31. Signs of Systemic Disease Flashcards
Common (30%) in long standing diabetes
Diabetic dermopathy
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)
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
Location of Bullous diabeticorum and Tx?
• Longstanding DM with
other complications
• Acral in location
• Often recurrent
• No effective treatment to
prevent
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
How would you describe this lesion: necrobiosis lipoidica?
Yellow atrophic plaques Multiple/bilateral, Usually shins
• Especially likely to ulcerate
in males with diabetes
Treatment for Necrobiosis lipoidica
Treatment difficult
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
What is this shit?
acanthosis nigrans skin tags
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)
AN 3 is releated to:
related to obesity, insulin resistance and
endocrinopathy (Type II DM, PCOS
>acromegaly, Cushing’s, hypothyroidism)
What type of AN is related to malignancy (especially gastric and lung)
AN 2
Common associations of Diabetes millitis?
Yeast, tinea, cellulitis, MRSA infections, neuropathic ulcers
What vascular diseases are associated with diabetes millitus?
Peripheral artieral disease and gangrenes
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
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
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
Primary adrenocortical insufficiency–inability to make cortisol. 80% of the ime is autoimmune-aBs are found
*especially post TB, vascular, neoplastic, genetic)
Addisons
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)
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
Etiology of Addisons
Overproduction of Cortisol by Adrenal Gland
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
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
5%
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.
