Journal of the American Academy of Dermatology
April 2002 • Volume 46 • Number 4
Special Report
Standard classification of
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)
Alvan Feinstein, MD (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 (a); the
Department of Dermatology, Mayo Clinic Scottsdale (b); the Department of Dermatology, Harvard Medical School, Boston (c); the Departments of
Medicine and Epidemiology and Public Health, Yale University, New Haven (d); the Department of Dermatology, University of California San
Francisco (e); and the 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 2002;46:584-7
- 16/1/120625
- doi:10.1067/mjd.2002.120625
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Rosacea is well recognized as a chronic cutaneous
disorder primarily of the convexities of the central face (cheeks, chin, nose,
and central forehead), often characterized by remissions and exacerbations.
Based on present knowledge, it is considered a syndrome, or typology,
encompassing various combinations of such cutaneous signs as flushing, erythema,
telangiectasia, edema, papules, pustules, ocular lesions, and rhinophyma [1].
In most cases, some rather than all of these stigmata appear in any given
patient.
Rosacea appears to be quite common, and in an epidemiologic
study in Sweden its prevalence was 10% [2].
It has been most frequently observed in patients with fair skin, but has also
been diagnosed in Asians and African Americans. Rosacea occurs in both men and
women and, although it may occur at any age, the onset typically begins at any
time after age 30 [3].
Despite its apparent high incidence, the nosology of rosacea is not well
established, and the term “rosacea” has been applied to patients and research
subjects with a diverse set of clinical findings that may or may not be an
integral part of this disorder. In addition to the diversity of clinical
manifestations, the etiology and pathogenesis of rosacea are unknown, and there
are no histologic or serologic markers.
Therefore, the National Rosacea
Society assembled a committee to develop a standard classification system that
can serve as a diagnostic instrument to investigate the manifestations and
relationships of the several subtypes and potential variants of rosacea.
Standard criteria for diagnosis and classification of patients are essential to
perform research, analyze results and compare data from different sources, and
may further serve as a diagnostic reference in clinical practice. The standard
terminology will 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 committee based
the standard classification system on present scientific knowledge and
morphologic characteristics. This avoids assumptions on pathogenesis and
progression, and provides a framework that can be readily updated and expanded
as new discoveries are made. As knowledge increases, it is hoped that the
definition of rosacea may ultimately be based on causality, rather than on
morphology alone.
The following provisional classification system
describes the primary features of rosacea and defines 4 subtypes and 1 variant.
Evolution from one subtype to another may or may not occur, and research to
investigate this process may provide important insight into the pathogenesis of
rosacea. Regardless of subtype, however, each individual characteristic may
progress from mild to moderate to severe. Early diagnosis and treatment are
therefore recommended.
Primary features
Rosacea
typically affects the convexities of the central face. The presence of one or
more of the following signs with a central face distribution is indicative of
rosacea. These signs are commonly transient, and each may occur independently.
Many patients may present with more than one of these diagnostic features.
- Flushing (transient erythema). A history of frequent blushing or flushing
is common.
- Nontransient erythema. Persistent redness of the facial skin is the most
common sign of rosacea.
- Papules and pustules. Dome-shaped red papules with or without accompanying
pustules, often in crops, are typical. Nodules may also occur. Although
patients with concomitant acne may exhibit comedones, comedones should be
considered part of an acne process unrelated to rosacea.
- Telangiectasia. Telangiectases are common but not necessary for a rosacea
diagnosis.
Secondary features
The following
signs and symptoms often appear with one or more of the primary features of
rosacea, but in some patients can occur independently.
- Burning or stinging. Burning or stinging sensations with or without
scaling or dermatitis may occur, especially on malar skin. [4]
- Plaque. Elevated red plaques without epidermal changes in the surrounding
skin may occur.
- Dry appearance. Central facial skin may be rough and scaling so as to
resemble dry skin and suggest an eczematous dermatitis, and may often include
the coexistence of seborrheic dermatitis. This “dryness” may be associated
with burning or stinging sensations, and may be caused by irritation rather
than the disease process.
- Edema. Edema may accompany or follow prolonged facial erythema or
flushing. Sometimes soft edema may last for days or be aggravated by
inflammatory changes. Solid facial edema (persisting hard, nonpitting edema)
can occur with rosacea, usually as a sequel of the papulopustular type, and
also independently of redness, papules and pustules, or phymatous changes.
- Ocular manifestations. Ocular manifestations are common, and range from
symptoms of burning or itching to signs of conjunctival hyperemia and lid
inflammation. Styes, chalazia, and corneal damage may occur in many patients
with rosacea in addition to cutaneous stigmata. The severity of ocular
manifestations may not be proportional to those of the skin.
- Peripheral location. Rosacea has been reported to occur in other
locations, [5]
but the frequency and occurrence of this are ill-defined. Rosacea in
peripheral locations may or may not be accompanied by facial manifestations.
- Phymatous changes. These can include patulous follicles, skin thickening
or fibrosis, and a bulbous appearance. Rhinophyma is the most common form, but
other phymas may occur (Table I).
Table I. Guidelines for the diagnosis of
rosacea
| Presence of one or more
of the following primary features: |
| Flushing (transient erythema) |
| Nontransient erythema |
| Papules and pustules |
| Telangiectasia |
| May include one or more
of the following secondary features: |
| Burning or stinging |
| Plaque |
| Dry
appearance |
| Edema |
| Ocular manifestations |
| Peripheral location |
| Phymatous changes |
The primary and secondary rosacea features described
above often occur together. The most common patterns or groupings of signs are
provisionally designated as specific subtypes of rosacea and are described here
(Table II).
Each subtype includes the fewest signs sufficient to make a diagnosis of the
subtype (though not necessarily limited to these), and patients may have
characteristics of more than one rosacea subtype at the same time.
Subtype 1: Erythematotelangiectatic
rosacea
Erythematotelangiectatic rosacea is mainly
characterized by flushing and persistent central facial erythema. The appearance
of telangiectases is common but not essential for a diagnosis of this subtype.
Central facial edema, stinging and burning sensations, and roughness or scaling
may also be reported. A history of flushing alone is common among patients
presenting with erythematotelangiectatic rosacea.
Subtype 2: Papulopustular
rosacea
Papulopustular rosacea is characterized by persistent
central facial erythema with transient papules or pustules or both in a central
facial distribution. However, papules and pustules also may occur
periorificially (that is, they may occur in the perioral, perinasal, or
periocular areas). The papulopustular subtype resembles acne vulgaris, except
that comedones are absent. Rosacea and acne may occur concomitantly, and such
patients may have comedones as well as the papules and pustules of rosacea.
Burning and stinging sensations may be reported by patients with papulopustular
rosacea.
This subtype has often been seen after or in combination with
subtype 1, including the presence of telangiectases. The telangiectases may be
obscured by persistent erythema, papules, or pustules, and tend to become more
visible after successful treatment of these masking components.
Subtype 3: Phymatous
rosacea
Phymatous rosacea includes thickening skin, irregular
surface nodularities, and enlargement. Rhinophyma is the most common
presentation, but phymatous rosacea may occur in other locations, including the
chin, forehead, cheeks, and ears. Patients with this subtype also may have
patulous, expressive follicles in the phymatous area, and telangiectases may be
present.
This subtype has frequently been observed after or in
combination with subtypes 1 or 2, including persistent erythema, telangiectases,
papules, and pustules. In the case of rhinophyma, these additional stigmata may
be especially pronounced in the nasal area.
Subtype 4: Ocular rosacea
The
diagnosis of ocular rosacea should be considered when a patient's eyes have one
or more of the following signs and symptoms: watery or bloodshot appearance
(interpalpebral conjunctival hyperemia), foreign body sensation, burning or
stinging, dryness, itching, light sensitivity, blurred vision, telangiectases of
the conjunctiva and lid margin, or lid and periocular erythema. Blepharitis,
conjunctivitis, and irregularity of the eyelid margins also may occur [6].
Meibomian gland dysfunction presenting as chalazion or chronic staphylococcal
infection as manifested by hordeolum (stye) are common signs of rosacea-related
ocular disease. Some patients may have decreased visual acuity caused by corneal
complications (punctate keratitis, corneal infiltrates/ulcers, or marginal
keratitis). [7]
Treatment of cutaneous rosacea alone may be inadequate in terms of lessening the
risk of vision loss resulting from ocular rosacea, and an ophthalmologic
approach may be needed. [8]
Ocular rosacea is most frequently diagnosed when cutaneous signs and
symptoms of rosacea are also present. However, skin signs and symptoms are not
prerequisite to the diagnosis, and limited studies suggest that ocular signs and
symptoms may occur before cutaneous manifestations in up to 20% of patients with
ocular rosacea. Approximately half of these patients experience skin lesions
first, and a minority have both manifestations simultaneously. [9]
Table II. Subtypes and variants of rosacea
and their characteristics
|
Characteristics |
| Subtype |
|
| Erythematotelangiectatic |
Flushing and persistent
central facial erythema with or without telangiectasia. |
| Papulopustular |
Persistent central
facial erythema with transient, central facial papules or pustules or
both. |
| Phymatous |
Thickening skin,
irregular surface nodularities and enlargement May occur on the nose,
chin, forehead, cheeks, or ears. |
| Ocular |
Foreign body sensation
in the eye, burning or stinging, dryness, itching, ocular
photosensitivity, blurred vision, telangiectasia of the sclera or other
parts of the eye, or periorbital edema. |
| Variant |
|
| Granulomatous |
Noninflammatory; hard;
brown, yellow, or red cutaneous papules; or nodules of uniform
size. |
Variants of rosacea, which do not represent morphologic
patterns or combinations as seen in rosacea subtypes, may occur. To date, the
committee has recognized one such variant.
Granulomatous
rosacea
Granulomatous rosacea is characterized by hard,
yellow, brown, or red cutaneous papules or nodules that may be severe and lead
to scarring. These lesions tend to be less inflammatory than papules and
pustules and sit upon relatively normal-appearing skin. They can vary in size
among patients but are monomorphic in each individual patient, and typically
appear on the cheeks and periorificial areas. Granulomatous rosacea may occur in
locations other than those in which the phymas are observed. The presence of
other rosacea signs is not needed for a diagnosis of the granulomatous rosacea
variant.
The committee noted that certain disorders may have
been prematurely identified as associated with rosacea or as a variant of
rosacea, and for clarity should be recognized at this time as separate entities.
There is insufficient basis at present to include the following conditions as
types of rosacea.
Rosacea fulminans
Popularly
known as pyoderma faciale, the grouping of this disorder as a type of rosacea is
premature. It is characterized by the sudden appearance of papules, pustules,
and nodules, along with fluctuating and draining sinuses that may be
interconnecting. The condition appears primarily in women in their 20s, and
intense redness and edema also may be prominent.
Steroid-induced acneiform
eruption
Steroid-induced acneiform eruption is not a variant
of rosacea and can occur as an inflammatory response in any patient during or
after chronic corticosteroid use. The same inflammatory response may also, of
course, occur in patients with rosacea.
Perioral dermatitis
Although
rosacea papules may appear in the perioral area, as noted earlier, perioral
dermatitis without rosacea symptoms cannot be classified as a variant of
rosacea. Perioral dermatitis is characterized by such stigmata as microvesicles,
scaling, and peeling.
This investigational instrument is intended to set the
stage for a better understanding of rosacea and its subtypes among researchers
and practitioners by fostering communication and facilitating the development of
a research-based classification system. As a provisional standard classification
system, it is likely to require modification in the future as the pathogenesis
and subtypes of rosacea become clearer, and as its relevance and applicability
are tested by investigators and clinicians. The committee welcomes reports on
the usefulness and limitations of these criteria.
The Committee thanks the following individuals who reviewed
and contributed to this document: Dr Joel Bamford, Department of Dermatology,
St. Mary's/Duluth Clinic; Dr Mats Berg, Department of Dermatology, Mälar
Hospital, Eskilstuna, Sweden; Dr Albert Kligman, Department of Dermatology,
University of Pennsylvania; Dr Mark Mannis, Department of Ophthalmology,
University of California-Davis; Dr Ronald Marks, Department of Dermatology,
University of Wales Medical Center, Cardiff, Wales; Drs Gerd Plewig and Claudia
Borelli, Department of Dermatology, Ludwig-Maximilians University, Munich,
Germany; Dr Alfredo Rebora, Department of Dermatology, University of Genoa,
Italy; Dr Diane Thiboutot, Department of Dermatology, Pennsylvania State
University; and Dr Guy Webster, Department of Dermatology, Thomas Jefferson
University. The final document does not necessarily reflect the views of any
single individual, and not all comments were incorporated.
1. Wilkin JK. Rosacea:
pathophysiology and treatment. Arch Dermatol 1994;130:359-62.
2. Berg M, Liden S. An epidemiological
study of rosacea. Acta Derm Venereol 1989;69:419-23.
3. Drake L. Survey maps typical
progression from rosacea's first appearance. Rosacea Review
1995;winter:2.
4. Lonne-Rahm
S-B, Fischer T, Berg M. Stinging and rosacea. Acta Derm Venereol
1999;79:460-1.
5. Jansen T, Plewig G. Rosacea:
classification and treatment. J R Soc Med 1997;90:144-50.
6. Macsai MS, Mannis MJ, Huntley AC.
Acne rosacea. In: Eye and skin disease. Philadelphia: Lippincott-Raven; 1996.
p. 335-41.
7. Chen DM, Crosby
DL. Periorbital edema as an initial presentation of rosacea. J Am Acad
Dermatol 1997;37:346-8.
8. Akpek EK, Merchant A, Pinar V, Foster
CS. Ocular rosacea: patient characteristics and follow-up. Ophthalmology
1997;104:1863-7.
9. Browning DJ, Proia AD. Ocular
rosacea. Surv Ophthalmol 1986;31:145-58.
Reprinted from Journal of the American Academy of Dermatology, Vol 50, Issue 6, Wilkin J. et. al., Standard classification of rosacea: Report of the National Rosacea Society Expert Committee on the Classification and Staging of Rosacea, Pages No. 584-587, Copyright (2002), with permission from American Academy of Dermatology.
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