Rosacea and rhinophyma

Rosacea is a chronic relapsing inflammatory skin disease with a high prevalence among adults of Northern European heritage with fair skin. Symptoms present in various combinations and severity, often fluctuating between periods of exacerbation and remission¹ .

Rosacea is characterised by recurrent episodes of facial flushing with persistent erythema, telangiectasia, papules and pustules. Ocular rosacea is usually bilateral and causes a foreign-body sensation. Rhinophyma is an enlarged nose associated with rosacea which occurs almost exclusively in men.

Aetiology²

The exact cause of rosacea is unclear. It is likely to be multifactorial involving genetic and environmental factors.

A systematic review found that compared with controls, people with rosacea were more likely to be infested with Demodex mites and had significantly higher skin density of Demodex mites than controls. However variability between studies was high and a causal relationship could not be confirmed.

Additional factors that may trigger or worsen rosacea include:

• Increasing age.

• Photosensitive skin types.

• Ultraviolet radiation exposure.

• Smoking.

• Heat or cold ambient temperature.

• Spicy foods and hot drinks.

• Alcohol.

• Emotional stress and exercise.

• Drugs such as calcium-channel blockers (may worsen vasodilatation and flushing) and topical corticosteroids.

Epidemiology

• In Europe, there is an increasing prevalence from South to North: in Germany prevalence is 2.2%, in Sweden 10% and in Estonia 22%³ .

• Rosacea is primarily a condition of the white population; it is three times more common in women than in men and has a peak age of onset between 30 and 60 years.

• For a diagnosis to be confirmed the erythema should have been present for at least three months.

Presentation

Symptoms

• Patients usually complain of the skin condition but direct enquiry may often reveal a long history of flushing back to early teens or before.

• The symptoms are initially intermittent but progress to a constant flushing with obvious telangiectasia.

• A few complain of gritty eyes and facial oedema.

Signs

The disease tends to be progressive but that does not mean that everyone will develop all features.

• The skin is not greasy as in acne and may be rather dry.

• Erythema and telangiectasias over the forehead and cheeks are variable.

• Although the usual areas affected are the nose, cheeks and forehead, other areas, such as the neck, chest and ears, can become involved.

• Sebaceous glands are prominent.

• The nose may be enlarged and distorted by rhinophyma.

• There may be periorbital oedema.
  

Rosacea with telangiectasia

Adult male rosacea side view

By Srecan, CC BY-SA 4.0, via Wikimedia Commons

Nose and cheeks displaying rosacea

By Michael Sand, Daniel Sand, Christina Thrandorf, Volker Paech, Peter Altmeyer, Falk G Bechara, CC BY 2.5, via Wikimedia Commons

Classification⁴

The first classification of rosacea was published in 2002, recognising rosacea as a syndrome that is comprehensively depicted by four distinct clinical subtypes defined as erythematotelangiectatic, papulopustular, phymatous, and ocular rosacea:

• Papulopustular rosacea (PPR) is the classical presentation. Patients are typically middle-aged women with a red central portion of their face that contains small erythematous papules surmounted by pinpoint pustules. They may have flushing. Telangiectasias are often present but may be difficult to distinguish from the erythematous background in which they exist.

• Phymatous rosacea shows marked skin thickenings and irregular surface nodularities of the nose, chin, forehead, one or both ears and/or the eyelids. There are four histological types of rhinophyma that include glandular, fibrous, fibroangiomatous and actinic.

• Ocular rosacea may precede the cutaneous form by years but often they develop together. The ocular signs include blepharitis, conjunctivitis, inflammation of the lids and meibomian glands, interpalpebral conjunctival hyperaemia and conjunctival telangiectasia. There may be stinging or burning of the eyes, dryness, irritation with light, or foreign body sensation. This may sometimes be confused with blepharitis.

• Erythematotelangiectatic rosacea shows central facial flushing, often with burning, stinging or itching. The redness usually spares around the eyes. They usually have skin with a fine texture that lacks a sebaceous quality typical of other types. The erythematous areas of the face at times appear rough with scale likely due to chronic, low-grade dermatitis. The burning or stinging is exacerbated when topical treatments are applied. The flushing often progresses to a permanent erythema and telangiectasias over the affected areas.

An updated rosacea classification was published in 2016, emphasising a more patient-centric phenotype approach:

• Diagnostic features:

  • Persistent centro-facial erythema associated with aggravation by trigger factors.

  • Phymatous changes.

• Major features:

  • Flushing/transient erythema.

  • Inflammatory papules and pustules, telangiectasia, ocular manifestations, lid margin telangiectasia, blepharitis, keratitis, conjunctivitis, and sclerokeratitis.

• Secondary features:

  • Burning sensation.

  • Stinging sensation, oedema, dry sensation of the skin.

Differential diagnosis

• Acne vulgaris, especially if it presents early in life. However, there are usually no comedones in people with rosacea. In addition, rosacea also tends to worsen with ultraviolet light, whereas acne tends to improve.

• Seborrhoeic dermatitis which is more likely to cause flaking and dryness of the skin. It can co-exist with rosacea though.

• Perioral dermatitis from use of potent steroids on the face.

• Systemic lupus erythematosus or discoid lupus erythematosus.

• Polymyositis and dermatomyositis.

• Photosensitive eruptions.

• Rhinophyma may resemble lupus pernio from sarcoidosis.

• Especially when flushes start in a woman aged around 50, hot flushes may suggest the menopause.

• Ophthalmic symptoms may not be recognised as part of the disease although they occur to some extent in over 50%.

Flushing

Causes of flushing are many and include:

• Heat or changes in temperature.

• Alcohol.

• Caffeine.

• Spicy foods.

• Stress or embarrassment.

• Sun or wind.

• Medication that causes vasodilatation.

Investigations

In all types of rosacea the diagnosis is usually made clinically after taking a history and examining the patient. Many patients only have mild symptoms and do not actually consult their doctor. A history of flushing preceding onset of the erythema and an association with triggers can be helpful.

Where the diagnosis is in doubt a skin biopsy can be helpful; however, this is unnecessary in most cases.

Management

Treatment of rosacea depends on the severity and type of rosacea present. Although rosacea's impact on physical health is limited, it has profound effects on a person's psychological well-being⁵ . Therefore, treating rosacea can greatly affect a person's quality of life. Although there are numerous treatments available, none of these is completely curative.

The latest advances in rosacea treatment include skin care and cosmetic treatments, topical therapies, oral therapies, laser- and light-based therapies, injection therapies, treatments for specific types of rosacea, treatments for systemic comorbidities, and combination therapies⁶ .

Non-drug

• Reassure patients of the benign nature of the condition and the relative rarity of any complications (including development of rhinophyma).

• Avoid precipitating or aggravating factors for their trigger factors of flushing.

• Facial massage may reduce oedema.

• Sunscreens should be applied daily.

• Avoid astringents, toners, menthols, camphor, waterproof cosmetics requiring solvents to be removed, or products containing sodium lauryl sulfate.

• Judicious use of cosmetics may improve appearance significantly and, in doing so, greatly reduce distress. If the skin is dry use emollients (hypoallergenic and non-comedogenic emollient creams).

• Avoid topical steroids.

Drugs

• Mild-to-moderate rosacea should be treated with a topical preparation.

• Topical metronidazole 0.75% is a common first-line option.

• Azelaic acid 15% gel is an alternative, especially in those with more inflammatory rosacea. It may be more effective but can cause sensitivity reactions in some patients.

• Moderate-to-severe papulopustular rosacea usually requires oral antibiotics. These are thought to act by virtue of their anti-inflammatory rather than antimicrobial action.

• Commonly used preparations are oxytetracycline 500 mg bd, lymecycline 408 mg od or doxycycline 40 mg od⁷ .

• Erythromycin 500 mg bd can be given as an alternative.

• There is some evidence that oral doxycycline as well as minocycline with topical azelaic acid or topical metronidazole leads to substantial improvements in inflammatory lesion counts compared to monotherapy⁸ .

• Isotretinoin is occasionally used for refractory cases⁸ .

• Ivermectin 1% is a topical cream which acts by binding selectively to glutamate-gated chloride ion channels that are present in invertebrate (but not mammalian) nerve and muscle cells. It causes parasite death by enhancing cell membrane permeability. In addition to killing the Demodex mites, ivermectin displays antimicrobial, antibacterial, and anti-inflammatory activities⁹ .

• Ivermectin 1% cream has been demonstrated to be significantly superior to metronidazole 0·75% cream and has achieved high patient satisfaction¹⁰ .

• Brimonidine is a novel therapeutic agent targeting the facial flushing and erythema of rosacea through its alpha-2 adrenergic receptor agonist activity¹¹ .

• Once-daily brimonidine gel 0.5% has a good safety profile and has been demonstrated to provide significantly greater efficacy relative to vehicle gel for the treatment of moderate-to-severe erythema of rosacea, as early as 30 minutes after application¹² .

• However, rebound erythema secondary to use of brimonidine can occasionally occur¹³ .

• A Cochrane review of treatments for rosacea has summarised that there is high-quality evidence to support the effectiveness of topical azelaic acid, topical ivermectin, brimonidine, doxycycline and isotretinoin for rosacea. Moderate-quality evidence is available for topical metronidazole and oral tetracycline. There is low-quality evidence for low-dose minocycline, laser and intense pulsed light therapy and ciclosporin ophthalmic emulsion for ocular rosacea¹⁴ .

Other treatments

• Laser treatment can obliterate telangiectasia.

• Camouflage treatments (available via The Red Cross) can be really effective.

Ocular rosacea⁷

• Patients with ocular rosacea should undertake regular lid hygeine (as in the treatment of blepharitis) using diluted baby shampoo (diluted 1:10 in warm water) and a cotton bud with warm compress.

• Artificial tears should be used at frequent intervals.

• Systemic tetracyclines are an effective treatment for ocular rosacea.

• Retinoids should be avoided in these patients. Retinoids can worsen their symptoms and lead to severe keratitis.

• If the patient is currently using topical corticosteroids on the face, these must be stopped.

Referral²

Consider arranging referral to a dermatologist if:

• Persistent or severe erythema, inflammatory papules and/or pustules have not responded to optimal management in primary care.

• Severe telangiectasia has not responded to self-management advice.

• An uncertain diagnosis.

Consider arranging referral to a local skin camouflage service (may be available through the local dermatology service). People may self-refer to the charity Changing Faces, which provides education from skin camouflage practitioners on the use and application of cosmetic camouflage creams and powders¹⁵ .

Consider arranging referral to a plastic surgeon if there is prominent non-inflamed phymatous disease.

Arrange referral to an ophthalmologist if:

• A serious eye complication, such as keratitis or anterior uveitis, is suspected.

• Other associated ocular symptoms are severe or do not respond to optimal management in primary care.

Prognosis²

The long-term prognosis of rosacea is variable:

• Rosacea may progress in severity and also transform to include additional clinical phenotypes. It is often characterised by repeated remissions and exacerbations.

• Progressive disease may include severe complications, such as rosacea fulminans

• The risk of relapse is high.

• A retrospective study of 234 people with different presentations of rosacea in dermatology clinics, with a median follow up of 17.5 months, found:

  • Partial remission in 61.5% of all participants.

  • Complete remission in 20.9% of all participants (median time to complete remission was 56 months).

  • People with inflammatory papules and pustules alone had a more favourable prognosis than other or mixed clinical presentations.

Rhinophyma^{16 17}

Rhinophyma is an advanced stage of rosacea affecting the nasal soft tissues and resulting in disruption of the nasal architecture, airway obstruction, and disfigurement of the nose. Rhinophyma presents with hypertrophy of the nasal soft tissues, erythema, telangiectasias, nodules, and lobules with a bulbous appearance. Significant psychosocial morbidity is associated with rhinophyma.

Treatment options vary, and include laser therapy, scalpel excision, electrocautery and the subunit method (uses six nasal flaps to provide exposure for removal of rhinophymatous tissue and enhance structure).

A review found that the subunit method had the highest complication and revision rates, followed by carbon dioxide laser therapy. Outcomes between carbon dioxide laser and scalpel therapy and electrocautery were equivalent. Patient satisfaction was common post-therapy regardless of the treatment method. The review found that over 89% of patients would recommend undergoing treatment for rhinophyma irrespective of treatment type.