1. Dr.Rohit kumar singh 1st yr ,P.G.Resident

2. Rosacea ia a latin word (Like-ROSES) Rosacea is a disease lacking an entirely satisfactory defination Chronic disorder Variable severity Variable presentation 3. Diagnostic criteria Recent diagnostic criteria is by one of the primary and one of many secondary criteria PRIMARY CRITERIA Intermittent or persistent erythema Telangiectasia Papules Pustules Photosensitivity 4. SECONDARY CRITERIA Burning /stinging Elevated red facial plaques with or without scales Dry/scaly skin Persistent facial edema Phymatous changes Ocular manifestations 5. Site- central convexities of the face i.e mid- forehead,nose,malar areas and chin Other sites where lesions can be seen are beard regions , neck ,scalp , shoulders and upper back Epidemiology- fair skin people(caucasians),but people of african and asian descent can also have the disease 6. ROSACEA VASCULAR INFLAMMATORY CHANGES CHANGES FLUSHING PAPULES TELENGIECTASIA PUSTULES (Initial and constant feature) 7. LATE COMPLICATION 1.Chronic lymphoedema- Thickening of the affected skin Leonine facies Peau d orange appearance Morbihans disease:chr upper facial erythematous oedema 2.Rhinophyma 3.Ocular blindness 8. Epidemiology Third to sixth decade, peak incidence in the fourth or fifth decade of life Affects both genders, but M:F(1:3)F> M Rhinophyma type afflicts mostly men. Also can be seen in children Acne rosacea affects white people, II-I-type classification (Fitzpatrick) and is rare in Africans or Asians. 9. Pathogenesis 1.Solar irradiation 2.Neurogenic aetiology 3.Oxidative stress 4.Abnormally high level of canthelicidin 5.Vasoactive mediators 10. 6.H.Pylori 7.Demodesidosis 8.HIV infection 9.Defect in trigeminal afferent pathway defect(new speculation) 11. SOLAR IRRADIATION dysfunction of unsupported facial blood vessels with endothelial damage Leakage Oedema inflammation 12. VEGF is increased Laser droppler- blood flow is increased markedly in papulopustular Capillaroscopy- neoangiogenesis,telangiectasis In rosacea vascular abnormalities are confined to the face 13. NEUROGENIC AETIOLOGY Lowered neural threshold for reaction to noxious stimuli may result in neurogenic inflammation Burning sensation Association with migraine 14. OXIDATIVE STRESS Reduced level of SUPEROXIDE DISMUTASE inflammatory Rosacea Response to metronidazole,tetracyclinsand azithromycin and their antioxidant properties 15. CANTHELICIDIN Endogeneous antimicrobial peptide Component of the innate immune system Trigger inflammation - leukocyte and angiogenesis 16. CANTHELICIN Serine proteases IL-37 PROINFLAMMATORY 17. VASOACTIVE PEPTIDES Proposed mediators Serotonin Bradykinin PGs Substance p Opioid peptides Gastrin As there is no associated sweating so it is stated that reason being vasoactive mediators and not neural neural reflex mechanism 18. H.PYLORI ( G-ve, microaerophilic) Increased level of gastrin( flushing) levels of histamine,PGs,leukotrienes(other cytokines) More prevalent with papulo-pustular rosacea Inconclusive data in this regard Drugs used in eradications for the H.Pylori also produces relief in rosacea patient 19. DOMODEX MITES infestation(demodecidosis) Two species 1.DEMODEX FOLLICULORUM 2.DEMODEX BREVIS Present in demis, eyelid in hair follicles,eyelash follicle,meibomian glands It is associated with the inflammatory response(papulopustular) 20. Presence of large number in the rosacea patient is a common association Also plays an important role in ophthalmic complication Metronidazole responsive 21. Found within follicular infundibula & sebaceous ducts 22. HIV INFECTION ASSOCIATED Recent studies point out that rosacea has been reported as a manifestation in the skin of HIV infection 23. DEFECT IN TRIGEMINAL AFFERENT NERVE PATHWAY repeated facial flushing ectatic vessels ,permanent vasodilation 24. Also intake of hot liquids heat up the tissues leads to countercurrent heat exchange with carotid artery signal relayed to hypothalamus dissipates heat through flushing and vasodilation 25. SUMMARY OF PATHOGENESIS Link ??? Between the vascular features and and the inflammatory feature link Vascular features Increased temperature Inflammatory feature 26. This may be explained by different behaviour of bacteria or perhaps folliculitis,at higher temperature Improvement of inflammmatory lesions after ablation of the telangiectatic vessels 27. Plewig-Kligman Stages Stage 1 Stage 2 Stage 3 28. EARLY ROSACEA (STAGE I) Flushers-blushers Often < Age 20 Nose/cheeks Recurrent episodes of flushing Eryhthema persists Few telagiectasias 29. STAGE I - TELANGIECTASIAS 30. STAGE I Telangiectasias becomes more prominent and widely distributed over central covexity of the face 31. STAGE II PAPULES & PUSTULES BEGIN, INCREASED ERYTHEMA AND TELANGIECTASIAS 32. STAGE III Dense erythema Papule,pustules, nodule Telangiectasias, severe,diffuse Variable erythematous plaques 33. Subtypes of rosacea(NRS -2002) 4 sub types 1.Erythematotelangiectatic 2.Papulopustular 3.Phymatous 4.Ocular One variant 1.Granulomatous rosacea 34. Erythemato-telangiectatic rosacea Central facial flushing ,burning or stinging Redness spares the periocular areas Skin with fine texture that lacks a sebaceous quality characteristics (common in other types) Erythematous areas rough and scaly (chronic,low grade dermatitis) Trigger factors emotional stress,alcohol,spicy food,exercise , hot liquids 35. Papulo-pustular rosacea Classic presentation of rosacea Typically in middle aged women Transient small erythematous papules surmounted by pin-point pustules Telangiectasias present but difficult to distinguish 36. Phymatous Marked skin thickening and irregular surface nodularities of the nose (most often) , chin ,forehead , one or both ears and eyelids Careful evaluation for basal cell carcinoma 4 distinct histological variant present a) Glandular b) Fibrous c) Fibroangiomatosis d) Actinic 37. RHINOPHYMA Craggy,irregular,bulbous swelling of the nose Predominantly affects men Whisky nose Irregular skin thickening Hypertrophy of soft tissue masses centered about the tip of the nose PSEUDORHINOPHYMA- seen with heavy eyeglasses ,obstructing the lymphatics and venous drainage of the nose Rhinophyma in female in ctaneous B cell neoplasms 38. Ocular rosacea May precede the cutaneous sign by years Clinical features eye stinging or burning , dryness , irritation with light or foreign body sensation Manifestations- Blepharitis Conjunctivitis Inflammation of lids and meibomian glands(modified sebaceous gland) Interpalpabralconjuctival hyperemia Conjunctival telangiectasias 39. 50 % cases of rosacea will have ocular involvement The severity of the eye involvement doesnot correlate with the severity of the facial involvement 40. Lupoid or Granulomatous rosacea Is a varaint of rosacea Charaterized by non-inflammatory ,hard,brown,yellow or red papules / nodules of the central face In 15% cases they have extrafacial lesions 41. Not variants of rosacea(as considered earlier) Rosacea fulminans(pyoderma faciale) Steroid induced acneiform eruptions Perioral dermatitis 42. Rosacea fulminans(pyoderma faciale) Most common in Women Age 15 46 yrs History of seborrhoea Characterized by rapid giant coalescent nodules on the face No comedones, telangietasias, erythema and remission with little or no scarring 43. Pediatric rosacea Poorly defined condition It is familial Most likely underreported because of tendency to recognize flushing and erythema as HEALTHY GLOW Condition may persists and progress in adulthood 44. Histopathology of rosacea Depends upon the stage of the disease but vascular dilation of upper and middermal vessels with perivascular and perifollicular lymphohistiocytic inflammation is generally present :PREDOMINANTLY DERMAL CHANGES,SOLAR ELASTOSIS,INCREASED MAST CELL DENSITY,LYMPHATIC CHANNELS MAY ALSO BE DILATED IN THE DEEPER DERMIS NON-PUSTULAR LESIONS Non-specific perivascular and perifollicular lymphohistiocytic infiltrate,accompanied by ocassional multinucleate d cells ,neutrophils and eosinophils 45. PAPULO-PUSTULAR LESIONS It demonstrate more pronounced granulomatous inflammation, perifollicular abscessess ,sometimes demodex may be abundant in nearby follicles GRANULOMATOUS ROSACEA It has a striking feature of caseating and non-caseating granulomata with negative stains for mycobacterium and fungi 46. Differential diagnosis Important ones are Acne vulgaris Acneiform eruption(steroid induced) Lupus miliaris disseminatus faciei Perioral dermatitis 47. Seborrhic dermatitis Nasal sarcoidosis(lupus pernio) Demodex folliculitis Carcinoid syndrome 48. Cutaneous lymphocytic infiltration(dermis) of the face includes 5Ls Jessners lymphocytic infiltrate Lymphocytoma cutis Polymorphic light eruptions Discoid lupus erythematosus Well-differntiated lymphocytic lymphoma 49. Jessners lymphocytic infiltrate Chronic benign T-cell lymphoproliferative disorder M > F , childrens can also be affected Lesions smooth raised ,non-scaly erythematous nodules or plaques usually on facial skin Usually asymptomatic(but some may complain burning or pruritic sensation) May involute spontaneously Waxes and wanes in severity 50. Winter exacerbations (in rosacea summer ) Histopathology Large circumscribed aggregation of lymphocytes around blood vessels and hair follicles Sometimes infiltrate can extend to subcutaneous tissue (fat) 51. Lymphocytoma cutis Also known as Spiegler-fendt sarcoid or lymphadenosis benigna cutis of bafverstedt Benign cutaneous B- cell lymphoproliferative condition Females > males 52. Lesion- solitary or grouped ,asymptomatic ,erythematous or violaceous papules ,nodules or plaques Site head(specially on ear lobes),rarely on trunk and limbs Can proceed to primary cutaneous B- cell lymphoma Can be in localized form or disseminated form 53. Histopathology Epidermis Spongiosis Edema and transmural infiltration of lymphocytes and plasma cells Granulomatous vasculitis Dermis Nodular infiltrate of lymphocytes,histiocytes eosinophils,plasma cells Germinal centres can seen 54. Polymorphic light eruptions Genetically determined delayed (type IV) hypersensitivity reaction to UVA > > UVB F:M(2-3:1) F>M Age of onset is 3rd decade Lesions papular (most common),papulovesicles ,plaques,vesicobullous,insect-bite like,erythema multiforme like lesion associated with pruritis. 55. Site sun exposed parts(can be extra facial also) Symmetrical in distribution 56. Histopathology Epidermis Spongiosis Dyskeratosis Exocytosis Basal cell vacuolization Dermis Moderate to dense perivascular infiltration Predominant T-cell with neutrophil,eosinophils 57. Discoid lupus erythematosus Benign disorder of the skin F:M(2:1), F>M, age of onset 4th decade Autoimmune disease Lesions-well defined circumscribed or discoid type of red scaly patches of variable size ,which heals with atrophy ,scarring and pigmentary changes(butterfly erythema) Site- face (most common MALAR region),scalp,ear ,arms, legs,trunk 58. History of joint pains,raynauds phenomenon,chilblains,photosensitivity In 15 % of the cases lesions on the face may appear similar to rosacea( but no pustular lesions in rosacea) In rosacea there may be low titre of ANA can be positive finding(but it is insignificant) No pustules as in rosacea Histopatology can differentiates between the two diseases 59. Histopathology Liquifactive degeneration of basal layer of the epidermis Atrophy of epidermis Hyperkeratosis Follicular plugging Incontinence of pigment A pachy dermal lymphocytic infiltrate with a few plasma cells and histiocytes around appendages,which may be atrophic 60. Sarcoidosis Multisystem disease defined by epitheloid cell granuloma without caseation different organs M:F(2:1) M>F age 25 bto 35 yrs Lesions- maculopapular and erythematous forms Papular forms Plaque form Nodular form Erythema nodosum Lupus pernio(nasal sarcoidosis, more common in women) 61. Sarcoidaosis affect nasal septum(nasal obstruction) Surface of nose is thickened(lupus pernio) Although telangiectatic ,is smooth but lacks the rugose peau d orange surface(in rhinophyma) 62. Site face,extremities(extensor aspects) and rarely trunk. Associated feature of ocular,neural,skeletal,genitourinary ,cardivascular system involvement. Histopathology Epidermis-hyperplasia,compact hyperkeratosis Dermis-lymphocytic infiltrate,granulomas in lower dermis, Subcutaneous tissue panniculitis (occasional) 63. ROSACEA VS. ACNE ADULTS PAPULES PUSTULES NO COMEDONES ERYTHEMA TELANGIECTASIAS TEENS PAPULES PUSTULES COMEDONES NO ERYTHEMA NO TELANGIECTASIAS 64. Other differential diagnosis DISEASE SIMILARITY DIFFERENCE ACNEIFORM ERUPTIONS ERYTHEMA,PAPULE,PU STULE,TELANGIETASIS AND INVOLVES CENTRAL 3rd OF THE FACE MONOMORPHIC ,EXTRAFACIAL SITE ,DRUG INDUCED,ATYPICAL AGE AND SITE SEBORRHEIC DERMATITIS BLEPHARITS ERYTHEMA SCALING,ECZEMATOUS CHANGE PARANASAL,NASOLABI AL,EXTRAFACIAL PERIORAL DERMATITIS ERYTHEMA PAPULES PERIORAL SMALLER LESIONS NO TELANGIECTASIS, FLUSHING,BLUSHING 65. Treatment 1.Avoidance of the trigger factors 2. Drugs both systemic and topical 3.Lasers 4.surgery 66. Triggers of rosacea Ingested or iatrogenic 1.Foods and drinks 2.drugs Cheeze niacin Chocolate nitroglycerine Spicy foods tobacco Soy sauce alcohol based vanilla nifedipine Dairy products cyclosporine Liver bromocriptine 67. 3.beverages 4.topical agents Red wine topical steroids Hot drinks cosmetics Beer acetone Vodka 68. Environmental causes 1.temperature 2.weather Sun heat sunny Over-heating heat Sun lamp stong wind Humidity 3.emotions Hot bath anger stress embarrasment 69. 4.activity Exercise Menopause Chronic cough straining 70. Systemic drugs 1.Oral tetracyclin first choice (200 mg bd) Also used in ocular rosacea Less efective for resistant lymphedema Marginally effective for rhinophyma Response in 7 to 14 days Continue upto 4wks or more 2.doxycycline(40 mg daily) 3.minocycline 4.oral metronidazole(200 mg bid) 71. 5.Azithromycin 6.Oral isotretinoin (10-60 mg /day) Is alternative in resistant rosacea Good response in rhinophyma Improves quality of life Not suitable for ocular rosacea Prolonged treatment 7.Oral dapsone in granulomatous rosacea 8.Sulpha drugs for demodex 72. Topical therapies 1.topical metronidazole gel( 1% ) is the major therapy in rosacea Also used in blepharitis 2.adapalene 3.benzoyl peroxide 4.tacrolimus 5.Clindamycin,erythromycin, 6.azelaic acid 20% cream 73. 7.0.025% retinoic acid cream 8.10% sulphur cream Role of topical steroids are limited 74. Telangiectasia Vascular lasers Intense pulsed light PERT(post erythema revealed telagiectasia) Lymphoedema of rosacea Antibiotic to decrease inflammation Low dose oral isotretoin0.1-0.2mg/day Oral ketotifen 1-2mg/day Prednisolone 30 mg daily Metronidazole initially 400 mg over 4 months 200 mg /day for continuation phase 75. Rhinophyma Surgical exision,lasers,dermabrasion Cryotherapy Radiofrequency Liquid nitrogen Ocular rosacea Artificial tears Oral tetracyclines > doxycyclins,erythromycin Retinoids are avoided 76. Flushing Clonidine 50 micrgram bd Oxymetazoline 0.05% daily Rilmenidine 1 mg daily Beta - blockers 77. REFERENCES ROOKS BOOK OF DERMATOLOGY 8TH EDITION. FITZPATRICK BOOK OF DERMATOLOGY. INDIAN ASSOCIATION OF DERMATOLOGIST, VENEROLOGIST, AND LEPROLOGIST. LEVER HISTOPATHOLOGY OF THE SKIN