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3: Pathologyof diseases of Epidermis, Dermis, Panniculus
Pathology of diseases of epidermis, dermis, panniculus Khin
107
Medical
Post-Graduate
08/01/2012

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Term
over production of keratin, decreased keratin desquamation -> marked thickening of keratin layer (hyperkeratinosis)
Definition
Ichthyoses
Term
Microscopic appearance: thick stratum corneum, thin epidermis
Definition
Ichthyoses
Term
Defect: unknown, possibly increased cohesiveness of cells of stratum corneum
Definition
Ichthyoses
Term
Inheirted as autosomal disorder;
defect on chromosome 12: ATP2A2 gene
Definition
Darrier Disease (Keratosis Follicularis)
Term
Keratosis Follicularis AKA
Definition
Darrier Disease
Term
Lesions:
tan colored papules 2-4 mm diameter
warty elevations : forehead, ears, back, scalp, chest
Definition
Darrier Disease (Keratosis Follicularis)
Term
Microscopy:
papule shows cleft above basal layer
root of cleft -> cone of compact keratotic material
Definition
Darrier Disease (Keratosis Follicularis)
Term
May occur at any age
Associated w/ arthritis, myopathy, enteropathy, AIDS
Definition
Psoriasis
Term
Site: elbows, knees, scalp, lumbosacral, intergluteal cleft, glans penis
Definition
Psoriasis
Term
Macro:
well demarcated, pink plaques covered by silvery scales
Definition
Psoriasis
Term
May occur as total body erytema and scaling -> erythroderma
Definition
Psoriasis
Term
Rarely, small pustules form on plaques
Definition
Pustular psoriasis
Term
Microscopic:
ACANTHOSIS w/ elongated rete ridges
p
PARAKERATOSIS
ABSENCE OF granular cell layer
MITOSES above basal layer
Dilation of blood vessels in dermis -> clnical erythema
Definition
Psoriasis
Term
Collections of neutrophils within superficial epithelium known as
Definition
Munro abscesses
Term
psoriasis lesions at site of trauma
Definition
Koebner phenomenon
Term
Chronic BLISTERING SKIN DISORDER, autoimmune in nature
Definition
Pemphigus Vulgaris
Term
affects all age groups, most common 40-60 yrs
Definition
Pemphigus Vulgaris
Term
Blister formation due to diminished cohesiveness of keratinocytes
Definition
Pemphigus Vulgaris
Term
outer epidermis separated from dermis -> suprabasilar blisters
Definition
Pemphigus Vulgaris
Term
Blisters contain:
lymphocytes, macrophages, eosinophils, neutrophils, esp in pervascular areas
Definition
Pemphigus Vulgaris
Term
Direct IF reveals intercellular deposits of IgG and complement
(in epidermal keratinocytes)
Definition
Pemphigus Vulgaris
Term
Lesions on scalp, *mucus membranes*, periumbilical area
Definition
Pemphigus Vulgaris
Term
Maybe associated w/ AI disorders : MG, SLE
Definition
Pemphigus Vulgaris
Term
Characterized by formation of blister in Basement membrane zone @ sites of trauma
Definition
Epidermolysis Bullosa
Term
Blisters noted @ birth or soon after
Definition
Epidermolysis Bullosa
Term
transmitted as AD trait
Bulla disease
Definition
Epidermolytic EB (EB Simplex)
Term
cytolysis of basal keratinocytes -> blisters heal by scarring
Definition
Epidermolytic EB (EB Simplex)
Term
Transmitted as AR trait (Bulla Disease)
Definition
Junctional EB
Term
blisters within lamina lucida
Definition
Junctional EB
Term
course of disease from benign -> severe and fatal in 2 years
Definition
Junctional EB
Term
transmitted as AD or AR trait
Definition
Dermolytic (or dystrophic) EB
Term
blisters beneath lamina densa
Definition
Dermolytic (or dystrophic) EB
Term
blisters heal leaving atrophic scarring
Definition
Dermolytic (or dystrophic) EB
Term
common autoimmune blistering disorder affecting elderly people
Definition
Bullous Pemphigoid
Term
Inner aspects of thighs, flexor surfaces of forearms, axilla, groin, lower abdomen
Definition
Bullous Pemphigold
Term
Detachment of epithellium from underlying CT, possibly due to immunologically mediated damage to basement membrane
Definition
Bullous Pemphigold
Term
Micro:
Subepidermal nonacantholytic blister; perivascular infiltrate of lymphocytes and EOSINOPHILS
Definition
Bullous Pemphigold
Term
Bulla contain:
eosinophils, lymphocytes, neutrophils, and fibrin
Definition
Bullous Pemphigold
Term
Acantholytic bulla
Definition
Pemphigous Vulgaris
Term
Nonacantholytic bulla
Definition
Bullous Pemphigold
Term
IF shows *linear BMZ deposits* of IgG and complement
Definition
Bullous Pemphigold
Term
Rare, chronic recurrent, pruritic disease; age of onset third nad fourth decades M>F
Definition
Dermatitis Herpetiformis
Term
Extensor surfaces - elbows and knees, upper back (shoulders), buttocks: bilateral and symmetrical; ORAL MUCOSA NOT AFFECTED
Definition
Dermatitis Herpetiformis
Term
Associated w/ gluten sensitivity
Definition
Dermatitis Herpetiformis
Term
Vesicles often grouped as true herpesvirus infection
Definition
Dermatitis Herpetiformis
Term
Neutrophils, eosiinophils, and fibrin at tips of dermal papillae (microabscesses)
Definition
Dermatitis Herpetiformis
Term
overlying basal cell vacuolization -> microscopic blisters - coalesce -> large subepidermal blisters
Definition
Dermatitis Herpetiformis
Term
IF testing shows granular deposits of IgA at tips of dermal papillae
Definition
Dermatitis Herpetiformis
Term
Individuals w/ HLA-B8 and HLA-DR3 particularly prone to this disease
Definition
Dermatitis Herpetiformis
Term
Acute self-limited dermatosis produced by allergic reaction to drugs (sulfonamides, penicillin, barbituates)
infections (herpes, mycoplasma, histoplasmosis, coccidiomycosis, typhoid), malignancy (carincomas, lymphomas), collagen-vascular diseases( SLE, dermatomyositis)
Definition
Erythema Multiforme
Term
Believed to be due to cell mediated immune injury
Definition
Erythema Multiforme
Term
Lesions may be widespread and present as macules, papules, vesicles, and bullae
Definition
Erythema Multiforme
Term
Target lesion (iris lesion) - central dark red zone occasionally w/ blister surrounded by paler area
Definition
Erythema Multiforme
Term
Severe, febrile form of erythema multiforme; more common in children; characterized by swelling, bleeding, and serosanguineous crusting of lips and oral mucosa. Conjunctiva, urethra and anogenital regions may also be affected
Definition
Erythema Multiforme
Term
Microscopic:
keratinocyte degeneration and necrosis -> vesiculation
Definition
Erythema Multiformes
Term
Dermis shows varying intensity of lympho-histiocytic infiltration.
Definition
Erythemia Multiforms
Term
Central nerosis surrounded by perivenular inflammation
Definition
Erythema Multiformes
Term
usually limited to skin
face, malar, scalp ears
Lesions start: slightly elevated papules w/ rough scale of keratin
later: disc shape w/ hyperkeratotic margin and pale center
may form disbursing scars
Definition
Chronic Cutaneous (discoid) Erythematosus
Term
irregular proliferation and thinning of epithelium
edema at DE junction
Definition
Chronic Cutaneous (Discoid) Erythematosus
Term
vacuolation of basal cells
patchy infiltrate of lymphocytes
thickening of basment membrane zone
Definition
Chronic Cutaneous (Discoid) Erythematosus
Term
Deposition of immune reactants along BMZ-> positive lupus band test
Definition
Chronic Cutaneous (Discoid) Erythematosus
Term
ANA seen in less than 10%
Definition
Chronic Cutaneous (Discoid) Erythematosus
Term
humoral and cell mediated mechanisms -> destruction of *pigment containing basal cells*
Definition
Chronic Cutaneous (Discoid) Erythematosus
Term
besides skin, MSK system and kidneys involved
upper chest, upper back, extensor surfaces of arms
Definition
Subacute Cutaneous Erythematosus
Term
lesions begin as erythematous papules which enlarge and form annular lesions

no signficant scarring
Definition
Subacute Cutnaeous Erythematosus
Term
ANA levels elevated in 70%
Definition
Subacute Cutaneous Erythematosus
Term
maculopapular rash of chest and extremities
delicate erythema of malar area of face (butterly rash) lasts a few hours / days

lesions heal w/o scarring
Definition
Acute SLE
Term
ANA levels elevated in more than 90% of patients
Definition
Acute SLE
Term
Common chronic skin / mucus membrane disease self-limiting

resolve in 1 to 2 years, oral persists several years
Definition
Lichen Planus
Term
Wickham striae: violaceous papules w/ white dots or lines
Definition
Lichen Planus
Term
Multiple lesions often symmetric; frequently on extremities and often on wrists and elbows.
Oral lesions: white reticularted areas on mucosa (buccal, lateral part of dorsum)
Definition
Lichen Planus
Term
Micro:
parakeratosis
saw tooth pattern of deep surface of epithelium
liquefaction degeneration at DE junction
Definition
Lichen Planus
Term
Anucleate, necrotic basal cells -> colloid or civatte bodies
Dermis 0> dense band like infiltrate of mononuclear cells T lymphocytes may predominant in upper dermis
Definition
Lichen Planus
Term
cause unknown, cell mediated injury to basal cells
Definition
Lichen Planus
Term
wheal formation from focal mast cell degranulation. mainly young adults
Definition
Urticaria
Term
Micro: scanty perivascular infiltrates of mononuclear cells; no increase in mast cells
Definition
Urticaria
Term
massive escape of fluid into tissues from BVs -> large edematous swellings
Definition
Angioedema
Term
Autosomal Dominant Disorder
mutation of C1-esterase inhibitor
Definition
Hereditary angioneurotic edema
Term
Involve GI tract an larynx. Laryngeal edema may be fatal
Definition
Heriditary angioneurotic edema
Term
AKA Allergic cutaneous vasculitis, leucocytoclastic vasculitis, hypersensitivity angiitis
Definition
Cutaneous Necrotizing Vasculitis
Term
circulatory immune complex deposited in vascular walls at site of injury or where venous circulation slowed
Definition
Cutaneous Necrotizing Vascultitis
Term
netrophils attracted to sites of deposition, release lusomal enzymes -> endothelial damage -> fibrin deposition
Definition
Cutaneous Necrotizing Vasculitis
Term
may be associated with HBV or HCV , RA, SLE, UC (ulcerative colitis), lymphoma
Definition
Cutaneous Necrotizing Vasculitis
Term
Micro: neutrophilic infiltration of vessel wall, fibrin deposition, extravasion of blood cells
Definition
Cutaneous Necrotizing Vasculitis
Term
Clincal: multiple purpuric papules 2-4mm usually on lower extremeities or sites of pressure. Small blood vessels in joints, GI, kidney may be involved
Definition
Cutaneous Necrotizing Vasculitis
Term
Pathology: separation of epidermal keratinocytes by edema fluid -> spongiosis
Definition
Allergic contact dermatitis
Term
occurs as response to indigestible antigens accidently implanted into skin (breast implant)
Definition
Granulomatous Dermatitis
Term
formation of granulomas
Pathology
Definition
Granulomatous Dermatitis
Term
granulomatous disease, cause uknown
lungs, skin, lymph nodes, spleen etc.

epitheloid granulomas NO central necrosis
skin lesions: papules, plaques, nodules in dermis/subcutis
Definition
Sarcoidosis
Term
besides skin. kidneys, heart, lungs, esophagus, SI may be involved

disorder of dermal CT
Definition
Scleroderma
Term
lesions in dermis
-large collagen bundles parallel to epidermis, hypocellular
-patchy lymphocyte and plasma cell infiltration
Definition
Scleroderma
Term
-sweat glands and hair follicles obliterated
-late stage: subcutaneous fat replaced by collagen
Definition
Scleroderma
Term
peak incidence 30-50 years
F:M 4:1
Definition
Scleroderma
Term
non pitting edema of hands or fingers
affected areas hard and tense, mask like face
late stage: skin all over body thick, dense, fibrotic, and fixed to underlying tissue
Definition
Scleroderma
Term
most common form of panniculitis
Definition
Erythema Nodosum
Term
acute onset often associated with infections (streptococcal, TB, coccidiomycosis, histoplasmosis) drugs (sulfonamides, OCs), sarcoidosis, 1BD
Definition
Erythema Nodosum
Term
Lesions: poorly defined, very tender, erythematous nodules, often involving lower legs
Definition
Erythema Nodosum
Term
May be accompanied by fever and malaise, lesions later become flat no residual scars
Definition
Erythema Nodosum
Term
CT septa widing with edema, fibrin deposition, neutrophilic infiltrate; later replaced by lymphohistiocytic infiltration w/ giant cells and eosinophils. No vasculitis
Definition
Erythema Nodosum
Term
Uncommon panniculitis, cause unknown
Primary affects adolescents and menopausal women
Definition
Erythema Induratum
Term
Considered as primary vasculitis of deep vessels supplying subcutaneous fat -> necrosis and inflammation of adipose tissue
Definition
Erythema Induratum
Term
Lesion: erythematous, slightly tender nodule, usually ulcerates and scars
Definition
Erythema Induratum
Term
Micro: necrotizing vasculitis affecting small to medium sized arteries and veins in deep dermis and subcutis follweod by granulatous inflammation and necrosis
Definition
Erythema Induratum
Term
Most common skin disease seen in derm
Definition
Acne Vulgarus
Term
Micro: neutrophilic inflammation surrounding ruptured sebaceous follicle, numerous macrophages
Definition
Acne Vulgarus
Term
Propionibacterium acnes break down sebaceous oils 0> irritating fatty acids -> inflammation
Definition
Acne Vulgarus
Term
Accumulation of sebum and epithelial debris -> distension and rupture of follicle and discharge of contents to surrounding dermis -> intense acute inflammatory reaction
Definition
Acne Vulgarus
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