Non-Cancerous Skin Disorders Flashcards
Group of epidermal diseases with thickened stratum corneum but thin epidermis, impaired tonofilament formation, excessive cornification, and increased basal cell proliferation.
Ichthyoses
Autosomal dominant and most common epidermal disease: caused by decreased of absent profilaggrin; marked by hyperkeratosis, small white scales, and a thin stratum granulosum.
Ichthyosis vulgaris
Heritable epidermal disease characterized by steroid sulfatase deficiency and delayed desmosomal dissolution (persistent cohesion) of the stratum corneum, with a preserved granular layer.
X-linked ichthyosis
Autosomal dominant epidermal disease caused by keratin tonofilament disassembly, leading to whorled, eosinophilic keratin filaments surrounding the nucleus, hyperkeratosis, and blistering.
Epidermolytic hyperkeratosis
Autosomal recessive epidermal disease characterized by TGM1 (transglutaminase 1) mutations and defective lamellar body secretion; granular layer is normal or thickened.
Lamellar ichthyosis
Autosomal dominant epidermal disease linked to ATP2A2 calcium pump defect that affects Ca-dependent desmosome assembly; characterized by mutlifocal papules and is also known as keratosis follicularis.
Darier disease
Epidermal disease where a genetic predisposition + trauma leads to persistent hyperplasia/hyperproliferation and altered differentiation; seronegative arthritis can develop and is linked to HLA-B27
Psoriasis
Epidermal disease featuring large, red, silvery-scaled plaques on the extensor surfaces with hyperplasia, hyperkeratosis, and parakeratosis (persistent nuclei); pinpoint bleeding can occur when plaques are removed.
Psoriasis
In psoriasis, the Munro microabscesses in the stratum corneum, spongiform pustules of Kogoi, “squirting papillae” of capillary loops, and increased arachidonic acid and leukotriene B in the epidermis are all related to an excess of what inflammatory cells?
Neutrophils
What epidermal disease is caused by autoantibodies (IgG) directed against keratinocyte membrane antigens (desmoglein 3)? What related disease involves IgG and desmoglein 1?
Pemphigus vulgaris; pemphigus foliaceus
What autoimmune epidermal disease involves diminished epidermal cohesiveness, intraepidermal bullae, easily ruptured blisters, and tombstone cells following acantholysis?
Pemphigus vulgaris
What basement membrane zone disease is usually noted at birth and features blisters that can range from minor to life-threatening?
Epidermolysis bullosa
Name the 3 types of epidermolysis bullosa. How does the Kindler syndrome relate to these types?
Epidermolytic (EB simplex), junctional, or dermolytic (dystrophic);
Kindler syndrome displays a mix of these cleavage planes but also shows poikiloderma (mottled pigmentation) and photosensitivity
What basement membrane zone disease is caused by autoantibodies (IgG) directed against basement membrane/lamina lucida antigens (BPAG1 and BPAG2)? It features eosinophil degranulation and tense blisters, but no acantholysis.
Bullous pemphigoid
What basement membrane zone disease is caused by immune complex deposition following IgA-mediated gluten sensitivity? Neutrophils accumulate in intensely pruritic plaques and subepidermal vesicles on the extensor surfaces.
Dermatitis herpetiformis
What self-limited basement membrane zone disease, characterized by “target” lesions, is often a reaction to a drug or infection? Name the more life-threatening version that can cause ulceration of the mucous membranes and internal organs.
Erythema multiforme minor; Stevens-Johnson syndrome is erythema multiforme major
What basement membrane zone disease is caused by immune complex deposition and lymphocyte-induced cell injury, including basal keratinocyte injury and vacuolization? The systemic form may be brought on by sun exposure
Systemic lupus erythematosus
Which form of SLE, limited to the skin, is ANA(-), has a thin epidermis and thick lamina densa, and features immune complexes mostly deep to the lamina densa?
Chronic cutaneous (discoid) LE
Which form of SLE is common in young- to middle-aged white women, can spread to the musculoskeletal system and kidneys, and features edema of the papillary dermis and a thickened lamina densa but nothing deeper?
Subacute cutaneous LE
Which form of SLE features a “butterfly” malar rash, maculopapular eruption that can spread t the kidneys and joints, and elevated ANA levels?
Acute systemic LE
Bullous SLE involves subepidermal blisters (beneath the lamina densa) and is caused by autoantibodies against what molecule?
Against type VII collagen
What basement membrane zone disease involves decreased epidermal turnover, melanophages (macrophages with ingested melanin), a dense infiltrate of lymphocytes and macrophages, and Wickham striae (white patches or streaks on oral mucous membranes)?
Lichen planus
Urticaria and angioedema are vascular hypersensitivity reactions mediated by what immunoglobulin? Which one occurs in the deeper dermis/subcutis, and what form is associated with a C1-esterase inhibitor mutation?
IgE; angioedema; hereditary angioedema
Cutaneous necrotizing vasculitis (a.k.a. allergic cutaneous vasculitis / leukocytoclastic vasculitis / hypersensitivity angiitis) is a vascular immune reaction characterized by what 2 observable features?
Neutrophil infiltration of vessel walls and palpable purpura (leukocytoclasia, or damage to neutrophils that leave dust-like nuclear remnants, may also be seen)
