Appendices

11.1 Appendix A: the rosacea “classification and staging” controversy

The nomenclature of acne rosacea has presented problems for many years. In 2002 and in 2004, an Expert Committee looked at the question and published a document “on the classification and staging of rosacea” [1].

The document contained changes that I felt were ill-advised and tended to mislead the public, as outlined in the following letter, published in the Journal of the American Academy of Dermatology:

To the Editor: For the past year or so, dermatologists have been the ambivalent recipients of referrals and self-referrals of patients who either believe or have been told that they have rosacea. And some of them do—the papular and papulopustular disorder known as acne rosacea in times past.

But many do not. Instead they present with a history of intermittent flushing (triggers varying from emotional overload to estrogen depletion) or a background facial erythema (sometimes demonstrably genetic but more commonly actinic) of varying color depth, or telangiectasia of the sun-exposed areas that rosacea favours, or all three.

Some have already been treated with the metronidazole-containing products that represent the standard of care, making the diagnosis a little difficult if the characteristic papules and pustules have disappeared. The problem is that these patients are usually complaining that their rosacea “is still there.” By this they mean the background erythema and telangiectasia that, alone or together, do not make a diagnosis of acne rosacea even though they are common companions of that disorder.

We dermatologists are presented with two problems, in addition to sorting out whether the patient actually has (or had) acne rosacea.

The first is education, actually re-education, defining the disorder for the patient and pointing out where he or she fits. This is a challenge, because an Expert Committee has recently suggested a change in the criteria for the diagnosis of rosacea (sic) and a new disorder, erythematotelangiectatic rosacea, has been included. Details were published in the June issue of the Journal. The criteria, also published online at http://www.rosacea.org/class/classystem.html, are such that anyone with persistent central facial erythema (with or without telangiectasia) fits this diagnosis, even though they suffer from nothing more than actinic (i.e., sun-induced) erythema, once known simply as “high colour” in the British literature.

I make no claim that these features are not part of rosacea, just that the diagnosis cannot hang on the vascular changes alone, because these features are quite capable of existing by themselves. I have begun to diagnose such patients as having “pseudorosacea.” It seems a better fit than “inconstant vasodilatory and actinic telangiectatic non-rosacea.”

The second problem is what to do about the patients’ unreasonable expectations. Patients are sent (or come driven by advertising) to us in the expectation that we will be able to “fix” them. Well, of the six components of rosacea, two (the papules and the pustules) are easily managed in most (but not all) cases by topical metronidazole or sulfur/sulfacetamide products, with or without oral antibiotics. It is not unreasonable to expect a good outcome here, and of course there will be a diminution of some of the erythema as the inflammation associated with these components lessens. That leaves us with the need to explain that the two vascular components are manageable only with a vascular laser (there are several) for the telangiectases or an intense pulsed light (IPL) unit for the background erythema, or both. While this presents dermatologists who own such equipment with a golden opportunity to market the procedure, one can understand that the somewhat suspicious medical public will wonder whether they are becoming victims of clever “bait and switch” marketing. The fifth component, the famous W. C. Fields rhinophyma, now referred to as “phymatous rosacea,” will require surgical reduction in one of several ways, usually requiring another referral. Sixth and last, if the patient responds to careful questioning that an itchy or scratching or gritty feeling in the eyes is part of the problem, then a diagnosis of ocular rosacea and a referral to an ophthalmologist should be considered.

So how should the front-line primary care practitioner confront suspected rosacea? I would suggest that the presence of papules and pustules at a minimum is required for a diagnosis of acne rosacea and treatment should be with topicals supplemented as needed with oral cyclines and other anti-inflammatories. Failure to respond should trigger a referral to a dermatologist for consideration of at least seven differential diagnoses mentioned in neither the above reference nor the above Web site (post adolescent acne, contact dermatitis, drug reaction, seborrheic dermatitis, perioral dermatitis, polymorphous light eruption, and facial psoriasis). In the absence of the papules and pustules, where only flushing and telangiectasia exist, actinic erythema and/or actinic telangiectasia would be better referring diagnoses. The consultant dermatologist should be able to confirm the diagnosis, consider the several alternatives, and direct the patient to appropriate care, including sun avoidance techniques and truly broad-spectrum sunscreens.

One further thought: the concept of marketing actinic telangiectasia as a form of rosacea (or pre-rosacea) amenable to topical pre-emptive or preventive therapy seems to be part of this whole picture. Proof is lacking that the former is a predictor or precursor of the latter, making such therapeutic innovations premature at this time. A multicenter phase IV clinical study is underway nevertheless. Meanwhile the predictive diagnosis of prerosacea must remain impossible to make until adequate and tested diagnostic criteria are developed. For now it might be fair to accept the diagnosis, but only when made retrospectively.

In any case, it would be best if the patient were not led to believe that the topicals will “cure” the problem, or, in the alternative, that these same topicals have actually failed to do what was expected. Unfulfilled unreasonable expectations tend to breed dissatisfied patients.

The Chair of the Expert Committee informs me by letter that he welcomes reports on the usefulness and limitations of these criteria. I write in the hope that this contribution will help with both patient care and patient-physician communication. [2]

The response from the Expert Committee is as follows:

To the Editor: The National Rosacea Society Expert Committee on the Classification and Staging of Rosacea read with interest the letter of Dr F. William Danby to this journal with comments relating to the standard classification of rosacea. As noted in its publication, and in the more recent publication of a standard grading system, this is a provisional system that is expected to require modification as the pathogenesis and subtypes of rosacea become clearer, and as its relevance and applicability are tested by researchers and clinicians The committee, therefore, welcomes reports on the usefulness and limitations of these criteria.

We believe that the letter by Danby contains a number of observations that are reasonable and accurate about the problems of treating patients with rosacea today; for example, that current therapies for rosacea offer limited success, especially in treating the erythema of rosacea, and this often poses a problem in managing patient expectations. However, we are concerned that some mistaken assumptions and implications about the classification system could easily be interpreted as critical and might cloud the intended benefits of the new system to researchers, physicians, and their patients.

It may first be useful to clarify what the standard classification system is not. First, it should be recognized that these standard diagnostic criteria have nothing to do with the promotion of treatment. In fact, they meticulously avoid treatment recommendations, either specific or general.

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