Rosacea Flashcards
Subtypes
Erythematotelengiectatic rosacea (most common)
Papulopustular rosacea
Phymatous rosacea
Ocular rosacea
Grading
Grade 1 (mild disease)
Grade 2 (moderate disease)
Grade 3 (severe disease)
Psychological, social amd occupational impact
Introduction
Chronic
Fluctuating severity
Middle aged
Pale Fair skinned sun-sensitive individuals —> Celtic “curse of the Celts”
Cause unknown
Approach to tretment dependant on subtype - Erythematotelengiectatic —> laser Papulopustular —> topical/oral ABs Phymatous —> surgery Ocular —> Ophthal referral
M develop more severe rosacea than F + more likely to develop rhinophyma
Associated diseases
Facial seb derm —> contributes to facial erythema, skin sensitvity —> needs to be Rx separately for optimal results
GI abnormalitites (skin and gut syndrome) i..e small intestime bacterial infection
Causes
UV light —> supported by facial distribution, mainly in the convexities + bald scalp males
Possibly subtype specific (erythematotelemgiectatic rosacea)
Altered innate immune response (papulopustular rosacea)
Demodex folliculorum mite proliferation in pilosebaceous follicles (skin and ocular inflammation of papulopustular roscea)
Alterations in the skin microenvironment i.e. changes in lipid profile, pH, skin brrier function —> facilitate overgrowth of commensal organisms
Upregulation of matrix metalloproteinases (phymatous rosacea)
Meibomian gland dysfunction (ocular rosacea) —> meibomian cysts (chronic inflammation of meibomian glands)
Risk factors
Family history (genetic predisposition) —> develop rosacea at earlier age than those without family hx
Skin types 1, 2
Causative organisms
Demodex folliculorum mites (papulopustular rosacea)
? Staph epidermidis
? Chlamydophila pneumoniae
Demodex-associated Bacillus oleronius
Environmental triggers
Mainly linked to erythematotelengiectatic subtype
Increased environmental temperature/diet —> transient increase in facial erythema, exacerbate flushing
- Ingestion of hot liquids
- Spicy foods
- Alcohol
- Large meals
Subtype 1: Erythematotelengiectatic rosacea
Skin type 1, 2
Complain of gradual increase in facial redness
Central face
+/- lateral cheeks
+/- ears
+/- sides of neck
Fixed facial erythema
Telengiectases
Transient rapid onset flushing d/t facial vascular reactivity with emvironmental temperature change + dietary components (hot liquids, spicy foods, alcohol)
Sensitive + dry skin —> easily irritated with skincare products previously well tolerated i.e. soaps, aftershaves, perfumed products, astringents
Burning, stinging sensation on the skin
Exacerbated by sunlight, wind exposure
Frequent actinic damage i.e. AKs, solar lentigines face, ears, scalp
MILD (grade 1)
Mild erythema
Occasional flushing
MODERATE (grade 2)
Moderate erythema
Telengiectases
Frequent flushing
SEVERE (grade 3)
Marked erythema
Many telengiectases
Severe flushing
DDX
Chronic photodamage
Seb derm (mimic erythematotelengiectatic rosacea or accompany papulopustular rosacea) —> orange red appearance, adherent large scales, distribution on scalp, eyebrows, ala nasi
Contact dermatitis
Systemic and subacute Lupus erythematosus, Dermatomyositis —> photodistributed facial erythema
Ulerythema ophryogenes (variant of keratosis pilaris) —> follicular keratoses, loss of eyebrow hair
Trichostasis spinulosa —> redness of nose + prominent follicular openings with plugging
COURSE/PROGNOSIS Persistent facial redness over time prominent malar telangiectasia increasingly sensitive facial skin i.e. burning, stinging Intolerance of temperature changes Facial flushing more problematic
GENERAL MX
As per general skin cares and photoprotection
Avoid triggers that provoke flushing -
- Hot drinks
- Spicy foods
- ETOH
Psychological counselling, group therapy sessions for flushing tendencies
TOPICALS (first line)
Topical brimonidine
INTRALESIONAL for recalcitrant flushing tendencies (third line)
Botox
SYSTEMICS for persistent flushing tendency (second line)
Low dose propanolol, carvedilol
VASCULAR LASER for erythema and telengiectasia, stabilises vascular reactivity —> effect for years, but relapses, no effect on subsequent papules/pustules (second line)
532nm KTP
595nm PDL
595nm Nd:YAG
SURGICAL (for disabling cases)
Selective sympathectomy —> beware high risk of serious adverse effects
Subtype 2: Papulopustular rosacea
Complain of developing groups of “spots” red bumps, pimples
No pain, itch or discomfort
Proximal cheeks Central chin Nose Central forehead \+/- scalp \+/- behind ears
Central facial erythema mostly related to Prominent perilesional Erythema —> coalescing into plaques of inflammatory erythema in severe cases
Mild facial oedema esp if widespread inflammatory lesions
Small dome-shaped papules may have tiny pustulation at apex/papulopustules (appear to be in different stages of evolution, untreated lesions wax and wane spontaneously over weeks) —> heal without scarring —> may leave post-inflammatory erythema
No nodules/cysts
+/- telengiectases
+/- flushing and skin sensitivity (not as prominent as erythematotelengiectatic rosacea)
+/- dryness/dermatitis in severe cases
MILD (grade 1)
Few papules/pustules <5
Mild perilesional erythema
Little tendency to flush
MODERATE (grade 2)
Several papules/pustules 5-10
Significant coalescing erythema around lesions
Tendency to temperature intolerance and flushing
SEVERE (grade 3) Many papules/pustules > 10 Plaques of coalescing erythema \+/- oedema \+/- scaling/dermatitis Marked intolerance of temperature change cold to heat —> flushing
DDX
Acne vulgaris —> oilynskin, open and closed comedones, cystic lesions, scarring
Granulomatous rosacea
Perioral dermatitis (d/t topical or inhaled CS) —> monomorphic small papulovesicles
Tinea faciei —> papules, pustules, asymmetrical, peripheral scale at the border, progressife enlargement, itch
Jessner’s lymphocytic infiltrate —> persistent facial erythematous papules, larger, no pustules, resistant to usual rosacea Rx, can scar, bx helpful
Pityriasis folliculorum (d/t profound infestation of facial hair follicles in some patients with papulopustular rosacea vs occur in isolation without significant inflammatory lesions) —> localised erythema, glazed/frosty skin surface, multiple fine follicular scales, mild itch +/- papules/pustules, dermoscopy = follicles with fine projecting keratinous material
Rosacea-like dermatoses (d/t EGFRi, tyrosine kinase inhibitors i.e. cetuximab, erlotinib OR frequent app fluorinated TCS) —> acneform eruption, sudden onset, acute inflammatory lesions
Lymphocytoma cutis
COURSE/PROGNOSIS
Recurrent episodic crops of papules > papulopustules centrofacial region
Persistent perilesional erythemaafter inflammatory lesions fade
May burn itself out
GENERAL MX
As per general skin cares and photoprotection
TOPICALS (creams are better tolerated if skin acutely inflamed) for papules and pustules
Metronidazole 0.75% gel or cream (Rozex)
Azelaic acid 15% gel
Ivermectin 1% cream
Compounded Sodium sulfacetamide 10% + sulphur 5% cream
Intial clearing phase —> apply BD before moisturiser for 6-8 weeks
As inflammatory lesions clear —> apply nocte before moisturiser
If skin remains clear after 3-4 months —> cease Rx, and continue regular moisturiser
If flares —> restart BD Rx
TOPICALS for perilesional erythema (no effect on papule and pustule formation)
Brimonidine (alpha receptor agonist)
SYSTEMIC (second line)
Antibiotics in full acne dosage but for shorter period of 6-8 weeks
- Tetracycline
- Doxycycline 50mg daily as effective as 100mg daily with less dverse effectsmand bacterial resistance
- Minocycline
- EES
- TMP
can be combined with topicals
TOPICALS (third line) —> If demodex proliferation relevant
Ivermectin 1% cream
Permethrin 5% cream
Crotamiton cream
SYSTEMIC (third line) —> inflammation tends to recur when discontinued —> alternative longer term Rx will need to be introduced when control achieved, before cessation
Metronidazole
Low dose Isotretinoin
Subtype 3: Phymatous rosacea
No associated pain, discomfort
May note unpleasant oiliness of the skin surface
May note malodorous greasy material discharge on squeezing the skin
Link to Demodex mites
May appear de novo (without preceding inflammatory changes)
OR
Have pre-existing papulopustular rosacea
Rhinophyma (enlargement of nose) Otophyma (enlargement of ears) Metophyma (enlargement of forehead) —> leonine facies Gnatophyma (jaw) Chin (mentophyma) Eyelids (blepharophyma)
Thickened nodular skin
Increased prominence of pores/patulous follicles (early disease)
Bulbous distorted features (advanced disease)
+/- prominent perinasal telengiectases
Flushing not common
MILD RHINOPHYMA (grade 1)
Puffiness of nose
Prominent folliclar openings (patulpus follicles)
No change in nasal contour
MODERATE RHINOPHYMA (grade 2)
Bulbous nasal swelling
Change in nasal contour without nodular distortion
SEVERE RHINOPHYMA (grade 3)
Marked nasal swelling
Nasal distortion with nodular component
DDX
Solid facial lymphoedema (Morbihan disease) —> ? Variant of rosacea i.e. lymphoedematous rosacea where lymphatic drainage rrom the face is defective, mimics rhinophyma when involves nose
Cutaneous TB
Lupus pernio (sarcoidosis of the nose) —> no patulous follicles, firm and indurated rather than soft and fleshy, cyanotic hue, bx non-caseating granulomas
Chronic cutaneous lupus erythematosus of the nose
Granuloma faciale —> indurated plaques with predilection for the nose, chronic, Rx resistant, characteristic bx
Malignancy i.e. SCC, BCC, lymphoma —> bx if atypical, progressive, unresponsive
lymphomacytoma cutis —> papules, nodules face and ears
COURSE/PROGNOSIS
Progressive —> enlargement to distortion unless treated
GENERAL MX
As per general skin cares and photoprotection
TOPICALS (first line)
For accompanying inflammatory lesions (creams are better tolerated if skin acutely inflamed) as for papulopustular rosacea -
- Metronidazole 0.75% gel or cream (Rozex)
- Azelaic acid 15% gel
- Ivermectin 1% cream
- Compounded Sodium sulfacetamide 10% + sulphur 5% cream
Intial clearing phase —> apply BD before moisturiser for 6-8 weeks
As inflammatory lesions clear —> apply nocte before moisturiser
If skin remains clear after 3-4 months —> cease Rx, and continue regular moisturiser
If flares —> restart BD Rx
For large occluded follicles -
- Skin peeling agent
SYSTEMIC (first line) for accompanying inflammatory lesions as in papulopustular rosacea -
Antibiotics in full acne dosage but for shorter period of 6-8 weeks
- Tetracycline
- Doxycycline 50mg daily as effective as 100mg daily with less dverse effectsmand bacterial resistance
- Minocycline
- EES
- TMP
PHYSICAL (first line) for telengiectases Electrocautery Vascular laser - 532nm KTP - 595nm PDL - 595nm ND:YAG
SYSTEMIC (second line) for rhinophyma —> progression when Rx ceased
Low dose isotretinoin 10-20mg daily for 2-6 months
PHYSICAL (third line) for rhinophyma —> excellent results, sustained long term
Laser ablation -
- 10600nm CO2
- 2940nm Er:YAG
SURGICAL (third line) for rhinophyma —> excellent results, sustained long term
Surgical remodelling -
- Hot wire loop
- Cold scalpel
Subtype 4: Ocular rosacea
Usually bilateral but severity in esch eye may vary
Dry gritty sensation
+/- itch
Watery eyes
Conjunctival hyperaemia/telengiectasia
Collarettes of scale around base of eyelashes
Swelling and erythema/telengiectasia of the eyelid margins
Blepharitis with crusting
Styes at the eyelid margins - chalazia (painless), hordeola (painful)
+/- Conjunctivitis, keratitis, episcleritis, scleritis, iritis
MILD (grade 1)
Mild itch, gritty feeling
Midl scaling/erythema of lid margins
Mild conjunctival injection
MODERATE (grade 2) Burning, stinging sensation Crusting/marked erythema of lid margins Collarettes and sleeves of keratin on the lash shafts Conjunctival injection Hordeolum/chalazion formation
SEVERE (grade 3) Pain/photophobia Blurred vision Loss of eyelashes (madarosis) Corneal changes Scleral involvement
DDX
Other causes of blepharitis
Dry eye syndrome
COURSE/PROGNOSIS Chronic Episodes of exacerbation Constant dry eyes —> susceptible to secondary bacterial infection (mainly staph) if not treated Intermittent inflammatory lesions i.e. chalazion, hordeola Conjunctival fibrosis Punctate keratitis Corneal revascularisation
GENERAL MX (first line)
Warm compresses before lid scrubs —> help to liquefy solidifying secretion from the meibomian glands —> facilitate removal
Daily lid hygiene/lid scrubs —> dilute several drops of baby shampoo in warm water in an eggcup —> apply the solution to the lid margins with a cotton bud
Oily tear-substitute lubricating eyedrops /aqueous gels
GENERAL MX (second line)
Careful lid massage to express contents of blocked meibomian glands
+ warm compresses
+ lid scrubs
TOPICALS (second line)
Metronidazole 0.75% gel applied with eyes closed
Sodium sulfacetamide ophthalmic ointment
Ciclosporin ointment
ABs (If secondary bacterial infection, mainly staph, suspected) -
- Fusidic acid ointment BD
- Erythromycin ophthalmic ointment BD
Tea tree oil preparations (anti Demodex effects) —> anecdotal, possible ICD/ACD, use with caution
SYSTEMICS (third line)
Oral Omega 3 fatty acids for rosacea related blepharitis
Full dose ABs similar to for papulopustular rosacea for 6-8 weeks -
- Tetracycline
- Doxycycline 50mg daily as effective as 100mg daily with less dverse effectsmand bacterial resistance
- Minocycline
- EES (preferable for children and during pregnancy)
- TMP
REFERRAL TO OPHTHAL (third line) if symptoms persist despite above
Best to avoid TCS in blepharitis
Clinical variants - Atypical distribution
May be asymmetrical
May involve non-centrofacial areas i.e. periocular, post auricular
M with papulopustular rosacea + androgenetic alopecia with simi;ar inflammatory lesions on the bald scalp —> respond to systemic Rx as for facial lesions
Clinical variants - Granulomatous rosacea
@
Acne agminata
Lupus miliaris disseminatus faciei
Lupoid rosacea of Lewandowsky
Persistent, firm, non-tender
Monomorphic
Red to brown dome-shaped papules or nodules arising in otherwise normal appearing skin
Resolves with significant scarring
Cheeks
Around mouth
Around eyes
HISTO
Granulomatous changes
+/- follicular rupture
+/- Foci of caseation necrosis
DDx
sarcoidosis
Complications
GENERAL
Cardiovascular disease
Social impact esp d/t flushing, F, rhinophyma
PAPULOPUSTULAR ROSACEA
Seb derm may accompany
RHINOPHYMA
BCC may be obscured
OCULAR ROSACEA
Inhibits contact lens wearing
Investigations
Typical clinical features —> clinical Dx
No specific serological or histopathological test to confirm Dx
General skincare for all subtypes of rosacea
DO
Soap free pH balanced wash + lukewarm water (not cold or hot) to wash face
Non-scented colour free Moisturisers may assist in restoring impaired epidermal barrier function -
- humectants i.e. glycerin
- Occlusives i.e. petrolatum
Sunscreen with both UVA and UVB with SPF 15+ or more all year round —> titanium dioxide and zinc oxide are usually well tolerated
Wear a hat
Use cosmetics and sunscreens that contain protective silicones
Camouflage -
- Water soluble light liquid foundation make-up containing inert green pigment helps to neutralize the perception of erythema
- Other camouflage make up
DONT
Avoid astringents, toners, and abrasive exfoliators amd skin peeling agents
Avoid cosmetics that contain ETOH, menthols, witch hazel, camhor, fragrance, peppermint, eucalyptus oil
Avoid water proof cosmetics and heavy foundations that are difficult to remove without irritating solvents or physcial scrubbing
Avoid procedures i.e. dermabrasion
Avoid environments that may overheat and/or dry the skin i.e. saunas, heater fans, open fireplaces
Facial dermatoses with an uncertain relationship to rosacea
Idiopathic facial aseptic granuloma
Rosacea conglobata and rosacea fulminans
Solid facial lymphoedema
Corticpsteroid-imduced roscea-like facial dermatosis
Periorificial facial dermatitis
Childhood granulomatous periorificial dermatitis
Idiopathic facial aseptic granuloma
Children 8 months - 13 years
Solitary inflammatory nodule on the cheek or eyelid
Superficially resembling insect bite
Asymptomatic nodules
Red/purple colour
Soft to palpatiom
Spontaenously involute pver several months
Rosacea conglobata and rosacea fulminans
ROSACEA CONGLOBATA
Rosacea-like eruption
GRADUAL onset
Young F
Marked facial erythema
Nodular abscesses
Indurated haemorrhagic plaques
Significant scarring
No comedones (unlike acne conglobata) Trunk spared (unlike acne conglobata)
ROSACEA FULMINANS @ pyoderma faciale
MORE ACUTE onset
Probably more severe variant of rosacea conglobata
Young F with oily skin
Often during or immediately after pregnancy
Arise de novo vs develop in patients with pre-existing rosacea
Inflammatory haemorrhagic plaques surmounted by pustules erupt abruptly
Major scarring
May be assoc with Acute ocular rosacea (conjunctivitis, severe keratitis)
Solid facial lymphoedema
If not preceded by hx of rosacea - @ Morbihan disease Solid facial lymphoedema lymphoedematous rosacea
DX of exclusion
CLINICAL FEATURES Firm pronounced non-pitting oedema Persistent Erythema Upper 2/3 face - Eyelids - Glabella - Nose - Cheeks
In many, No clinical evidence of preceding or concomitant rosacea/other inflamamtory dermatoses —> idiopathic
? Recurrent inflammation —> structural damage to the draining lymphatic vessels
DDX
Dermatomyositis
Chronic actinic dermatitis
Chronic ACD
MX
Difficult
Long term isotretinoin 40-80mg daily for up to 2 yrs —> improvememt not seen till at least 6 months
Thalidomide
Corticosteroid-induced rosacea-like facial dermatoses
CAUSE
Potent TCS on the face, esp fluorinated steroids (triamcinolone, betamethasone, dexamethasone)
Sometimes even mild TCS i.e. hydrocortisone 1% in children
Corticosteroid nasal sprays
Topical tacrolimus 0.1% ointment
If TCS use continues —> fixed erythema, telengiectasia similar to idiopathic rosacea
PATHOGENESIS
Not well understood
Alterations in skin microbiome —> effect on skin innate immune system
CLINICAL FEATURES
Marked sensitity to the slightest irritant
Itching
Burning
Intense erythema
When Rx discontinued —> eruption flares —> state of dependence on steroid
Steroid application produces prompt transient improvement in the symptoms
MX
Withdraw TCS —> anticipate a flare of the rosacea —> introduce a less potemt TCS to reduce severity of the flare
Topical or oral ABs as used for idiopathic rosacea i.e. doxycyline, EES, TMP —> help suppress flares in the early stages of steroid withdrawal
Topical calcineurin inhibitors i.e. pimecrolimus, tacrolimus —> beware risk of rosacea-like eruption
COURSE/PROGNOSIS
May tale several weeks/months to subside
Eventually resolution occurs if TCS avoided
Periorificial dermatitis (PD)
@
Perioral dermatitis
Periocular dermatitis
Periorbital dermatitis
Similarities to corticosteroid-induced rosacea-like facial dermatosis, but different clinical distribution
CAUSE
Prolonged TCS use -
- Potent TCS
- Occasional reports of hydrocortisone 1%
Steroid asthma inhalers/nebs (perioral dermatitis)
Steroid containing ophthalmic preparation (periocular/periorbital dermatitis)
PATHOGENESIS
Alterations in skin microbiome
Skin barrier impairment
ASSOCIATIONS
Atopic eczema
Irritant products on the skin
CLINICAL FEATURES
The potent TCS may have been used or borrowed for a different indication
Patchy erythema
Tiny papules and pustules
Lower face and chin
Leaves a small border of unaffected skin around the mouth
DDX Rosacea - PD has no flushing or telengiectasia Granulomatous rosacea - firmer larger papules - less background erythema - granulomatous histology Granulomatous periorificial dermatitis in children - Does not spare perilabial skin - No pustules - Predominantly M Seb derm - Nasolabial area - Not usually circumoral - Scalp, ears, eyebrows commonly involved ACD - Does not usually spare the perilabial area as in PD Late onset acne vulgaris - Larger papules than PD - Comedones, cysts
HISTO
Mild spongiosis
Perifollicular inflammation and pustules
Granulomas and demodex mites not found
MX
Stop TCS —> warn an initial flare is expected
Substitute with milder TCS when more potent TCS stopped —> diminish subsequent flare
Avoid cosmetics
4 week course of -
- Topical erythromycin
- Topical metronidazole
- Oral tetracycline
COURSE/PROGNOSIS
Prominent remission after short course of broad spectrum ABs —> relapse in small minority
If untreated + steroids continued —> persist for years
Childhood granulomatous periorificial dermatitis
Prepubertal children of African descent
Unknown aetiology
Asymptomatic
Flesh-coloured dome-shaped papules
Perioral and periocular skin
+/- other parts of the head and neck
Involves immediate perilabial skin (unlike usual periorificial dermatitis)
Diascopy —> confirms granulomatous nature
Resolves spontaneously with a few months to 3 years without Rx
May leave milia/pitted scars
HISTO
Non-caseating epitheliod granulomas
Perivascular inflammatory infiltrate
