Pathology - Integumentary Diseases 1

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Congenital Syphilis

Back 1

Epi: 1 per 7500 live births
Eti: Treponema pallidum
RF: Lack of maternal prenatal care, Maternal high-risk sexual practices
Path: Transplacental transmission of spirochete Treponema pallidum
Sx: Asymptomatic at birth, secondary syphilis symptoms, multi-organ failure
Px: Perinatal mortality rate of 40%
Tx:
Note: Generally NOT a/w developmental abnormalities

Front 2

Staphylococcal Scalded Skin Syndrome

Back 2

Epi: 1/ 1 million, mostly in kids and mostly in males
Eti:Staphylococcus aureus
RF: Immune deficiency, day care
Path: exfoliative toxins released by S. aureus to system causes intraepidermal cleavage of the granular layer
Sx: fever, skin rash, broad areas of desquamation
Px: Skin heals and resolves in several weeks
Tx:
Note:

Front 3

Neonatal Herpes Infection

Back 3

Epi: 1/ 4000 live births
Eti: Herpes Virus
RF: Maternal herpes infection, prolonged rupture of membranes, vaginal delivery
Path: After contact, HSV replicates in epidermal and mucosal epithelial cells, cytolytic to a number of cell types and creates persistent infection of lymphocytes and macrophages
Sx:
Px:
Tx:
Note: Many types of the infection (Keratoconjunctivitis, Mucocutaneous, Encephalitis, Disseminated

Front 4

Oral Candidiasis

Back 4

Epi: 1/ 60 immunocompetent patients taking antibiotics, Increased in immunocompromised
Eti: Candida Albicans
RF: Antibiotic treatment, Primary immunodeficiency, Secondary Immunodeficiency
Path: Immune defect or disruption in normal bacteria flora which allows Candida to enter
Sx: Erythematous plaques with adherent white material, involves tongue and mucosa of the mouth
Px:
Tx:
Note:

Front 5

Mongolian Spots

Back 5

Epi: incidence varies with race, happens in pigmented races more
Eti: Melanocyte migration
RF:
Path: Caused by aberrant trapping of migrating melanocytes in the dermis
Sx: Irregular gray-blue area of discoloration commonly located in the sacral area and buttocks
Px: Resolves by year 6
Tx:
Note:

Front 6

Neonatal Systemic Lupus Erythematous

Back 6

Epi: rare
Eti: acquired autoimmune disease due to transplacental passage of IgG autoantibodies
RF:
Path: Transplacental passage of IgG autoantibodies from mother to fetus
Sx: hyperkeratosis with follicular plugging overlying an interface dermatitis with basilar vacuolar changes on the scalp face and periorbital region "raccoon eye" appearance
Px: Increased incidence of congenital heart block and sudden cardiac death, almost all cases resolve by month 6, Increased risk of autoimmune disease the rest of life
Tx:
Note:

Front 7

Allergic Contact Dermatitis

Back 7

Epi: 1/ 80
Eti:
RF: Any type of chronic dermatitis which allows easier contact of Langerhans cells with the allergen thru inflammed, porous skin
Path: Molecules responsible for ACD bind to carrier proteins on Langerhans cells in epidemis, Langerhans cells present antigen to CD4+ (T helpers), Sensitization then occurs to that chemical
Sx: Pruritis, Erythematous papules that vesiculate
Px: Self resolving
Tx:
Note: Common allergens are poison ivy, nickel, latex, formaldehyde

Front 8

Hereditary Angioedema

Back 8

Epi: 1/ 50,000 in USA
Eti:
RF:
Path: Hereditary deficiency of inhibitor first component of complement pathway (C1-Esterase inhibitor)
Sx: Onset during late childhood or adolescense, may or may not be preceded by erythematous lesions, more commonly effects extremities, but can effect mucosa or respiratory tract
Px: Increased incidence of Autoimmune Disease, Malignant Lymphoma
Tx:
Note: Type 1 is the most common type

Front 9

Anaphylaxis

Back 9

Epi: Non fatal 1/150, Fatal 1/350,000
Eti: Allergen
RF:
Path: Exposure to allergen elicits generation of IgE antibodies that bind to mast cells which degranulate and release histamine systemically
Sx: Urticaria, Angioedema, Wheezing, Hypotension, Shock, Death
Px:Low mortality rate with treatment
Tx: Antihistamines and possible Epinephrine
Note:

Front 10

Acute Eczematous Dermatitis

Back 10

Epi: 1/8 children
Eti:
RF: Bronchial asthma, Allergic rhinitis, Atopic dermatitis
Path: Disordered immune response in helper T-cells and an imbalance of cytokines
Sx: Early childhood onset, Ill-defined erythematous areas commonly with scaling Generalized skin dryness, intensely pruritic, Distribution on skin creases, cheeks, forehead, scalp, extensor surfaces
Px: May resolve spontaneously, Increased incidence of secondary bacterial infection
Tx:
Note:

Front 11

Psoriasis

Back 11

Epi: 1/75 usually young adults
Eti:
RF: Positive family history
Path: Hyperadhesive epithelial cells causing plaques, immune dysregulation in T-cell lymphocytes, Hyperplasia of epidermis associated with intense CD4+ lymphocyte inflammation
Sx: Lesion is commonly found on extensor surfaces with erythematous plaques (dry silvery scale), punctate areas of bleeding if removed (Auspitz sign), dystrophic nail changes
Px: Chronic and persistent dermatosis that waxes and wanes, some develop Psoriatic Arthritis
Tx:
Note:

Front 12

Seborrheic Dermatitis

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Epi: 1/25
Eti:
RF:
Path: Unknown, but presence of Malassezia sp. fungal organisms is probably a contributing condition
Sx: Commonly located in the oily and hair bearing areas of the head and neck, waxy and greasy scaling (dandruff) to widespread thick adherent crusts
Px: Chronic and persistent waxes and wanes
Tx:
Note:

Front 13

Discoid Lupus Erythematosus

Back 13

Epi: 1/5000, more common in females and blacks
Eti:
RF:
Path: Photodamage exposes or creates a heat shock protein within keratinocytes which act as an antigen target for T-cell mediated epidermal cell cytotoxicity, Deposition of immunoglobulin and and complement
Sx: Erythematous papule or plaque with scaling, Alterations in skin pigmentation, Systemic symptoms are rare, but occur
Px: Minority of patients develop SLE
Tx:
Note:

Front 14

Pemphigus Vulgaris

Back 14

Epi: 1/85,000, Increased incidence in Ashkenazi Jews
Eti: Pemphigus autoantibodies
RF: Positive Family Hx
Path: Pemphigus autoantibodies bind to keratinocyte cell surface molecules desmoglein and cause the loss of cell-to-cell adhesion.
Sx: Flaccid bulla arise on normal skin, Oropharyngeal lesions
Px: Healing without scarring, mortality rate around 10%
Tx:
Note:

Front 15

Bullous Pemphigoid

Back 15

Epi: 1/200,000, mostly in older adults or elderly
Eti:
RF:
Path: IgG autoantibodies directed against the hemidesmosomal BP antigens lead to complement activation and chemotaxis of neutrophils and eosinophils followed by inflammatory injury
Sx: Group of vesicles on an erythematous base
Px: Healing of bullous erosions without scarring, spontaneous remission
Tx:
Note:

Front 16

Dermatitis Herpetiformis

Back 16

Epi: 1/3000 usually adults but can occur at any age, Northern European whites
Eti:
RF:
Path: Deposition of IgA at the epidermo-dermal junction or in the basement membrane of the mucosa of a viscus which leads to complement fixation and neutrophil chemotaxis resulting in vesicle formation
Sx: Skin changes are common a/w intense PRURITIS, Usually presents on the extensor surfaces, groups of vesicles erythematous base
Px: Presentation of disease have underlying CELIAC DISEASE
Tx:
Note:

Front 17

Erythema Multifome

Back 17

Epi: M>F 2:1, usually in older children
Eti:
RF:
Path: UNKNOWN, but possibly hypersensativity rxn triggered by Medications (ADULTS) and Infections (Children), Dermis and epidermis accumulate activated T-cells and macrophages which release a large amount of cytokines
Sx: Usually occurs 10-14 days after beginning medication, Symmetric erythematous rash usually beginning on face and trunk with rapid progression to generalized rash
Px: Most have complete resolution in 12-15 days after last dosage of medication
Tx:
Note:

Front 18

Erythema Nodosum

Back 18

Epi: 1/5,000, M<F 1:4
Eti:
RF:
Path: UNKNOWN, Probably hypersensitivity reaction that occurs in relation to infection, systemic inflammatory disease or medication use
Sx: Lymphadenopathy, arthralgias that may be a/w effusions or arthritis are common, erthematous tender nodules that may be up to several inches in diameter and almost always on extensor surfaces
Px: Most resolve spontaneously in several months
Tx:
Note: Common causes include Strep, TB, Sarcoidosis, Lymphoma

Front 19

Nevus Flammeus

Back 19

Epi: 1/150 usually whites
Eti:
RF: (+) FMHx
Path: Congenital malformation of the superficial dermal blood vessels probably due to developmental paucity of sympathetic nervous system innervation
Sx: Usually located in one of three divisions of the 5th cranial nerve, Flat discrete violaceous macule that varies in size
Px: Increased incidence of Glaucoma
Tx:
Note: Many different forms ( Sturge-Weber Syndrome, Osler-Weber-Rendu Syndrome, Von Hippel-Lindau Syndrome ((increased incidence of Renal Malignancy))

Front 20

Systemic Vasculitis

Back 20

Epi: 1/250,000
Eti:
RF:
Path: Generally caused by aberrant immune responses that result in either direct endothelial injury or deposition of immune complexes into the endothelium leading to vascular wall inflammation
Sx: Fatigue, Myalgia, Fever, Palpable purpura, Urticaria, Oliguria, Melena
Px: Highly variable, Increased incidence of Chronic leg ulcer
Tx:
Note: Many Primary Vasculitis diseases (GIANT CELL ARTERITIS is MOST COMMON)

Front 21

Stasis Dermatitis

Back 21

Epi:
Eti:
RF:
Path: Breakdown of physiological mechanisms causing pooling of the blood in the lower extremities leading to venous hypertension. Ischemia and hypoxia ensue and it leads to vessel hemorrhaging
Sx: Commonly involves the lower legs, pruritis, edema, desquamation, varicosities
Px: Increased incidence of hyperpigmentation, lipodermatosclerosis, ulceration, and course is chronic and progressive
Tx:
Notes:

Front 22

Chronic Leg Ulcer

Back 22

Epi:
Eti:
RF: Postphlebitis Syndrome, CHF, Obesity, Varicose Veins
Path: Breakdown of physiological mechanisms causing pooling of blood. Pooling causes vascular hypertension, ischemia, necrosis and ulceration.
Sx: Chronic pruritis, Edema, Erythema and Desquamation, Serosanguinous drainage
Px:
Tx:
Notes: Most common type of chronic leg ulcer

Front 23

Thrombotic Thrombocytopenia Purpura

Back 23

Epi: usually adults, F>M (3 to 1)
Eti:
RF:
Path: Primary injury is endothelial cell injury which triggers cascade of events that results in microvascular lesions. Platelets are consumed causing formation of platelet thrombi.
Sx: Fever, Polyarthralgias, Petechiae, Shortness of breath, Nausea, Hematuria, Edema, Bleeding
Px: Majority resolve without sequelae
Tx:
Notes:

Front 24

Hemolytic Uremic Syndrome

Back 24

Epi: usually children,
Eti:
RF:
Path: Primary injury is endothelial cell injury which triggers cascade of events that results in microvascular lesions. Platelets are consumed causing formation of platelet thrombi
Sx: Fever, Infectious Enterocolitis, Bleeding, Oliguria, Hypertension
Px: Majority resolve without sequelae
Tx:
Notes:

Front 25

Idiopathic Thrombocytopenia Purpura

Back 25

Epi: Usually young children, 1/20000 (M>F 3 to 1)
Eti:Viral prodome
RF:
Path: Prodrome, somehow, induces IgG antibodies which cross react with the glycoprotein antigens on platelets and then are destroyed leading to manifestations of bleeding
Sx: Petechiae, Purpura, Usually on lower extremities, epistaxis, gingival bleeding
Px: Most pediatric cases resolve spontaneously, most adults display chronic thrombocytopenia
Tx:
Notes:

Front 26

Warfarin-Induced Skin Necrosis

Back 26

Epi: 1/5000 who begin warfarin therapy (F>M 1 to 9)
Eti:
RF:
Path: Unknown, but Warfarin-induced Protein C Deficiency resulting in spontaneous thrombosis in the blood vessels of the skin causing broad ranged hemmorrhage and skin necrosis
Sx: Begins shortly after treatment, unilateral or bilateral, sudden onset of painful sharply demarcated erythematous hemorrhage, bullous formation and skin necrosis
Px: Increased incidence of Sepsis, Distal Extremity Ischemia, Surgical Debridement with Skin Grafting
Tx:
Notes:

Front 27

Congenital CMV

Back 27

Epi: 1/200 live births
Eti: Betaherpesvinnae cytomegalovirus
RF:
Path: Contact secretions containing CMV leading to viremia in which the virus crosses the placental barrier, infected cell manifests large intranuclear inclusion "owl's eye"
Sx: CNS damage, ventriculomegaly, chorioentinits, hepatosplenomegaly, thrombocytopenia, sensoral/neural hearing loss
Px:
Tx:
Note: Most COMMON congenital infection in US