dermatologic manifestations of chronic disease shelbi hayes. m.d. saints dermatology october, 26...

Dermatologic Manifestations of Chronic Disease

Shelbi Hayes. M.D.

Saints Dermatology

October, 26 2012

I. Creating a Framework for Evaluating Skin Lesions

II. Application of the framework to the most common manifestations

of chronic disease

I have no financial disclosures.

Creating a Framework

Question #1

Is this a primary or secondary lesion?

Macule Patch

Papule Plaque

Pustule Nodule

Pustule Nodule

Vesicle Bulla

Pustule

Vesicle

Bulla

Wheals

Wheals

Morphologic categories

Macular-Patch

Papular

Papulosquamous (scaly papules)

Nodular

Pustular

Vesicular-bullous

Urticarial

Petechial

Telangiectasia

Burrow

Poikiloderma

Hyperkeratotic/scale

Atrophic

Secondary Lesions

Crust

Erosions and ulcers

Excoriations

Fissures

Scars

Lichenification

Atrophy

Creating a Framework

Question #2

Is there scale?

Scale or No Scale?

Scale indicates the disease process involves the epidermis.

Lack of scale indicated the disease process affects the dermis or subcutaneous fat.

Exception: Tinea Incognito, Early Vesiculobullous Lesions

Creating a Framework

Question #3

What is the configuration?

Configuration

Annular

Arcuate

Geographic

Discrete

Confluent

Serpiginous

Linear

Reticulated

Creating a Framework

Question #4

What is the color?

Color

PinkVioletOrangeBlueGreenYellowBlackBrown

Color

Color

Pink—Pityriasis roseaViolet—Lichen planusOrange—Juvenile xanthogranulomaBlue—Amiodarone skin pigmentationGreen—PseudomonasYellow—XanthomasBlack—EscharBrown—Café au lait spots

Creating a Framework

Question #5

What is the distribution?

Immunosuppression

Herpes Simplex

Herpes Simplex

Caused by HSV-1 and HSV-2Infections occurs at the primary site, transported via neurons to dorsal root ganglion where latency is establishedPain, tenderness or tingling occur often before reactivation.Grouped vesicles on erythematous base, however you may not see the primary lesion when the patient presents!!

Herpes Simplex VirusEczema Herpeticum

Herpes Simplex VirusEczema Herpeticum

Herpes Zoster

EM-SJS-TEN

Spectrum of epidermal damage +/- mucosal involvementEM minor = no mucous membraneEM in kids usually secondary to HSV, drugs in adultsSJS-TEN constitute one of the few derm emergenciesTreat in burn unit, frozen section of bx to check for necrosis, little inflammationFluids, infection prophylaxis, consult ophtho and uro as indicated

Erythema Multiforme

Erythema Multiforme Major

Also thought to be a hypersensitivity reactionAs with EM minor, but with involvement of ≥2 mucosal surfaces (precedes rash by 1-2 days)Pronounced constitutional symptoms common

Stevens-Johnson Syndrome

Is SJS separate entity from EM major?

Some feel SJS is a distinct entity as the rash is more erythematous and less acral than EM major

EM major is more commonly triggered by infections and SJS by drugs.

Stevens-Johnson Syndrome

Stevens-Johnson Syndrome

Stevens-Johnson Syndrome

Toxic Epidermal Necrolysis

Nikolski’s Sign = separation of the epidermis from the dermis by rubbing skin between the lesions

Toxic Epidermal Necrolysis (TEN)

A life-threatening, exfoliating disease of the skin and mucous membranes

Hallmark is full-thickness necrosis of the epidermis with separation at the dermoepidermal junction.

SJS vs TEN

Some use %BSA to define with:

<10% = SJS

>30% = TEN

Histologically SJS has a much higher density cell infiltrate (T-lymphocytes) vs TEN (low density macrophages and dendrocytes)

TEN - Pathogenesis

Majority of cases are likely adverse drug reactions (foreign antigen response).

Mean time from drug to onset = 13.6 days

Higher risk drugs– NSAIDS [38%]– Antibiotics [36%] (sulfonamides)– Anticonvulsants [24%] (phenobarb, lamotrigene)– Corticosteroids [14%]

Use Trimethoprim-Sulfamethoxazole Judiciously.

Up to 17% of patients can have an adverse cutaneous reaction.

Occurs within the first 3 weeks.

Warn Patients to alert you immediately.

Do not prescribe if the patient has a family history of sulfa allergy.

TEN - Clinical Features

Initial symptoms (1-3 days)– Fever (100%)– Conjuctivitis (32%)– Pharyngitis (25%)– Pruritis (28%)– Headache, myalgias, arthralgias, vomiting,

and diarrhea may occur

TEN - Clinical Features: Mucosal Involvement

Erosive mucosal lesions (1-3 days before skin eruption) occur in 97%– Oral (93%)– Ocular (78%)– Genital (63%)– Anal

TEN - Clinical Features:Skin Eruption

Burning / painful skin rash

Usually begins on face / upper trunk

Begins as one of:– Diffuse erythema– Irregular bullae– Poorly defined dusky or erythematous

macules

Scalp usually spared

Multisystem Involvement

GI - Mucosal sloughing in esophagus (dysphagia, GI bleeding)

Resp - Tracheal/bronchial erosions(Respiratory decompensation)

Renal – Glomerulonephritis

Profound fluid and electrolyte disturbances

Dermatophytes

Named for area involved: tinea capitis, corporis, manum, facei, pedis, cruris, etc.

If there is scale, do KOH exam.

Words of a famous dermatologist:

“If it is scaly, SCRAPE it!”

Tinea Pedis

Tinea Cruris-Don’t use steroids!

Tinea Incognito

Scabies

Scabies

Caused by Sarcoptes scabiei

Pregnant female mite burrows in the stratum corneum, lays eggs about 2-3 per day. Eggs hatch after about a week.

See burrows, papules, vesicles.

In immunocompromised and elderly, can be crusted and hyperkeratotic (Norwegian also called Crusted Scabies).

Scabies

Scabies love babies!

Scabies love warm, occluded places:– axilla, webspaces, groin, head of

penis

Distribution

Pruritic, erythematous papules on the head of the penis=scabies until proven otherwise.

Scabies burrow

Crusted Scabies

Verruca Vulgaris

Verruca Vulgaris

Liquid Nitrogen

Candida Antigen

IL Bleomycin

Curretage and cautery

Condyloma

Treatment for CA

Avoid liquid nitrogen

Apply Podophyllin in the office and Rx imiquimod at home.

S, Pniewski T, Malejczyk M, Jablonska S. Imiquimod is highly effective for extensive, hyperproliferative condyloma in children.Pediatr Dermatol. 2003 Sep-Oct;20(5):440-2. Sharquie KE, Al-Waiz MM, Al-Nuaimy AA. Condylomata acuminata in infants and young children. Topical podophyllin an effective therapy.

Notify CPS?

CPS should be notified of concerns of possible sexual abuse when ano-genital warts are diagnosed in any child older than 3 years.

It also is important for CPS to be educated by the reporting medical provider of other possible nonsexual modes of transmission for the ano-genital warts.

Hornor G.Ano-genital warts in children: Sexual abuse or not?

J Pediatr Health Care. 2004 Jul-Aug;18(4):165-70.

Notify CPS?

For children younger than 3 years, CPS should be notified if other risk factors are noted during assessment, such as an abnormal genital examination, the presence of another sexually transmitted disease, or psychosocial information that warrants investigationHornor G.Ano-genital warts in children: Sexual abuse or not? J Pediatr Health Care. 2004 Jul-Aug;18(4):165-70.

RecommendationsChild 2 years or younger – No report to child protective services needed unless one of the following

is present: Abnormality noted on ano-genital examination that is of concern for sexual abuseAnother sexually transmitted diseasePsychosocial/behavioral issue that is of concern for sexual abuseParental concern of sexual abuse that warrants investigation

Child 3 years or older – Report concerns of possible sexual abuse to child protective services– Nonleading interview of child regarding sexual abuse concerns (should

be completed by a trained forensic interviewer)

Hornor G.Ano-genital warts in children: Sexual abuse or not?J Pediatr Health Care. 2004 Jul-Aug;18(4):165-70. Review.

Molluscum Contagiosum

Molluscum Contagiosum

Caused by pox virusCharacteristic umbillicated papules, molluscum bodies on biopsyMay be an STD in adults – suprapubic and genital lesionsGiant molluscum in AIDS pts, ddx in this pop. includes crypto and other fungal infectionsTx includes cryo, curettage, cantharidin, imiquimod or nothing – they will spontaneously resolve

Auto-Immunity

Lupus

ACLE

Lupus

SCLE

Lupus DLE

Lupus

Must evaluate all forms of cutaneous lupus for systemic lupus– ANA, anti-ds DNA, anti-Ro (especially with

SCLE), complement levels, UA

Review current medications

Treatment is a combination of system steroids and steroid sparing agents (especially Plaquenil), mild cases may be treated with only topical steroids

Dermatomyositis

Dermatomyositis

Scalp involvement is relatively common and manifests as an erythematous to violaceous, scaly dermatitis.

Clinical distinction from seborrheic dermatitis or psoriasis is occasionally difficult.

Nonscarring alopecia may occur and often follows a flare of systemic disease.

Dermatomyositis

Heliotrope rash Gottron papulesMalar erythemaPoikiloderma in a photosensitive distributionViolaceous erythema on the extensor surfaces,Periungual and cuticular changes

Dermatomyositis

In 40% of patients, the skin disease may be the sole manifestation at the onset. Muscle disease may occur concurrently, precede, or follow the skin disease by weeks to years.

The disease is often intensely pruritic.

Systemic manifestations may occur.

ROS: arthralgias, arthritis, dyspnea, dysphagia, arrhythmias, and dysphonia.

Dermatomyositis

Malignancy is possible in any patient with DM, but it is much more common in adults older than 60 years. All adults must be screened.

Children with DM may have an insidious onset that hides the true diagnosis until the dermatologic disease is clearly observed

Vasculitis

Vasculitis

Characterized by size of vessel.

Most common cutaneous disease involves small vessels, i.e. leukocytoclastic vasculitis (“Palpable Purpura”).

Medium sized vessel disease includes PAN, Wegeners, and Churg-Strauss.

Vasculitis

Acronym for DDx of LCV: Mt Sinai Hospital Center

Meds/Malig Strep/Serum sickness Henoch Schonlein/HCV Connective tissue disease/Cryoglobulinemia

HSP usually <10 y.o. but can be adults, subsequent to URI. IgA around blood vessels Watch renal function.

Vasculitis Treatment

1. Identify and eliminate underlying cause.

2. If arthralgias present consider starting NSAIDS.

3. Colchicine, dapsone, and immunosuppressive agents may be used if vasculitis is chronic.

Fluid Overload

Stasis Dermatitis

Stasis Dermatitis

Stasis Dermatitis

Typically affects middle-aged and elderly patients.

Occurs on the lower extremities in patients with chronic venous insufficiency and venous hypertension.

Prevalence is 6-7% in patients older than 50.

This finding makes stasis dermatitis twice as prevalent as psoriasis and only slightly less prevalent than seborrheic dermatitis.

Stasis Dermatitis

Insidious onset of pruritus affecting one or both lower extremities.Reddish-brown skin discoloration is an early sign and may precede the onset of symptoms.The medial ankle is most frequently involved, with symptoms progressing to involve the foot and/or the calf.H.O. dependent leg edemaH.O. factors that worsen peripheral edema (CHF, HTN with diastolic dysfunction)

Stasis Dermatitis

Treatment is two-fold:– Relief of symptoms – Treatment of underlying venous insufficiency

For pruritus and eczematous component:

Class IV or V topical corticosteroids and emollients (AVOID NEOMYCIN)

Daily use of support stockings

Id Reaction

Autosensitzation dermatitis

Most often pts with stasis and contact dermatitis

Follows primary lesions by days to weeks

Treatment includes treatment of inciting event, topical and IM steroid

Pruritus

Extremely common in patients with chronic renal failure

Much more common in patients on renal dialysis vs peritoneal dialysis

Independent marker for mortality for patients of hemodialysis

Pruritus

Antihistamines of some help– Doxepin

Topical capsaicin cream or Sarna lotion

Efficient hemodialysis

UVB

Diabetes

Eruptive Xanthomas

Patients with poorly controlled glucose and elevated triglycerides

Resolution with tight glucose control

Necrobiosis Lipoidica Diabeticorum

0.03% of patients with diabetes

Resolution or progression is not related to glucose control

Very difficult to treat–Topical or IL steroid–Topical tacrolimus–Surgical excision (often recur)

Acanthosis Nigricans

Associated with obesity and insulin resistance

Improved with weight loss and glucose control

Treatment includes topical retinoids and salicylic acid

Diabetic Bullae

Appears on background of normal skin

Resolves spontaneously

Culture fluid for secondary infection of it appears cloudy

Diabetic Dermopathy

Patients with poorly controlled diabetes

Correlates with vacsular damage secondary to diabetes

No treatment needed– thought to improve with improved

glucose control

“More is missed by not looking than by not knowing”

M. McKay, M.D.