Dermatologic Signs

of Systemic Disease
Zharlah Gulmatico-Flores, MD, FPDS
COMMON CUTANEOUS DISORDERS
 Seborrheic dermatitis
• common chronic, superficial inflammatory disease of the
scalp, nasolabial folds, ears and central chest

• characterized as thin, erythematous plaques with fine and

greasy scale.

• it can be associated with Parkinson’s diseases, and HIV

• treatment includes medicated shampoos: zinc pyrithione,

selenium sulfide, salicylic acid, tar or ketconazole
 Seborrheic keratosis
• common benign cutanenous neoplasms

• warty, age-related hyperkeratotic papules and plaques

that appear anywhere on the body, most commonly on
the trunk.

• if appear suddenly in great numbers (sign of Leser-Trelat)

it indicates adenocarcinoma of the GI.

• No treatment is necessary. If symptomatic, can be

removed by curettage or cryotherapy
 Urticaria
• hives, most often caused by medication or food or infection.
These are very pruritic edematous, evanescent wheals that
resolve within 24 hours.

• acute urticaria last less than 6 weeks.

• wheals in fixed location for more than 24 hours suggest the

possibility of urticarial vasculitis and warrants skin biopsy.

• chronic urticaria often requires RAST or skin-prick patch testing

• Treatment includes: antihistamines, oral corticosteroids,

immunosuppressants such as sulfasalazine and cyclosporine
 Erythema multiforme
• cutaneous hypersensitivity reaction, caused by herpes
simplex or Mycoplasma pneumonia and less commonly by
drug sensitivity.

• characterized as papules, plaques, vesicles or bullae often

targetoid or iris appearance occur in the skin often in acral
distribution and with mucosal surfaces.

• self-limited usually resolving within 2 to 4 weeks

• Treatment: suppressive oral antiviral agents such as

acyclovir, valacyclovir or discontinuation of causative
medications and supportive care
 Vitiligo
• charatercized by focal or generalized distribution of
depigmented macules and patches,

• commonly occurs in periorificial areas or at sites of trauma.

• often associated with autoimmune thyroid disease, insulin

dependent diabetes mellitus, pernicious anemia or
addison’s disease.

• treatment: sunscreems, potent topical stroids, topical

calcineurin inhibitors, NBURVB, psoralen or total
depigmentation for extensive disease
 Erythema Nodosum
• most common type of panniculitis

• painful nodules on the shins

• occurs in young women with a peak incidence between

20 and 40.

• most common in pediatric population with streptococcal

pharyngitis.

• Treatment: identification and elimination of cause, bed

rest, NSAIDS.
 Pemphigus vulgaris
• uncommon chronic and debilitationg blistering disease
characterized by painful mucosal erosions and flaccid
blisters that become erosive.

• biopsy reveals suprabasilar acantholysis and

intraepidermal bullae formation

• Treatment: good wound care, systemic corticosteroids,

immunosuppresants, rituximab, IV immunoglobulin and
plasmapharesis.
 Bullous Pemphigoid
• most common bullous disease and is characterized by
large, tense subepidermal blisters.

• occurs in elderly patients ages 65 and 75 years.

• Prognosis: influenced by age and general condition of the

patient.

• Treatment: topical and systemic corticosteroids, steroid-

sparing immunosuppresants and macrolides with
niacinamide.
INTERNAL MALIGNACIES
 Cutaneous Metastasis

• uncommon, would usually originate from lung, large

intestine and kidney.

• are flesh-colored to violaceous nodules that appear in

close proximity to the primary neoplasm.

• treatmentL depends on the primary neoplasm

 Paget’s Disease
• uncommon condition characterized by unilateral
eczematous plaque of the nipple and areaola.

• strongly associated with invasive carcinoma of the breast

• Extramammary paget’s disease typically present as

eczematous plaque of anogenital or axillary regions, often
associated with underlying apocrine carcinoma or
underlying GUT or GIT CA.

• Treatment: surgical excision, radiation therapy, and

photodynamic therapy
 Acanthosis Nigricans
• characterized as smooth, velvet, hyperkeratotic plaques in
intertriginous area.

• Type 1 is associated with malignancy. It is considered as a

marker for adernocarcinoma.

• Type II is familial type.

• Type III is associated with obesity and insulin resistance.

• Treatment: determine the underlying problem. Topical

treatment loike tretinoin, calcipotriol, urea and SSA maybe
helpful
 Cowden’s Syndrome

• autosomal dominant syndrome caused by PTEN gene.

• characterized by multiple tricholemmomas, cancer of the

breast or endometrium, thyroid disease, mental
retardation and fibrocystic disease of the breast.
 Sweet’s Syndrome
• acute febrile neutrophilic dermatosis associated with
leukemia.

• characterized as painful erythematous to violaceous

plaques on the face, extremities and trunk.

• may occur with inflammatory bowel disease, bowel

bypass and pregnancy.

• treatment: systemic corticosteroids and NSAIDS.

 Amyloidosis
• may be a sign of multiple myeloma

• affected patients have papules on the eyelids and

extremities that become purpuric and ecchymotic

• patients may have prominent macroglossia

• primary systemic amyloidosis has poor prognosis

• treatment includes chemotherapy and stem cell

transplantation
Paraneoplastic pemphigus
• characterized by intractable stomatitis and blisters on the
trunk and extremities

• DIF - deposition of IgG intercellularly and at the dermal-

epidermal junction.

• with strong association with Hodgkin’s lymphoma, CLL

and Castleman’s disease with or without mysthenia
gravis.

• Treatment: systemic corticosteroids, steroid sparing

immunosuppressants, rituximab and plasmapheresis.
Erythema Gyratum Repens

• rare but very distinctive skin disease characterized by

reddened concentric bands in whorled or wood grain
pattern

• patients has severe pruritus and peripheral eosinophilia.

• strong association with lung cancer

CARDIOVASCULAR DISEASE
LEOPARD SYNDROME

• multiple lentigenes occur with LEOPARD syndrome

• lentigines, electrocardiographic changes, ocular telorism,

pumonary stenosis, abnormal genitalia, retarded growth,
and deafness.
 CARNEY syndrome

• encompasses LAMB syndrome (lentiginese, atrial myoma,

mucocutanrous myxomas and blue nevi) and NAME
syndrome (nevi, atrial myxoma, myxoid neufibromas and
ehphelids)
Pseudoxanthoma Elasticum

• characterized by yellow papules over redundant skin folds

on the neck, abdomen, and groin.

• skin biopsy reveals swollen, fragmented elastic fibers.

• fundoscopic examination: angoid streaks in Bruch’s

membrane

• mutation n the gene ABCC6 on chromosome 16

Ehlers Danlos Syndrome

• characterized by joint hyperextensibility, hypermobility,

skin and vessel fragility and fish-mouth scars.

• associated with mitral valve prolapse, blue sclerae,

vascular aneursym, aortic dissection, PVD, hernia and
angina.

• Type IV are prone to arterial rupture and have highest

mortality.
 Sarcodoisis
• multisystem granulomatous disease of the lungs, bones,
CNS, lymph nodes, eyes and skin.

• skin diseases include red to purple indurated plaques of

the nose, midfacial papules, annular plaques, and
plaques or nodules on the trunk and extremitis.

• treatment: systemic corticosteroids, intralesional

corticsteroids for localized disease, methotrexate,
thalidomide, antimalarials and tumor necrosis factor
inhibitors
RHEUMATIC DISEASES
 Psoriatic Arthritis

• asymmetric fusiform swelling of the distal interphalangeal

joints associated with oligoarthritis and tenosynovitis

• 50% of affected patients have the HLA-B27 genotype

• treatment: INF- inhibitors, metrotrexate, NSAIDS, and

steroid sparing immunosuppresants
 Lupus Erythematosus
• is an autoimmune photosensitive dermatosis

• characterized by atrophic, scarring plaques on sun-exposed areas

• Subacute CLE is characterized as annular pink to red plaques in a

sun-exposed, shawl-like distribution on the chest, back and arms.
Unlike DLE, there is no scarring.

• Serology is often positive in SCLE antinuclear antibody and

antibodies to Ro/SSA antigen

• Treatment: sun protection, intralesional, topical, and systemic

corticosteroids, antimalarials; dapsone and immunosuppressants
 SCLERODERMA
• localized form which is known as morphea which begins
as erythematous patches that evolve into dusky,
hypopigmented, indurated plaques with violaceous
borders, usually on the trunk.

• CREST syndrome (calcinosis, Raynaud’s phenomenon,

esophageal dysmotility, sclerodactyly, telangectasia)

• Treatment: vasodilating drugs, phototherapy,

methotrexate and cyclophosphamide
 Reactive Arthritis

• Reiter’s syndrome with conjunctivitis, urethritis and

diarrhea

• affected patients, usually men, often have vesicles and

crusted plaques on the penis and erythematous pustules
and papules on the palms and soles that can mimic
pustular psoriasis.

• treatment: topical corticosteroids, cyclosporine, acitretin

 Erythema Chronicum
Migrans
• hallmark of Lyme disease, early infection with the tick-
borne spirochete Borrelia burgdoferi

• develops as red macule or papule at the site of tick bite

and gradually enlarges to an annular, reddened plaque

• late sequelae include meningoencephalitis, myocarditis,

and peripheral neuropathy

• treatment: doxycycline
 Dermatomyositis
• connective tissue disease characterized by symmetric
roximal muscle weakness, photosensitivity, papules and
plaques on the hands, elbows and knees (Gottron’s papules)
and periorbital edema with violaceous hue (heliotrope)

• other features include poikiloderma, periungual

telangiectasias and cuticular hypertrophy.

• Diagnostic criteria: creatine kinase or aldolase level, positive

Jo-1 antibody, elevtromyographic changes.

• treatment: systemic corticosteroids, methotrexate, and other

steroid-sparing immunosuppressants, TNF inhibitors.
GASTROINTESTINAL DISEASE
Dermatitis Herpetiformis

• a chronic intensely pruritic blistering characterized by

symmetric grouped vesicles, papules and wheals on the
elbows, knees, scalp and buttocks.

• biopsy reveals deposition of IgA at the dermal-epidermal

junction

• treatment icludes dapsone, sulfapyridine and gluten-free

diet.
 Acrodermatitis
Enterohepathica

• an inherited or acquired condition characterized by

pustules, bullae, scaling in an acral and periorificial
distribution, and concomitant zinc deficiency

• mutation in SLC39A which encodes an intestinal zinc

transporter.

• treatment is zinc supplementation

 Necrolytic Migratory
Erythema

• rare disease characterized by erythematous scaly plaques

on acral, intertriginous, and periorificial areas, in
association with an islet cell tumor of the pancreas.

• associated signs include hypoglycemia, diarrhea, weight

loss and atrophic glossitis

• treatment is removal of the tumor

 Hepatitis C Virus-
Associated Skin Disorders
• leukocytoclastic vasculitis (cutaneous small vessel vasculitis)
associated with circulating type II cryoglobulins, usually yields
palpable purpura on the lower extremities.

• Treatment of the hepatitis C infection often leads to resolution of

the vasculitis.

• Lichen planus characterized by violaceous, flat, polygonal papules,

often on the flexor aspects of the wrists, trunk, medial thighs,
genitalia, and oral mucosa. Lichen planus also occurs with primary
biliary cirrhosis and hepatitis B virus immunization. Oral erosive
lichen planus is the most common expression of lichen planus in
hepatitis C patients. Treatment includes topical and intralesional
corticosteroids, topical immunomodulators, and phototherapy.
 Hepatitis C Virus-
Associated Skin Disorders

• Lichen planus characterized by violaceous, flat, polygonal

papules, often on the flexor aspects of the wrists, trunk,
medial thighs, genitalia, and oral mucosa.

• Lichen planus also occurs with primary biliary cirrhosis and

hepatitis B virus immunization. Oral erosive lichen planus is
the most common expression of lichen planus in hepatitis C
patients.

• Treatment includes topical and intralesional corticosteroids,

topical immunomodulators, and phototherapy.
 Hepatitis C Virus-
Associated Skin Disorders

• Necrolytic acral erythema, characterized by pruritic

keratotic plaques on the upper and lower extremities, is a
distinctive finding in hepatitis C infection and can
resemble a deficiency dermatosis.
 Muir-Torre Syndrome

• is a disorder characterized by one or more sebaceous

tumors (adenoma, epithelioma, carcinoma) and one or
more internal neoplasms, usually colorectal or
genitourinary, rarely lymphoma

• mutation of the DNA mismatch repair genes, most often

MSH-2.

• treatment is isotretinoin
Peutz-Jeghers Syndrome

• characterized by lentigines on the skin and mucosa and

hamartomas of the stomach, small intestine and colonal
polyps

• 10-18x greater risk of cancer

• treatment includes regular and routine endoscopy and

symptomatic treatment for hypogeusia and diarrhea
Pyoderma Gangrenosum

• neutrophilic dermatosis chracterized by painful ulcers with

boggy, undermined edges and a border of gray or purple
pigmentation

• legs are the most common site

• treatment includes local wound care, systemic and

intralesional corticpsteroids, cyclosporine and infliximab.
RENAL DISEASE
 Nephrogenic Systemic
Fibrosis

• resembles Scelroderma.

• characterized as thick, indurated plaques on the

extremities and trunk.

• treatment: immunosuppresive agents, phototherapy,

topical steroids, retioids, and photopheresis
ENDOCRINE
Diabetes Mellitus