Lecture 4 - Cutaneous Manifestations of Systemic Diseases Flashcards
Diabetic foot is characterized by an _____ that does not heal and is surrounded by a callous ring. It can progress to ______, infection of the bone.
Diabetic ______ is a circumscribed, atrophic lesion, very common on the anterior lower leg, and it is typically asymptomatic.
Diabetic ____ are the fluid-filled blister looking lesions, typically on the lower anterior leg as well.
Think about dad’s legs!
Ulcer
Osteomyelitis
Diabetic Dermopahty
Diabetic Bullae
Necrobiosis ________ is a diabetic lesion that appears very similar to psoriasis –> sharply circumscribed and multicolored plaques, especially on the lower anterior _____. Keep in mind it is much more common in _____ (men or women?) at a ratio of 3:1. Microscopically, it appears as a necrobiotic granulomata of the _____ (epidermis or dermis?).
Necrobiosis Lipoidica
Leg
Women
Dermis
Xanthomas are plaques or nodules of abnormal lipid deposition as ___ cells in skin or tendons. These are a sign of a variety of ________ disorders, so it’s important to test cholesterol levels in these patients.
Foam cells
Lipoprotein disorders
While xanthomas are typically related to hypercholesterolemia, Eruptive Xanthomas are typically caused by hyper________. These are typically seen on the forearms, but can present as Xanthelasmas, which are yellowish plaques on the _____. These can be treated cosmetically.
Hypertriglyceridemia
Eyelid
Hypersensitivity Vasculitis results from _______ complex deposition in post capillary venules. It appears as palpable non-blanching _______, and patients often have elevated ______ ______ rates (ESR). Because this is a hypersensitivity reaction, it’s important to treat the underlying cause. This type of vasculitis is also called ________ vasculitis, and you’ll be able to see _____ dust on microscopic imaging as a result of apop.
Immune complex
Purpura
Erythrocyte Sedimentation Rates (ESR)
Leucocytoclastic vasculitis
Nuclear dust
Lupus Erythematosus (LE) is a spectrum of diseases ranging from Acute cutaneous to Subacute cutaneous to Chronic cutaneous. Which one is almost always associated with Systemic LE (SLE), and which one is usually not associated with SLE? Which one is typically localized to the head and neck and causes scarring like on Seal’s face?
While we don’t know the exact pathogenesis, we know that _____ exposure exacerbates the symptoms.
Look for patients presenting with ______ rash on the face, alopecia, and mucus membrane erosion.
Acute cutaneous is almost always associated with SLE, and Chronic is usually not.
Chronic causing scarring like on Seal’s face.
Sun exposure
Butterfly rash
Immunofluorescence of LE typically reveals which Ig(s) along with complement at the dermal-epidermal junction?
IgA, IgE, and IgG
Dermatomyositis (DM) is an autoimmune disease that targets the skin and/or _____. It’s most commonly seen in _____ aged people. Juvenile DM is associated with vasculitis, calcinosis, and _____. There is an increased incidence of this disease with malignancy in people over _____ years old. Patients often present with a violaceous heliotrope rash of the _____ and periorbital region with edema.
Muscle
Middle aged people
Arthritis
55 years old
Eyelids
Scleroderma is typically a multisystem disease that results from increased _____ deposition in the skin. Think about how this will present microscopically –> lots of pink staining collagen of course.
Patients often present with taut leathery skin, especially noticeable in the _______, where they may also present with lesions on the knuckles from skin tearing. Because the vasculature is often involved, these patients can present with ______ phenomenon (whitening of the outstretched fingers from lack of blood flow.)
Many patients have auto-Abs to DNA _________-1.
Collagen
Fingers
Reynaud’s phenomenon
DNA Topoisomerase-1
Localized Scleroderma is called _____.
Morphea
Sarcoidosis is a disease characterized by non-_______ granulomatous inflammation in many organ. It is 10 X more common in ______ ______ (which race?).
Patients can present with Lupus pernio, which is a doughy enlargement of the nose, similar to ______, but smoother in appearance. Patients also often present with increased ______-converting enzyme.
Look for nodular collections of epithelioid histiocytes w/ a few giant cells containing ______ bodies.
Non-caseating granulomatous inflammation
African Americans
Rosacea
Angiotensin-converting enzyme
Asteroid bodies
There are 2 forms of Neurofibromatosis. Both are _____ dominant and patients with either of these two forms will present with ____-__-___ spots (Especially in the ______ of NF1 patients) and neurofibromata.
The NF1 form affects the gene for _________ on chromosome ______. These patients will present classically with Lisch nodules (pigmented hamartomas of the iris) and plexiform neurofibromas.
The NF2 form affects the ______ gene on chromosome ____. These patients present classically with bilateral _______ _______. Keep in mind this form is ______ (more or less?) common than NF1.
Autosomal
Cafe-au-lait spots
Axilla
Nerofibromin
Chromosome 17
Merlin
Chromosome 22
Acoustic Schwannomas
More
Tuberous sclerosis is an ______ dominant disease that affects either the _____1 or _____2 tumor suppressor genes. Look for a triad of _____, _______ disability, and congenital ________ spots in early manifestations. This disease arises from ectoderm or mesoderm. Keep in mind the spots shine under ______ lamp.
Autosomal dominant
TSCS1 or TSCS2
Seizures, Intellectual disability, and Congenital Hypopigmented spots.
Wood’s lamp
_______ cell Histiocytosis is a group of idiopathic disorders characterized by infiltration of tissues by ______ cells (tissue resident dendritic cells). Clinical syndromes include Eosinophilic Granuloma, Hand-Schuller-Christian, and Letterer-Siwe disease. Which of the three is the most aggressive?
These patients often present with skin rashes localized to the ______, but mucosal tissues can be involved. Look for ______ bean shaped histiocytes and Bierbeck’s granules (_____ _____ shaped cells visible on EM).
Langerhans Cell histiocytosis
Langerhans cells
Letterer-Siwe disease is most aggressive.
Trunk
Coffee bean shaped histiocytes
Tennis racket shaped cells on EM.
Mastocytosis syndromes arise from mutations in ___-____, affecting cellular proliferation. _____ sign is a good indicator a patient has Mastocytosis, and it involves stroking the lesion which causes an itch and wheal reaction. What are the cells involved in this disease (and the reaction mentioned) releasing?
C-kit
Darier sign
They release Histamine
