Rosacea Flashcards

1
Q

Subtypes

A

Erythematotelengiectatic rosacea (most common)

Papulopustular rosacea

Phymatous rosacea

Ocular rosacea

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2
Q

Grading

A

Grade 1 (mild disease)

Grade 2 (moderate disease)

Grade 3 (severe disease)

Psychological, social amd occupational impact

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3
Q

Introduction

A

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

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4
Q

Associated diseases

A

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

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5
Q

Causes

A

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)

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6
Q

Risk factors

A

Family history (genetic predisposition) —> develop rosacea at earlier age than those without family hx

Skin types 1, 2

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7
Q

Causative organisms

A

Demodex folliculorum mites (papulopustular rosacea)

? Staph epidermidis

? Chlamydophila pneumoniae

Demodex-associated Bacillus oleronius

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8
Q

Environmental triggers

A

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
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9
Q

Subtype 1: Erythematotelengiectatic rosacea

A

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

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10
Q

Subtype 2: Papulopustular rosacea

A

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

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11
Q

Subtype 3: Phymatous rosacea

A

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

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12
Q

Subtype 4: Ocular rosacea

A

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

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13
Q

Clinical variants - Atypical distribution

A

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

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14
Q

Clinical variants - Granulomatous rosacea

A

@
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

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15
Q

Complications

A

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

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16
Q

Investigations

A

Typical clinical features —> clinical Dx

No specific serological or histopathological test to confirm Dx

17
Q

General skincare for all subtypes of rosacea

A

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

18
Q

Facial dermatoses with an uncertain relationship to rosacea

A

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

19
Q

Idiopathic facial aseptic granuloma

A

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

20
Q

Rosacea conglobata and rosacea fulminans

A

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)

21
Q

Solid facial lymphoedema

A
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

22
Q

Corticosteroid-induced rosacea-like facial dermatoses

A

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

23
Q

Periorificial dermatitis (PD)

A

@
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

24
Q

Childhood granulomatous periorificial dermatitis

A

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