviolet
treatment in the Goeckerman method

dihydroxyanthralin
Anthralin exerts its direct effect on
keratinocytes and leukocytes by
suppressing neutrophil superoxide
generation and inhibition of monocyte
derived IL-6, IL-8, and TNF-alpha
 Highly irritating and stains skin, clothing
and bedding

tazarotene
Appears to treat psoriasis by
modulating keratinocyte differentiation
and hyperproliferation, as well as
suppressing inflammation
 Produces local irritation
 Combination with topical corticosteroids
aids in patient acceptance

Vitamin D
Vitamin D3 affects keratinocyte differentiation
partly through its regulation of epidermal
responsiveness to calcium
 Has been shown to be very effective in the
treatment of plaque-type and scalp psoriasis
 Combination therapy with high-potency
steroids may provide greater response

Salicylic acid
Aids in removing of the scale and
promoting the efficacy of other topical
agents
 Usually in a 3% to a 5% concentration
 Widespread application may lead to
salicylate toxicity
 Tinnitus, acute confusion, refractory
hypoglycemia

Ultraviolet light





In most instances sunlight improves psoriasis
remarkably
However, burning of the skin may cause
Koebner’s phenomenon and an exacerbation
UVB
Tar applications or baths before UVB
exposure have been credited with enhancing
its effects
Maintenance UVB phototherapy after clearing
contributes to the duration of remission

Narrow band UVB has been shown to
be more effective in treating psoriasis
than broadband
Goeckerman technique

2 to 5% tar preparation is applied to
the skin, and a tar bath is taken at least
once daily. The excess tar is removed
with mineral or vegetable oil and
ultraviolet light is given
Ingram technique
Consists of a daily coal tar bath in a solution
such as 120 ml liquor carbonis detergens to
80 L of warm water
 This is followed by exposure to an ultraviolet
light for daily increasing periods
 Anthralin paste is then applied to each
psoriatic plaque
 Talcum powder is sprinkled over the lesions,
and a stockinette dressing is applied

PUVA therapy





UVA given 2 hours after ingestion of 8methoxypsoralen (Oxsoralen), and given two
to three times a week
Maintenance treatment is needed
Risk of enhanced carcinogenesis is proven
Reversal of the usual ratio of squamous cell
carcinomas to basal cell carcinomas
Accelerated actinic elastosis, melanocyte
dysplasia, and possible increased incidence of
melanoma should be considered
Surgical treatment
Denervation by surgery has long been
known to abolish psoriatic plaques
 Excision to the depth of the reticular
dermis
 In a group of 14 patients with
pharyngeal colonization by streptococci
unresponsive to antibiotic therapy 9
cleared after tonsillectomy

Lasers in psoriasis
Psoriatic plaques can be cleared by
destruction of the upper dermis
 Blood vessels in the psoriatic dermal
papillae can be selectively destroyed
with yellow lasers effectiveness with
flashlamp pulsed dye laser
 Some success with CO2 resurfacing
laser

hyperthermia
22 chronic psoriatic plaques in 9 patients
were heated to 42 – 45 C with ultrasound for
30 minutes 3 times a week for 4 to 10
treatments. 15 completely cleared
 Microwave hyperthermia may produce
significant complications such as pain over
bony prominences and hypotension

Occlusive treatment
Report of clearing of psoriatic lesion by
occluding them with tape
 Has been shown that hydrocolloid
occlusion (with Actiderm) had a similar
effect on small lesions

Systemic treatment
Corticosteroids
 Methotrexate
 Cyclosporine
 Diet
 Oral antimicrobial therapy
 Retinoids
 dapsone

Corticosteroids
The side effects of orally administered
prednisone are so dangerous that its
use should be limited to patients with
unusual individual circumstances
 There is a great risk of “rebound” or
induction of pustular psoriasis when it is
stopped

Methotrexate
Was the first effective systemic drug for
psoriasis and is the standard for systemic
therapy
 Methotrexate has greater affinity for
dihydrofolic acid reductase than has folic acid
 The synthesis of deoxyribonucleic acid is
blocked when dihydrofolic acid reductase is
bound and thereby cell division is decreased

May also affect the inflammatory elements of
psoriasis
 Indications for its use include psoriatic
erythroderma, moderate to severe psoriatic
arthritis, acute pustular psoriasis, more than
20% total body surface involvement, localized
pustular psoriasis, psoriasis that affects
certain areas of the body so that normal
function and employment are prevented, lack
of response to phototherapy, PUVA, or
retinoids

Prior to start of treatment ensure that
there is no history of liver or kidney
disease
 Alcohol abuse, cirrhosis, severe illness,
debility, pregnancy, anemia should be
considered
 AST, ALT, bilirubin, serum albumin,
creatinin. Alkaline phosphatase, CBC,
platelets, Hep B and C serology, and UA
should all be evaluated prior to tx

Most patients with no risk factors for
liver disease the first liver biopsy should
be obtained at approximately 1.0 to 1.5
g of cumulative methotrexate and
repeated every subsequent 1.5 g until a
total of 4 g is reached
 3 divided oral doses 12 hrs apart
weekly, once weekly doses orally and
single weekly subcutaneous injections
are effective treatment schedules

Cyclosporine
The therapeutic benefit of the
immunosupressive features of
cyclosporine in psoriatic disease may be
related to down modulation of
proinflammatory epidermal cytokines
 Biopsies of the kidney in 30 patients
after 2 years of cyclosporine treatment
found features consistent with
cyclosporine-induced nephrotoxicity

Another study concluded that cyclosporine
may be used long-term (5-10 years) in
severe, recalcitrant psoriasis if renal function
is closely monitored
 Induction of therapy with 2.5 to 3.0 mg/kg
given as a divided dose twice daily and may
be increased to 5.0 mg/kg/day until a clinical
response is noted

Diet
Controlled studies evaluating various dietary
restrictions, especially those of low protein
intake, showed no appreciable difference in
the course of psoriasis
 Another study found psoriasis to be more
severe in patients trying herbal remedies,
vitamin therapy, and dietary manipulation


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
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





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
Acitretin, avoid pregnancy for up to 3 years
following cessation of therapy
dapsone

Use is limited largely to palmoplantar
eruptions
Combination therapy
Patient on methotrexate may have their
dose minimized with concomitant
topical steroid use
 PUVA with Acitretin
 PUVA with cyclosporine
 PUVA with methotrexate
 Topical Dovonex with Acitretin,
cyclosporine, methotrexate, and
phototherapy

The principle behind combination therapy is
the use of agents win which the side effects
profile is different or partly opposing
 Hydroxyurea and sulfalsalazine have been
used in severe cases and in combination with
other modalities, such as PUVA
 Particularly useful in topical therapy, where
the risk profiles are considerably less

Reiter’s syndrome
Characteristic clinical triad consisting of
urethritis, conjunctivitis, and arthritis
 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
 Occurs chiefly in young men of HLA-B27
genotype

Clinical features
Any one of the triad of urethritis, conjunctivitis,
or arthritis may occur first, accompanied by
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

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

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
 Lesions on the soles start as pustules,
become crusted or hyperkeratotic

The eruption is known as keratoderma
blennorrhagicum
 Penile lesions are frequent
 Characterized by perimeatal balanitis,
these become crusted and form
circinate lesions
 Similar lesion are seen on the vaginal
mucosa of women

Nails become thick and brittle, with
development of subungal keratosis
 Endocarditis, pericarditis, and
myocarditis, as well as aortic
insufficiency occur in some
 In children conjunctivitis is the most
frequent complaint

etiology

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% of
cases of Reiter’s syndrome
 This rises to 90 to 100% in patients
with accompanying sacroiliitis, uveitis,
or aortitis
 May be caused by abnormalities in the
production and elicitation of immune
mediators or T cells

Laboratory findings
No specific changes are characteristic of
this syndrome
 A leukocytosis of 10, 000 to 20,
000/mm3 and an elevated sed rate are
the most consistent findings

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
Usually mucocutaneous lesions are self
limited and clear within a few months
 Topical steroids are helpful
 NSAIDs for the joint disease
 Methotrexate for severe disease
 Cyclosporine
 Acitretin

The course is characterized by
exacerbations and remission in about
on third of patients
 A chronic deforming arthritis occurs in a
bout 20% of patients, with significant
disability resulting, chiefly from foot
deformities

Subcorneal pustular dermatosis
(Sneddon-Wilkinson Disease)






Chronic pustular disease which occurs chiefly
in middle-aged women.
Pustules are superficial and arranged in
annular and serpiginous patterns, especially
on the abdomen, axillae and groins
Sometimes vesicles are present
Cultures from pustules are sterile
Oral lesions are rare
Some cases occur in association with and IgA
monoclonal gammopathy
Subcorneal pustular dermatosis
(Sneddon-Wilkinson Disease)
Dapsone appears to be effective in
most cases
 Sulfapyridine
 Acitretin
 Narrow band UVB
 Corticosteroids, colchicine, and TCN
with niacinamide
 Without treatment this is a chronic
condition with remissions of variable
duration

Eosinophilic pustular folliculitis

Also referred to as sterile eosinophilic
pustulosis
Five times more common in males
 Peak age of onset in the third decade,
although a number of pediatric cases
have been reported
 Characterized by pruritic, follicular
papulopustules arranged in groups
 Plaques may form

Eosinophilic pustular folliculitis
Distribution is usually asymmetrical,
with the face, trunk, and upper
extremities most often afflicted
 Cause is unknown, however, numerous
studies have implicated chemotactic
substances, ICAM-1, and
cyclooxygenase-generated metabolites
 Typical course is one of spontaneous
remissions and exacerbations lasting
from 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
 Must be distinguished from HIVassociated EF

Eosinophilic pustular folliculitis
Another variant has been described that
occurs early in childhood
 Patients develop sterile pustules and papules
preferentially over the scalp and; however,
scattered clusters of pustules may occur over
the trunk and extremities
 High-potency topical steroids are the
treatment of choice
 Recurrent exacerbations and remissions
usually occur with spontaneous remission
