Transcript Paraneoplastic Syndromes and Cutaneous Signs of Malignancy

Cutaneous Manifestations of
Internal Disease
Ronald Buckley, D.O.
Dermatology Department
Lakeview Medical Center
Suffolk,Virginia
April 02 2011
Course Objectives:
• Cutaneous findings may provide valuable
clues to the diagnosis of underlying internal
disease.
• During this presentation, a limited number
of internal diseases manifestations on the
skin will be reviewed.
Course Objectives
• At the conclusion of this talk, the audience will recognize
the cutaneous manifestations of internal diseases
involving: integument disorders, blistering diseases,
internal cancers, cardiovascular disease, pulmonary
disease, rheumatic disease, gastrointestinal, and metabolic
disease.
• Goals
– Help the primary care physician to establish the proper diagnosis
and management.
– Determine the need for referral to the appropriate specialist.
Overview
1.
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3.
4.
5.
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7.
8.
Common Benign
Cutaneous Disorders
Blistering Diseases
Internal Cancers
Cardiovascular Disease
Pulmonary Disease
Rheumatic Disease
Gastrointestinal Disease
Metabolic Disease
Common Benign Cutaneous
Disorders
• Seborrheic Dermatitis
– Chronic and common inflammatory disease.
– Location: scalp, face, chest and intertriginous areas.
– Clinical appearance: erythematous, often greasy,
scaling patches and plaques favor hair bearing areas.
Scalp pruritus is common.
– Internal Association: HIV/AIDS and Langerhan Cell
Histiocytosis (Letterer-Siwe, Hand Schuller-Christian
and eosinophilic granuloma).
Seborrheic Dermatitis
Seborrheic Dermatitis
Common Benign Cutaneous
Disorders
• Seborrheic Dermatitis
– Differential Diagnosis: eczema, psoriasis, tinea
infection, zinc deficiency and lupus
erythematosus.
– Treatment: shampoos, sulfacetamide, azeleic
acid, coal tar, topical steroids and topical or oral
antifungal.
Tinea Facei
Common Benign Cutaneous
Disorders
• Seborrheic keratoses
– Extremely common benign tumor.
– Location: Anywhere but most commonly on the
trunk.
– Clinical appearance: warty plaques and “stuck
on” appearance, “greasy” with pseudohorn
cysts.
Seborrheic keratoses
Common Benign Cutaneous
Disorders
• Seborrheic keratoses
– Internal Association: underlying
adenocarcinoma of GI tract if they appear
suddenly in great numbers (sign of LeserTrelat).
– Differential Diagnosis: Malignant Melanoma.
– Treatment: reassurance, cryotherapy, curettage
or excision.
Common Benign Cutaneous
Disorders
• Urticaria and Angioedema
– Transient lesions most often triggered by
medication (PCN, Sulfa, ASA) or food (nuts,
eggs, milk, shellfish), and less so by infection.
– Location: Localized, regional, or generalized.
– Clinical appearance: edematous papules and
plaques with oval, annular, polycyclic,
serpiginous, and bizarre shapes.
Urticaria
Urticaria
Common Benign Cutaneous
Disorders
• Urticaria and Angioedema
– Internal Association: Urticarial vasculitis-type II
immune complex reaction.
– Urticarial plaques that persists for 24-72 hrs.
– Associated with purpura, scaling, and hyperpigmentation.
– Differential diagnosis: erythema multiforme, SLE,
bullous pemphigoid, mastocytosis.
– Treatment: elimination of known causes,
antihistamines, systemic steroids, sulfasalazine and
cyclosporine.
Urticarial
Vasculitis
Angioedema
Common Benign Cutaneous
Disorders
• Erythema multiforme
– Relatively common- hypersensitive, acute and
often recurrent inflammatory process.
– Location: backs of hands and feet, extensor
surfaces of forearms and arms and less
commonly on the trunk.
– Clinical Appearance: targetoid, morbilliform,
plaques, vesicles or bullae.
Erythema multiforme
Common Benign Cutaneous
Disorders
• Erythema multiforme
– Internal Association: HSV, mycoplasma,
connective tissue diseases and drugs.
– Differential diagnosis: urticaria, drug eruption
bullous pemphigoid.
– Treatment: oral antiviral for herpes infection;
discontinuation of offending medication; oral
prednisone and supportive care.
Common Benign Cutaneous
Disorders
• Vitiligo
– Type of acquired leukoderma. Autoimmune
disease.
– Location: focal (periorificially -mouth ,orbits,
vagina, anus), segmental and generalized.
– Clinical appearance: Depigmented macules
and patches.
Vitiligo
Vitiligo
Common Benign Cutaneous
Disorders
• Vitiligo
– Internal Association: thyroid dz, diabetes mellitus,
pernicious anemia or Addison’s dz.
– Differential Diagnosis: pityriasis alba, tinea versicolor,
postinflammatory hypopigmentation, hypopigmented
mycosis fungoides.
– Treatment: sunscreen, topical steroids, tacrolimus or
pimecrolimus, phototherapy, skin grafting and
depigmentation.
Common Benign Cutaneous
Disorders
• Erythema nodosum
– Most common type of panniculitis.
– Location: lower legs (most common in females), knees,
arms, rarely face and neck.
– Clinical Appearance: painful, indurated, tender, red to
violaceous nodules.
– Internal Association: Infectious ( streptococcal
pharyngitis), drug sensitivity (sulfonamide,oral
contraceptives), inflammatory bowel dz and
sarcoidosis.
Erythema Nodousm
Erythema Nodosum
Common Benign Cutaneous
Disorders
• Erythema nodosum
– Differential diagnosis: nodular vasculitis and
other panniculitis.
– Treatment: elimination of known triggers, bed
rest, NSAIDs, colchicine, and supersaturated
iodide.
Life Threatening Cutaneous Disorder
• Exfoliative Dermatitis (Erythroderma)
– Potentially a life-threatening reaction pattern involving
the skin.
– Location: generalized .
– Clinical appearance: generalized and confluent redness
and scaling of the skin with associated systemic
“toxicity”, generalized lymphadenopathy, pruritus,
malaise, chills, and fever.
– Causes: most commonly are preexisting skin diseases:
– Psoriasis, atopic dermatitis and drug hypersensitivity.
Exfoliative dermatitis
Exfoliative
dermatitis
Life Threatening Cutaneous Disorder
• Exfoliative Dermatitis (Erythroderma)
– Internal Association: Cutaneous T Cell Lymphoma
(less commonly), leukemia and pityriasis rubra pilaris.
– Differential diagnosis: lichen planus, pemphigus
foliaceus, ichthyosiform erythroderma, acute graftversus-host disease.
– Treatment: hospitalized in single room with attention to
the patient need for temperature control. Water baths
with bath oils followed by application of bland
emollients.
Life Threatening Cutaneous Disorder
• Exfoliative Dermatitis
(Erythroderma)
– Treatment: Oral and
topical corticosteroids
for remission induction
only.
– Supportive (cardiac,
fluid, electrolyte,
protein replacement).
Blistering Disorders
• Pemphigus vulgaris
– Serious acute or chronic debilitating blistering
autoimmune disease of the skin and mucous membrane.
Often fatal unless treated with immunosuppresive
agents.
– Location: mouth or generalized. Predilection for the
scalp, face chest, axillae, groin, umbilicus.
– Clinical appearance: Vesicles and bullae, flaccid,
easily ruptured, and weeping, arising on normal skin.
Extensive erosions that bleed easily with crusting.
Nikolsky’s sign.
Blistering Disorders
• Pemphigus Vulgaris
– Diagnosis: Bx of the skin and mucous
membrane, DIF (IgG/C3) and circulating autoantibodies(IgG).
– DDX: BP, Steven-Johnson, EBA
– Tx: Immunosuppressive agents, systemic
corticosteroids, plasmapheresis, topical
corticosteroids and antibiotics, rituximab and
IVIg.
Pemphigus Vulgaris
Pemphigus Vulgaris
Blistering Disorders
• Bullous pemphigoid
– Chronic autoimmune mediated bullous eruption
in patients over 60 yrs of age.
– Location: Generalized or localized and
randomly distributed. Rarely involve oral
mucosa.
– Clinical appearance: Large tense firm-topped
bullae. May arise in normal or erythematous
skin and often pruritic.
Bullous Pemphigoid
Blistering Disorders
• Bullous pemphigoid
– Diagnosis: Clinical, confirmed by histopathology and
immunopathology (IgG at DEJ)
– Differential Diagnosis: bullous SLE, EBA, cicatricial
pemphigoid and DH.
– Treatment: excellent prognosis with treatment; topical
and systemic corticosteroids, TCN, niacinamide, steroid
sparing immunosuppressants.
Blistering Disorders
• Dermatitis herpetiformis (DH)
– Intensely pruritic chronic and recurrent
eruption.
– Location: symmetrical groups on the elbows,
knees, scalp, and buttocks.
– Clinical appearance: tiny vesicles, papules, and
urticarial wheals.
Dermatitis Herpetiformis
Blistering Disorders
• Dermatitis herpetiformis (DH)
– Internal association: small-bowel malabsorption.
Gluten-sensitive enteropathy.
– Diagnosis: Clinical, confirmed by histopathology and
immunopathology.
• Circulating IgA antibodies (antiendomysial antibodies) in 70%
pts.
– Differential Diagnosis: linear IgA dermatosis, BP,
contact dermatitis and bullous LE.
– Treatment: dapsone, sulfapyridine, gluten-free diet.
Blistering Disorders
• Epidermolysis bullosa acquisita (EBA)
– Rare autoimmune bullous disease.
– Location: hands, feet, elbows, knees and
mucous membrane.
– Clinical appearance: skin fragility, milia,
scarring alopecia, and nail dystrophy, tense
blisters on inflammatory or noninflammatory
base.
Epidermolysis bullosa acquisita
Epidermolysis bullosa acquisita
Blistering Disorders
• Epidermolysis bullosa acquisita (EBA)
– Diagnosis: Clinical, confirmed by
histopathology and immunopathology.
– Differential Diagnosis: bullous pemphigoid
bullous LE.
– Treatment: Poor response to topical and
systemic therapy. Cyclosporine and high dose
intravenous immunoglobulins may be effective.
Skin Disease and Internal Cancer
• Cutaneous metastases
– Occur in less that 5% of patients with metastatic cancer.
– Generally reflect the most prevalent cancer in the
general population (breast, lung, or GI tract)
– Clinical appearance: skin-colored to violaceous nodules
in close proximity to the primary neoplasm.
– Location: most commonly seen on the head, neck and
trunk.
– DDx: cyst, adnexal tumor, neurofibroma and lipoma
Metastatic
Carcinoma
Metastatic Carcinoma
Skin Disease and Internal Cancer
• Mammary Paget’s disease
– Unilateral breast cancer-nearly always associated with
underlying intraductal carcinoma.
– Clinical appearance: eczematous plaque of the nipple
and areola with exudate that persists for longer than a
few weeks. Resistant to topical therapy.
– Differential Diagnosis: allergic or irritant contact
dermatitis, psoriasis, BCC, Bowens dz.
– Treatment: Referral to oncologist, surgery, radiation,
photodynamic therapy.
Paget’s Disease
Skin Disease and Internal Cancer
• Extramammary Paget’s Disease
– Neoplasm of the anogenital and axillary skin.
– Histologically and clinically similar to Paget’s dz of
the breast. Often represents an intraepidermal
extension of primary adenocarcinoma of underlying
adnexal (apocrine) carcinoma or underlying cancer of
GI tract or GU tract.
– Location: vulva, scrotum, penis, perianal, perineal,
axilla,umbilicus and sternum.
– Clinical appearance: erythematous plaque +/- scaling,
crusting, and exudation. Borders sharply defined.
Extramammary
Paget’s Disease
Skin Disease and Internal Cancer
• Extramammary Paget’s Disease.
– Differential Diagnosis: eczematous dermatitis,
intertriginous Candida infection, tinea corporis,
erythrasma, Bowen’s dx, HPV induced intraepithelial
neoplasia, superficial spreading melanoma.
– Treatment: surgical excision.
Skin Disease and Internal Cancer
• Acanthosis nigricans
– most likely triggered by
factors that stimulate
epidermal keratinocyte and
dermal fibroblast
proliferation.
– benign form- the factor likely
insulin or an insulin-like
growth factor
– malignant AN- stimulating
factor secreted either by the
tumor or in response to the
tumor.
Acanthosis
Nigricans
Acanthosis Nigricans
Skin Disease and Internal Cancer
• Acanthosis Nigricans
– Location: groin, axillae, neck.
Malignant form has sudden
onset and more extensive
distribution- face and dorsal
and palmar surfaces of hands .
– Clinical appearance: smooth,
velvet-like, hyperkeratotic
plaques of intertriginous areas.
– Internal association:
Adenocarcinoma – GI, lung,
breast. Most common causes
are obesity, insulin-resistant
diabetes and medications.
Tripe Palms
Skin Disease and Internal Cancer
• Acanthosis Nigricans
– Differential diagnosis: confluent and reticulated
papillomatosis, Dowling-Degos dz.
– Treatment: treatment of underlying disorder,
including removal of malignant tumor.
Skin Disease and Internal Cancer
• Gardner’s syndrome
– Autosomal dominant cancer syndrome
– Characterized by:
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•
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Colonic polyposis
Osteomas (maxilla, mandible, skull)
Scoliosis
Epidermoid cysts
Soft-tissue tumors (fibromas, desmoid tumors, lipomas)
Pigmented ocular fundus lesions
– Adenocarcinoma of colon develops in 60% of pts by
the age of 40.
Epidermal Inclusion Cyst
Skin Disease and Internal Cancer
• Muir-Torre Syndrome
– Autosomal dominant cancer syndrome.
– Characterized by at least one sebaceous tumor
(adenoma, sebaceoma, epithelioma, or carcinoma) and
one internal neoplasms, usually colorectal(47%),
GU(21%), breast CA(12%) and hematologic(9%)
– Clinical appearance: small asymptomatic papules or
nodules that resembles cysts or benign growths to waxy
papules located mainly of the face, trunk and scalp.
Sebaceous Adenoma
Sebaceous Neoplasms
Skin Disease and Internal Cancer
• Cowden’s Syndrome
– Autosomal dominant.
• Mutations in tumor suppressor gene PTEN
– Characterized by:
• Multiple tricholemmomas (resemble warts) around the mouth,
nose and ears.
• Breast and thyroid cancer
• Fibrocystic disease of the breast
• Thyroid adenoma
• Mental retardation
Skin Disease and Internal Cancer
• Hirsutism
– Appearance of excessive coarse, male-type pattern,
hair in woman.
– Associated androgen excess (adrenal or ovarian tumor).
• Hypertrichosis
– Excessive growth of vellus hairs in any hair-bearing
areas
– Associated with malignancy, metabolic disorders or
medications (minoxidil and cyclosporine).
Hirsutism
Skin Disease and Internal Cancer
• Sweet’s syndrome (acute neutrophilic
dermatosis)
– Strong association with acute myelocytic or
myelomonocytic leukemia.
– Clinical appearance: abrupt onset of tender or
painful reddened plaques or nodules
occasionally with vesicles, bullae or pustules on
the face, extremities, and trunk usually in
middle-aged women.
Sweet’s Syndrome (acute febrile
neutrophilic dermatosis)
Sweet’s
Syndrome
Skin Disease and Internal Cancer
• Sweet’s syndrome (acute neutrophilic dermatosis)
– Associated systemic symptoms: fever, peripheral
leukocytosis, arthralgias, myalgias and conjunctivitis.
– Differential diagnosis: erythema multiforme, SLE,
deep fungal infection, pyoderma gangrenosum,
cutaneous metastases.
– Treatment: systemic corticosteroids, NSAIDs and
dapsone.
Skin Disease and Internal Cancer
• Amyloidosis
– May be a sign of multiple myeloma.
– Clinical: waxy, smooth, shiny, flat-topped or
spherical papules, nodules or plaques. “Pinch
Purpura”
– Location: midface, tongue, scalp, body folds,
axillae, umbilicus, anogenital area.
– Treatment: no effective therapy, chemotherapy
and stem cell transplantation.
Primary systemic amyloidosis
Macroglossia
(Amyloidosis)
Purpura (Amyloidosis)
Skin Disease and Internal Cancer
• Dermatomyositis
– An inflammatory systemic disease;
– Characterized: proximal muscle weakness,
photosensitivity, violaceous (heliotrope)
inflammatory changes of eyelids and periorbital
area, papules and plaques of hands, elbows, and
knees (Gottron’s papules).
– Elevated creatine kinase or aldolase level,
positive Jo-1 antibody, and EKG changes.
Heliotrope erythema
Gottron’s papules
Dermatomyositis
Skin Disease and Internal Cancer
• Dermatomyositis
– Other features: scaly, telangiectatic plaques
with atrophy and hypopigmentation
(poikiloderma) on face, neck, trunk ,
extremities, malar erythema and nail
abnormalities (periungal telangiectases and
cuticular hypertrophy).
Dermatomyositis
Skin Disease and Internal Cancer
• Dermatomyositis
– Diagnosis: requires muscle bx, electromyogram,
measurement of muscle enzymes (aldolase, creatine
kinase, transaminases).
– Internal association: Adenocarcinoma of breast, GI
tract or lung in adults.
– Differential diagnosis: SLE, photosensitive drug
eruption.
– Treatment: systemic corticosteroids, MTX,
immunosuppressants and TNF alpha inhibitors.
Skin Disease and Internal Cancer
• Paraneoplastic pemphigus
– Autoimmune disease with clinical and
histological features of EM and PV.
– Clinical appearance: intractable stomatitis and
blisters , conjunctival reactions, polymorphous
skin lesions.
– Location: oral mucosa, trunk, extremities.
Paraneoplastic pemphigus
Skin Disease and Internal Cancer
• Paraneoplastic pemphigus
– Internal association: non-Hodgkin’s
lymphoma, chronic lymphocytic leukemia,
thymoma.
– Diagnosis: histopathology and immunopathology.
– Treatment: based on underlying dz.
Skin Disease and Internal Cancer
• Erythema gyratum repens
– Rare by most distinctive
skin eruption
– Clinical appearance:
reddened concentric bands
in a wood grain or whorled
pattern.
– Internal association: lung,
breast, cervical and GI
cancers.
– Treatment: removal of
cancer clears the eruption.
Erythema gyratum repens
Skin Disease and Cardiovascular
Disease
• Multiple Lentigines
– LEOPARD Syndrome (Moynahan’s)
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Lentigines
Electrocardiographic changes
Ocular telorism
Pulmonary stenosis
Abnormal genitalia
Retarded growth
Deafness
LEOPARD
Skin Disease and Cardiovascular
Disease
• Multiple Lentigines
– NAME
•
•
•
•
Nevi
Atrial myxoma
Myxoid neurofibromas
Ephelides
Skin Disease and Cardiovascular
Disease
• Multiple Lentigines
– LAMB Syndrome
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Lentigines
Atrial myxoma
Mucocutaneous myxomas
Blue nevi
Skin Disease and Cardiovascular Disease
• Pseudoxanthoma elasticum (PXE)
– An inherited or acquired defect of elastic tissue.
• Mutations in the gene ABCC6 on chromosome 16 have been
linked to PXE.
– Clinical appearance: yellow cobblestone lesions over
redundant skin folds in flexural location arranged in
linear distribution – “plucked chicken”.
– Location: Neck and axillae most common sites.
Pseudoxanthoma Elasticum (PXE)
Angioid Streaks
Skin Disease and Cardiovascular Disease
• Pseudoxanthoma elasticum (PXE)
– Internal association: HTN, peripheral vascular
and coronary dz, retinal and GI hemorrhage,
strokes and angioid streaks.
– Differential diagnosis: cutis laxa, EhlersDanlos syndrome.
– Treatment: no definitive therapy.
Skin Disease and Cardiovascular Disease
• Ehlers-Danlos syndrome
– An inherited defect of collagen tissue at least eleven
types.
– Clinical appearance: hyperextensibility, hypermobility,
skin fragility, “fish-mouth” scars.
– Internal association: MVP, blue sclerae, aneurysm,
aortic dissection, hernias, angina, GI bleeding and
peripheral vascular dz.
– Treatment: protection of skin and treatment of systemic
findings.
Ehlers-Danlos Syndrome
Ehlers-Danlos
Syndrome
Skin Disease and Pulmonary Disease
• Sarcoidosis
– Chronic multisystem granulomatous inflammation.
– Clinical appearance: red to violaceous plaques of the
nose (lupus pernio), midfacial papules, annular plaques,
nodules or plaques on trunk and extremities. Erythema
nodosum-mt common cutaneous manifestation.
– Higher prevalence in women and African Americans.
– Differential diagnosis: trichoepitheliomas, rosacea,
granuloma annulare.
– Treatment: systemic corticosteroids, antimalarials,
MTX, thalidomide and TNF alpha inhibitors.
Sarcoidosis
Sarcoidosis
Skin Disease and Rheumatic Disease
• Psoriatic arthritis
– A distinct form of arthritis with negative rheumaotoid
factor.
• HLA-B27 genotype in 50% of affected patients.
– Clinical appearance: five types:
• Asymmetric arthritis-most common type with “sausage”
fingers and toes
• Symmetric arthritis
• Distal interphalangeal joint disease
• Arthritis mutilans
• Ankylosing spondylitis
Psoriatic
Arthritis
Psoriatic Arthritis
Skin Disease and Rheumatic Disease
• Psoriatic arthritis
– Differential diagnosis: rheumatoid arthritis.
– Treatment: Biologic agents, NSAID’s,
antimalarials, MTX, retinoids, cyclosporine.
Skin Disease and Rheumatic Disease
• Lupus erythematosus
– A multisystem autoimmune photosensitive dermatosis.
– Clinical forms:
• 1. DLE- localized to head or neck with scarring plaques
usually negative ANA.
• 2. SCLE-annular plaques on trunk and arms with positive
ANA, Ro(SS-A), La(SS-B).
• 3. SLE-malar erythema, photosensitivity, oral ulcers, discoid
plaques, alopecia, positive- ANA, dsDNA, Ro(SS-A), La(SSB), sm.
Discoid Lupus Erythematosus
Systemic Lupus Erythematosus
(SLE)
Skin Disease and Rheumatic Disease
• Lupus erythematosus.
– Clinical forms:
• 4. Neonatal LE- noticed in first month, photosensitive,
papulosquamous and annular patterns, congenital heart block
50% of cases. Maternal passage of antibodies- Ro, La, U 1RNP, Antiphospholipids.
• 5. Drug-Induced LE- rash uncommon, ANA, ssDNA,
antihistone positive. Procainamide, Hydralazine, Isoniazid.
Symptoms clear with stopping the med.
– Treatment: Sun protection, steroids, dapsone and
immunosuppressants.
Neonatal LE
Skin Disease and Rheumatic Disease
• Reiter’s syndrome
– Triad: urethritis, conjunctivitis, and
oligoarthritis.
– Circinate balanitis-vesicles and crusted plaques.
– Keratoderma blennorrhagicum-erythematous
pusutles and papules on palms and soles.
– Sacroiliitis-present 50% of pt (HLA-B27).
– Treatment: topical corticosteroids for skin dz.
Skin Disease and Gastrointestinal
Disease
• Hepatitis C infection association:
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Leukocytoclastic vasculitis
Cutaneous B-cell lymphoma
Cryoglobulinemia
Pruritus
Porphyria cutanea tarda
Urticaria
Prurigo nodularis
Lichen planus
Leukocytoclastic Vasculitis
Skin Disease and Gastrointestinal
Disease
• Peutz-Jeghers Syndrome
– Characterized: lentigines on skin and mucosa-lips,
buccal mucosa, periorbital region, dorsal of fingers and
soles.
– Associated: benign polyps (hamartomas) of small
intestine with low malignant potential.
– DDx: LEOPARD syndrome, Carney complex and
Cronkhite-Canada syndrome
– Tx: regular and routine endoscopy and symptomatic
treatment.
Peutz-Jeghers Syndrome
Skin Disease and Gastrointestinal
Disease
• Pyoderma gangrenosum
– Neutrophilic dermatosis with painful ulcers with boggy,
undermined edges and a border of gray or purple
pigmentation.
– Associated: inflammatory bowel dz, rheumatoid
arthritis or paraproteinemia usually IgA gammopathy.
– Differential diagnosis: infection, vasculitis, factitious
disorder.
– Treatment: systemic, intralesional and topical
corticosteroids; cyclosporine, infliximab and
immunosuppressants.
Pyoderma Gangrenosum
Skin Disease and Metabolic Disease
• Porphyrias
– A group of inherited or acquired blistering
disorders of heme biosynthetic pathway.
– Porphyria cutanea tarda (PCT)
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•
•
•
Most common porphyria.
Hepatic porphyria (acquired/sporadic).
Deficiency uroporphyrinogen decarboxylase.
Accumulation of by products in urine/serum.
Porphyria Cutanae Tarda (PCT)
Porphyria Cutanae Tarda (PCT)
Skin Disease and Metabolic Disease
• Porphyrias
– Porphyria cutanae tarda (PCT)
• Precipitating factors: alcohol ingestion, estrogen
administration, dinitrochlorobenzene, carbon, tetrachloride,
dialysis, hepatitis B or C infection.
• Clinical appearance: photosensitivity, skin fragility, bullae,
erosions of sun-exposed skin, milia and hypertrichosis.
• Differential diagnosis: bullous SLE, EBA, pseudoporphyria.
• Treatment: phlebotomy, antimalarials.
Test
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
Exfoliative dermatitis
Dermatitis herpetiformis
Acanthosis nigricans
Gardners’s syndrome
Muir-Torre Syndrome
Cowden’s Syndrome
Erythema nodosum
Erythema Multiforme
Amyloidosis
Sweet’s Syndrome
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A. HSV/Mycoplasma
B. Strep. Pharyngitis
C. Psoriasis, Eczema, drug
D. Gluten sensitive enteropathy
E. Obesity-Insulin resist. DM
F. Colonic Polyposis
G. Colorectal/GU CA
(Sebaceous tumors)
• H. Breast & Thyroid CA
(Tricholemmomas)
• I . Multiple Myeloma
• J. AML
References
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1. Longshore S, Tomecki K. The Cleveland clinic disease management project. Skin
signs of systemic disease. 2002;1-8. www.
Clevelandclinicmeded.com/diseasemanagement/dermatology/skinsigns
2. Von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am
Acad Dermatol. 1994;31:535.
3. Poole S. Fenske NA. Cutaneous markers of internal malignancy. I, II J Am Acad
Dermatol. 1993;28:1,147.
4. Fitzpatrick T, Johnson R et al. Color atlas and synposis of Clinical Dermatology 2 nd
edition. 1994.
Habif T. Clinical Dermatology: A color guide to diagnosis and treatment. 3 rd edition.
1996.
Arndt K, Leboit P et al. Cutaneous medicine and surgery: an integrated program in
dermatology. 1996.
WWW. Dermnet.com (digital images)
WWW. Dermatlas.org