Systemic derm Diseases Flashcards
What is dermatomyositis?
-Rare inflammatory muscle and skin disease, causing proximal muscle weakness and a skin rash
Which gender and in what ages is dermatomyositis more common in?
Women
5-10 years and over 40
Adult dermatomyositis is associated with what?
Malignancy and collagen vascular disease
What is the heliotrope sign in dermatomyositis?
Red-purple edematous erythema on the upper palpebral
What is Gottron’s sign found in dermatomyositis?
Red-purple, keratotic and atrophic erythema / macules on the extensor surface of finger joints
Where is the erythema usually seen with dermatomyositis?
Extensor surfaces of extremity joints, and slight raised red-purple erythema over the elbows or knees
What are the labs that can be elevated with dermatomyositis?
Creatinine kinase
What are the antibodies that can be found with dermatomyositis?
Anti-jo-1
Is the rash seen with dermatomyositis pruritic?
Yes–can awaken from sleep
What happens to the hair with dermatomyositis?
Localized Alopecia
What can happen to the lesions with dermatomyositis?
Calcinosis
What are the systemic s/sx of dermatomyositis?
Fever
Malaise
Weight loss
What are the GI findings of dermatomyositis? (3)
- Esophageal impairment / dysphagia
- GERD
- Ulcers and infections
What are the cardiac findings of dermatomyositis? (2)
AV defects
Tachyarrhythmias
What is the gait that is characteristic of dermatomyositis?
Tip-toe gait
What is the treatment for dermatomyositis?
- Steroids
- Immunosuppressants (MTX)
- Avoid sun + sunscreen
What is the prognosis for dermatomyositis?
Gradual disability in most, but 20% have spontaneous remission
True or false: there is an increased risk of malignancy in dermatomyositis
True
What are the psych / neuro s/sx of SLE?
Psychosis
Szs
What are the lung / CV findings of SLE?
Pleuritis and pericarditis
What is the natural h/o SLE?
Flares and remissions
Who is SLE more common in?
Black women of child -bearing ages
What is the discoid rash with SLE?
Erythematous, raised patches with adherent keratotic scaling and follicular plugging
What is the malar rash seen with SLE?
Fixed erythema (flat or raised) over the malar eminences
What are the oral findings of SLE?
Oral or nasopharyngeal ulceration that are usually painless
What are the hematological disorders found with SLE?
- hemolytic anemia
- Leukopenia
What are the abs that are found with SLE? (3)
- anti dsDNA
- anti Smith
- ANA
What infectious disease do patients with SLE test positive for?
Syphilis
True or false: the rash seen with SLE is photosensitive
True
What is discoid lupus? What areas of the body are particularly affected? What are the lesion characteristics?
A chronic skin condition of sores with inflammation and scarring favoring the face, ears, and scalp and at times on other body areas.
These lesions develop as a red, inflamed patch with a scaling and crusty appearance, that are well demarcated.
True or false: there is a high incidence of systemic disease in discoid lupus
False–low incidence
What are the drugs that can cause SLE?
HCTZ
CCBs
ACEIs
What antifungal drug can cause subacute cutaneous SLE?
Terbinafine
Who usually gets drug induced lupus?
White females
What is Subacute cutaneous lupus erythematosus? Who does it usually affect, what what are the characteristic skin lesions?
a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as polycyclic annular lesions or psoriasiform plaques
What is the treatment for cutaneous lupus?
- Sunscreens
- Topical steroids TID
- Tacrolimus
What are the systemic therapies for SLE?
- Antimalarials
- MTX
- Isotretinoin
What is scleroderma?
Systemic autoimmune disease characterized by sclerosing of the skin and sometimes internal organs
What does scleroderma present with, usually?
Thickening of the hand and raynaud’s phenomenon
What are the three stages of the skin part of scleroderma?
edematous
Indurative
Atrophic
What are the components of CREST syndrome?
Calcinosis Raynaud's Esophageal dysfunction Sclerodactyly Telangiectasias
What is the order of color changes with Raynaud’s?
White
Blue
Red
What is the morphea type of scleroderma? How does it start and progress?
Localized form of scleroderma, that usually starts with one to several circumscribed areas of purplish induration, that progresses to a raised, white sacr with liac ring
Raised white scar with lilac ring = ?
Morphea
What is the treatment for morphea?
Intralesional steroids or hydroxychloroquine
What is the linear scleroderma form of moephea?
Linear patch of sclerotic skin that crosses joint lines, and may lead to joint contractures
What is the en coup de sabre form of morphea?
a type of linear scleroderma characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal scalp
What is Reiter’s syndrome?
Reactive arthritis that usually occurs following an infection
Triad of:
- Inflammatory arthritis
- Conjuntivitis / uveitis
- Urethritis
“can’t see, can’t pee, can’t climb a tree”
What is the HLA haplotype associated with Reiter’s syndrome?
HLA B27
What are the skin findings of Reiter’s syndrome?
Keratoderma blenorrhagicum
What is mycosis fungoides?
T cell lymphoma that presents with rash-like patches that are pruritic, similar to psoriasis
What is Paget’s disease of the bone?
Chronic bone deforming disorder that results from excessive breakdown and formation of bone, causing affected bones to weaken, change shape, and produce pain.
What are the lab findings of Paget’s disease of the bone?
Elevated alk phos
What are the major symptoms of Paget’s disease?
bone pain
hearing loss
What is the characteristic histological finding of Paget’s disease of the bone?
Jigsaw eosinophilia
What are the skin manifestations of Paget’s disease of the bone?
None, but skull deformity may occur
What causes Paget’s disease of the breast?
Ductal CA that migrates to the skin
What is extramammary Paget’s disease?
a rare, slow-growing, usually noninvasive intraepithelial (in the skin) adenocarcinoma outside the mammary gland and includes Paget’s disease of the vulva and the extremely rare Paget’s disease of the penis.
What are the s/sx of extramammary Paget’s disease?
itching, burning, and soreness
What is the treatment for Kaposi’s sarcoma?
Excision or chemo
What is Sweet’s syndrome (acute febrile neutrophilic dermatosis)? S/sx?
Skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.
What are the characteristic lab findings of Sweet’s syndrome? (4)
ESR elevated
CRP elevation
Leukocytosis
Neutrophilia
What is the treatment for Sweet’s syndrome?
Systemic corticosteroids
What is Sweet’s syndrome associated with?
Underlying hematological or visceral malignancy or pregnancy
What are the skin findings with sweet’s disease?
Tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.
What is the classic skin triad of hypothyroidism?
- Edematous eyelids
- Swollen lower lip
- Droopy facial appearance
What are the characteristics of diabetic dermopathy?
bruises in the lower extremities
What is the associated of granuloma annulare?
pre-DM
What is necrobiosis lipoidica diabeticorum?
Necrotizing skin condition in DM pts that is usually asymptomatic, but may become tender and ulcerate when injured
What is waterhouse friederichsen syndrome?
N meningitidis causes hemorrhage into adrenal glands, causing insufficiency
What are two diseases that can cause acanthosis nigricans?
DM II
Cushing’s
What are the crystals found in gout?
Monosodium urate
What is pseudogout? S/sx?
- Accumulation of Ca pyrophosphate in CT, with the knee joint most commonly affected.
- Joint pain + cellulitis-like rash over the area
What is the difference in the hand findings of osteoarthritis vs RA?
OA = MCP RA = DIP and PIPs
What is psoriatic arthritis? S/sx?
- Inflammatory arthritis that can develop in people with psoriasis
- Inflammatory arthralgias
- Dactylitis (sausage joint)
What are the nail findings of psoriatic arthritis?
Onycholysis, pitting and hyperkeratosis
What are the lesions like with psoriasis? What makes the pain better?
Large, scaly white-red Plaques that get better in the sun
What, generally, is psoriasis? S/sx?
Autoimmune disease characterized by an abnormally excessive and rapid growth of the epidermal layer of the skin
large, pruritic plaques
What is the treatment for psoriatic arthritis?
MTX or steroids
What causes the elevated Ca in sarcoidosis?
Granulomas of sarcoid can produce vit D
What is lupus pernio? What is it pathognomonic for?
a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis.
What are the mouth findings in Peutz-Jeghers syndrome?
Patches of hyperpigmentation in the mouth and on the hands and feet
What is osler-Weber-Rendu disease (Hereditary hemorrhagic telangiectasia)? S/sx?
- AD genetic disorder, that leads to abnormal blood vessel formation in the skin, mucous membranes, and organs
- Telangiectasis in the mouth, epistaxis, GI bleeds
What is the inheritance pattern of Peutz-jeghers syndrome?
AD
