Autoimmune Flashcards
(59 cards)
What is Cutaneous Lupus Erythematosus (CLE)?
Cutaneous Lupus Erythematosus (CLE) is a diverse group of autoimmune connective tissue disorders localized to the skin, which can be associated with systemic lupus erythematosus (SLE) to varying degrees.
How is Cutaneous Lupus Erythematosus (CLE) classified?
Acute (ACLE)
Subacute (SCLE)
Intermittent (Lupus Tumidus)
Chronic (CCLE), which includes discoid lupus (DLE), lupus profundus, and chilblain lupus erythematosus.
What are the features of localized Acute Cutaneous Lupus Erythematosus (ACLE)?
Localized ACLE presents as a malar ‘butterfly’ rash, characterized by redness and swelling over both cheeks, sparing the nasolabial folds, and lasting hours to days.
What are the features of generalized Acute Cutaneous Lupus Erythematosus (ACLE)?
Generalized ACLE presents as diffuse or papular erythema of the face, upper limbs (sparing the knuckles), and trunk, resembling a morbilliform drug eruption or viral exanthem.
What is Toxic Epidermal Necrolysis-like Acute Cutaneous Lupus Erythematosus?
Toxic Epidermal Necrolysis-like ACLE is associated with lupus nephritis or cerebritis and must be distinguished from drug-induced toxic epidermal necrolysis in a patient with SLE.
Where do SCLE skin lesions typically occur, and what triggers them?
SCLE skin lesions typically occur on the trunk and upper limbs and are triggered or aggravated by sun exposure.
What are the common presentations of SCLE skin lesions?
SCLE presents as a psoriasiform papulosquamous rash or annular, polycyclic plaques with central clearing.
What is Intermittent Cutaneous Lupus Erythematosus?
Intermittent Cutaneous Lupus Erythematosus, better known as lupus tumidus, is a dermal form of lupus erythematosus.
Discoid Lupus Erythematosus (DLE): Prevalence in CCLE
DLE is the most common form of CCLE, accounting for 80% of cases, particularly prevalent and severe in patients with skin of color.
DLE: Lesion Characteristics
Presents as destructive scaly plaques with follicular prominence (carpet tack sign) which can result in scarring alopecia.
Dermatomyositis: Gottron’s Sign
Violaceous erythematous papules over knuckles, elbows, knees, and malleoli.
Dermatomyositis: Nailfold Signs
Telangiectasia and hemorrhage on the nailfold.
Dermatomyositis: Heliotrope Erythema
Violaceous erythema and swelling of the upper part of the face, with potential involvement of the whole face, neck, and upper chest.
Dermatomyositis: Organ Involvement
Involves the heart (~40%), smooth muscles, lungs, and rarely the kidneys and liver; calcinosis of fasciae may occur.
Dermatomyositis: Diagnostic Tests
Elevated creatine kinase, aldolase, MRI of affected muscles, and possibly muscle biopsy. Important to search for tumors, especially in adults (≈ 50% are paraneoplastic).
Dermatomyositis: Initial Therapy
Corticosteroids at 1-2 mg/kg body weight to start.
Dermatomyositis: Immunotherapy
Intravenous immunoglobulins (2 mg/kg body weight/month for up to a year), methotrexate (15-25 mg/week), and mycophenolate.
Dermatomyositis: General Management
Rest in the beginning, with careful and mild physiotherapy as tolerated.
Morphea: Lesion Characteristics
Asymmetric sclerotic plaques, usually 2–15 cm in diameter.
Morphea: Active Lesion Appearance
Active lesions may have a lilac border with central hypo- or dyspigmentation; older lesions often become hyperpigmented.
Morphea: Depth of Sclerosis
Sclerosis may extend deeply into fat or underlying structures such as fascia, muscle, and bone, potentially causing disability.
Pemphigus Vulgaris: Demographic Associations
More common in Europeans and Indians compared to Africans.
Pemphigus Foliaceus: Demographic Associations
More common in Africans compared to Europeans and Indians; associated with HLA B8 in South Africa.
Pemphigus Vulgaris: Affected Areas
Affects skin and mucous membranes. Always check the mouth
