Erythematotelangiectatic rosacea (ETR) is characterized by persistent facial erythema and flushing along with telangiectases, central face edema, burning and stinging, roughness or scaling, or any combination of these signs and symptoms (Fig. 81-1). Mild, moderate, and severe subtypes are recognized. In contrast, PPR manifests as persistent, central-face erythema with papules and pustules that predominate in convex areas (Fig. 81-2).9 Again, mild (see Fig. 81-2A), moderate, and severe forms (see Fig. 81-2B) are distinguished. Burning and stinging of the facial skin may occur in PPR, but occurs less commonly compared with ETR. Flushing is often less severe in PPR compared with ETR. In both subtypes, erythema spares the periorbital areas. Edema can be mild or severe. Severe edema may take on the plaque morphology of solid facial edema.1,23 This occurs most often on the forehead and glabella and it less commonly affects the eyelids and upper cheeks.
Phymatous rosacea is characterized by patulous follicular orifices, thickened skin, nodularities, and irregular surface contours in convex areas (Fig. 81-3). Here also, mild, moderate, and severe subtypes are distinguished. Phyma most often occurs on the nose (rhinophyma), but may also develop on the chin (gnathophyma), forehead (metophyma), eyelids (blepharophyma), and ears (otophyma).24 Women with rosacea do not develop phyma, perhaps for hormonal reasons, but they can manifest sebaceous or glandular features characterized by thickened skin and large follicular orifices.9
Ocular rosacea may develop before cutaneous symptoms in up to 20% of affected individuals25 (Fig. 81-4). In half of patients, ocular symptoms develop after skin symptoms. In a minority, skin and eye symptoms present simultaneously.26 Ophthalmic rosacea severity does not coincide with cutaneous rosacea severity. Ocular involvement may manifest as blepharitis, conjunctivitis, iritis, scleritis, hypopyon, and keratitis; mild, moderate, and severe subtypes are recognized (see Fig. 81-4).26 Blepharitis is the most common feature, characterized by eyelid margin erythema, scale, and crust, with the variable presence of chalazia and staphylococcal infections due to underlying meibomian gland dysfunction.25 Photophobia, pain, burning, itching, and foreign body sensation may be part of the ocular symptom complex. In severe cases, rosacea keratitis may lead to vision loss.
Granulomatous rosacea is considered the only true rosacea variant.1 Granuloma formation is a histologic feature of the condition; the clinical features of granulomatous rosacea include yellow–brown or red papules or nodules that are monomorphic and located on the cheeks and periorificial facial skin27 (Fig. 81-5). Upon diascopy, these papules reveal apple-jelly-like change in color similar to sarcoidosis or lupus vulgaris. The background facial skin is otherwise normal. Other signs and symptoms of rosacea are not required to make a diagnosis of granulomatous rosacea.
Rosacea is a clinical diagnosis; histology may be helpful when the facial distribution is atypical or when granuloma formation is suspected. In ETR, a sparse, perivascular lymphohistiocytic infiltrate is accompanied by dermal edema and ectatic venules and lymphatics.16 Severe elastosis may be present. Similar features are found in the papulopustular subtype, but the inflammatory infiltrate also surrounds hair follicles and sebaceous glands. Phymatous rosacea is characterized by prominent elastosis, fibrosis, dermal inflammation, sebaceous hyperplasia, and hypertrophy of sebaceous follicles.16,28 Epithelialized tunnels undermine the hyperplastic tissue and are filled with inflammatory debris. D. folliculorum mites may be found in all types of rosacea within the follicular infundibula and sebaceous ducts.28
Approach to patient. Diagnosis and therapy of the rosacea subtypes. aRegimen recommendation based on author experience. ETR = erythematotelangiectatic; S/S = sodium sulfacetamide/sulfur.
Systemic diseases that must be differentiated from rosacea include polycythemia vera, connective tissue disorders (lupus erythematosus, dermatomyositis), carcinoid syndrome, mastocytosis, and neurologic causes of flushing. Neurologic causes include brain tumors, spinal cord lesions, orthostatic hypotension, migraine headaches, and Parkinson disease.29 Unilateral auriculotemporal flushing may follow parotid gland injury or surgery.30
Medication-induced flushing has been associated with all vasodilators, calcium channel blockers, nicotinic acid (niacin), morphine, amyl and butyl nitrite, cholinergic drugs, bromocriptine, thyroid releasing hormone, tamoxifen, cyproterone acetate, systemic steroids, and cyclosporine.29,31 The flush associated with nicotinic acid may be blocked with aspirin or indomethacin.32 Disulfiram, chlorpropamide, metronidazole, phentolamine, and cephalosporins induce flushing when they are combined with alcohol.29 Amiodarone has induced rosacea and multiple chalazia.33 Food additives, including sulfites, sodium nitrite, nitrates, and monosodium glutamate, may also cause flushing.29 Dumping syndrome following gastric surgery is characterized by flushing, sweats, tachycardia, and abdominal pain.
Cutaneous conditions that may mimic rosacea include topical steroid-induced acneiform eruption (formerly steroid-induced rosacea), acne vulgaris, perioral dermatitis, inflammatory keratosis pilaris, and chronic photodamage. In particular, acne vulgaris (see Chapter 80) and rosacea may coexist, although rosacea most often begins and reaches its peak incidence in the decades after acne declines. The primary differentiating feature between acne vulgaris and rosacea is the presence of open and closed comedones in acne alone.2
Rosacea fulminans, also known as pyoderma faciale and rosacea conglobata, occurs mainly in women in their 20s.2,34,35 It is characterized by the sudden onset of confluent papules, pustules, nodules, and draining sinuses on the chin, cheeks, and forehead within a background of diffuse facial erythema. Rosacea fulminans has proved controversial in its classification and was not included as a rosacea subtype or variant by the NRS Expert Committee.1,2
Perioral dermatitis (see Chapter 82) differs from rosacea in its facial distribution, signs, symptoms, and patient demographic. It is characterized by perioral, and sometimes periorbital, microvesicles, micropustules, scaling, and peeling. It affects younger women and also occurs in children. Central face erythema and inflammatory papules are not features of perioral dermatitis.9 Therapy includes topical and oral antimicrobials. Perioral dermatitis is exacerbated by topical steroid use.
Steroid-induced acneiform eruption (see Chapter 80) can mimic PPR. With prolonged use of topical steroids on the face, monomorphic inflammatory papules may develop.1 The treatment is discontinuation of the topical corticosteroid and initiation of an oral tetracycline, a topical antimicrobial, a topical calcineurin inhibitor, or a combination of these agents.36,37 This regimen is generally continued for 1 to 3 months, and relapse does not tend to occur as long as topical steroids are not reintroduced.
In chronic photodamage (see Chapter 90), telangiectases and erythema are prominent features. However, unlike rosacea, actinic damage affects the periphery of the face and neck, the upper chest, and the posterior auricular skin. Hyperpigmentation and hypopigmentation are additional feature of sun damage not observed in rosacea. Chin involvement is both mental and submental in rosacea, while in chronic photodamage there is submental sparing.9