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Standard grading system for rosacea: Report of the
National Rosacea Society Expert Committee on the Classification and Staging of Rosacea
Jonathan Wilkin, MD Chairman (a)
Mark Dahl, MD (b)
Michael Detmar, MD (c)
Lynn Drake, MD (c)
Matthew H. Liang MD, MPH (d)
Richard Odom, MD (e)
Frank Powell, MD (f)
Sections
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- From the Division of Dermatologic and Dental
Drug Products, Food and Drug Administration, Rockville, Maryland (a);
Department of Dermatology, Mayo Clinic Scottsdale, Arizona (b);
Department of Dermatology, Harvard Medical School, Boston, Massachusetts (c);
Department of Medicine, Harvard Medical School, Boston, Massachusetts (d)
Department of Dermatology, University of California San Francisco (e);
and Regional Centre of Dermatology, Mater Misericordiae Hospital, Dublin. (f)
- The opinions set forth in this report are those of the committee members and do not represent the Food and Drug Administration in any way.
- Reprint requests: The National Rosacea Society,800 S Northwest Highway, Suite 200, Barrington, IL 60010
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J Am Acad Dermatol 2004;50:907-12.
- 01909622
- doi:10.1016/jaad.2004.01.048
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A standard classification system for rosacea was published in the April 2002
issue of the Journal of the American Academy of Dermatology.[1]
Developed by the National Rosacea Society Expert Committee on the Classification
and Staging of Rosacea and reviewed by rosacea experts worldwide, it describes
primary and secondary features of rosacea and recognizes 4 patterns of signs and
symptoms, designated as subtypes.
To enhance the utility of the system for both clinicians and researchers, the
committee has devised a standard method for assessing gradations of the severity
of rosacea. In addition to the classification system, a standard grading system
is often essential to perform research, analyze results, and compare data from
different sources, and in turn provides a common reference for diagnosis,
treatment, and assessment of results in clinical practice.
[2] and [3].
Standard parameters and terminology also facilitate clear communication among a
broad range of basic, clinical, and other researchers; practicing
dermatologists; primary care physicians; ophthalmologists and other specialists;
health and insurance administrators; and patients and the general public.
The standard grading system rates the primary and secondary features of
rosacea established by the standard classification system, and provides a global
assessment of subtypes by both the physician and the patient. Beyond clinical
manifestations, additional factors are important in determining the severity of
rosacea from the patient's viewpoint. These may include the psychological,
social, or occupational effects of the disorder, [4]
and other potential factors such as responsiveness to treatment.
For optimal utility, the grading system is designed to be reproducible and
easily performed based on observation in clinical practice, while forming a
consistent framework for more comprehensive measurements that may be developed
for specific research studies. Moreover, as with the standard classification
system, this grading system is an investigative instrument that can be readily
modified based on clinical experience or updated and expanded as new discoveries
are made.
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Rosacea is a chronic cutaneous disorder affecting primarily the convexities
of the central face (cheek, nose, chin, and central forehead). It is a syndrome
or typology encompassing various combinations of signs and symptoms. In most
cases, some rather than all of these features appear in any given patient, and
they are often characterized by remissions and exacerbations.
[5] and [6].
The committee based the standard classification system on current scientific
knowledge and morphologic characteristics to avoid assumptions on pathogenesis
and progression, which are at present incompletely understood. As knowledge
increases, the definition of rosacea may ultimately be based on causality rather
than on morphology alone.
The committee first identified primary and secondary features of rosacea, and
then delineated subtypes based on the most common patterns or groupings of these
features. The primary signs of rosacea include flushing (transient erythema),
nontransient erythema, papules and pustules, and telangiectasia. The presence of
one or more of these features with a central face distribution is indicative of
rosacea. Secondary features, which often appear with one or more of the primary
features but can occur independently, include burning or stinging, plaques, dry
appearance, edema, ocular manifestations, peripheral locations, and phymatous
changes.
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For clinicians assessing patients, primary signs and symptoms may be graded
as absent, mild, moderate, or severe (0-3), and most secondary features may be
graded simply as absent or present (Table I). Researchers are encouraged to provide more detailed assessments. In some
situations, more detailed or finer distinctions, perhaps supplemented by
advanced technology, might be possible. Certain clinicians also may wish to use
some of these other more comprehensive analytic methods, especially when based
on visual observation.
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Table I. Rosacea clinical scorecard
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Primary features
Flushing (transient erythema)
Clinically, physicians should determine the presence or absence of flushing
through patient history, and may ask about frequency, duration, extent, and
severity. Noting the presence or absence of accompanying sweating may also be
helpful. Perimenopausal flushing should not be considered significant unless it
is accompanied by other characteristics of rosacea.
Researchers may grade flushing from 0 to 3 based on intensity and frequency.
In addition, duration of flushing may be noted, because some episodes are very
transient (eg, from embarrassment) and some are not (eg, from ingestion of
alcohol). Specific time frames may also be identified.
Nontransient erythema
For clinicians, nontransient (persistent) erythema may be graded from 0 to 3.
Although inflammation (papules, pustules, plaques) or dry appearance may obscure
the level of erythema, underlying redness should be evaluated disregarding this
effect. Inflammation or dry appearance may be noted, but perilesional erythema
should not be included in this assessment.
In clinical studies, researchers may use instruments or other measurements to
score erythema beyond a score of 0 to 3. For example, erythema may be assessed
objectively with an appropriate device.
Papules and pustules
A modified version of the descriptive grading system established for acne
vulgaris is recommended and shown in Table II. [7]
Few to several papules and pustules, with no plaques, are scored as "mild."
Several to many papules and pustules, with no plaques, are considered
"moderate." Numerous and/or extensive papules and pustules, with or without
plaques, are considered "severe."
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Table II. Severity grading of rosacea papules and pustules
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Researchers should record the number of papules and pustules, and note the
presence or absence of plaques.[1]
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Telangiectasia
Telangiectasia may be graded in the clinical setting from 0 to 3. If erythema
is intense, it may be difficult to definitively score telangiectasia, because
erythema may mask some telangiectases, which become more visible if redness
fades. This phenomenon has been described as posterythema-revealed
telangiectasia.[5] On the other hand,
the presence of one or two isolated telangiectases in the absence of any other
primary signs of rosacea may be insufficient for a diagnosis.
Researchers also should count telangiectases, if feasible, at least in
specified areas. Nasal and malar telangiectases should be identified
independently, and be qualitatively described as fine and threadlike to coarse.
Secondary features
Burning or stinging
In the clinical setting, burning or stinging may be reported by the patient
and, if present, may be weighed into the overall assessment of severity.
Researchers should seek out this information, record the locations of both
symptoms if present, and use a systematic method of assessing both symptoms.
Plaques
In clinical practice, plaques may be noted. Plaques may be defined as
confluent areas of inflammation, often seen as larger red areas among papules
and pustules without epidermal changes in the surrounding skin. In research
studies, they may be further differentiated by severity, location, or other
criteria.
Dry appearance
In clinical practice, rough, dry-appearing skin may be noted. In research,
this may also be stratified based on such criteria as distribution and severity.
If scaling is noted, it may represent coexisting seborrheic dermatitis or
irritation.
Edema
In clinical practice, edema may be identified by location (eg, periorbital,
glabellar, malar) through patient history and examination. If present, it may be
noted as acute, chronic recurrent, or chronic persistent and, if chronic, as
pitting or nonpitting. Researchers may assign a grade of 0 to 3 according to
extent and degree of swelling.
Ocular manifestations
Clinicians may identify ocular manifestations by looking for tearing, redness
of bulbar and/or palpebral conjunctivae, telangiectasia of conjunctiva and lid
margin, lid or periocular erythema, or styes, and by inquiring about symptoms of
foreign-body sensation, gritty feeling, burning, stinging, itching, dryness,
light sensitivity, blurred vision, or decreased visual acuity.
[8] Cases that are moderate to severe, progressive, or
not responding to treatment, or where vision is affected, may require an
ophthalmologic consultative approach. Treatment of cutaneous rosacea alone
may be inadequate to reduce the risk of vision loss.
[9]
Researchers may wish to stratify the ocular manifestations as mild
(signs/symptoms affecting eye margin, meibomian gland), moderate (signs/symptoms
affecting inner lid, fluid secretion, eye surface), or severe (corneal damage
and potential vision loss).
Peripheral location
Clinicians and researchers may determine the presence of any extrafacial
signs and symptoms, and note the anatomic sites. Common extrafacial locations
may include the neck, chest, scalp, ears, and back. The diagnosis of rosacea in
locations other than the face may be problematic in the absence of diagnostic
clinical or histologic features.
Phymatous changes
In the clinical setting, severity may be rated from 0 to 3, with 1 being
patulous follicles but no contour changes, 2 being a change in contour without a
nodular component, and 3 indicating a change in contour with a nodular
component. Researchers may also note any vascular findings or inflammatory
changes.
Global assessment of subtypes
Because the potential manifestations of rosacea are so numerous and varied,
the committee concluded that global assessment can be most easily and
meaningfully performed by subtype. The standard classification system
established the following subtypes of rosacea, which are described in depth in
the standard classification system.[1]
The following descriptions include the minimum signs and symptoms required to
diagnose each subtype, and patients may have characteristics of more than
one rosacea subtype at the same time.
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Subtype 1: erythematotelangiectatic rosacea
Subtype 1 (Fig 1) is characterized by flushing and persistent central facial erythema.
Telangiectases are common but not essential for the diagnosis.
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Fig 1. Subtype 1, erythematotelangiectatic rosacea, is characterized by
flushing and persistent central facial erythema. Telangiectases are common but
not essential for diagnosis. A, Mild; B,
moderate; C, severe.
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Subtype 2: papulopustular rosacea
Subtype 2 (Fig 2) includes persistent central facial erythema with transient papules,
pustules, or both in a central facial distribution. Burning and stinging may
also be reported.
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Fig 2. Subtype 2, papulopustular rosacea, includes persistent central
facial erythema with transient papules, pustules, or both in central facial
distribution. A, Mild; B, moderate;
C, severe.
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Subtype 3: phymatous rosacea
This subtype (Fig 3) may include thickening skin, irregular surface nodularities, and
enlargement. Phymatous rosacea occurs most commonly as rhinophyma but may appear
elsewhere, including the chin, forehead, cheeks, and ears. Patulous, expressive
follicles may appear in the phymatous area, and telangiectases may be present.
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Fig 3. Subtype 3, phymatous rosacea, may include thickening skin, irregular
surface nodularities, and enlargement. Patulous, expressive follicles may
appear in phymatous area, and telangiectases may be present.
A, Mild; B, moderate; C,
severe.
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Subtype 4: ocular rosacea
Ocular rosacea (Fig 4) may include watery or bloodshot appearance (interpalpebral conjunctival
hyperemia), foreign-body sensation, burning or stinging, dryness, itching, light
sensitivity, blurred vision, telangiectasia of the conjunctiva and lid margin,
or lid and periocular erythema. Blepharitis, conjunctivitis, and irregularity of
the eyelid margins also may occur. Meibomian gland dysfunction presenting as
chalazion, or chronic infection as manifested by hordeolum (stye), are common.
Some patients may experience loss of vision as a result of corneal complications
(punctate keratitis, corneal infiltrates, ulcers, or marginal keratitis). An
ophthalmologic consultative approach to treatment may be required.
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Fig 4. Subtype 4, ocular rosacea, may include watery or bloodshot
appearance, telangiectasia of conjunctiva and lid margin, or lid and
periocular erythema. Blepharitis, conjunctivitis, and irregularity of eyelid
margins also may occur. A, Mild; B,
moderate; C, severe.
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For clinicians, global assessment for each subtype should be performed with a
standard rating of 0 to 3, based on a composite of the severity of the signs and
symptoms. The evaluation may also take into consideration the duration of signs
and symptoms through patient history, and their extent at time of examination.
For researchers, additional detail and assessment technology may be added beyond
the basic rating system to provide further data and precision.
The committee noted that the ultimate goal of diagnosis and treatment of
rosacea is both to control the disorder and to minimize the discomfort of the
patient. Patient participation in evaluation is, therefore, essential. The
patient may provide a 0 to 3 global assessment of the severity of their
condition in general terms that encompasses both the physical manifestations of
rosacea and its impact on quality of life, which may include psychological,
social, and occupational effects.
Patients might be informed of potential primary and secondary features of
rosacea before their global assessments to aid them in evaluating their
individual conditions more thoroughly. Of particular concern is ocular rosacea,
which patients may not associate with cutaneous rosacea and that may require
further evaluation.
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In developing a standard grading system for rosacea, the committee attempted
to design a basic examination process that is practical, useful, and similar to
the usual examinations currently performed in clinical practice. To aid
clinicians in evaluating their patients, the committee has developed a standard
diagnostic flow chart (Table I). Superimposed on this basic
standard system, researchers are encouraged to study and explore features beyond
the minimum, using more sensitive and reproducible systems and applying new
technology and methodologies that may further advance the scientific knowledge of rosacea.
This investigational instrument is intended to help provide a foundation for
better understanding of rosacea among practitioners and researchers by
establishing a common language for communication and facilitating the
development of a research-based approach to diagnosis and treatment. The
scorecard (Table I) is included for those who wish to have a
more detailed investigative record of the patient's disorder.
As with the standard classification system, this grading system is considered
provisional and is subject to modification as the pathogenesis and subtypes of
rosacea become clearer, and as its relevance and applicability are tested by
investigators and clinicians. The National Rosacea Society Expert Committee
welcomes comments on the usefulness and limitations of these criteria.
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The committee thanks the following individuals who reviewed and contributed
to this document: Dr Joel Bamford, Department of Dermatology, St Mary's/Duluth
Clinic, Duluth, Minnesota; Dr Mats Berg, Department of Dermatology, Uppsala
University, Uppsala, Sweden; Dr Joseph Bikowski, Department of Dermatology,
University of Pittsburgh, Pittsburgh, Pennsylvania; Dr Albert Kligman,
Department of Dermatology, University of Pennsylvania, Philadelphia,
Pennsylvania; Dr Ronald Marks, Department of Dermatology, University of Wales
Medical Center, Cardiff, United Kingdom; Dr Gerd Plewig, Department of
Dermatology, Ludwig-Maximilians University, Munich, Germany; Dr Bryan Sires,
Department of Ophthalmology, University of Washington, Seattle, Washington; Dr
Diane Thiboutot, Department of Dermatology, Pennsylvania State University,
Hershey, Pennsylvania; Dr Guy Webster, Department of Dermatology, Thomas
Jefferson University, Philadelphia, Pennsylvania; and Dr Mina Yaar, Department
of Dermatology, Boston University, Boston, Massachusetts. The final document
does not necessarily reflect the views of any single individual, and not all
comments were incorporated.
The National Rosacea Society is a 501(c)(3) nonprofit
organization whose mission is to support rosacea research, including the
awarding of research grants, and to provide educational information on rosacea
to physicians, patients, and the public. Reports or inquiries should be directed
to the National Rosacea Society, 800 S Northwest Hwy, Suite 200, Barrington, IL
60010; telephone 1-888-662-5874; E-mail: rosaceas@aol.com.
1.
Wilkin J, Dahl M, Detmar M, Drake L, Feinstein A, Odom R, et al. Standard
classification of rosacea: report of the National Rosacea Society expert
committee on the classification and staging of rosacea. J Am Acad Dermatol
2002;46:584-7.
2. C.E. Gessert and J.T.M. Bamford, Measuring the severity of rosacea: a review.
Int J Dermatol 42 (2003), p. 444.
3.
Henderson CA, Charles-Holmes S, McSween R, Ilchyshyn A. A system for grading
rosacea severity. Br J Dermatol 1995;133(Suppl):34
4.
Drake L. Rosacea takes emotional toll. Rosacea Rev 1998;summer:2.
5.
J.K. Wilkin, Rosacea: pathophysiology and treatment. Arch Dermatol
130 (1994), pp. 359-362.
6.
G. Plewig and A.M. Kligman, Editors, Acne and rosacea (3rd ed.),
Springer, Berlin (2000).
7.
Pochi PE, Shalita AR, Strauss JS, Webster SB. Report of the consensus conference
on acne classification. J Am Acad Dermatol 1991;24:495-9.
8.
Macsai MS, Mannis MJ, Huntley AC. Acne rosacea. In: Eye and skin disease.
Philadelphia: Lippincott-Raven; 1996. p. 335-41.
9.
E.K. Akpek, A. Merchant, V. Pinar and C.S. Foster, Ocular rosacea: patient
characteristics and follow-up. Ophthalmology 104 (1997),
pp. 1863-1867.
Reprint requests: National Rosacea Society, 800 S Northwest
Highway, Suite 200, Barrington, IL 60010,USA.
*1
Supported by the National Rosacea Society.
Conflicts of interest: None identified.
The opinions set forth in this report are those of the committee members and
do not represent the Food and Drug Administration in any way.
*2
The National Rosacea Society is a 501(c)(3) nonprofit organization whose mission
is to support rosacea research, including the awarding of research grants, and
to provide educational information on rosacea to physicians, patients, and the
public.
Reprinted from Journal of the American Academy of Dermatology, Vol 50, Issue 6 , Wilkin J. et. al., Standard grading system for rosacea: Report of the National Rosacea Society Expert Committee on the Classification and Staging of Rosacea,
Pages No. 907-912, Copyright (2004), with permission from American Academy of Dermatology.
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