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Rosacea
Author: Agnieszka Kupiec-Banasikowska, MD, Consulting Staff, Division of Dermatology, Georgetown University Medical Center
Coauthor(s): Mana Ogholikhan, MD, Staff Physician, Division of Dermatology, Georgetown University Hospital; Ravi Ratnavel, MD, Consulting Staff, Department of Dermatology, Stoke Mandeville, Thames Valley Nuffield, Paddocks Hospitals, UK

Introduction
Background
Rosacea is a common condition characterized by symptoms of facial flushing and a spectrum of clinical signs, including erythema, telangiectasia, coarseness of skin, and an inflammatory papulopustular eruption resembling acne.
Definition and subtypes
An expert committee assembled by the National Rosacea Society explicitly defined and classified rosacea in April 2002 into 4 different subtypes based upon specific clinical signs and symptoms. This was an important step in the treatment of rosacea. Currently, the therapeutics of rosacea empirically target the signs and symptoms of the disease because investigators do not understand the details of its pathophysiology. Therefore, this classification system aides clinicians in treatment by highlighting the preponderance of one or more of the clustering signs of presentation and, thus, helps to specify which therapeutic approach to initiate.
The diagnosis of rosacea is a clinical diagnosis. Skin biopsy may be necessary to exclude other disease states that mimic the clinical presentation of rosacea. For example, the clinician must exclude polycythemia vera, connective tissue diseases (eg, lupus erythematous, dermatomyositis, mixed connective tissue disease), photosensitivity, carcinoid mastocytosis, long-term application of topical steroids, contact dermatitis, and photosensitivity before making the diagnosis of rosacea. Rosacea is defined by persistent erythema of the central portion of the face lasting for at least 3 months. Supporting criteria include flushing, papules, pustules, and telangiectasias on the convex surfaces. Secondary characteristics are burning and stinging, edema, plaques, a dry appearance, ocular manifestations, and phymatous changes. The prevalence of these findings designates the subclassification of the presentation and, additionally, the therapeutic options.

Erythematotelangiectatic type
Central facial flushing, often accompanied by burning or stinging, is the predominant sign in erythematotelangiectatic rosacea (ETR). The redness usually spares the periocular skin. These patients usually have skin with a fine texture that lacks a sebaceous quality characteristic of other subtypes. The erythematous areas of the face at times appear rough with scale likely due to chronic, low-grade dermatitis. Frequent triggers to flushing include acutely felt emotional stress, hot drinks, alcohol, spicy foods, exercise, cold or hot weather, and hot baths and showers. These patients also report that the burning or stinging is exacerbated when topical agents are applied.

Papulopustular rosacea
Papulopustular rosacea (PPR) is the classic presentation of rosacea. Patients are women of middle age who predominately present with a red central portion of their face that contains small erythematous papules surmounted by pinpoint pustules. One may elicit a history of flushing. Telangiectasias are likely present but may be difficult to distinguish from the erythematous background in which they exist.

Phymatous rosacea
Phymatous rosacea is defined as marked skin thickenings and irregular surface nodularities of the nose, chin, forehead, one or both ears, and/or the eyelids. Four distinct histologic variants can occur with rhinophyma (associated changes of the nose) that include glandular, fibrous, fibroangiomatous, and actinic. The mainstays of treatment are isotretinoin topical application and surgical correction. This varies from other rosacea subtypes.

Ocular rosacea
Ocular manifestations may precede the cutaneous signs by years. Yet, frequently they develop concurrently with dermatologic manifestations. The ocular manifestations include blepharitis, conjunctivitis, inflammation of the lids and meibomian glands, interpalpebral conjunctival hyperemia, and conjunctival telangiectasias. Patients may describe eye stinging or burning, dryness, irritation with light, or foreign body sensation. Ocular rosacea, similar to phymatous rosacea, has a distinct therapeutic management. Therefore, dermatologists must ask their patients specifically about ocular symptoms and perform a thorough physical examination to rule out this type of rosacea.

Pathophysiology
The etiology of rosacea is unknown. However, several factors such vasculature, climatic exposures, matrix degeneration, chemicals and ingested agents, pilosebaceous unit abnormalities, and microbial organisms likely play a role in its development. Furthermore, the distinct subtype of rosacea is likely determined by a patient's unique sensitivity to these triggers.

Vasculature
Increased blood flow to the blood vessels of the face and increased numbers of blood vessels that are closer to the surface of the face are thought to be responsible for the redness and flushing associated with rosacea. Furthermore, vasodilatation, the normal response to hyperthermia, is thought to be more pronounced or exaggerated in those with rosacea.

Climatic exposures
Evidence exists that suggests that harsh climatic exposures damage cutaneous blood vessels and dermal connective tissue. This also includes exposure to solar irradiation, which may explain why rosacea predominately affects the facial convexities and has a tendency to flare in the spring. However, other studies suggest the contrary, in that most patients' symptoms do not worsen in the sunlight and do not flare with an acute exposure to ultraviolet light.

Dermal matrix degeneration
Rosacea involves associated damage to the endothelium and degeneration of the dermal matrix. However, it is not known whether the initial damage is in the dermal matrix and this leads to poor tissue support of cutaneous vessels and causes pooling of serum, inflammatory mediators, and metabolic waste or whether the initial abnormality exists in the cutaneous vasculature and this leads to leaky vessels and delayed clearance of serum proteins, inflammatory mediators, and metabolic waste, thus causing matrix degeneration.

Chemicals and ingested agents
Spicy foods, alcohol, and hot beverages may trigger a flushed face in patients with rosacea. However, most evidence does not support that dietary factors play a main role in the pathogenesis. Moreover, certain medications such as amiodarone, topical steroids, nasal steroids, and high doses of vitamins B-6 and B-12 may cause flares for patients with rosacea.

Perivascular versus perifollicular inflammation
There may be inflammatory infiltrates in a perivascular and/or a perifollicular location. However, evidence is conflicting regarding which location predominates. To answer this question, more studies need to be designed to categorize subtypes of rosacea because the answer varies depending on the subdivision.

Microbial organisms
Demodex species (mites that normally inhabit human hair follicles) may play a role in the pathogenesis of rosacea. Support suggests that Demodex prefers skin regions that are affected in rosacea, such as the nose and cheeks. Studies also support that an immune response of helper-inducer T cell infiltrates occurs, surrounding the Demodex antigens in patients with rosacea. Yet, conflicting evidence indicates that Demodex does not induce an inflammatory response in patients with rosacea. Moreover, Demodex is found in large numbers of healthy individuals without rosacea. More studies need to be performed to determine whether Demodex truly is pathogenic.
Also, inconclusive evidence suggests that Helicobacter pylori is associated with the etiology of rosacea. However, many of the studies have not controlled for confounding variables that influence H pylori prevalence, such as sex, age, socioeconomic status, and medications. Furthermore, these studies were not statistically powered to account for the ubiquitous nature of H pylori infection.

Frequency
United States
Accurate incidence data are not available, but persons with rosacea are disproportionately of fair-skinned European and Celtic origin.
International
A study in Sweden revealed an incidence of 1 in 10 middle class workers. The caseating granulomatous variant (acne agminata) may more commonly occur in people of Asian or African origin.

Mortality/Morbidity
A spectrum of clinical features is seen, and progression may be step-wise. The condition ranges from minor cosmetic disability to severe disfiguring disease.

Clinical
History
•Patients are likely to have a background of facial flushing, often dating to childhood or the early teens. In adult life, flushing may be increasingly precipitated by hot drinks, heat, emotion, and other causes of rapid body temperature changes. Some patients report flushing with alcohol, which is not specific.

•The symptoms are usually intermittent but can progressively lead to permanently flushed skin. The latter may be described as high color and is associated with the development of permanent telangiectasia.

•A few individuals report a gritty quality of the eyes and facial edema.

Physical
The disease consists of a spectrum of symptoms and signs, with most patients failing to develop every stage of disease. Variable erythema and telangiectasia are seen over the cheeks and the forehead. Inflammatory papules and pustules may be predominantly observed over the nose, the forehead, and the cheeks. Extrafacial involvement uncommonly occurs over the neck and the upper part of the chest. Prominence of sebaceous glands may be noted, with the development of thickened and disfigured noses (rhinophyma) in extreme cases. Unlike acne, patients generally do not report greasiness of the skin; instead, they may experience drying and peeling. The absence of comedones is another helpful distinguishing feature. Ocular lymphedema may be prominent but is mostly uncommon. The condition generally does not produce scarring.
•Rhinophyma may occur as an isolated entity without other symptoms or signs of rosacea. It can be disfiguring and therefore distressing for patients. Some authorities consider rhinophyma to represent a different disease process.

•Lymphedema may be marked periorbitally, and on occasion, it is the presenting symptom.

•Symptoms of ocular rosacea may be accompanied by conjunctival injection, and rarely, chalazion and episcleritis may occur.

•Rosacea fulminans (pyoderma faciale) is fortunately a rare complication characterized by the development of nodules and abscesses with sinus tract formation accompanied by systemic signs.

•Both seborrhea and seborrheic dermatitis/blepharitis are not uncommonly observed in patients with rosacea. The reasons for these associations are not well understood.

•A rare caseating granulomatous variant of rosacea (acne agminata/lupus miliaris disseminatus faciei) can manifest with inflammatory erythematous or flesh-colored papules distributed symmetrically across the upper part of the face, particularly around the eyes and the nose. The lesions tend to be discrete, and surrounding erythema is not a marked feature but may be present. This pattern of rosacea is sometimes associated with scarring and may be resistant to conventional treatment.

Causes
A rosacealike syndrome (including perioral dermatitis) can result from the indiscriminate use of potent corticosteroids on the face. A number of aggravating factors may be recognized. Excess wind and UV light (weathering) exposure may accelerate the disease process. See Pathophysiology for more information.

References

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