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Dermatology: Lecture 7

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Pruritic/sore white atrophic patches, fissures, & scarring in the genital/perianal region
Most common in post-menopausal women
What is the presentation of Lichen Sclerosus?
Autoimmune
What is the etiology of Lichen Sclerosus?
Diagnosis: Clinical, can confirm with biopsy.
Treatment:
Avoid friction, gentle skin care, barrier cream
Topical steroids, calcineurin inhibitors, retinoids, and estrogen creams
What is the diagnosis and treatment for Lichen Sclerosis?
Lichen Sclerosis
Clustered vesicles & bullae often in an annular or targetoid distribution on the skin and mucous membranes/eyes
"String of beads" and "cluster of jewels" signs
All ages but most common in the elderly
What is the presentation of Linear IGA disease?
Autoimmune
IgA antibodies attack hemidesmosomes and the basement membrane
May be triggered by an infection
or medication
What is the etiology of Linear IGA disease?
Diagnosis: Skin biopsy with direct IF
Treatment:
Dapsone
Stop offending drug if medinduced
What is the diagnosis and treatment of Linear IGA disease?
Linear IGA Disease
Firm, rubbery, round nodules on the trunk or extremities
Usually asymptomatic
Vary in size; may be single or multiple
What is the presentation of Lipomas?
Benign tumor of fat cells; cause unknown
Familial variants
What is the etiology of Lipomas?
Diagnosis: Clinical
Treatment:
1. Cosmetic. Surgical excision.
How do we diagnose and treat lipomas?
Erythema, itching, swelling, and blistering of the skin, esp.
when rubbed/scratched (Darier sign)

Types of Mastocytosis
Solitary mastocytoma (small red/brown plaque)
Urticaria pigmentosa (maculopapular lesions)
Diffuse (generalized erythema, thickened skin)
Telangiectatic (extensive telangiectasias)
Systemic (bone marrow/organ involvement)
What is the presentation of Mastocytosis?
Mast cell proliferation & accumulation in the skin and/or organs
KIT mutation, usually
spontaneous
Various triggers
What is the etiology of Mastocytosis?
Diagnosis:
Darier sign; skin biopsy
High serum tryptase suggests systemic
Treatment:
Avoid triggers
Antihistamines, mast cell stabilizers
Immune/chemotherapy or bone marrow transplant for severe systemic cases
What is the diagnosis and treatment of Mastocytosis?
Mastocytosis
Rash of small red papules
Often in skin folds
AKA "Prickly heat"
itchy, warm, prickly tingling sensation
What is the presentation of miliaria rubra?
Plugging of sweat ducts
What is the etiology of Miliaria Rubra?
Diagnosis: Clinical
Treatment:
Avoid heat, sweating, & friction
Menthol, camphor, calamine, and topical steroids for itch
What is the diagnosis and treatment of Miliaria Rubra?
Miliaria Rubra
Hardened, indurated plaques
White, flesh colored, or red-violet
More common in women
What is the presentation of Morphea?
Localized scleroderma
Cause unknown; runs in families
What is the etiology of Morphea?
Diagnosis: Clinical
Treatment:
Topical steroids or calcineurin inhibitors
Systemic immunomodulators in more extensive cases
What is the diagnosis and treatment of Morphea?
Morphea
Tender, yellow-brown patches w/central atrophy & telangiectasias on the shins of diabetics
Prone to ulceration
What is the presentation of Necrobiosis Lipoidica Diabeticorum?
Unknown
What is the etiology of Necrobiosis Lipoidica Diabeticorum?
Diagnosis: Clinical. Biopsy can confirm.
Treatment:
Mild cases don't require
treatment
Topical steroids under occlusion
What is the diagnosis and treatment of Necrobiosis Lipoidica Diabeticorum?
Necrobiosis Lipoidica Diabeticorum
Excoriations, erosions, and even ulcerations within patients reach
What is the presentation of Neurotic Excoriation?
Compulsive skin picking &
scratching
Psychological disorder
What is the etiology of Neurotic Excoration?
Diagnosis: Clinical
Treatment:
Aimed at the underlying
disorder
May require referral to
Psych
What is the diagnosis and treatment of Neurotic Excoriation?
Neurotic Excoriation
Brown macules or papules. Can be congenital or new up to 40.
What is the presentation of Nevi?
Benign proliferation of melanocytes
Dysplastic nevi (+ABCDs) run in families and are at risk of developing
into melanoma
What is the etiology of nevi?
Diagnosis: Clinical. Dysplastic Nevi should be closely monitored or biopsied.
Treatment:
1. Moderate-severely atypical dysplastic nevi are surgically excised.
What is the diagnosis and treatment of Nevi?
Thickened, firm, tender, erythematous or hyperpigmented nodular plaques
Can occur alongside vasculitis
What is the presentation of Panniculitis?
Inflammation of Subcutaneous fat. Variety of causes like erythema nodosum or cutaneous lupus.
What is the etiology of Panniculitis?
Diagnosis: Biopsy
Treatment:
Treat the underlying cause
Rest, elevation,
compression
What is the diagnosis and treatment of Panniculitis?
Panniculitis
Painful, thin-walled, flaccid bullae and erosions on the skin and mucous membranes
What is the presentation of Pemphigus Vulgaris?
Autoimmune disease.

Autoantibodies attack the desmosomes (proteins that connect keratinocytes)
What is the etiology of Pemphigus Vulgaris?
Diagnosis: +Nikolsky sign. Skin biopsy with direct IF.
Treatment:
1. Oral steroids and other systemic immunosuppressants.
What is the diagnosis and treatment of Pemphigus Vulgaris?
Pemphigus Vulgaris
Irritation, erythema, cracking, and fissuring of the corners of the lips.
What is the presentation of Angular Cheilitis?
Irritants/allergens (contact dermatitis)
Staph aureus or Candida
Nutritional deficiencies (B2, iron)
What is the etiology of Angular Cheilitis?
Diagnosis: Clinical. Positive/negative culture or KOH prep.
Treatment:
Ointment; topical steroid,
antifungal, or antibiotic
B complex vitamin, iron
What is the diagnosis and treatment of Angular Cheilitis?
Angular Cheilitis
Fragile skin, bullae, and erosions, on dorsal hands/forearms/feet
What is the presentation of Porphyria Cutanea Tarda?
Increased porphyrins -> Photosensitivity
Type 1: Acquired
Type 2: Genetic
What is the etiology of Porphyria Cutanea Tarda?
Diagnosis:
Skin biopsy
Tea colored urine, which fluoresces coral pink under Wood's lamp
Elevated 24-hour urine porphyrins
Treatment:
Avoid causative agents
Opaque sun block
Phlebotomy
What is the diagnosis and treatment of Porphyria Cutanea Tarda?
Porphyria Cutanea Tarda
Intensely pruritic nodules on the
extremities & trunk with 2°
excoriations, crust, scale
What is the presentation of Prurigo Nodularis?
Unknown; likely exacerbated by chronic scratching
Can be associated with systemic disease (i.e. liver, kidney, autoimmune, HIV)
What is the etiology of Prurigo Nodularis?
Diagnosis:
Clinical
Screen for systemic disease
Treatment:
Emollients, steroids, antihistamines, capsaicin or menthol creams
Light therapy
What is the diagnosis and treatment for Prurigo Nodularis?
Prurigo Nodularis
Inflamed, follicular papules in shaved areas.
What is the presentation of pseudofolliculitis Barbae?
Foreign body inflammatory reaction to ingrown hairs as a result of shaving
Risk factors: African American, curly hair
What is the etiology of psuedofolliculitis Barbae?
Diagnosis: Clinical
Treatment:
Shave less frequently (and in the direction of the follicle), or use electric clippers
Hydrocortisone cream, tretinoin, benzoyl peroxide
What is the diagnosis and treatment of pseudofolliculitis barbae?
Pseudofolliculitis Barbae
Small yellow papules in linear or reticular distribution -> coalesce into "cobblestone" plaques
Soft, saggy, wrinkled skin
What is the presentation of Pseudoxanthoma Elasticum?
Rare genetic disorder resulting in abnormal elastic tissue in the skin, blood vessels, and eyes
What is the etiology of pseudoxanthoma elasticum?
Skin findings -> cosmetic (laser, plastic surgery)

Prevent and treat eye, blood vessel, and cardiac complications
What is the diagnosis and treatment of pseudoxanthoma elasticum?
Pseudoxanthoma Elasticum
Enlarging, painful ulcerations with purple border -> cribriform scarring.

it occurs at site of trauma.
What is the presentation of pyoderma grangrenosum?
Auto-inflammatory, related to neutrophil dysfunction
A/w systemic diseases such as IBD, leukemia, RA
What is the etiology of pyoderma grangrenosum?
Diagnosis: Clinical
Treatment:
Procedures make it worse!
Topical/IL steroids, calcineurin inhibitors, oral antibiotics, oral steroids, systemic immunomodulators
What is the diagnosis and treatment of pyoderma grangrenosum?
Pyoderma Gangrenosum
Macular -> papular -> petechial rash
Spreads from ankles/wrists -> palms/soles -> limbs -> trunk
Fever, headache, myalgias
What is the presentation of rocky mountain spotted fever?
Rickettsia rickettsii infection, transmitted by ticks
What is the etiology of rocky mountain spotted fever?
Diagnosis: Blood serology
Treatment:
Early treatment is critical3-5% mortality rate
Doxycycline, chloramphenicol
What is the diagnosis and treatment of rocky mountain spotted fever?
Rocky Mountain Spotted Fever
Thickened, tightly-bound skin, sclerodactyly, Raynaud's, telangiectasias, calcinosis, nail changes, ulcers, hypo/hyperpigmented patches
What is the presentation of scleroderma?
Autoimmune dz causing fibrosis of skin, blood vessels, organs
What is the etiology of Scleroderma?
Diagnosis: ANA, anti-centromere, Scl-70 antibodies
Treatment:
Symptom support, systemic immuno-modulators.
How do we diagnose scleroderma?
Scleroderma
True. 98% are harmless
T/F: Spider bites are almost always harmless
"Bulls Eye" blister. it is a large necrotic ulcer.
What does a brown recluse bite look like?
Brown recluse spider bite
Flu-like prodrome -> painful rash spreading from the trunk to the limbs, face, and mucous membranes
Macules, targetoid lesions, purpura, flaccid blisters -> widespread skin sloughing (SJS <10%, TEN >30%)
Complications: dehydration, malnutrition, electrolytes, infection, ARDS, shock, multisystem organ failure, DIC, death
Tell me about the presentation and complications of Stevens Johnson Syndrome (Toxic Epidermal Necrolysis)
Drug reaction; unknown mechanism
Typically occurs within days to a month
>200 drugs implicated, but antibiotics and seizure meds are most common
What is the etiology of Stevens Johnson Syndrome (Toxic Epidermal Necrolysis)?
Diagnosis:
Skin biopsy
Monitor labs for complications
Treatment:
Patient must be hospitalized, preferably in the burn ICU
Stop offending drug
Manage pain, fluids, electrolytes, temperature
Wound care, treat infections
IV steroids (controversial), IVIG, plasmapheresis, systemic immunomodulators
How do we diagnose and treat Stevens Johnson Syndrome?
Acute onset of fever & tender red
papules, plaques, or nodules
Eye inflammation, oral erosions
What is the presentation of sweets syndrome?
Reactive; associated with an underlying systemic disease, i.e. malignancy, RA, IBD
What is the etiology of sweets syndrome?
Diagnosis:
Skin biopsy ->
neutrophilic infiltrate
Elevated WBC, ESR
Treatment:
Systemic steroids
How do we diagnose and treat Sweets Syndrome?
sweets syndrome
Presentation
1-3 mm flesh-colored or yellow firm papule(s)
Eyelids > cheeks > axillae, genitals
What is the presentation of Syringoma?
Benign sweat duct tumors
What is the etiology of Syringoma?
Diagnosis: Clinical or biopsy to rule out BCC or xanthalasma
Treatment:
1. Cosmetic
2. Laser or electrosurgery
What is the diagnosis and treatment of Syringoma?
Syringoma
Red or purple dilated blood
vessels visible on the skin or
mucous membranes
What is the presentation of Telangiectasia?
Variety of causes.

Rosacea, estrogen, alcoholism,
hereditary telangiectasia
syndromes, scleroderma, etc
What is the etiology of Telangiectasia?
Diagnosis: Clinical, further workup depending on underlying cause
Treatment:
Treat underlying issue
Otherwise, considered cosmetic
(laser)
What is the diagnosis and treatment for Telangiectasia?
Telangiectasia
Diffuse, profound non-scarring alopecia
Nails may have Beau lines
What is the presentation of Telogen Effluvium?
Sudden stress on the body (Illness, surgery, childbirth, etc.)
Up to 70% anagen hair -> telogen phase
Shedding starts ~2 months later
What is the etiology of Telogen Effluvium?
Diagnosis:
Clinical
Hair pluck test
Rule out other causes
Treatment:
Self limited
Gentle handling of hair
Adequate nutrition
What is the diagnosis and treatment of Telogen Effluvium?
Irregular patches of hair loss, broken hair shafts. Usually starts in late childhood.
What is the presentation for Trichotillomania?
Psychiatric disorder resulting from compulsive hair pulling.

Often occurs alongside OCD and anxiety disorders.
What is the etiology of Trichotillomania?
Diagnosis: Clinical and ruling out other causes
Treatment:
- Psychosocial support and psychiatry
What is the diagnosis and treatment of Trichotillomania?
Hamartomas in the skin, brain, eye, kidney and/or heart. Seizures can also cause developmental delay. Skin findings are present in 70% of cases. There are 4 categories of these findings.

Angiofibromas: Small reddish papules in butterfly distribution on face
Ungual Fibromas: Firm, flesh colored papules arising from nail folds
Shagreen Patch: "pebbly", orange peel-like plaque, usually on lower back
Ash Leaf Marks: Leaf-shaped white macules or patches
What is the presentation of Tuberous Sclerosis?
Tuberous Sclerosis
Genetic mutation in TSC
AD inheritance; 2/3 sporadic
What is the etiology of tuberous Sclerosis?
Diagnosis: Clinical, imaging for internal dz
Treatment:
- Multidisciplinary
- Considered cosmetic for skin findings.
How do we diagnose and treat Tuberous Sclerosis?
Relatively painless ulceration
Shins & medial malleoli most common sites
Often a/w edema, stasis dermatitis, lipodermatosclerosis, varicose veins
What is the presentation of venous leg ulcers?
Inefficiency of venous valves in the legs
Most common cause of lower leg ulcers
What is the etiology of venous leg ulcers?
Diagnosis: Clinical, venous ultrasound
Treatment:
Wound care
Compression and elevation
Adequate nutrition
Consider referral to vascular surgery
What is the diagnosis and treatment of venous leg ulcers?
Venous Leg Ulcers
Venous Leg Ulcers
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