Transcript Exfoliative Dermatitis - Osteopathic Medical School

Andrews’ Diseases of the SkinChapter 10-pg 239-253 &
Chapter 11
Boris Ioffe, D.O.
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
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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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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
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Histology
– similar to those seen in psoriasis
– the primary lesion is epidermal
– An intraepithelial spongiform pustule is formed
by infiltration of pmn’s
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Treatment
– topical mechlorethamine, topical steroids, PUVA,
fluorouracil, and sulfapyridine
– Acitretin, low dose cyclosporine, Acitretin plus
calcipotriol
Palmoplantar Pustulosis
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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
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Patches begin as
erythematous areas
in which pustules
form
Start as pinheadsized, enlarge and
coalesce to form
small lakes of pus
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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
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May be predisposed to
joint disease and
possibly SAPHO
syndrome-Synovitis,
Acne, Pustulosis,
Hyperostosis and
Osteoarthritis
It’s resistant to most
treatments
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Acitretin is reportedly
effective(1mg/kg/day)
Low-dose cyclosporine
(1.25mg/kg/day3.75mg/kg/day)
Intramuscular Kenalog
(40-60mg)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
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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
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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
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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
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Acropustulosis of infancy
Pompholyx
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AKA dyshidrosis
A vesicular eruption of palms and soles
characterized by spongiotic intraepidermal
vesicles and often accompanied by burning or
itching
Hyperhidrosis may be present
Usually bilateral and symmetrical
Bullae may form
Contents are clear and colorless
Attacks generally last a few weeks
Lesions dry-up and desquamate rather than
rupture
Pomphylox
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Etiology- stress, atopy, and topical as well
as ingested contactants
Histopathology: spongiotic vesicles in the
epidermis
Differential dx:
– dermatophytid, contact dermatitis, atopic
dermatitis, drug eruption, pustular psoriasis of
palms and soles, acrodermatitis continua, and
pustular bacterid
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Rarely, T-cell lymphoma can present with
similar clinical findings, but biopsy of the
vesicles will be diagnostic
Pomphylox
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Tx: high potency corticosteroid creams
Triamcinolone acetonide
intramuscularly or a short course of
oral prednisone is rapidly effective
Oral or topical psoralen + UVA (PUVA)
is effective but costly & inconvenient
In more severe forms,
immunosuppressive mycophenolate
mofetil has been effective
Lamellar Dyshidrosis
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AKA dyshidrosis
lamellosa, keratolysis
exfoliativa
A superficial exfoliative
dermatosis of the palms
and sometimes soles
Referred to as recurrent
palmar peeling
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Involvement is bilateral
Can occur in association
with dyshidrosis
Often exacerbated by
environmental factors
Differential dx:
dermatophytosis,
chronic contact
dermatitis
Lamellar Dyshidrosis
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Tx: difficult
Spontaneous involution can occur in a few
weeks for some
Most tends to be chronic and relapsing
Tar creams (Zetone cream) usually helps
5% tar in gel (Estar Gel) is an excellent tx
Lac-Hydrin lotion and Carmol 10 or 20 are
often effective
NB-UVB may be helpful
Lamellar Dyshidrosis
Palmoplantar
Keratoderma
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AKA tylosis, keratosis, hyperkeratosis
Characterized by excessive formation of keratin on
the palms and soles
Acquired
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–
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Keratosis Punctata of the Palmar Creases
Punctate Keratoses of the Palms and Soles
Porokeratosis Plantaris Discreta
Keratoderma Climactericum
Congenital
Punctate Keratosis of the
Palms and Soles
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Primary lesion is a 1-5mm round to
oval, dome-shaped papule distributed
over left hand and hypothenar
eminence
Main symptom is pruritis
Lesions number from 1 to >40
Affects mainly blacks
There’s a potential risk of developing
lung and colon cancer
Punctate Keratosis of the
Palms and Soles
Keratosis Punctata of the
Palmar Creases
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Common most often in black pts
Primary lesion is a 1-5mm depression
filled with a conical keratinous plug
Primarily, in creases of palms or
fingers, occasionally in soles
Lesions are multiple
Friction aggravates lesions causing
them to become verrucoid or
surrounded by callus
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Punctate
keratoses of
the palmar
creases in an
AfricanAmerican
PPPK-punctate
palmoplantar
keratoderma
Porokeratosis Plantaris
Discreta
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Occurs in adults, Female:Male (4:1)
Characterized by sharply marginated,
rubbery, wide-based papule that does not
bleed on removal
Lesions are multiple, painful, 7-10mm in
diameter
Usually on wt bearing areas of sole, beneath
metatarsal heads
Tx: foot pads to redistribute wt, surgical
excision, blunt dissection
Keratoderma
Climactericum
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Characterized by hyperkeratosis of
palms and soles beginning at about
the time of menopause
Descrete, thickened, hyperkeratotic
patches most pronounced at pressure
sites
Fissuring may be present
Tx: keratolytics -- 10% salicylic acid,
lactic acid creams, etc.
Hereditary syndromes
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These have palmoplantar keratoderma
as a feature
– Unna-Thost
– Papillon-Leferve
Unna Thost
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Dominant inheritance; congenital thickening
of epidermal horny layer of the palms and
soles
Usually symmetrical
Epidermis becomes thick, yellowish,
verrucous, and horny
Striate and punctate forms occur
Unna Thost
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Occasionally nails become thickened
5% salicylic acid may help
Lac Hydrin 12% may be tried
Acitretrin or isotretinoin may be
considered, but need for lifetime tx
makes them impractical
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Focal
palmoplantar
keratosis of the
striate type on
the sole
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Diffuse nonepidermolytic
palmoplantar
keratosis
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Diffuse epidermolytic palmoplantar
keratosis with diffuse hyperkeratosis
Papillon-Lefevre
Syndrome
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Palmoplantar hyperkeratosis with
peridontosis
Usually develops within the first few months
of life but may occur in childhood
Well demarcated, erythematous,
hyperkeratotic lesions on palms and soles
Transverse grooves of fingernails may occur
Papillon-Lefevre
Syndrome
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Early onset peridontal
disease has been
attributed to damage
and alteration in PMN
function caused by
Actinomyces
actinomycetemcomitans
Disease associations
include: acroosteolysis,
and pyogenic liver
abcesses
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There are asymptomatic
ectopic calcifications in
the choroid plexus and
tentorium
Therapy may retard
both dental and skin
abnormalities
Treatment with Acitretin
in four siblings was
reported to be effective
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Papillon-Lefevre syndrome: plantar
keratoderma
Mutilating Keratoderma
of Vohwinkel
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Palmoplantar hyperkeratosis of the
honeycomb type-associated with starfishlike keratosis on backs of hands and feet;
linear keratoses of the elbows and knees,
and annular constriction (pseudo-ainhum) of
the digits, this may progress to
autoamputation
More than 30 cases have been reported
world-wide
More common in women and in whites
Onset is in infancy or early childhood
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Vohwinkel’s mutilating syndrome: A.) diffuse
keratoderma of palms with B.) pseudoaainhum
formation
Palmoplantar
Keratodermas &
Malignancy
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Diffuse, waxy keratoderma of palms and
soles occurring as an AD trait associated
with esophageal carcinoma
Other related factors are oral leukoplakia,
esophageal srictures, squamous carcinoma
of tylotic skin, carcinoma of larynx and
stomach
Acquired forms of palmoplantar
keratodermas have also been associated
with carcinoma of esophagus, lung, breast,
bladder and stomach
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Focal PPK in association
with carcinoma of the
esophagus
Acrokeratoelastoidosis of
Costa
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AD, more common in women
Small, round, firm papules occurring over
dorsal hands, knuckles, and lateral margins
of palms and soles
Appears in early childhood and progress
slowly
Most often asymptomatic
Significant histologic finding is dermal
elastorrhexis
Therapies: liquid nitrogen, salicylic acid,
tretinoin, and prednisone have been tried
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Focal
acrokeratoelastoides:
multiple skin-colored
papules at the
margin of the palmar
skin
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Path: nonepidermolytic
palmoplantar
keratosis,
acanthosis and
hypergranulosis
Exfoliative Dermatitis
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Universal or very extensive scaling and
itching erythroderma
Often associated with hair loss
Initially with erythematous plaques, which
spread rapidly
Onset accompanied by general toxicity
Skin becomes scarlet and swollen and may
ooze a straw-colored exudate
Desquamation is evident within a few days
Etiology
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Most common is
preexisting dermatoses:
(53%);
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– atopic dermatitis, chronic
actinic dermatitis,
psoriasis,seborrheic
dermatitis, vesicular
palmoplantar eczema,
pityriasis rubra pilaris,
and contact dermatitis
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Drug eruptions(5%);
– allopurinol, gold,
carbamazepine,
phenytoin, and quinidine
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Cutaneous T-cell
lymphoma(13%);
Sezary syndrome and
mycosis fungoides
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Paraneoplstic (2%);
carcinoma of the
lung and carcinoma
of the stomach
Leukemia cutis (1%)
Idiopathic (26%)
Mortality rate at a
mean follow-up
interval of 51
months was 43%
Histology
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Most commonly, histology is
nonspecific
Hyperkeratosis & focal parakeratosis
Epidermis shows mild acanthosis,
scant superficial upper dermal infiltrate
of mononuclear cells
May be small areas of spongiosis
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Generali
zation
after
withdra
wal of
methotr
exate
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Exfoliation
of scale
with
underlying
erythema
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Generalized
erythema
with thick
scale and
crusted
fissures on
the plantar
surface
Treatment
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Topical steroids, soaks, and compresses
Acitretin and cyclosporin-useful in psoriatic
erythroderma, and isotretinoin in
erythroderma caused by RPR; methotrexate
Systemic corticosteroids in severe cases
Discontinuing the offending drug in druginduced cases
* Subungual
hyperkeratosi
s and distal
dystrophy
Parapsoriasis, Pityriasis
Rosea, Pityriasis Rubra
Pilaris
Parapsoriasis
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Group of macular scaly eruptions with
slow evolution
These are all markedly chronic,
resistant to treatment, and are without
subjective symptoms
They are divided into: pityriasis
lichenoides chronica, pityriasis
lichenoides et varioliformis acuta, and
parapsoriasis en plaques
Pityriasis Lichenoides
Chronica
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Erythematous,
yellowish, scaly
macules and lichenoid
papules
They persist
indefinitely without
change
Mainly on sides of
trunk, thighs, and
upper arms
May be confused with
psoriasis and
secondary syphilis
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Tx- UV light is
beneficial; however
intense doses may be
needed for good results
PUVA has been reported
to be effective
Oral tetracycline may be
used with
antihistamines
PLC is a benign disease
that clears
spontaneously in a few
yrs to months
Pityriasis Lichenoides
Chronica
PLEVA
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AKA: parasoriasis
lichenoides,
Habermann’s disease,
Mucha-Habermann
disease and
parapsoriasis
varioliformis acuta
Sudden appearance of a
polymorphous eruption
composed of macules,
papules, and occasional
vesicles
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May run an acute,
subacute, or chronic
course
Papules are usually
yellowish or brownishred, round lesions,
which tend to crust,
become necrotic and
hemorrhage
PLEVA
PLEVA
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* Usually a benign, selfWhen exanthem heals it
limited disorder, but
leaves a smooth,
may be more chronic
pigmented, depressed,
and severe
varioliform scar
 Maybe a spectrum of
Favorite sites are
cutaneous T-cell
anterior trunk, flexural
lymophoma
arms, and axillae
 Differential dx:
Palms and soles are
– leukocytoclastic angiitis,
papulonecrotic
involved infrequentlytuberculid, psoriasis,
mucous membranes are
lichen planus, varicella,
not
PR, drug eruptions,
Generalized
maculopapular syphilid,
viral, rickettsial diseases,
lymphadenopathy can
lymphomatoid papulosis
occur
PLEVA
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Histologically of PLEVA is characterized by
epidermal necrosis, with prominent
hemorrhage and primarily a dense
perivascular infiltrate of lymphocytes in the
superficial dermis
Absence of neutrophils simplifies the
distinction between leukocytoclastic angiitis
Lymphomatoid papulosis differs by the
presence of large, atypical mononuclear
cells in the dermal infiltrate
PLEVA-Tx
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No one tx is reliably effective
Tetracycline and erythromycin are worth
trying
UVB and PUVA
Methotrexate, 2.5-7.5mg every 12 hrs for 3
doses 1 day each week
Several serious reactions a few of them
fatal have occurred with simultaneous
administration of methotrexate and NSAIDs
Dapsone and pentoxifylline(Trental), 400mg
twice daily
Parapsoriasis en Plaques
* Small-plaque parapsoriasis is characterized
by non-indurated, brownish,
hypopigmented, or yellowish red scaling
patches, round to oval, with sharply defined
borders
* Most lesions occur on the trunk and are
between
1 –5cm
* Patches may persist for years to decades
and do not progress to lymphoma
Large Plaque
Parapsoriasis
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Has patches 5-15 cm; otherwise is
similar to small-plaque type
Prognosis is benign, especially if
pruritis is severe
10% may eventuate in T-cell
lymphoma
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Large plaques
parasporiasis:
large, variably
erythematous
and mildly
poikilodermatou
s patches in the
bathing trunk
region
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Small plaque
parasporiasis:
small(<5),
erythematous,
slightly scaly
patches
Treatment
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First line: UV radiation -- either natural or
UVB
Lubricants and Topical steroids
PUVA but only if UVB fails
Use of PUVA or high-potency topical streroids
should be limited due to long-term adverse
effects
LPPP has the potential to develop lymphoma
– thus, justifying more intense tx
Vitamin D2 daily–250,000 units over 2-4
months has been effective
Pityriasis Alba
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AKA-pityriasis streptogenes, furfuraceous
impetigo, pityriasis simplex, pityriasis sicca
faciei, and erthema streptogenes
Characterized by hypopigmented, round to
oval, scaling patches on face, upper arms,
neck, or shoulders
Color is white (but never actually
depigmented) or light pink
Scales are fine and adherent
Patches are usually sharply demarcated;
edges may be erythematous and slightly
elevated
Pityriasis Alba
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Lack of any early specifically follicular
localization helps to distinguish this lesion
from follicular mucinosis
Vellus hairs are not lost in pityriasis alba, nor
does hypesthesia to cold occur, as often
happens in follicular mucinosis
Usually asymptomatic; however there may be
mild pruritis
Disease mainly occurs in children and
teenagers
It is particularly a cosmetic problem in darkskinned individuals
Pityriasis Alba
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Etiology unknown
Excessively dry skin appears to be
contributory
Most lesions disappear with time
Repigmentation can be accelerated with
treatment
Emollients and bland lubricants
Low-strength corticosteroids plus Lac-Hydrin
are helpful
Others have recommended PUVA
Pityriasis Rosea
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Mild inflammatory exanthem of unknown
origin ?viral
Characterized by salmon-colored papules and
patches which are oval and covered with a
collarette of scale
Disease frequently begins with a single herald
patch, which may persist a week or more,
then involutes
PR
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Appears rapidly and last from 3-8
weeks
Peak: ages 15-40
Typically in Spring and Autumn
More common in women
Pityriasis Rosea
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Mainly affects the trunk
Oral lesions are
relatively uncommon,
but present as
aphthous lesions
Herald Patch
Pityriasis Rosea
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Papular PR is an unusual form common in
black chidren under age 5
Inverse PR is unusual, but not rare
Relapses and recurrences are frequently
observed
A PR-like eruption can occur as a rxn to
captopril, arsenicals, gold, bismuth,
clonidine, methoxypromazine,
tripelennaminehydrochloride, or barbituates
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Inverse pityriasis rosea: oval annular
plaques in groin
Treatment
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Supportive
UVB should be used after acute
inflammatory stage has passed
Topical corticosteroids
Antihistamines
Emollients
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PR: There is focal parakeratosis, mild
acanthosis, spongiosis, perivascular
lymphocytes, and focal erythrocyte
extravasation
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PR: papules and annular plaques
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PR: oval and round plaques, some with central
scale and others with a collarette of scale
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PR in darkly
pigmented skin: it
tends to be more
papular than in
lightly pigmented
skin-note
associated
hyperpigmentatio
n
Pityriasis Rubra Pilaris
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Chronic skin disease characterized by small
follicular papules, disseminated yellowish pink
scaling patches, and often, solid confluent
palmoplantar hyperkeratosis
Disease generally manifests itself first by
scaliness and erythema of the scalp
PRP
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Involvement is usually symmetrical and diffuse,
with islands of normal
Hyperkeratosis of palms and soles called, the
“sandal”
Nails may be dull, rough, thick, and brittle
Itching in some cases
Koebner’s phenomenon may be present
A number of cases have been associated with
Kaposi’s sarcoma, leukemia, basal cell, lung,
unknown primary metastatic and hepatocellular
carcinoma
PRP
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PRP may classified into familal or acquired
types
in respect to the onset of the disease in
childhood or adulthood
Griffth’s classification: Type I, the classic
adult type, is seen most commonly, with
80% involuting in 3 years
Three types of juvenile-onset forms account
for up to 40% of cases and have a poor
prognosis for involution
PRP
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Etiology unknown??AD
Either sex affected
Possible related to
deficiency of
vitamin A
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Histology:
hyperkeratosis,
follicular plugging, and
focal parakeratosis at
follicular orifice
Inflammatory infiltrate
in dermis is composed
of mononuclear cells
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PRP: psoriasiform dermatitis with
follicular plugging
Treatment
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Symptomatic:
 Methotrexate
emollients-- Lac-Hydrin
2.5mg alternating
A several-month course
with 5mg daily
of isotretinoin in doses
 Monitor and treat
of 0.5 – 2 mg/kg/day
Vitamin A in doses of
secondary
300,00 to 500,000 untis
infections
daily, with possible
addtion of vitamin E,
400 units 2-3 times
daily
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Pityriasis rubra
pilaris: diffuse
erythroderma
with
desquamation
and follicular
hyperkeratosis
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Pityriasis rubra
pilaris: follicular
papules and
confluent orangered scaly plaques
with islands of
sparing
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Pityriasis rubra pilaris: orange-red waxy
keratoderma of the palms