CHAPTER 21 has a great impact on life plaque psoriasis, not,

Pustular Eruptions quality and however, to ppp2

of Palms and Soles the ability to work. investigation
Ulrich Mrowietz Epidemiology of the apolipoprotein E
Pustular eruptions of the PPP has a worldwide alleles
palms and distribution. It is a e2, e3, and e4 in chronic
soles include palmoplantar rare condition, but the exact plaque and guttate
pustulosis incidence is psoriasis as well as in PPP in
(PPP), acrodermatitis not known. acitretin
continua (Hallopeau Females show a higher responders and
disease), and infantile prevalence nonresponders showed
acropustulosis than males, with a ratio of that the frequency of the e4
(Table 21-1). The entities approximately allele was significantly
present 3: 1. Onset of the disease higher in the psoriasis groups
with chronic and persistent occurs but not in PPP patients as
eruptions of mostly between the ages of compared to
sterile, purulent vesicles. 20 and 60 healthy controls.3 in chronic
• PALMOPLANTAR years; rarely, the condition plaque psoriasis
PUSTULOSIS occurs after and psoriatic arthritis an
PPP is a chronic pustular the sixth decade of Hfe, and association
dermatosis In 10 percent with tumor necrosis factor
localized on the palms and of the patients the onset is (TNF)-a-238
soles only. before and -308 promotor
High resistance to treatment the age of 20 years. polymorphisms have
and a been found' however, the
high recurrence rate are Genetics association has
characteristic. HLA typing of patients with oot been found in P1'1'.4
Histologically, it is PPP reveals Studies from Japan provide
characterized by no increased frequency of evidence
intraepidermal vesicles filled any of the for phenotypic and genetic
with known psoriasis-linked heterogeneity
neutrophils. alloantigens. 1 In of PPP according to its
Previously, PPP was a direct comparison among association!
classified as a chronicplaque provocation with tonsillitis.
form of pustular psoriasis and psoriasis, guttate psoriasis, In patients
many and in which PPP was not
textbooks describe PPP in PPP, the three major associated with
their respective candidate genes tonsillitis, the phenotype
chapter of psoria is. Today, within the PSORSl region frequency of
PEP fs (HLA-Cw~6, the TNF-~2 allele of the
regarded as its own entity. HCR~WWCC, and CDSN'5) TNF-~ gene and
The involvement of palms showed a of the allele B of the TNF-a
and soles high association to guttate gene (TNF-a.
and chronic
pB) was significantly higher smoking. In two Swedish percent of patients. I?
as compared surveys, 95 Scintigraphic investigations
to controls.s percent of 1'1'P patients were showed stemocostoclavicular
These findings further smokers at joint involvement to be
substantiate onset of disease and cessation present in 16
the notion that PPP and of smoking of 73 (22 percent) patients. IS
psoriasis represent was the most important For this
different entities. measure to condition, the term SAPHO
Etiology treat the disease.tO,ll There is (synovitis,
The cause of 1'1'1' is not evidence acne, pustulosis,
known. An imbalance from immunohistochemical hyperostosis, osteitis)
of the protease/antiprotease studies that has been established. 19
system nicotinic acetylcholine Clinical manifestations
in the skin consisting of receptors are of SAPHO syndrome are
decreased modulated in lesJonal skin similar
antileukoprotease from smoking whether they occur in relation
(ela6nJSKALP) activity PPP patients when compared to severe
in pustular psoriasis has been to mokers acne (mostly acne
discussed and healthy controls conglobata) or PPP
as a possible mechanism of suggesting an abnormal (see Chap. 78). The primary
pustule response to nicotine in lesion consists
formation. 6 Exacerbation of PP1'.12 of a sterile abscess containing
1'1'1' has Case reports have revealed neutrophils.
been observed after patch multiple The site of predilection is the
testing with provocation factors of 1'1'1' anterior chest wall. SAPHO
metals and was accompanied including syndrome
by elevated thyroid-diseases,13 can be associated with
leukotriene B4 levels in Ilelicob(l(ler pylori infection, pseudoinfectious
plasma l4 and psychologic factors arthritis. Involvement of the
and pustules? such sacroiliacal
In a long-term survey from as anxiety.15 joints may be present.20
Japan, the PPP is also seen in patients
incidence of PPP was found Disease Associations with
to be pOSitively An association of PPP and chronic recurrent multifocal
correlated to heavy smoking osteomyelitis as well as with
osteoarthritis
(more than 20 cigarettes per of the anterior chest wall was nOlUnfectious inflammatory
day), tonsillitis first described bone lesions.
and seasonal factors such as in Japan. 16 As reported by
high Swedish Clinical Features
humidity and high authors, involvement of the
temperature.a.9 manubriostemal The primary lesions are
The most striking association joint is present in 6 percent pustules of
in 1'1'1' is and of the sternoclavicular nearly equal size measuring
joints in 10 two to four
mm in diameter. Crops of However, in severe eruptions, The lesions of PPP are
pustules usually pain and sterile; a moderately
arise within a few hours on the inability to stand, walk, or increased white blood cell
the typical- do manual count
appearing palmar and plantar work may greatly reduce the may occasionally be ob
skin quality erved, but all other laboratory
(Fig. 21-1). Involvement of life. As remission begins, tests are usually normal.
usually is symmetric fewer pustules In patients with an infectious
but unilateral location on are produced, but the skin trigger,
palms may remain infection-associated
and/or soles is seen. Single erythematous and laboratory pa~
lesions then hyperkeratotic, rameters, such as C-reactive
become surrounded by an resembling eczema. protein,
erythematous Remissions last for may be increased. Increased
ring. Sometimes, the pustules days, weeks, or months until levels of
extend pustulation anti-gliadin antibodies may
to the dorsa of the fingers, the again occurs. occasionally
feet, or over the volar wrists be found.
(see Fig. Histopatology
21-1C). Episodes of new Diagnosis and Differential
Histologically, there is an Diagnosis (Box 21-1)
pustular eruptions
intraepidermal PPP is a distinct entity. The
occur at varying intervals and cavity 6lled with
remain course of the
polymorphonuclear disease, together with the
strictly confined to the sites leukocytes associated with
of characteristic
spongiform morphology, permits the
predilection. changes within the
As pustules become older, proper diagnosis.
surrounding epidermis The disease must be
their yellow (Fig. 21-2). Eosinophils and
color changes to dark brown, differentiated
mast from Mdyshidrotic~
so cells are present in increased
that in untreated PPP, the eczematous dermatitis
numbers in (pompholyx), especiaUy
lesions show PPP biopsies from lesional
various shades of color (see when pustules
skin. Another due to secondary infection
Fig. 21-1C hallmark is the inability to
and D). Dried pustules are are
visualize the present (see Chap. 16). In
shed within epidermal part of the eccrine
approximately 8 to 10 days. that condition,
duct in PPP the onset is also acute, but
There are no symptoms other specimens indicating an
than clear vesicles
involvement of of various sizes are scattered
itching or a burning the acrosyringium.21
sensation, which on the
may precede new crops of laboratory finding palms, soles, and volar and
lesions. interdigital
aspects of the fingers. These diet was found to have a a therapeutic effect,
may coalesce beneficial particularly when
and secondarily become effect on lesions in psoriasis combined with these
pustular and PPP.14 compounds on an
because of secondary Among topical treaoncnts every second- or third~day
bacterial infection. only a few basis; this
Pustular variants of tinea of procedures have bt:en may also delay cutaneous
the palms identified as being side effcctS.
and soles or pusrules of therapeutic bene£it.:z5 Psoralen and ultraviolet A
developing in infected Unilateral involvement light
scabies may resemble PPP. and/or PPP ""Jith a limited (PVVA) treatment has been
Bacterial effect on used alone
cultures or demonstration of daily living activities should or in combination with
hyphae be treated systemic retinoids
or mites dearly separate these with topical agents. Due to and found to be effective.
entities the fact that PUVA bath or PUVA with
from PPP. skin penetration through application
Rare clinical variants such as palmar and plantar of the photosensitizer in an
vesicopustular skin is highly restricted even ointment or
mycosis fungoides palmaris in acute gel formulation s.howed
et disease with focal skin barrier beneficial effects
plantaris,22 pustular disruption in case-controlled studies.26
localized vasculitis, only certain drugs can be In disabling PPP and in PPP
23 or palmoplantar used effectively. with a high
involvement of Potent and superpotent frequency of relapse systemic
generalized pustular psoriasis steroids are the therapy is
may also drugs of choice and may be preferred. A systematic
resemble PPP. used under review found retinoids
plastic film or hydrocolloid to be the only drugs with
Treatment occlusion, particularly proven
PPP is difficult to treat and in the beginning of thCl<lpy, efficacy. However, as there is
aU reporred Subsequent topical therapy a female
treatments have a high with vita~ preponderance in PPP, the
recurrence rate. min D3·analogues such as use of systemic
The management of PPP is calcipotrioll retinoids is restricted at least
summarized calcipotriene, tazarotene, or in women
in Box 21-2. anthralin with childbearing potential.
The first measure in the may prevent a quick relapse Other sys~
management in some patients. temic agents with efficacy are
of PPP is cessation of However, every prolonged methotrexate
smoking. In patients topical (lO to 25 mg/week) and
with proven gluten steroid use is usually needed cyclosporine.
intolerance, a glutenfree to maintain It has been shown that
cyclosporine is effective
in a low-dose regimen (1 to 2
mgt
kg/day) over a I-year
period.17 Systemic
steroids are of questionable
value. Withdrawal
is usually followed by a
rebound.
Efficacy of fumaric add ester
therapy in
PPP has been decribed,l$
There is debate as to whether
TNF-o:.
antagonist~ may be
beneficial in PPP.
Whereas in chronic plaque
type psoria~
sis and generalized pustular
psoriasis
the anti-TNF 0: monoclonal
antibody infliximab
was found to be highly
effective,
this agent was found to be
both of
beneficial effect and to
worsen PPP.29.30
In the SAPHO syndrome,
infliximab led
to a complete remission of
osteoarticular
disease but PPP deteriorated
during
treatment.:>! Efalizumab, an
anti-CDlla
monoclonal antibody, seems
to be beneficial
in PPP, but experience is
limited.