CMS Final: Derm E2

which type of urticaria is IgE dependent?

acute (<6 weeks)

which type of urticaria results from stroking/scratching skin?

dermographism

which condition is a malar rash/butterfly rash associated with?

cutaneous lupus erythematosus

how long does a butterfly rash last and what makes it worse?

lasts hours-weeks;
exacerbated by sun exposure

does a butterfly rash include the nasolabial folds?

no they are spared

what vascular manifestations are seen in cutaneous lupus erythematosus?

periungal erythema
livedo reticularis
raynauds
vasculitis

what sign/sx is seen in ACUTE cutaneous lupus?

butterfly rash

what sign/sx is seen in SUBACUTE cutaneous lupus?

small, erythematous, slightly scaly papules that evolve into psoriasiform plaques or annular plaques→seen on shoulders, forearms, neck and upper torso

what is the most common type of chronic cutaneous lupus?

discoid

what skin manifestation is seen in chronic cutaneous lupus?

discrete, erythematous, indurated plaques covered by a well-formed adherent scale that extends into dilated hair follicles → healed plaques leave depressed central scars, atrophy, telangiectasia, and hyper/hypopigmentation

which form of lupus presents as painful indurated plaques or firm subcutaneous nodules?

lupus panniculitis (profundus)
→ upon resolution they are left with subcutaneous atrophy and scarring

what is the first line of treatment for cutaneous lupus?

sunscreen + topical/intralesional/oral corticosteroids

what treatment is used for refractory lupus?

antimalarials (-quin)

what is a hallmark of scleroderma?

widespread vascular dysfunction and progressive fibrosis of skin and internal organs

what are some clinical features of scleroderma?

edema, skin thickening/hardening, Raynaud's, painful ulcerations at DIP and PIP, sclerodactyly

what is the first line of treatment for scleroderma?

methotrexate

scleroderma, lupus, sjrogans, dermatomyositis, polymyocitis, hypothyroidism

rule out raynauds?? idk what she said about these dx in class

what is a big risk factor for raynaud's?

smoking

what are some scleroderma associated syndromes?

CREST!

C = calcinosis cutis
R = raynaud's
E = esophageal dysfunction
S = scerlodactyly
T = telangiectasia

which subtype of raynaud's is due to underlying disease?

secondary

what is defined as an exaggerated vascular response to cold temperature or emotional distress?

Raynaud's phenomenon

what is the first line treatment for Raynaud's?

CCB (amlodipine or nifedipine)

2nd line = PDE5i

what is the first line of treament for dermatomyositis?

hydroxychloroquine
2nd line = add methotrexate
for pruritis → topical steroids/calcineurin inhibitors

how does dermatomyositis commonly present?

progressive, symmetric, proximal muscle weakness and cutaneous findings

what cutaneous findings are associated with dermatomyositis?

Gottron's papules (fingers), Heliotrope eruption (eyelids), Shawl sign (back)

How is Behcet's diagnosed?

Recurrent Oral Ulceraction + 2 of following:
-recurrent apthae-like genital ulcers
-uveitis
-skin lesions
-+pathergy test

what is the first line of treatment for Behcet's?

topical glucocorticoids for aphthous ulcers

systemic → prednisone

what severe complication is associated with Behcet's?

ocular disease → uveitis → blindness

which subtype of angioedema affects face/extremities, laryngeal edema, angioedema of bowel wall?

hereditary angioedema

what is a pathognomonic finding for IgA vaculitis?

leukocytoclastic vasculitis in postcapillary venules with IgA deposition on bx

what are risk factors for IgA vaculitis?

age 4-7 &
S. pyogenes infection

which condition is defined as "systemic necrotizing vasculitis that typically affects medium-sized muscular arteries with additional involvement of small arteries"?

polyarteritis nodosa

what is a hallmark feature of neurofibromatosis?

cafe au lait spots

which type of NF is most associated with complication of acoustic neuroma?

NF2 → associated with Schwannomatosis

which condition is associated with cutaneous and mucosal erythema and edema with subsequent desquamation?

Kawasaki disease

does Kawasaki disease include the palms and soles?

yes → erythema

how is Kawasaki disease diagnosed?

presence of fever last 5+ days with no explanation PLUS 4/5 of the following:
1. bilateral bulbar conjunctival injection
2. oral mucous membrane changes (strawb tongue)
3. peripheral extremity changes
4. polymorphous rash
5. cervical LAD

what is the treatment for Kawasaki disease?

IVIG and aspirin

what is a complication of Kawasaki disease?

coronary artery aneurysm (was on exam)

which subtype of sarcoidosis is associated with diffuse, violaceous, soft doughy infiltrations on the nose, cheeks or earlobes?

lupus pernio

which subtype of sarcoidosis is associated with papules and translucent yellow/red with apple jelly appearance on diascopy?

papular

what is seen on diascopy in sarcoidosis?

"apple jelly" semitranslucent yellowish/brown color

what is a hallmark of sarcoidosis?

noncaseating granulomas

what is the triad associated with granulomatosis with polyangiitis (GPA) (Wegners)?

1. necrotizing granuloma in upper resp tract and lungs
2. vasculitis involving both arteries and veins
3. glomerulitis

which condition shows necrotizing vasculitis of small arteries/veins with intra or extravascular granuloma formation on histo?

GPA

which drug is used for prevention of opportunistic infections in GPA?

bactrim

what is the treatment for induction of GPA?

cyclophosphamide or Rituximab PLUS prednisone

what is the treatment for maintenance of GPA?

methotrexate or Rituximab or azathioprine

which autoimmune condition is characterized by chronic inflammatory subepidermal blistering?

bullous pemphigoid

what is seen in the prodromal phase of bullous pemphigoid?

weeks/months of pruritic eczematous papular or urticaria-like skin lesions

what is the initial therapy for bullous pemphigoid?

high potency topical steroid (clobetasol) OR oral glucocorticoids OR doxy

which diagnosis should be suspected in a patient >60 y/o with blistering skin disease, desquamative gingivitis/mucositis, and unexplained pruritic urticarial plaques?

bullous pemphigoid

which disease has a prodrome of fever, fatigue, malaise, arthralgias that may precede nodules 1-3 weeks?

erythema nodosum

Where is erythema nodosum most commonly found?

shins

where is a neurofibroma most commonly found?

trunk

what is the diagnostic criteria for neurofibromatosis?

2 of the following:
- > 6 cafe au lait lesions >5mm prepuberty or 15> mm postpuberty
- multiple freckles in axillary+inguinal areas
- >2 neurofibromas or one plexiform neurofibroma
- bone legions
- bilateral optic nerve glioma
- >2 Lisch nodules
- 1st degree relative with NF1

what drug is used to treat pruritis of neurofibromas?

gabapentin

which inherited disorder is characterized by benign hamartomas of the brain, eyes, heart, lungs, liver, kidneys, and skin?

tuberous sclerosis

which blood test should always be checked in patients with xanthomas?

lipid panel

what is the most common xanthoma?

Xanthelasma

what is the cause of scurvy?

Vitamin C deficiency

what are common clinical features of scurvy?

petechiae, extensive ecchymoses, bleeding gums, loss of teeth, hemorrhage into long bones and intracerebral hemorrhage (Severe)

what disease of zinc deficiency is seen in infants?

Acrodermatitis Enteropathica → d/t genetic disorder of zinc absorption

what is the triad of Acrodermatitis Enteropathica?

1. erythematous, dry, scaly plaques of face/anogenital area
2. alopecia
3. diarrhea

which disease is caused by Niacin deficiency?

Pellagra

what is a chemically induced skin irritation that is exacerbated by direct sunlight?

phototoxicity

what is an abnormal cutaneous response that occurs after exposure to UV or visible light?

photosensitivity

how long does UVA damage take to develop?

4-16 hours, fades in 48-72 hrs

how long does UVB damage take to develop?

6-24 hours, fades in 72-120 hrs (more harmful)

what is the criteria to refer to burn center?

- suspected inhalation injury
- moderate-severe burns
- circumferential partial or full thickness burns
- burns to hands, feet, face, and perineum
- chemical burns
- carbon monoxide poisoning
- inadequate family support of abuse
- severe co-morbidities (COPD, CAD, DM, renal insuff)

what is a delayed-type hypersensitivity reaction to an allergen whose antigenicity changes after exposure to UV?

photoallergic

what is chronic photodamage also known as?

Dermatoheliosis (photoaging)

what is characterized by wrinkled, leathery, premature aged, cigarette-paper skin?

dermatoheliosis

what is characterized by circumscribed flat, brown macules with uniform color in a scattered distribution?

solar lentigo

what deadly condition does solar lentigo mimic?

melanoma (solar lentigo is UNIFORM in color)

what is appears as an exaggerated sunburn secondary to phototoxic agent that has been applied/ingested?

phototoxic dermatitis

which fitzpatrick type is most affected by phototoxic dermatitis?

ALL

what is the most common idiopathic photodermatosis?

polymorphous light eruption (PMLE)

what is the cause of PMLE?

genetic susceptibility or delayed-type hypersensitivity

what is skin hardening associated with?

PMLE → treated with PUVA

what type of hypersensitivity is solar urticaria?

immediate type 1 → sunlight induced wheals

what is the etiology of porphyria cutanea tarda?

deficiency of uroporphyrinogen decarboxylase enzyme (UROD)

which disease is characterized by fragile skin, superficial erosions, chronic scarring and alopecia?

porphyria cutanea tarda (vampire disease) (liver skin condition)

which condition shows pink fluorescence under a woods lamp?

porphyria cutanea tarda (vampire disease)****

How are burns classified?

classified according to their depth

which degree of burn involves destruction of the epidermis and varying depths of dermis?

2nd → partial thickness

which degree of burn involves only the epidermis?

1st → superficial

which degree of burn involves destruction of epidermis, dermis, and subq tissue?

3rd → full thickness

which degree of burn involves subq tissue, muscle, fascia, and bone?

4th → full thickness and can involve muscle and/or bone

which degree of burns can destroy nerve endings?

3rd and 4th

what is the Lund-Browder Chart?

A detailed version of the rule of nines chart that takes into consideration the changes in body surface area brought on by growth. → most accurate

What is the rule of nines used for?

divides the body into 11 sections each accounting for 9%; mainly used to determine the amount of fluids lost in a burn → fastest

what is the treatment for superficial burns?

topical mupirocin or bacitracin

what is the treatment for partial thickness burns?

silvadene

which type of burn appears waxy, white, and leathery gray?

3rd degree

what burn injuries are most common in young children?

scald injuries → evaluate for child abuse

what is the leading cause of death with burns?

systemic infection

Burn Center Referral Criteria

on exam!

which skin condition starts with a "Herald patch"?

pityriasis Rosea → single round or oval, sharply demarcated lesion on chest, neck or back

which condition is characterized by pink/hyperpigmented, scaly, oval, papulosquamous lesions?

pityriasis rosea