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
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.