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ROSACEA AND RELATED CONDITIONS

Rosacea

Etiology and pathogenesis

Rosacea is characterized by papules and pustules on the cheeks and nose of an adult. Comedones are absent. Fair-skinned patients (particularly Celts) are prone to this disease. Black-skinned patients are rarely affected.

    The exact cause of rosacea is unknown. Demodex mites (see later in this chapter) and gastrointestinal colonization with Helicobacter pylori have been proposed as the reason for rosacea, although never proved to have a true causal association. Recent evidence suggests that the warm milieu of the highly vascularized nose and cheeks causes resident bacteria to secrete a greater amount and a different composition of proteins as compared to with normal flora.

Clinical

Clinically, one sees inflammatory papules and pustules of the central face, especially the nose (Fig.10.23). A Maltese cross distribution has been described in which the nose, each cheek, central forehead, and point of the chin are affected. The areas immediately around the mouth and the eyelids are characteristically spared, although, paradoxically, the eyelids may be affected in isolation in a variant of rosacea.

    Erythematous papules usually outnumber pustules. The erythema may extend for 1 - 2 cm beyond the center. Comedones are absent. When severe, the redness may be confluent (Fig. 10.24).

     Telangiectasias may accumulate over time, and ocular involvement (e.g. blepharitis, conjunctivitis, and ocular dryness) may occur as well. Facial telangiectasias, a reddish appearance of the face, and periodic flushing are often associated. Hot liquids and a hot environment may induce flushing. When this flush is the main component, diagnosis may be difficult. Unilateral rosacea may suggest demodicosis (Fig.10.25). Enlargement of the nose (rhinophyma; Fig.10.26) occurs in the minority, and patients should be reassured that this is uncommon; it is more common in men, and the degree of rosacea present may be minimal. Tremendous edema may develop in rosacea (Fig.10.27), sometimes without preceding erythema or other skin lesions.

Differential diagnosis

There are three common differential diagnoses that arise.

Treatment

Tetracycline or oxytetracycline (250 - 500mg once or twice a day) is the mainstay of therapy for the papules and pustules of rosacea. In fact, these agents are more effective for rosacea than for acne, and a response is usually seen within 2 - 3 weeks. Patients can often maintain a clear face with a 500-mg capsule every day or every other day. It should be made clear to the patient that therapy may be lifelong. Control is the rule, not cure. It is also important to stress at the outset that papules and pustules may respond much better than the redness component, as explaining this at an early stage may avoid false expectations. Alternative oral antibiotics include doxycycline (100mg once or twice a day), minocycline (50 - 100mg once to twice a day), and azithromycin.

     Topical therapy is also available for rosacea, but it is not nearly as effective as oral tetracyclines. Sodium sulfacetamide 10% - sulfur 5% lotion, or metronidazole 0.75% or 1% cream every day, are modestly effective topical agents. Clindamycin lotion or benzoyl peroxide are alternatives. Combining an oral tetracycline with one of these initially is a good approach for more severe cases. Alternatively, topical therapy may be used to prevent relapse when oral medications are stopped. Isotretinoin has been employed for severe cases, although it has no product license for this indication. A low dose (e.g. 20 - 40mg/day) is usually given for 4 - 5 months.

     Topical retinoids have little benefit. Any trigger factors of flushing (e.g. hot liquids) should be avoided.
It is important to help the patient understand that this therapy will not improve any telangiectasias. These so - called broken blood vessels do not respond to medical therapy. They can, however, be cleared nicely with the pulsed dye laser.

     The excessive tissue of rhinophyma may be removed surgically to give excellent cosmetic results. Both cold steel and laser techniques may be employed.

     It is vital to avoid use of topical steroids in rosacea. While they transiently reduce redness, their effect is of limited duration, and suddenly stopping them may cause a rebound flare. In either of these situations, a stronger agent may be applied to control the disease, thus worsening the situation and increasing the risk of local side effects (see Ch. 3 and perioral dermatitis, later in this chapter). It is all too easy for the unwary to become trapped in this vicious cycle.

Figure 10.26  (a,b) Severe rhinophyma. (c) Severe rhinophyma in a black patient. (Panels a and b courtesy of Michael O. Murphy, M.D.)

Figure 10.27  Edema of the forehead from rosacea. The inflammation of rosacea may cause significant edema. Edema about the eyes or of the entire face occurs.

Ocular rosacea

A chronic blepharitis, conjunctivitis, and ocular dryness are common in patients with rosacea (Fig. 10.28). Keratitis may occur.

     Tetracycline or oxytetracycline is effective, as is doxycycline (100mg twice a day) or minocycline (100mg twice a day). Artificial tears are helpful for the dryness.

Figure 10.28  Ocular rosacea. Approximately half of patients with rosacea will have ocular rosacea as well. Dry eyes and irritation are common.

Steroid rosacea

Etiology and pathogenesis

The chronic use of a high-potency or oral steroid can result in an acneiform eruption called steroid rosacea. The exact mechanism is unknown.

Clinical

Relatively monomorphic erythematous papules and follicular pustules are characteristic (Fig. 10.29). Comedones are absent.

Differential diagnosis

The diagnosis is generally apparent from the clinical setting. The most important differential, especially if the patient has had prolonged oral steroid therapy, is to ensure that the lesions (especially pustules) do not represent opportunistic infection.

     The differential may include the following.

Figure 10.29  Steroid rosacea resulting from prolonged use of a high-potency topical steroid. Although the initial rash may be gone, the patient is continuing to use the medication, hoping that the new rash will improve.

Treatment

The offending steroid should be stopped, but the patient must be made aware that the disease may flare for the first 1 - 2 weeks. Tetracycline or oxytetracycline (e.g. 500mg twice a day) or minocycline (e.g. 50 -100mg twice a day), with topical metronidazole (0.75% gel twice a day) or benzoyl peroxide daily, are effective therapies and will blunt this flare.

Demodicosis

Clinical

Although the human Demodex folliculorum mite is a normal component of the facial skin, some patients can develop a rosaceaform pustular rash of the face (Fig. 10.25) in the setting of excessive numbers of Demodex mites, as seen on KOH scraping. Often, the rash is 'unilateral'. Topical tacrolimus has precipitated this condition.

Treatment

Topical permethrin 5% cream and oral metronizadole have been used.

Figure 10.30  Rosacea fulminans. (a) The acute onset of large, deep, inflammatory nodules and abscesses on the face of a young adult woman is characteristic of rosacea fulminans, formerly called pyoderma faciale. (b) Close-up view.

Rosacea fulminans

Etiology and pathogenesis

The acute onset of large, deep, inflammatory nodules and abscesses on the face of a young adult woman is characteristic of rosacea fulminans, formerly called pyoderma faciale (Fig.10.30). The cause is unknown. Bacterial cultures show the usual acne pathogens. Constitutional symptoms are generally absent (in contrast to AF). Comedones are absent, as are typical acne lesions on the chest and back. Increased facial oiliness prior to onset is typical.

Clinical

The chin, cheeks, and forehead are preferentially affected by inflammatory plaques with a variable amount of pustules. A localized form may occur, with a solitary lesion affecting the jaw, chin, or one cheek.

Differential diagnosis

The important differential is from infected lesions, such as staphylococcal abscesses. The main differentials are as follows.

Treatment

Prednisone or prednisolone at a dose of 0.5 - 1.0mg/kg per day is given for the first 1 - 2 weeks to gain control of the outbreak. Then isotretinoin (e.g. 0.2 - 0.5mg/kg per day) is begun. The oral corticosteroid may be tapered over 2 - 3 weeks and the isotretinoin continued until resolution occurs. Warm compresses and a high-potency topical steroid may be used during the first 1 - 2 weeks. If staphylococcal infection is a possibility when the patient is first seen, even though rosacea fulminans is the likeliest diagnosis, an antibiotic that might be useful for either condition (such as erythromycin 1 - 2g daily) may be given while awaiting confirmation that bacterial cultures are negative and before starting a systemic corticosteroid that would be contraindicated in the presence of infection; the advantage of erythromycin over tetracyclines is that it can be given concurrently with isotretinoin if required, so the treatments can overlap. Note that pus for culture from a fluctuant lesion should be obtained by aspiration; formal incision and drainage should not be done, as it causes too much scarring. Dapsone has also been used as alternative therapy.

PRACTICE POINTS

Crusted folliculitis of the scalp

Etiology and pathogenesis

Some adult men complain of very itchy bumps on the scalp. They usually scratch away any primary lesion such that, at the time of the examination, only a crusted papule hidden in the hair is seen. On occasion, the true primary lesion, a pustule, is found (Fig.10.31). Previous authors have called this condition acne necrotica, and others have opted for calling it P. acnes folliculitis, as they have found P. acnes in the follicles. Still others have found staphylococci, Malassezia spp., or gram-negative bacteria. Indeed, there may be several organisms that can cause crusted folliculitis of the scalp, some that are easily cultured and others that are not.

     Finally, some consider this to be a variant of rosacea or of seborrheic dermatitis.

Figure 10.31  Crusted folliculitis of the scalp. Pustules occur first, but they are usually scratched away quickly.

Clinical

As documented earlier, the lesions may be a pustule, a crusted papule, or simply a mark from previous excoriation.

Differential diagnosis

The differential is usually between the following.

Treatment

Picking should be discouraged. A potent topical steroid may be prescribed if the itch is significant. The patient must be encouraged not to scratch. An oral antibiotic (e.g. tetracycline, 500 - 1000mg/day) is often effective and should be tried. Other antibiotics (such as amoxicillin) may be used on the basis of bacteriology results, but culturing the causative organism (and knowing whether it is a cause, a relevant contributor, or simply a contaminant) may be difficult. Some have considered seborrheic dermatitis to be contributory, therefore this should be cleared with ketaconazole shampoo or a tar shampoo every day or every other day. A suggested regimen is ketoconazole shampoo daily combined with tetracycline 500mg b.i.d. Finally, isotretinoin has been recommended for therapy-resistant cases.

Perioral dermatitis (periorificial dermatitis)

Etiology and pathogenesis

Perioral dermatitis is an inflammatory condition that occurs around the mouth. It has a strong female predilection, and most patients are young or early middle-aged adults. Its cause is unknown, although prolonged use of a potent topical steroid is one definite predisposing and aggravating factor. Some cases undoubtedly start out as seborrheic dermatitis, which the patient treats with a potent topical steroid. Still others seem to arise de novo. Histologically, perioral dermatitis resembles rosacea, and it is unfortunate that the 'dermatitis' part of its name has been used, as it encourages treatment with topical steroids as for eczema, rather than steroid avoidance (as discussed earlier for rosacea).

     One recent study compared the follicular organisms in patients with perioral dermatitis versus those with seborrheic dermatitis. In all patients with perioral dermatitis and in two normal subjects, 20 - 70% of sample hairs were positive for fusiform bacteria. Malassezia-positive hairs were rarely seen in these cases. Seborrhoeic dermatitis showed the opposite results. Perioral dermatitis may tend to develop under fusiform bacteria- rich conditions, rather than Malassezia-rich conditions as in the case of seborrhoeic dermatitis.

Figure 10.32  Periorificial dermatitis. (a) Periorificial papules are seen. Itching and burning are common in this disease of young women. (b) Tiny erythematous papules, pustules, and a small amount of scale occur about the mouth in periorificial dermatitis. (c) Confluent erythema of the nasolabial fold is a classic sign, and a narrow zone about the lips is typically spared. The use of a potent topical steroid may trigger or contribute to the eruption. Comedones are absent.

Clinical

Multiple red papules, and occasionally pustules, around the mouth in a woman aged 20 - 30 years, are characteristic (Fig.10.32). Often there is a sensation of burning or itching. Confluent erythema of the nasolabial fold is characteristic. As a rule, perioral dermatitis is bright red and papular, whereas seborrheic dermatitis is red-brown and scaly. Rarely, children may be affected. Variants occur around the nostrils and eyes (Fig. 10.33), and are treated similarly. For this reason, the term periorificial dermatitis is preferred by some.

Figure 10.33  Periorficial dermatitis about the eyes. Usually, the outer, lower area is affected. This condition is a variant of perioral dermatitis. Oral tetracycline is effective.

Figure 10.34  Papular periorficial dermatitis in an older teenaged boy. This condition is thought to represent a papular variant of periorificial dermatitis in the darker-skinned patient.

Differential diagnosis

The clinician should consider the following.

Treatment

Perioral dermatitis has many similarities to rosacea, one of them being its responsiveness to tetracycline. Indeed, tetracycline or oxytetracycline (250 - 500mg twice daily) is the best current therapy. It will clear the condition within 4 - 8 weeks. Some patients may be able to stop the tetracycline then and remain clear, while others may need to keep it on hand for occasional flares, and finally some may need to take a low-maintenance dose (from one per day to one per week). Doxycycline or minocycline are effective oral alternatives. Topical therapy (e.g. metronidazole 0.75 - 1% or benzoyl peroxide) is significantlys less effective, but may be preferred by some.

     If any topical steroids are being used, they should be discontinued. Patients may be told that their skin has become 'addicted' to the steroid, so it may flare for several weeks on its discontinuation. If the patient had been on a high-potency topical steroid, one of low potency may be given for 10 days to blunt the flare.

     For children aged 12 years or younger, tetracyclines must be avoided. Topical metronidazole gel or cream, 0.75 -1%, twice daily is the preferred treatment. Alternatively, one can try topical clindamycin, topical erythromycin or benzoyl peroxide, or both.

Lupus miliaris disseminatus faciei (‘acne agminata’)

Etiology and pathogenesis

Lupus miliaris disseminatus faciei (LMDF) is a papular eruption of the face that histologically shows tuberculoid granulomas. No relation to tuberculosis has been found, however, and LMDF is thought to be a form of granulomatous rosacea.

Figure 10.35  Lupus miliaris disseminatus faciei. Despite its name, no relationship to lupus is present. This condition is thought to represent a form of granulomatous rosacea.

Clinical

Multiple, symmetric, flesh-colored to brown papules are distributed on the forehead, temples, eyelids, chin, and cheeks (Fig. 10.35).

Differential diagnosis

This includes acne, plane warts, papular sarcoidosis, miliary forms of lymphocytoma cutis, and histiocytoses. The latter conditions do not normally enter the differential of other disorders discussed in this chapter, but do need to be considered in this condition due to its chronicity and therapeutic resistance.

Treatment

This condition is often difficultto treat. Tetracycline or erythromycin (1 g/day) can be tried. Isotretinoin and dapsone have been reported to be helpful. None of these options are consistently reliable.

White/Cox: Diseases of the Skin, 2ed.(c) 2006, Elsevier Inc. All rights reserved.