Skin Deep:

Dermatologic
Manifestations of
Systemic Diseases

Katty L. Go, M.D.
Dermatology
Scope

Common Rheumatic
cutaneous diseases
disorders Gastrointestinal
Internal
diseases
malignancies
Cardiovascular
Renal diseases
diseases Endocrine and
Pulmonary metabolic
diseases diseases
Common cutaneous
disorders

Seborrheic dermatitis
Seborrheic keratoses
Urticaria
Erythema multiforme
Vitiligo
Erythema nodosum
Seborrheic Dermatitis

Associated with
Parkinsons
disease
HIV infection
CVA unilateral
Seborrheic keratoses

If appear
suddenly and
in great
numbers
adenoCA of
GIT
Urticaria

Medication
(penicillin, sulfa,
aspirin)
Food (shellfish,
nuts, chocolates)
Infection
Erythema Multiforme

Infection (herpes
simplex virus,
mycoplasma
pneumoniae)
Drug
(sulfonamides,
barbituates,
antibiotics)
Vitiligo

Autoimmune
thyroid disease
Insulin
dependent DM
Pernicious
anemia
Addisons disease
Erythema nodosum

Infection
Drug
hypersensitivity
Inflammatory
bowel disease
(Crohns disease))
sarcoidosis
Internal Malignancies

Cutaneous matastases
Pagets disease
Acanthosis nigricans
Cowdens syndrome
Sweets syndrome
Amyloidosis
Paraneoplastic pemphigus
Erythema gyratum repens
Cutaneous malignancies

Prevalence: 0.7%-
10%
In men, CA lung,
colon, kidney
In women, CA
breast, colon
Sites: scalp, neck,
trunk
Pagets Disease

Breast CA
Ductal CA in situ
Extramammary
Pagets in
anogenital or
axillary
adnexal CA, CA
GUT, colon
Acanthosis Nigricans

Type 1
malignant AN
(adenoCA
gastric)
Type 2 familial
Type 3
obesity, DM
Cowdens Syndrome

Autosomal dominant
Mutations in the
tumor suppressor
gene PTEN
Tricholemmomas
mouth, nose, ears
CA breast,
endometrium,
thyroid
MR
Fibrocystic breast
Sweets sydrome

Acute myelocytic
or
myelomonocytic
leukemia
Inflammatory
bowel disease
Bowel bypass
syndrome
Amyloidosis

Multiple myeloma
Purpura and
ecchymosis after
pressure or
rubbing blood
vessel fragility
from amyloid
infiltration of
vessels
Paraneoplastic
Pemphigus

Non Hodgkins
lymphoma
Chronic
lymphocytic
leukemia
Pulmonary
(bronchiolitis
obliterans)
Erythema Gyratum
Repens

Lung CA strong
association
Less: CA breast,
cervical, GI
Severe pruritus
Peripheral
eosinophilia
Cardiovascular disease

LEOPARD Syndrome
Carney complex
Pseudoxanthoma elasticum
Ehlers-Danlos syndrome
LEOPARD Syndrome
Lentigines
Electrocardiograp
hic changes
Ocular telorism
Pulmonary
stenosis
Abnormal
genitalia
Retarded growth
Deafness
Carney Complex

LAMB lentigines,
atrial myxoma,
mucocutaneous
myxoma, blue nevi
NAME nevi, atrial
myxoma, myxoid
neurofibromas,
ephelids
Pseudoxanthoma
elasticum

Yellow papules
over redundant
skin folds from
elastic fiber abn
With HPN, CAD,
retinal/GI
hemorrhage,
stroke
Ehlers-Danlos Syndrome

Hyperextensibility,
hypermobility,
vessel fragility, fish
mouth scars
Mitral valve
prolapse, blue
sclerae, vascular
aneurysm, aortic
dissection, hernias,
GI bleeding
Pulmonary disease

Sarcoidosis
Sarcoidosis

Granulomatous
disease lungs,
bones, CNS, lymph
nodes, eyes, skin
Lupus pernio red
to purple indurated
plaques on the
nose
EN on shins
Rheumatic disease

Psoriatic Arthritis
Lupus Erythematosus
Scleroderma
Reactive arthritis
Erythema Chronicum
Migrans
Dermatomyositis
Psoriatic Arthritis

5-10% of patients
70% as
asymmetric
fusiform swelling
DIP
Oligoarthritis,
tenosynovitis
50% - HLA-B27
genotype
Lupus Erythematosus

Localized with scarring
(discoid)
Subacute annular
pink/red plaques sun-
exposed areas
Systemic malar rash,
photosensitivity, oral
ulcers, discoid plaques,
bullae, purpura,
calcinosis cutis,
alopecia
Scleroderma

Localized form
(morphea)
Systemic 2 forms:
CREST calcinosis,
Raynauds,
esophageal
dysmotility,
sclerodactyly,
telangiectasia
Progressive
systemic sclerosis
Reactive Arthritis

Reiters syndrome
with conjunctivitis,
urethritis, diarrhea
Crusted plaques
penis
Papules pustules on
palms and soles
Erythema Chronicum Migrans

Lyme disease
Tick borne
spirochete Borrelia
burgdoferi
Annular plaque on
bite site
Fever arthralgia
myalgia Bells
palsy
Dermatomyositis

Proximal muscle
weakness,
symmetric
Photosensitivity
Gottrons papules
Heliotrope
Poikiloderma face,
neck trunk
extremities
Malar erythema
Periungal
Gastrointestinal disease

Dermatitis Herpetiformis
Acrodermatitis Enteropathica
Necrolytic Migratory Erythema
Hepatitis C virus-associated skin
disorders
Gardners Syndrome
Hereditary Hemorrhagic Telangiectasia
Muir-Torre Syndrome
Peutz-Jeghers Syndrome
Pyoderma Gangrenosum
Dermatitis Herpetiformis

Grouped vesicles
papules elbows,
knees scalp buttocks
IgA antibodies
against smooth
muscle cell
endomysium in 70%
Gluten sensitive
enteropathy
Acrodermatitis
Enteropathica

Acral periorificial
pustules, bullae
w/ zinc deficiency
Mutation in
SLC39A that
encodes an
intestinal zinc
transporter
Necrolytic Migratory
Erythema

Red scaly plaques
acral,
intertriginous,
periorificial areas
Islet cell tumor of
pancreas
Hyperglycemia,
diarrhea, weight
loss, atrophic
glossitis
Hepatitis C Virus-
Associated Skin Disorders

Leucocytoclastic
vasculitis
Lichen planus
Necrolytic acral
erythema
Prophyria cutanea
tarda
Gardners Syndrome

Autosomal dominant
Colonic polyposis
Osteomas (maxilla,
mandible, skull)
Scoliosis
Epidermal cysts
Soft tissue tumors
(fibromas, desmoids,
lipomas)
Hereditary Hemorrhagic
Telangiectasia

Osler-Weber-
Rendu Syndrome
Autosomal
dominant
Telangiectasia
skin and oral
mucosa, lungs
liver brain eyes
GIT
Muir-Torre Syndrome

Sebaceous tumors
(adenoma,
epithelioma,
carcinoma)
w/ internal CA
colorectal GU
lymphoma
Mutation of MSH-2
Peutz-Jeghers Syndrome

Autosomal
dominant
Lentigines
periorbital,
fingers, toes
Hamartomas
polyps GIT
10-18x risk for GI
CA
Pyoderma Gangrenosum

Painful ulcers
extend
centrifugally
Underlying RA,
inflammatory
bowel disease,
IgA
gammopathy
 Renal Disease

Nephrogenic Systemic Fibrosis
Birt-Hogg-Dube Syndrome
Nephrogenic Systemic
Fibrosis

Resemble
scleroderma
In end stage renal
disease, on
dialysis or after
kidney transplant
Maybe
progressive
Birt-Hogg-Dube
Syndrome

Multiple
fibrofolliculomas and
trichodiscomas (skin
colored dermal papules
face trunk)
Inc risk renal
onococytoma and
chromophobe renal CA
Pneumothorax from
pulmonary cyst rupture
Endocrine and metabolic
disease

Porphyrias
Pseudoporphyria
DM related skin
conditions
Porphyrias

Photosensitivity, skin
fragility, bullae,
erosions on sun
exposed skin,
hypertrichosis
Def
uroporphyrinogen
decarboxylase
uroporphyrin urine
and serum
Pseudoporphyria

No enzyme defect
NSAIDs
(naproxen),
furosemide,
tetracycline
most common
causes
DM related skin

Diabetic
dermopathy
Diabetic thick skin
Acanthosis
nigricans
Yellow nails and
skin (palms and
soles)
Acquired
perforating
disorders (thick
DM related skin

Calciphylaxis
Necrobiosis
lipoidica
diabeticorum
Diabetic
bullae
ALL WE HAVE TO DECIDE IS WHAT
TO DO WITH THE TIME THAT IS
GIVEN US.
LORD OF THE RINGS

I FEEL AT EASE WITH WHATEVER WILL
COME, NOT BECAUSE I AM STRONG BUT
BECAUSE THIS IS PART OF LIFE. IT IS
NOTHING TO BE FEARED.
ANGELINA JOLIE-PITT