Diseases 3 Flashcards
Cutaneous Lupus erythematosus
- definition
- etiopathogenetic
-autoimmune CT inflammatory disease
- genetic and environmental factors
- exogeneous: UV light, viruses, chemical substances, smoking
- individual: hormonal status, immune response, mother anti-SSa, SSb transplacental transport
- genetics: complement defects, variation in some genes
Cutaneous Lupus erythematosus
-pathogenesis
- predisposing factors
- keratinocytes apoptosis
- transportation of apoptotic material
- activation of T cells
- migration of activated CLA + T cells
- production of cytokines
- basal cell apoptosis
Cutaneous Lupus erythematosus
-classification
Acute
Subacute
Chronic - discoid or cilblain
Intermittent
Cutaneous Lupus erythematosus
-clinical features
Discoid lupus
- chronic sun exposed areas
- primary lesion: erythematous well-circumscribed, persistent scaly papules and plaques, with telangiectasia, centrifugally growing
- progressive signs: atrophy in center of plaque, centrifugally spreading
- regressing lesion: central and peripheral hypopigmentation, healing with scarring (may cause alopecia)
Subacute
- neck, shoulders and forearms - spares face
- small erythematous lesions developing into either annular or psoriasiform lesions
- lesions are symmetrically
- no scars, change in pigmentation and atrophy
Cutaneous Lupus erythematosus
- diagnosis
- treatment
-skin biopsy, serology - ANA/ Anti -SSA and SSB
- eliminate risk factors - sun avoidance, sunscreen usage, vit D, stop smoking
- topical - corticosteroids, calcineurin inhibitors, retinoids
- systemic - thalidomide, antimalaria drugs, SGCS
Scleroderma
- definition
- etiopahogenesis
-chronic, cutaneous inflammatory disease characterized by hardening of the skin and affects internal organs
- environmental factors and mechanical skin trauma
- t cells release cytokines which control fibroblast function
- blood vessel disturbances, activated t cells, fibroblast caused an abnormal CT production
Scleroderma
-classification
Local (morphea)
-strictly limited hardened skin area
Generalized
-multiple hardened skin patches
Systemic
Scleroderma
-clinical features
morphea
- circumscribed sclerotic plaque with ivory center and red-violet periphery
- starts as erythematous patch that slowly spreads
- asymmetrical
- skin atrophy, hypo/hyperpigmentation
- trunk, face, extremities, sword- stroke - head, forehead, eyelid, hands
systemic
-acral systemic sclerosis - type 1 (only hands and forearms), 2 (starts on hands, spreads to arms and trunk), 3 (starts on the trunk, severe facial involvement)
-CREST syndrome - calcinosis of soft tissue, Raynaud phenomena, esophagus function disorders, sclerodactyly - finger skin hardening, telangiectasia
Scleroderma
- diagnosis
- treatment
- CBC, liver function, ESR, CRP
- serology: ANA, anti-scl-70
- serum protein electrophoresis
-physical therapy, prevent dry skin, phototherapy, immunosuppressive therapy (ex: methotrexate), organ specific therapy in some cases
Vitiligo
- definition
- etiopathogenesis
- acquired chronic multifactorial pigmentation disorder
- white patches = loss of functioning epidermal and sometimes hair follicles melanocytes
-metabolic abnormalities, oxidative stress, generation of inflammatory mediators, cell detachment and autoimmune responses (innate immune inflammation and t cell mediated melanocyte destruction)
Vitiligo
-pathogenic steps
- Genetic predisposition
- Triggering factors
- intrinsic - emotional stress, nutritional deficiency
- extrinsic - sunburn, drugs, infection, chemicals, cosmetics - Melanocyte stress
- Altered inflammatory innate and adaptive immune responses
Vitiligo
-classification
Non-segmental
- symmetrical bilateral patches
- more common
- hands, forearms, neck, scalp, feet, face
- immune cells attack melanocytes
Segmental
- unilateral depigmentation that does not cross midline
- limited to a dermatome
- more common in children
- neurochemicals damage melanocytes
Mixed
-evolves into non-segmental vitiligo
Vitiligo
-clinical features
- white or hypopigmented macules or patches
- oval, round, linear
- borders well demarcated
- inflammatory vitiligo: pruritus, elevated lesions, erythematous margins
- leukotrichia (body hair) involvement
- Koebnerisation - occurrence on areas subjected to repeated trauma
Vitiligo
- diagnosis
- treatment
- wood lamp examination - “milk-white” fluorescent aspect
- skin biopsy
- labs - ANA, CBC, thyroid function test
- topical immunosuppression, phototherapy, oral immunosuppression, surgical
- sunscreen, cosmetics (to hide it)
