Dermatologic Manifestations of systemic disease

Leser-Trelat (paraneoplastic syndrome)

Abrupt increase in size or number of seborrheic keratoses that can occur before, during, or after diagnosis of gastric/colon adenocarcinoma, breast carcinoma, and lymphoma.

Hypertrichosis lanuginosa acquisita

Appearance of lanugo hairs in generalized areas of skin in association w/ cancers. predominantly in females

Nephrogenic Fibrosing Dermpoathy

Assoc. w/ exposure to gadolinium-containing contrast agents during MRI in patients w/ advanced renal disease Thickened hard immobile joint contracture w/ difficulty walking. 5% mortality

Peutz-Jegher's

Autosomal dominant condition w/ possibly malignant intestinal polyps and mucocutaneous pigmentation.

Perforating folliculitis control itching

Dermal material is eliminated transepidermally causing scaly papules in hair-bearing areas that may be pruritic asso. w/ renal failure condition? treatment?

Xanthomas

Develop due to intracellular and dermal lipid deposition. May be due to lipid disorder or paraproteinemia

Necrobiosis lipoidica

Disorder of collagen degeneration w/ granuloma formation and fat deposition commonly seen w/ DM but can occur w/o diabetes. Asymptomatic shiny red-brown patches on shins

Eruptive xanthomas

Erythematous to yellow/orange papules seen in severe chylomicronemia and hypertrigliceridemia (high triglcyerides) on hands, extremities, buttocks that regress when lipid state is stabilized

tendinous xanthoma on achilles tendon

Familial Hypercholesterolemia most commonly has a skin manifestation as what/where?

Gardner syndrome

Familial polyposis of colon. Autosomal dominant. Mutation in APC gene. Epidermoid cysts seen. Congenital Hyperpigmentation of retinal pigment epithelium (CHRPE)

Tuberous/Tuboeruptive xanthomas

Fatty growths that indicated underlying disorder of lipid/cholesterol metabolism. Pink/yellow papules or nodules on extensor surfaces. Slower to resolve w/ therapy.

Tendinous Xanthomas

Firm smooth nodular lipid deposits that can affect achilles, extensor tendons of hands/knees/elbows. Assoc. w/ underlying lipid disorder

Tripe Palm

Form of palmoplantar keratoderma. Velvety thickened skins of palms w/ ridged and rugose appearance. May herald malignancy of stomach, GU or lungs

Acquired Ichthyosis

Generalized scaling of skin most commonly seen w/ hodgkin's lymphoma "fish-like scales"

Necrolytic Migratory Erythema

Glucagon-producing tumor of pancreas often have what skin manifestation?

Acanthosis Nigricans treat DM - Keratolytic agents like lactic acid cream

Hyperpigmented velvety papules/plaques seen in association w/ DM. Commonly in axilla, inguinal, neck folds assoc. w/ skin tags condition? treat?

Acrokeratosis Paraneoplastica

Lesions on hands, occurs w/ malignancies of upper respiratory or digestive tracts. very rare.

Paget's disease

Malignant intraepithelial condition of nipple and skin around the nipple. Occurs almost exclusively in women w/ breast cancer.

Calciphylaxis renal disease wound care aggressive. normalize serum calcium

Microvascular occlusion w/ deposition of calcium salts in skin due to dysregulation of its metabolism. Dermatologic emergency. Super painful violaceous patches w/ necrosis. high mortality rate from sepsis condition? assoc. w/ what? treatment

Diabetic Dermopathy

Most common skin finding in patients w/ diabetes - shin spots in up to 50% of diabetics

Chlamydia

Most common trigger of reactive arthritis

Tuberous Sclerosis

Mutating hamartin or tuberin genes resulting in epilepsy, intellectual disability and angiofibromas.

Diabetic Ulcer

Occurs over pressure points on feet that have been diabetic for many years. Can often result in loss of limb. often associated w/ thick callus

Bullous Diabeticorum

Painless rapid developing blisters in small percentage of diabetics. Appear like bullous pemphigoid.

Familial dysbetalipoporteinemia

Palmar xanthomas are associated w/ what?

renal disease perforating disorders

Perforating folliculitis Elastosis perforans serpiginosa Reactive perforating collagenosis Kyrle's disease all are assoc. what what type of systemic disease?

Zinc deficiency

Periorificial, intertirignous, and acroal distribution of eczematous lesions

Kyrle's disease

Predominant material is extruded keratin, can have elastic and collagen as well. Almost always on lower extremity. Assoc. w/ kidney disease

Wilson Disease zinc

Rare autosomal recessive disease causing copper accumulation in tissues that presents in first or second decade of life presenting w/ nausea, vomiting, stomach ache or neurologic abnormlaties condition? treatment?

Marasmus

Results from total calorie starvation. Most commonly in infants that are malnourished.

Kwashiorkor

Severe from of protein malnutrition. Hyperpigmented patches/plaques that desquamate leaving raw surface often in friction areas. Flag sign - alternating bands of light and dark hair.

Sarcoidosis

Skin lesions occurring in tattoos. Condition?

Elastosis Perforans Serpiginosa

Transepidermal elimination of abnormal elastic fibers assoc. w/ renal disease, osteogenesis imperfecta, down's syndrome. Flesh colored to red umbilicated papules often occuring on extermities, face, trunk.

Reactive perforating collagenosis

Transepithelial elimination of altered collagen related to superficial trauma. Initially small papule that enlarges, spontaneously regresses, leaving scarring w/ brown adherent crust seen w/ kidney disease.

Pellagra

Vitamin B3 deficiency assoc. w/ 4 D's - dermatitis, dementia, diarrhea, and death.

Scurvy

Vitamin C deficiency - corkscrew hairs, perifollicular hemorrahge, gingival hypertrophy w/ bleeding gums

Perforating disorders Calciphylaxis Nephrogenic Fiborsing Dermopathy Pruritis

What are 4 major skin manifestations of renal disease

Eruptive Tuberous/Tuboeruptive Tendinous Plane

What are the types of xanthomas

Acanthosis Nigricans Diabetic dermopathy Diabetic ulcers Skin tags Necrobiosis Lipoidica Diabeticorum Bullous Diabeticorum

What are the various skin manifestations of DM

Chest abdomen, pelvis Most commonly - ovarian cancer

What cancers should be screened for after diagnosis w/ dermatomyositis

Arthritis, erythema nodosum, bilateral hilar lymphadenopathy Sarcoidosis

What is lofgren syndrome? Where is it seen?

Plane xanthomas

Xanthelasma is what type of xanthoma

Plane xanthomas

Yellow/orange patches or slightly raised papules or plaques that form due to increased lipid deposition due to elevated serum lipid. common in ages 40-60 commonly around eyelids

Type 1 - autosomal dominant mutation of tumor suppressor gene on chromosome 17 - cafe au lait, neurofibromas and axillary/groin freckling type 2 - acoustic neuromas, less common, mutation of TSG on 22. fewer cafe au lait.

differentiate neurofibromatosis type 1 and type 2

Xeroderma pigmentosum

rare autosomal recessive disorder of nucleotide excision repair - can't repair sun damaged keratinocytes. Extreme photosensitivity and high skin cancer rate.

Paget disease

what can mimic nipple eczema

If patients scratch they create more lesions

whats the koebner phenomenon