Psoriasis

14
Bahar Shafaeddin Schreve
and Wolf-Henning Boehncke

Abstract
Psoriasis is a common, chronic-recurrent inflammatory dermatosis. Its
most typical clinical manifestation are well-demarked, erythemato-
squamous plaques, namely on the extensor sites of the extremities. In this
case, the diagnosis can easily be made clinically. However, this chronic
plaque-type manifestation accounts for only around 70 % of cases. There
are numerous other clinical manifestations, including inverse psoriasis
lacking scales, acute exanthematic as well as localized manifestations, and
finally pustular forms, some of which may represent pathogenetically dis-
tinct entities. In these cases, biopsies might be necessary to establish the
diagnosis and to exclude numerous differential diagnoses.
Clinical assessment of psoriasis comprises both aspects accessible by
physicians’ observation as well as patient-reported outcomes. The best-
known tool to assess chronic plaque-type psoriasis is the Psoriasis Area
and Severity Index (PASI), quantifying the involved body surface area
along with the extent of redness, infiltration, and scaling. Given its limita-
tions, numerous modifications of the PASI have been proposed. An alter-
native approach is to assess psoriasis more globally, e.g. by the Physician
Global Assessment (PGA). The most widely used tool to document
patient-reported outcomes is the Dermatology Life Quality Index (DLQI),
a 10-item questionnaire that can also be used to assess other dermatoses.

Keywords
Plaque-type psoriasis • Guttate psoriasis • Pustular psoriasis • Erythroderma •
Inverse psoriasis • Palmoplantar psoriasis • Assessment • Psoriasis area and
severity index • Physician global assessment • Dermatology life quality index

B. Shafaeddin Schreve, MD (*)

Faculty of Medicine, University of Geneva, W.-H. Boehncke, MD, MA
Rue Gabrielle-Perret-Gentil 4, Geneva 1211, Service de Dermatologie et Vénéréologie,
Switzerland Hopitaux Universitaires de Genève, Geneva
e-mail: bahar.shafaeddin@gmail.com Switzerland

© Springer International Publishing Switzerland 2016 129

A. Adebajo et al. (eds.), Psoriatic Arthritis and Psoriasis: Pathology and Clinical Aspects,
DOI 10.1007/978-3-319-19530-8_14
130
Psoriasis is among the most common skin
diseases, with a wide spectrum of clinical mani-
festations. This inflammatory skin disease is char-
acterized by its chronic- relapsing course and its
genetic predisposition. The hallmark of this dis-
ease is altered epidermal differentiation and
hyperproliferation as well as inflammation. It clas-
sically manifests by erythemato-squamous
plaques but there are also other manifestations. It
is one of the most prevalent inflammatory skin dis-
eases, affecting around 2 % of the Caucasian pop-
ulation (Europe and North America). The disease
is rarer in some genetically distinct populations
(Native Americans, African Americans, Eskimos).
Based on disease severity, family history, and
genetics, two types of plaque-type psoriasis can
be differentiated: Type I psoriasis is character-
ized by an early onset (<40 years), a severe
course, positive family history, and an HLA cw6
positive genotype, while type II psoriasis patients
have a later onset (>50 years), a milder course, a
negative family history, and no association with
HLA cw6.
The heredity of the disease is polygenetic, but
is triggered by numerous exogenous and endoge-
nous factors. Exogenous factors comprise trauma
(Koebner effect), sunburn, irritative topical ther-
apy, and mechanic trauma. Endogenous factors
are infections (streptococcal), drugs (beta-block-
ers, lithium, and chloroquine) and emotional
stress. Patients often improve during the summer
and worsen in winter, reflecting how the disease
is influenced by different environmental factors.
The role of mechanical stress on the disease is
illustrated by “Koebner’s phenomenon” (or iso-
morphic response) illustrated in Fig. 14.1, where
psoriasis skin lesions are triggered by sites of
injury or trauma. Drug-induced flaring of psoria-
sis is not completely understood. Beta-adrenergic
blockers may induce epidermal hyperprolifera-
tion by decrease of intraepidermal cyclic AMP,
lithium may increase proinflammatory cyto-
kines and stimulate leukocyte recruitment, and
chloroquine blocks epidermal transglutaminase
which is involved in terminal differentiation of
keratinocytes. Infections, especially streptococ-
cal infections of the upper respiratory tract, are
recognized triggers of psoriasis. There is also
B. Shafaeddin Schreve and W.-H. Boehncke
Fig. 14.1 Koebner Phenomenon in plaque-type
psoriasis. Typical psoriatic lesions can be triggered
through physical trauma such as scratching or cutting
(e.g. surgical procedures)
exacerbation or initial manifestation of psoriasis
in patients infected by HIV. Alcohol ingestion is
also a trigger factor.
Clinical Manifestation
The classic manifestation is plaque-type psoria-
sis, exhibiting well delineated, red, infiltrated
plaques covered with silver white scales
(Fig. 14.2). Lesions are most active at their edge,
sometimes conferring nearly an annular appear-
ance. Scales are easily removed by scratching,
and removal results in the appearance of small
blood droplets (Auspitz sign) (Fig. 14.3). This is
caused by the thinning of the epidermal layer
overlying the tips of the dermal papillae which
contain dilated and tortuous capillaries, which
bleed readily when the scale is removed. Plaques
may coalesce into polycyclic or serpiginous pat-
terns, and are usually distributed symmetrically.
Predilection sites comprise elbows and knees, the
scalp (where they usually do not extend beyond
the hairline and may cause non-scarring alopecia;
(Fig. 14.4), periumbilical and lumbar regions, but
any anatomical site may be affected, including
nails. Nail changes are common, and often con-
sist in pitting (best seen under oblique light), oil
spots (yellow-brown spots under the nail plate)
and dystrophy. Psoriatic arthritis, a seronegative
inflammatory arthritis which occurs in the
presence of psoriasis may affect up to 30 % of
14 Psoriasis 131

Fig. 14.2 Chronic plaque-type psoriasis in predilection sites, which include the lumbar (left) and periumbilical (right)
areas

Fig. 14.3 Auspitz phenomenon in chronic plaque-type psoriasis. Scales can easily be removed by gently scratching the
lesion, leaving a glossy erythematous area on which blood droplets appear rapidly

Fig. 14.4 Scalp psoriasis. The scalp is another predilec- Typically, the border of the lesion(s) goes about two fin-
tion site. Well-defined erythemato-squamous plaques are gers wide beyond the scalp
often found behind the ears or on the forehead (above).
132 B. Shafaeddin Schreve and W.-H. Boehncke

patients (discussed separately). Pruritis is In inverse psoriasis, lesions are located in

common, especially in scalp and anogenital intertriginous sites such as the groins and axilla,
psoriasis. The morphological variants of psoria- sparing the typical predilection areas. In this
sis are illustrated in Table 14.1. type of psoriasis, lesions are sharply defined,
dark red moist plaques, and lack the typical
Table 14.1 Clinical classification of psoriasis scaling (Fig. 14.5) rendering diagnosis difficult.
A pustular pattern may be present on the palms
Clinical classification of psoriasis
and soles.
Psoriasis vulgaris
Acute guttata psoriasis, is an exanthematic
Chronic plaque-type
Acute exanthematic type
inflammatory form of psoriasis (guttata meaning
Inverse psoriasis
droplet in Latin), and is relatively rare (2 % of all
Isolated psoriasis of the nails psoriasis). This form is characterized by an erup-
Psoriasis pustulosa tion of disseminated dark pink or red keratotic
Pustular palmoplantar psoriasis papules of 1–2 cm of diameter, with or without
(Königsbeck-Berber) scaling, generally appearing on the trunk (but
Acrodermatitis continua suppurativa (Hallopeau) may affect any site of the body), usually sparing
Generalized pustular psoriasis (von Zumbusch) the palms and soles (Fig. 14.6). This form occurs
Impetigo herpetiformis often preceded by a beta-hemolytic streptococcal
Pustulous psoriasis of the type of erythema annulare or a viral infection 2–3 weeks prior, especially in
centrifugum children and adolescents. It is a self-limiting dis-
Erythrodermic psoriasis ease, resolving within 3–4 months of onset, but
Psoriatic arthritis its long term prognosis is unknown. Some studies

Fig. 14.5 “Inverse psoriasis” spares the typical predilection sites and affects intertriginous areas instead. In this case,
the erythema is still sharply demarked, but scaling is usually absent
14 Psoriasis
Fig. 14.6 Guttate psoriasis is characterized by the eruptive manifestation of multiple, monomorphic, erythematous
keratotic papules, maturing into small plaques
indicated that one third of patients develop into
classic chronic plaque disease
Pustular psoriasis must be differentiated
from other pustular dermatoses. The pustules are
2–5 mm, deep seated, yellow, develop into red-
dish macules and crusts, and are present in areas
of erythema and scaling and normal skin. The
lesions are not associated to hair follicles, and are
always sterile. This is a rare form of psoriasis
which occurs in adults, and rarely in children.
There are known precipitating factors and
patients may or may not be known for stable
plaque-type psoriasis. Pustular psoriasis is cate-
gorized in localized forms and generalized
disease.
Generalized pustular psoriasis (von
Zumbusch) is an acute form of psoriasis with
abrupt onset of disseminated small, monomor-
phic inflammatory sterile pustules in painful
inflamed skin. The pustules may evolve into
major bullae. Patients often show signs of sys-
temic inflammation such as fever. Triggers of
generalized pustular psoriasis are infections,
abrupt withdrawal of systemic treatment, and
sometimes withdrawal of potent topical
corticosteroids. Severe forms of generalized
pustular psoriasis may also affect the oral cav-
133
ity (stomatitis geographica, stomatits areata
migrans, lingua geographica). These patients
are often seen in an emergency setting due to
their acute symptoms. Laboratory examina-
tions show a polymorphonouclear leukocytosis
with white blood cells reaching 20,000/
μL. Bacterial cultures of tissue show sterile
pustules. Blood cultures should be performed
because of the risk of superinfection, particu-
larly with S. aureus. Generalized HSV infec-
tion must be ruled out with Tzanck tests and
viral cultures must be established. Generalized
pustular drug eruptions (acute generalized
exanthematous pustulosis) may have a similar
presentation. The course of this disease is char-
acterized by relapses and remissions over a
period of years. It may precede or be followed
by psoriasis vulgaris. The prognosis may be
dire in the elderly.
Palmoplantar pustular psoriasis consists of
yellow-brown sterile pustules located on the
palms and soles. a quarter of patients with PP
psoriasis also have chronic plaque psoriasis. This
form of disease has a different demographic (pre-
dominating in women with a 9:1 ratio, more fre-
quent in current or previous smokers (95 %) and
a later onset (fourth and fifth decades) and differ-
134
Fig. 14.7 Erythrodermic psoriasis. Psoriasis is one of the dermatoses which can cover the complete skin, resulting in
the clinical picture of erythroderma
ent causes. A severe manifestation of localized
pustular psoriasis is acrodermatitis continua sup-
purativa affects toes and fingers and confluent
pustules, primarily in the area of nails, forming
major bullae. This disease is persistent for years
and characterized by periods of remission and
exacerbations. It may result in loss of finger and/
or toe nails.
The differential diagnosis of pustular psoriasis
comprises fungal infections, dyshidrotic eczema-
B. Shafaeddin Schreve and W.-H. Boehncke
tous dermatitis, irritant or allergic contact derma-
titis, herpes simplex virus (HSV) infection (if
localized in one site).
Erythrodermic psoriasis is defined as pso-
riasis affecting the whole body surface
(Fig. 14.7). It may arise from any type of psoria-
sis and may develop progressively or acutely.
Factors inducing erythroderma are irritating
treatments, sunburns, discontinuation of steroid
therapy. This is potentially a life-threatening
14 Psoriasis 135

condition with complications such as protein Table 14.2 Differential diagnosis of psoriasis
loss, impaired thermoregulation, electrolyte Manifestations of
loss, impaired response, superinfection, and psoriasis Differential diagnosis
cardiac failure. Psoriasis vulgaris Bowen’s disease
Parapsoriasis en plaques
Mycosis fungoides
Nummular ecznema
Differential Diagnosis CDLE
Tinea
Typical chronic plaque-type psoriasis affecting Reiter’s disease
Seborrhoic dermatitis
the classical predilection sites can easily be rec-
Acute exanthematic Syphilis II
ognized. The differential diagnosis comprises type Psoriasiform drug reaction
seborrheic dermatitis, seborrhiasis, and lichen HIV exanthema
simplex chronicus. Besides, psoriasifrom drug Pityrisis lichenoides chronica
Irritated pityriasis rosea
eruptions, caused by drugs such as beta blockers,
SCLE
gold, and methyldopa, are also to be considered.
Isolated psoriatic Tinea
Tinea corporis, often showing much smaller plaque Eczema
scales, can quickly be excluded with a KOH CDLE
examination. Mycosis fungoides may sometimes Psoriasiform lupus vulgaris
Pagetoid reticulosis
mimick plaque-type psoriasis, as it may present
Basal cell carcinoma lichen
with scaly plaques. Typically plaques in the case simplex chronicus
of mycosis fungoides are ovally shaped and Isolated psoriasis of Tinea amiantacea
localized on the trunk Rather than the typical pre- the scalp Seborrrhiasis
dilection sites of psoriasis. Inverse psoraisis Intertrigo
Acute rashes consisting of erythemato- Candidosis
Extramamary Paget’s disease
squamous lesions in a patient with positive per-
Generalized Acute generalized pustulosa
sonal or family history are also easy to diagnose. pustular psoriasis
The presence of an Auspitz phenomenon is also a Pustular Hand –and –feet dermatitis
helpful clue to this diagnosis. palmoplantar Palmoplantar pustulosis
When the lesions are less typical, it may be psoriasis
difficult to diagnose the disease. One must search
for subtle history (i.e. medication, recent infec-
tions), perform further diagnostic tests (syphilis Table 14.2 summarizes the most important
serology, HIV) and a biopsy may be required. differential diagnoses of psoriasis.
One must always take into account the possibility
of drug-induced psoriasis, but in the absence of
distinct patient and family history of psoriasis, it Assessment Tools for Psoriasis
may be difficult to distinguish a drug-induced
psoriasis from a psoriasiform drug eruption. Accurate and reliable assessments tools are
The differential diagnosis of acute guttata pso- important to document the severity of psoriasis
riasis comprises maculopapular drug eruption, and to quantify the effect of the treatment.
secondary syphilis, and pityriasis rosea, while in Historically, the extent of the lesions as well as
the case of inverse psoriasis fungal infections, redness, infiltration, and scaling were assessed,
Hailey-Hailey, intertrigo, and extramammary eventually leading to the development of the
Paget disease should be considered. Glucagonoma Psoriasis Area and Severity Index (PASI, see
syndrome is an important differential as lesions below). The PASI is often used in the context of
are similar to inverse psoriasis. Nail psoriasis clinical trials, while many practicing dermatolo-
might at times mimick onychomycosis, as it may gists find it impractical for use in the daily prac-
cause onychodystrophy. tice. Thus, alternative measures such as the extent
136
of the lesions in percent of the body surface
(Body Surface Affected – BSA), or global assess-
ments (Physician Global Assessment – PGA) are
widely used.
In recent years, the importance of the patients’
perspective became increasingly acknowledged.
Thus, tools to document patient reported out-
comes (PROs) were developed, with the
Dermatology Life Quality Index (DLQI) being
currently the most widely used tool for this pur-
pose in the indication of psoriasis.
In many countries, the combination of PASI,
BSA, and DLQI is being used as a reference
point to classify psoriasis as being either “mild”
or “moderate-to-severe”, with the cut-off being a
PASI, BSA, or DLQI of 10 (“rule of 10s”).
The PASI is currently the most widely used
assessment tool in the context of clinical trials.
Many trials look at a 75 % reduction in this score
as the primary end point (PASI75). It takes into
account the extent of the lesions, along with red-
ness, infiltration, and scaling. Head, arms, trunk,
and legs are being assessed separately.
The body surface area is commonly estimated
using the rule of nines: In an adult, the head
accounts for 9 % of the body surface area, each
arm represents 9 %, the front of the trunk is 18 %,
the back is 18 %, and each leg accounts for 18 %
of the body surface area. When assessing the per-
cent of affected body surface, the patient’ hand
can be used as a hint, with the palm correspond-
ing to approximately 1 % of the patient’s body
surface. Once the percentage of the affected body
surface is established for a given anatomic site,
the percentage is transformed into a numerical
score. The severity of redness, infiltration, and
scaling is done based on a scale from “0” to “3”,
corresponding to “none”, “mild”, “moderate”,
and “severe”. Finally, the results for the different
anatomical sites are “weighted”, using a multi-
plier, the addition of the resulting numbers gives
the final absolute PASI. To facilitate the calcula-
tion of the PASI, score sheets, calculators, and
even aps for smartphones exist.
The PASI shows considerable intra-rater and
inter-rater variability, the former often diminish-
ing with experience, while the latter can be
reduced through vigorous PASI training. The
B. Shafaeddin Schreve and W.-H. Boehncke
Table 14.3 Example of a 6-point static Physician Global
Assesment (PGA)
Physician Global Assessment (PGA)
5 severe Very marked plaque elevation,
scaling, and/or erythema
4 moderate to Marked plaque elevation, scaling,
severe and/or erythema
3 mild to Moderate plaque elevation, scaling,
moderate and/or erythema
2 mild Slight plaque elevation, scaling, and/
or erythema
1 almost clear Intermediate between mild and clear
0 clear No signs of psoriasis
(postinflammatory
hyperpigmentation may be present)
domain usually showing the largest variability is
the estimation of the area component. Additional
limitations of the PASI include its low power of
discrimination in mild psoriasis and its limitation
to plaque-type psoriasis. Moreover, affection of
different anatomical sites is not weighted with
regard to the significance for the patient:
Involvement of visible areas such as hands or
face is assessed in the same way by the PASI as
sites that can easily be covered by clothes (such
as the trunk), but the impact on the patient is
different.
Given the limitations of the PASI on one hand
and the concerns of practicing dermatologists to
use the PASI as part of their daily practice on the
other hand, different types of Physician Global
Assessment (PGA) were developed. Principally,
static and dynamic scales can be differentiated,
the latter aiming at assessing improvement of
psoriasis over time. Numerous static PGAs have
been developed, where the investigator assigns a
single estimate of the patient’s overall severity of
disease on a 5, 6 or 7 point ordinal rating ranging
from clear to very severe psoriasis. Table 14.3
gives an example of a 6-point static
PGA. Generally, this method is a more intuitive
one and does not integrate plaque morphology or
BSA.
The Lattice System Physicians’ Global
assessment tool (LS-PGA) was developed to
address deficiencies in other assessments of pso-
riasis: The PASI can be difficult to use and has no
clear clinical frame of reference, while numerous
14 Psoriasis
PGA systems exist in parralel. The LS-PGA
addresses these deficiencies by providing a clear
method of rating and clinical description of the
results in an eight step method. This system has a
more quantitative approach to global assessment
of disease severity as it integrates the BSA
involved and the plaque morphology. It gives
more weight to plaque elevation than to scale or
redness, which is not the case in the PASI. This is
due to the fact that scaling and redness may vary
with environmental conditions and other factors
(emollients, ambient conditions). Plaque eleva-
tion is given more weight as it may be more
related to inflammation and proliferation, hall-
marks of psoriasis activity.
PASI, PGA, and LS-PGA show high correla-
tions with each other. The LS-PGA has the
advantage of having a better reproducibility from
one session to the next than the PGA and less
variation from one physician to another when
compared to the PASI.
None of the above-mentioned assessment
tools take into account the patient’s perspective.
The currently most widely used tool to document
the latter is the Dermatology Life Quality Index
(DLQI). This tool was developed as a simple
compact and practical questionnaire and has been
shown to be reliable and valid in dermatology
clinical settings. It contains ten questions, the
137
answers of the patients yielding between 0 and 3
points. The score ranges from 0 to 30, with 0
being the best score documenting absence of any
burden of disease, and 30 documenting the maxi-
mum burden of disease. A change of five or more
points is considered to be clinically meaningful.
It is suggested that a DLQI below five should be
a treatment goal in psoriasis. The DLQI is
intended for use in dermatology at large and
therefore does not specifically focus on psoriasis.
Other questionnaires evaluating perception and
social and emotional impact of a disease, such as
the SF 36 or EuroQOL 5D, can generally be used
in all clinical specialties, but their use to assess
psoriasis in particular is often limited to clinical
studies.
In recent years, several experts have suggested
additional tools to document patient reported out-
comes, e.g. the Patient Benefit Index (PBI). It
will be important to carefully validate and evalu-
ate the new tools, as well as potential new tools
attempting to “objectively” measure disease
activity and severity in psoriasis. Ideally, this
should be done through a rigid, well-defined pro-
cess. A good example for a comprehensive
approach to develop clinical assessment tools is
OMERACT in rheumatology. With IDEOM,
there is now an initiative aiming at implementing
a similar approach in dermatology.