Standard grading system for rosacea: Report of the National Rosacea Society Expert Committee on the Classification and Staging of Rosacea
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SPECIAL REPORT
Standard grading system for rosacea: Report of
the National Rosacea Society Expert Committee
on the Classification and Staging of Rosacea
Committee members: Jonathan Wilkin, MD, Chair,a Mark Dahl, MD,b
Michael Detmar, MD,c Lynn Drake, MD,c Matthew H. Liang, MD, MPH,d
Richard Odom, MD,e and Frank Powell, MDf
Rockville, Maryland; Scottsdale, Arizona; Boston, Massachusetts;
San Francisco, California; and Dublin, Ireland
A standard classification system for rosacea
was published in the April 2002 issue of the
Journal of the American Academy of Derma-
tology.1 Developed by the National Rosacea Society
Expert Committee on the Classification and Staging
facilitate clear communication among a broad range
of basic, clinical, and other researchers; practicing
dermatologists; primary care physicians; ophthal-
mologists and other specialists; health and insurance
administrators; and patients and the general public.
of Rosacea and reviewed by rosacea experts world- The standard grading system rates the primary
wide, it describes primary and secondary features of and secondary features of rosacea established by the
rosacea and recognizes 4 patterns of signs and standard classification system, and provides a global
symptoms, designated as subtypes. assessment of subtypes by both the physician and
To enhance the utility of the system for both the patient. Beyond clinical manifestations, addi-
clinicians and researchers, the committee has de- tional factors are important in determining the se-
vised a standard method for assessing gradations of verity of rosacea from the patient’s viewpoint. These
the severity of rosacea. In addition to the classifica- may include the psychological, social, or occupa-
tion system, a standard grading system is often es- tional effects of the disorder,4 and other potential
sential to perform research, analyze results, and factors such as responsiveness to treatment.
For optimal utility, the grading system is designed
compare data from different sources, and in turn
to be reproducible and easily performed based on
provides a common reference for diagnosis, treat-
observation in clinical practice, while forming a con-
ment, and assessment of results in clinical prac-
sistent framework for more comprehensive mea-
tice.2,3 Standard parameters and terminology also
surements that may be developed for specific re-
search studies. Moreover, as with the standard
From the Division of Dermatologic and Dental Drug Products, classification system, this grading system is an inves-
Food and Drug Administration, Rockvillea; Department of tigative instrument that can be readily modified
Dermatology, Mayo Clinic, Scottsdaleb; Departments of based on clinical experience or updated and ex-
Dermatologyc and Medicine,d Harvard Medical School, Boston; panded as new discoveries are made.
Department of Dermatology, University of California San
Franciscoe; and Regional Centre of Dermatology, Mater
CLASSIFICATION OF ROSACEA
Misericordiae Hospital, Dublin.f
Supported by the National Rosacea Society. Rosacea is a chronic cutaneous disorder affecting
Conflicts of interest: None identified. primarily the convexities of the central face (cheek,
The opinions set forth in this report are those of the committee nose, chin, and central forehead). It is a syndrome or
members and do not represent the Food and Drug Administra- typology encompassing various combinations of
tion in any way. signs and symptoms. In most cases, some rather
The National Rosacea Society is a 501(c)(3) nonprofit organization
whose mission is to support rosacea research, including the than all of these features appear in any given patient,
awarding of research grants, and to provide educational informa- and they are often characterized by remissions and
tion on rosacea to physicians, patients, and the public. exacerbations.5,6
Reprint requests: National Rosacea Society, 800 S Northwest The committee based the standard classification
Highway, Suite 200, Barrington, IL 60010. system on current scientific knowledge and mor-
J Am Acad Dermatol 2004;50:907-12.
0190-9622/$30.00 phologic characteristics to avoid assumptions on
© 2004 by the American Academy of Dermatology, Inc. pathogenesis and progression, which are at present
doi:10.1016/j.jaad.2004.01.048 incompletely understood. As knowledge increases,
907
908 Wilkin et al J AM ACAD DERMATOL
JUNE 2004
Table I. Rosacea clinical scorecard
Primary features
Flushing (transient erythema) 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Nontransient erythema 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Papules and pustules 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Telangiectasia 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Secondary features
Burning or stinging 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Plaques 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Dry appearance 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Edema 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
If present: 䊐 Acute 䊐 Chronic
If chronic: 䊐 Pitting 䊐 Nonpitting
Ocular manifestations 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Peripheral location 䊐 Absent 䊐 Present
If present: List location(s) ________________________________________________
Phymatous changes 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Global assessment
Physician ratings by subtype
Subtype 1: Erythematotelangiectatic 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Subtype 2: Papulopustular 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Subtype 3: Phymatous 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Subtype 4: Ocular 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
Patient’s global assessment 䊐 Absent 䊐 Mild 䊐 Moderate 䊐 Severe
the definition of rosacea may ultimately be based on Primary features
causality rather than on morphology alone. Flushing (transient erythema). Clinically,
The committee first identified primary and sec- physicians should determine the presence or ab-
ondary features of rosacea, and then delineated sub- sence of flushing through patient history, and may
types based on the most common patterns or group- ask about frequency, duration, extent, and severity.
ings of these features. The primary signs of rosacea Noting the presence or absence of accompanying
include flushing (transient erythema), nontransient sweating may also be helpful. Perimenopausal flush-
erythema, papules and pustules, and telangiectasia. ing should not be considered significant unless it is
The presence of one or more of these features with accompanied by other characteristics of rosacea.
a central face distribution is indicative of rosacea. Researchers may grade flushing from 0 to 3 based
Secondary features, which often appear with one or on intensity and frequency. In addition, duration of
more of the primary features but can occur indepen- flushing may be noted, because some episodes are
dently, include burning or stinging, plaques, dry very transient (eg, from embarrassment) and some
appearance, edema, ocular manifestations, periph- are not (eg, from ingestion of alcohol). Specific time
eral locations, and phymatous changes. frames may also be identified.
Nontransient erythema. For clinicians, non-
GRADING OF ROSACEA transient (persistent) erythema may be graded from
For clinicians assessing patients, primary signs 0 to 3. Although inflammation (papules, pustules,
and symptoms may be graded as absent, mild, mod- plaques) or dry appearance may obscure the level of
erate, or severe (0-3), and most secondary features erythema, underlying redness should be evaluated
may be graded simply as absent or present (Table I). disregarding this effect. Inflammation or dry appear-
Researchers are encouraged to provide more de- ance may be noted, but perilesional erythema
tailed assessments. In some situations, more detailed should not be included in this assessment.
or finer distinctions, perhaps supplemented by ad- In clinical studies, researchers may use instru-
vanced technology, might be possible. Certain clini- ments or other measurements to score erythema
cians also may wish to use some of these other more beyond a score of 0 to 3. For example, erythema
comprehensive analytic methods, especially when may be assessed objectively with an appropriate
based on visual observation. device.
J AM ACAD DERMATOL Wilkin et al 909
VOLUME 50, NUMBER 6
Table II. Severity grading of rosacea papules and represent coexisting seborrheic dermatitis or
pustules irritation.
Severity Papules/pustules Plaques
Edema. In clinical practice, edema may be iden-
tified by location (eg, periorbital, glabellar, malar)
Mild Few None
through patient history and examination. If present,
Moderate Several None
Severe Many Present
it may be noted as acute, chronic recurrent, or
chronic persistent and, if chronic, as pitting or non-
pitting. Researchers may assign a grade of 0 to 3
according to extent and degree of swelling.
Papules and pustules. A modified version of Ocular manifestations. Clinicians may identify
the descriptive grading system established for acne ocular manifestations by looking for tearing, redness
vulgaris is recommended and shown in Table II.7 of bulbar and/or palpebral conjunctivae, telangiec-
Few to several papules and pustules, with no tasia of conjunctiva and lid margin, lid or periocular
plaques, are scored as “mild.” Several to many pap- erythema, or styes, and by inquiring about symp-
ules and pustules, with no plaques, are considered toms of foreign-body sensation, gritty feeling, burn-
“moderate.” Numerous and/or extensive papules ing, stinging, itching, dryness, light sensitivity,
and pustules, with or without plaques, are consid- blurred vision, or decreased visual acuity.8 Cases
ered “severe.” that are moderate to severe, progressive, or not
Researchers should record the number of papules responding to treatment, or where vision is affected,
and pustules, and note the presence or absence of may require an ophthalmologic consultative ap-
plaques.1 proach. Treatment of cutaneous rosacea alone may
Telangiectasia. Telangiectasia may be graded be inadequate to reduce the risk of vision loss.9
in the clinical setting from 0 to 3. If erythema is Researchers may wish to stratify the ocular man-
intense, it may be difficult to definitively score tel- ifestations as mild (signs/symptoms affecting eye
angiectasia, because erythema may mask some tel-
margin, meibomian gland), moderate (signs/symp-
angiectases, which become more visible if redness
toms affecting inner lid, fluid secretion, eye surface),
fades. This phenomenon has been described as
or severe (corneal damage and potential vision loss).
posterythema-revealed telangiectasia.5 On the other
Peripheral location. Clinicians and researchers
hand, the presence of one or two isolated telangi-
may determine the presence of any extrafacial signs
ectases in the absence of any other primary signs of
and symptoms, and note the anatomic sites. Com-
rosacea may be insufficient for a diagnosis.
mon extrafacial locations may include the neck,
Researchers also should count telangiectases, if
chest, scalp, ears, and back. The diagnosis of rosa-
feasible, at least in specified areas. Nasal and malar
cea in locations other than the face may be prob-
telangiectases should be identified independently,
lematic in the absence of diagnostic clinical or his-
and be qualitatively described as fine and threadlike
tologic features.
to coarse.
Phymatous changes. In the clinical setting, se-
Secondary features verity may be rated from 0 to 3, with 1 being patu-
Burning or stinging. In the clinical setting, lous follicles but no contour changes, 2 being a
burning or stinging may be reported by the patient change in contour without a nodular component,
and, if present, may be weighed into the overall and 3 indicating a change in contour with a nodular
assessment of severity. Researchers should seek out component. Researchers may also note any vascular
this information, record the locations of both symp- findings or inflammatory changes.
toms if present, and use a systematic method of
assessing both symptoms. Global assessment of subtypes
Plaques. In clinical practice, plaques may be Because the potential manifestations of rosacea
noted. Plaques may be defined as confluent areas of are so numerous and varied, the committee con-
inflammation, often seen as larger red areas among cluded that global assessment can be most easily
papules and pustules without epidermal changes in and meaningfully performed by subtype. The stan-
the surrounding skin. In research studies, they may dard classification system established the following
be further differentiated by severity, location, or subtypes of rosacea, which are described in depth in
other criteria. the standard classification system.1 The following
Dry appearance. In clinical practice, rough, descriptions include the minimum signs and symp-
dry-appearing skin may be noted. In research, this toms required to diagnose each subtype, and pa-
may also be stratified based on such criteria as dis- tients may have characteristics of more than one
tribution and severity. If scaling is noted, it may rosacea subtype at the same time.
910 Wilkin et al J AM ACAD DERMATOL
JUNE 2004
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.
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.
Subtype 1: erythematotelangiectatic rosa- of the eyelid margins also may occur. Meibomian
cea. Subtype 1 (Fig 1) is characterized by flushing gland dysfunction presenting as chalazion, or
and persistent central facial erythema. Telangiec- chronic infection as manifested by hordeolum
tases are common but not essential for the (stye), are common. Some patients may experience
diagnosis. loss of vision as a result of corneal complications
Subtype 2: papulopustular rosacea. Subtype 2 (punctate keratitis, corneal infiltrates, ulcers, or mar-
(Fig 2) includes persistent central facial erythema with ginal keratitis). An ophthalmologic consultative ap-
transient papules, pustules, or both in a central facial proach to treatment may be required.
distribution. Burning and stinging may also be For clinicians, global assessment for each subtype
reported. should be performed with a standard rating of 0 to 3,
Subtype 3: phymatous rosacea. This subtype based on a composite of the severity of the signs and
(Fig 3) may include thickening skin, irregular surface symptoms. The evaluation may also take into con-
nodularities, and enlargement. Phymatous rosacea sideration the duration of signs and symptoms
occurs most commonly as rhinophyma but may ap- through patient history, and their extent at time of
pear elsewhere, including the chin, forehead, examination. For researchers, additional detail and
cheeks, and ears. Patulous, expressive follicles may assessment technology may be added beyond the
appear in the phymatous area, and telangiectases basic rating system to provide further data and
may be present. precision.
Subtype 4: ocular rosacea. Ocular rosacea (Fig The committee noted that the ultimate goal of
4) may include watery or bloodshot appearance diagnosis and treatment of rosacea is both to control
(interpalpebral conjunctival hyperemia), foreign- the disorder and to minimize the discomfort of the
body sensation, burning or stinging, dryness, itch- patient. Patient participation in evaluation is, there-
ing, light sensitivity, blurred vision, telangiectasia of fore, essential. The patient may provide a 0 to 3
the conjunctiva and lid margin, or lid and periocular global assessment of the severity of their condition
erythema. Blepharitis, conjunctivitis, and irregularity in general terms that encompasses both the physicalJ AM ACAD DERMATOL Wilkin et al 911
VOLUME 50, NUMBER 6
Fig 3. Subtype 3, phymatous rosacea, may include thickening skin, irregular surface nodu-
larities, and enlargement. Patulous, expressive follicles may appear in phymatous area, and
telangiectases may be present. A, Mild; B, moderate; C, severe.
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.
manifestations of rosacea and its impact on quality This investigational instrument is intended to
of life, which may include psychological, social, and help provide a foundation for better understanding
occupational effects. of rosacea among practitioners and researchers by
Patients might be informed of potential primary establishing a common language for communication
and secondary features of rosacea before their and facilitating the development of a research-based
global assessments to aid them in evaluating their approach to diagnosis and treatment. The scorecard
individual conditions more thoroughly. Of particular (Table I) is included for those who wish to have a
concern is ocular rosacea, which patients may not more detailed investigative record of the patient’s
associate with cutaneous rosacea and that may re- disorder.
quire further evaluation. As with the standard classification system, this
CONCLUSION grading system is considered provisional and is sub-
In developing a standard grading system for ro- ject to modification as the pathogenesis and sub-
sacea, the committee attempted to design a basic types of rosacea become clearer, and as its relevance
examination process that is practical, useful, and and applicability are tested by investigators and cli-
similar to the usual examinations currently per- nicians. The National Rosacea Society Expert Com-
formed in clinical practice. To aid clinicians in eval- mittee welcomes comments on the usefulness and
uating their patients, the committee has developed a limitations of these criteria.
standard diagnostic flow chart (Table I). Superim- The committee thanks the following individuals who
posed on this basic standard system, researchers are reviewed and contributed to this document: Dr Joel Bam-
encouraged to study and explore features beyond ford, Department of Dermatology, St Mary’s/Duluth Clinic,
the minimum, using more sensitive and reproduc- Duluth, Minnesota; Dr Mats Berg, Department of Derma-
ible systems and applying new technology and tology, Uppsala University, Uppsala, Sweden; Dr Joseph
methodologies that may further advance the scien- Bikowski, Department of Dermatology, University of Pitts-
tific knowledge of rosacea. burgh, Pittsburgh, Pennsylvania; Dr Albert Kligman, De-912 Wilkin et al J AM ACAD DERMATOL
JUNE 2004
partment of Dermatology, University of Pennsylvania, Society expert committee on the classification and staging of ro-
Philadelphia, Pennsylvania; Dr Ronald Marks, Department sacea. J Am Acad Dermatol 2002;46:584-7.
of Dermatology, University of Wales Medical Center, 2. Gessert CE, Bamford JTM. Measuring the severity of rosacea: a
Cardiff, United Kingdom; Dr Gerd Plewig, Department of review. Int J Dermatol 2003;42:444.
3. Henderson CA, Charles-Holmes S, McSween R, Ilchyshyn A. A sys-
Dermatology, Ludwig-Maximilians University, Munich,
tem for grading rosacea severity. Br J Dermatol 1995;133(Suppl):
Germany; Dr Bryan Sires, Department of Ophthalmology,
34.
University of Washington, Seattle, Washington; Dr Diane 4. Drake L. Rosacea takes emotional toll. Rosacea Rev 1998;sum-
Thiboutot, Department of Dermatology, Pennsylvania mer:2.
State University, Hershey, Pennsylvania; Dr Guy Webster, 5. Wilkin JK. Rosacea: pathophysiology and treatment. Arch Derma-
Department of Dermatology, Thomas Jefferson University, tol 1994;130:359-62.
Philadelphia, Pennsylvania; and Dr Mina Yaar, Depart- 6. Plewig G, Kligman AM, editors. Acne and rosacea. 3rd ed. Berlin:
ment of Dermatology, Boston University, Boston, Massa- Springer; 2000.
chusetts. The final document does not necessarily reflect 7. Pochi PE, Shalita AR, Strauss JS, Webster SB. Report of the consen-
the views of any single individual, and not all comments sus conference on acne classification. J Am Acad Dermatol 1991;
24:495-9.
were incorporated.
8. Macsai MS, Mannis MJ, Huntley AC. Acne rosacea. In: Eye and skin
disease. Philadelphia: Lippincott-Raven; 1996. p. 335-41.
REFERENCES 9. Akpek EK, Merchant A, Pinar V, Foster CS. Ocular rosacea: pa-
1. Wilkin J, Dahl M, Detmar M, Drake L, Feinstein A, Odom R, et al. tient characteristics and follow-up. Ophthalmology 1997;104:
Standard classification of rosacea: report of the National Rosacea 1863-7.You can also read
