31. Signs of Systemic Disease Flashcards by Meghan May

Common (30%) in long standing diabetes

Diabetic dermopathy

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50 yo man with uncontrolled diabetes comes in with this on his legs, how would you describe the lesions?

Atrophic, pink and hyperpigmented macules and (looks like scars)

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Longstanding diabetic comes in with these ickies on his leg… what are they? Are they common

Bullous diabeticorum
• Rarer- 0.5% of patients with DM
• Male: Female 2:1

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Location of Bullous diabeticorum and Tx?

• Longstanding DM with
other complications
• Acral in location
• Often recurrent
• No effective treatment to
prevent

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Diabetic presents with these all over here shins… Gross, what is this?

Necrobiosis lipoidica
• Rare- only 0.03% of patients
with DM
• DM in only 11-65% (not all

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How would you describe this lesion: necrobiosis lipoidica?

Yellow atrophic plaques Multiple/bilateral, Usually shins
• Especially likely to ulcerate
in males with diabetes

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Treatment for Necrobiosis lipoidica

Treatment difficult

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Young woman comes in worried about this discoloration on her neck and armpits. What disease do we expect her to have?

Common in TYPE II DM
• Marker for insulin resistance
• Velvety hyperpigmented
thickening of the skin
• Intertriginous/flexures
• Less extensor surfaces/face
• +/- skin tags

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What is this shit?

acanthosis nigrans skin tags

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What are some other reasons for ancanthosis nigrans besides DM Type II

• AN 1- familial
• AN 2-malignancy (especially gastric and lung)
• AN 3- related to obesity, insulin resistance and
endocrinopathy (Type II DM, PCOS
>acromegaly, Cushing’s, hypothyroidism)

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AN 3 is releated to:

related to obesity, insulin resistance and
endocrinopathy (Type II DM, PCOS
>acromegaly, Cushing’s, hypothyroidism)

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What type of AN is related to malignancy (especially gastric and lung)

AN 2

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Common associations of Diabetes millitis?

Yeast, tinea, cellulitis, MRSA infections, neuropathic ulcers

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What vascular diseases are associated with diabetes millitus?

Peripheral artieral disease and gangrenes

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Young lady comes in with a nail infection and mild diffuse alopecia. Her skin is fine and smooth due to increased sweating and has hyperpigmentation with itching. DDx?

HYperthyroidism

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Your performing a physical exam on an young woman and notice she has dry, cold, rough skin with scales on her feet. She has a dry cough and thin brittle nails and is misisng the lateral thid of the eyebrows. Dx?

Hypothyroidism

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Occurs in some with Grave’s disease- about 1-5% (not in all with hyperthyroidism)
• Cutaneous infiltration of skin of shins (rare other sites) with MUCIN
• “Peau d’orange” (like the skin of an orange), skin colored to brown red, firm
• Can occur during Grave’s or following treatment of same

Pretibial myxedema

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Primary adrenocortical insufficiency–inability to make cortisol. 80% of the ime is autoimmune-aBs are found

*especially post TB, vascular, neoplastic, genetic)

Addisons

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Symptoms of Addisons

Hyperpigmentation: MSH like effect of ACTH

diffuse, sun exposed, sites of trauma/scars, axillae, perineum, nipples, palmar creases, nevi, mucous membranes, hair, nails

Loss of ambisexual hair in post-pubertal woman

fibrosis and calcification of ear (rare)

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Symtpsoms of Cushings

Moon (round ) facies
Dorsicervical fat pad (“buffalo
hump”)
Truncal obesity
Spindly limbs
Striae distensae
Easy bruisability
Slow wound healing
Acne and hirsutism

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Etiology of Addisons

Overproduction of Cortisol by Adrenal Gland

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Multisystem disorder that can have big affect on the skin. Has several recognizable ‘subsets’ and is often diagnosed by appearance, timing and pathology

Lupus Erythematous

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Why is Lupus considered a Spectrum of Disease?

• Overall, about 80% of systemic lupus (SLE) patients have problems with skin • Some lupus patients (chronic cutaneous lupus) ONLY have problems with their skin

What skin related SLE systoms do we suspect to see?

Skin related • Malar (cheek) erythema\* • Discoid (chronic/thick) lesions\* • Oral ulcers\* • Photosensitivity (sun sensitivity)\*

What are the multisystem involvements seen in SLE

Multisystem • Arthritis • Serositis • Nephropathy • CNS disorder • Hematologic disorder • Immunologic • Abnormal ANA

Does SLE target any specific group of people?

Females \> Males (6:1 for SLE) Young \> Old African American \> Caucasian

Pt comes in with ACLE, what regions are we expecting to see

• Generalized or photodistributed, exanthematous (rashy) eruption • Often brought on by the sun but not always • “Malar rash” = “butterfly rash” • Strongly associated with SLE

Young girls comes to the office dt what her mother suspected was sunburn. It looked erythematous and there is a malar rash over her face. Whats the Dx and what can you tell the mom to expect?

Acute = resolves quickly in hours to days WITHOUT scarring • Female:male = 8:1

Most common type of Chronic Cutaneous Lupus Erythematosus

Discoid

15-30% of SLE pts have some of these lesions

discoid CCLE

\_\_\_\_% of those with CCLE will devo Systemic SLE (especially if lesions are widespread

Discoid CCLE is typically seen:

• Usually face/ ears/ scalp/ arms but can be generalized • Mucosal (lips and mouth) involvement in 25%

• Atrophic (thin) scarring with telangiectasias (blood vessels), follicular scales, and too much or too little pigment. • Leaves scars! This is why it is challenging especially if scarring alopecia

``` Chronic cutaneous (discoid) lupus erythematosus ```

Differential Dx

``` Chronic cutaneous (discoid) lupus erythematosus ```

Dx:

Discoid Lups: Leaves scars!

Newest subset of skin disease in lupus, described in 1979 • May also meet ARA criteria for SLE • Usually positive for different ANA types than SLE • Very photosensitive yet usually not on face

Subacute cutaneous lupus erythematosus

What is the pathology of Neonatal Lupus?

Due to SSA/B antibodies crossing the placenta

Young pt comes in with proximal weakness and Elevated CK. Abnormal myositis of biopsy and abnormal MRI of muscles. Dx.

Dermatomyositis

Skin findings of Dermatomyositis

SKIN “Heliotrope rash” (eyelids) Photosensitive “poikiloderma”/ dermatitis Gottron’s papules Positive ANA (+/-) Elevated CRP/ESR

What are these little ickies and who would we expect ot see them on?

Gottrons papules, Dermatomyositis

Young girl comes in with this rash on her face and muslce weakness, hard to get up stairs and get out of bed. What would we expect to see in her labs

Dermatomyositis ``` Positive ANA (+/-) Elevated CRP/ESR ```

Describe this rash

Dermatomyositis: poikiloderma rash on trunk and extremities

Nailfold capillary changes are seen in :

Dermatomyositis

What must we absolutely kepe on an eye on in a female pt with dermatomyositis?

Associated cancer in adults 10-50% – GU, **ovarian**, colon most common – Also breast, lung, pancreatic and lymphoma – Always consider OVARIAN in women with DM • DM may overlap with other CTDs • Interstitial lung disease may be fatal

Sarcoidosis is characterized by what histologically?

Non-caseating granulomas

Sarcoidosis is a multisystem disease. What are two of the more commonly invovled organ systems?

Pulmonary 90% of time and can be asytomatic Skin 20-35 and easy to biopsy

What skin presentation do we expect to see with sarcoidosis?

can be pleomorphic common is red-brown macules and papules on face, typically around eye and nose lesions can be small papules or larger plaques (may have associdated erythema nodosum) \*\*\*\*If you have cutaneous sarcoidosis... do systemic workup!!!)

What is diascopy and when would we use this technique?

Placing glass slide over suspected sarcoidosis... will reveal the brown-red pigment better (also helps to visualize telengactasia)

African American Pt comes in with fever and has brown-red papules and plaques around the eyes and nose. During a workup it's confirmed he has Hilar adenopathy, Erythema nodosum and joint stiffness. Dx?

Lofgren’s syndrome- very commom in sarcoidosis (Spring; better prognosis- will often resolve Can make diagnosis without tissue)

Pt comes in with Causes fragile blisters from trauma and what he suspectes as sunburn. During labs you note he has elevated ALT, AST and a history of Hep C. What explains the blistering on hands?

Porphyria cutanea tarda (Caused by defect uroporphyrin decarboxylase which breaks down heme proteins)

What disease states or 'stressors' can cause porphyria cutanea tarda?

Enzyme system is “stressed” by chronic hepatitis C infection of the liver, but PCT can also be caused by some drugs, alcohol and iron overload in the liver (hemachromatosis)

Little boy is on platelet and RBC transfusions for a blood disorder. The Doctor is worried about iron overload because if he has a defect in this enZ it can lead to Porphyria cutanea tarda

Caused by defect uroporphyrin decarboxylase which breaks down heme proteins

Besides Fragility and bullae and scars on the hands.. what else do we expect in pt with Porphyria cutanea tarda

Hypertrichosis of the face

• Crohns disease has specific skin involvements including:

granulomas in the skin • Involvement of oral mucosa with granulomatous inflammation • Fistulae to skin

Non-specific association of Crohns disease?

* Erythema nodosum * Pyoderma gangrenosum * Nutritional problems * Other

What is the association of IBD we see?

Pyoderma gangrenosum

Describe this lesion and its associated disease

IBD: A sterile, rapid ulceration of the skin caused by neutrophilic infiltration • Characteristic undermined, dusky border

What diseases is pyoderma grangrenosum associated with?

inflammatory arthritis, hematologic problems and malignancies. Also idiopathic

This is a consequence of gluten sensitivity. What is it called?

Dermatisis Herpetiformis

Dermatitis herpetiforms reaction in the body?

Reaction to gluten in grains--\> immune rxn to small bowel that laters the mucosa--\> leasds to antiB formatio--\> aBs can enter blood and attach to skin--\> dermatitis herpetiformis

Common involvement of Dermatitis Herpetiformis

Extensor surface and above ass crack RIDICULOUSLY pruitic!

Pt comes in with outbreak of lesions on her elblows and some on her lower back. Very itchy! They look simular to a herpes outbreak. What is the Tx you would recommend?

Pt has Dermatitis herpetiformis Best treatment is complete avoidance of gluten - after 3 months, TTG antibody levels decrease, mucosa of gut normalizes and symptoms of DH abate

What is the reasoning behind using Dapsone as tx for pt with dermatitis herpetiformis?

Dapsone is also effective treatment for relief of DH (almost immediate for itching), but does not effect the gut or production of autoantibodies.

31. Signs of Systemic Disease Flashcards

(66 cards)