DD 03-10-14 10-11am Skin Signs of Systemic Disease - Dunnick Flashcards Preview

Unit 3 D&D > DD 03-10-14 10-11am Skin Signs of Systemic Disease - Dunnick > Flashcards

Flashcards in DD 03-10-14 10-11am Skin Signs of Systemic Disease - Dunnick Deck (65):
1

Clubbing – defn.

Nail plate is enlarged & excessively curved (>180 degree angle btwn proximal nail fold & nail plate)

Caused by enlarge of the soft tissue of the distal digit

2

Clubbing – etiologies

Rarely idiopathic/primary

Pulmonary disease (idiopathic, pulmonary fibrosis, lung cancer)

Cardiac disease (cyanotic congenital heart disease)

GI disease (Crohn disease, Ulcerative colitis, Proctitis)

Malignancies (Thyroid / Thymus cancer, Hodgkin disease)

Hypoxemia (possibly related to long-term cannabis smoking)

3

Terry’s Nails

Liver cirrhosis in 82% (also in normal individuals)
Leukonychia affects entire nail except for 1-2 mm distal band

4

Half-n-half nails

Distal nail is normal, proximal nail is white

In 10% of pts w/chronic renal failure (also in normal individuals)

5

Proximal Subungual White Onychomycosis

Associated w/HIV disease

Usually due to Trichophyton rubrum (more specifically called tinea ungum)

6

Kaposi’s Sarcoma (KS)

Usually related to immunosuppression

ENDOthelial malignancy, triggered by HHV-8

Slowly progressive, not very common

Generally brownish purple/red patches

7

Classic Kaposi’s sarcoma

Occurs mostly in elderly men of Eastern European descent

8

Lymphadenopathic Kaposi’s sarcoma

Aggressive form primarily in equatorial Africa
Affects young men & is rapidly fatal

9

AIDS-Associated Kaposi’s Sarcoma

More frequent in homosexual pts w/AIDS

Incidence declining w/better anti-retroviral therapy against HIV

10

Therapy for Kaposi’s Sarcoma

Radiation therapy
Excision
Interferon alpha
Chemotherapy

11

Thyroid disease – signs/symptoms

- Exophthalmos
- Pretibial myxedema
- May cause alopecia areata (if see alopecia, test TSH)

12

Alopecia areata – association

T cells fighting off hair cells, so…

Associated w/other autoimmune diseases:
- thyroid
- vitiligo
- IBD

13

Alopecia areata – Clinical Non-Scarring Alopecia

Round/oval patches of hair loss
Short “exclamation point” hairs, broader at distal end
Hairs often re-row w/ depigmentation

14

Alopecia areata – Clinical subtypes

Patch focal
Ophiasis pattern
Diffuse variant
Alopecial totalis (all scalp hair)
Alopecia unversalis (all body hair)

15

Vitiligo - characteristics

Development of total white macules / patches

Histology show complete absence of melanocytes

16

Vitiligo – Associations

Most commonly thyroid disease (Hashimoto thyroiditis, Graves’ disease) – screen TSH levels

Other endocrine disorders (Diabetes Mellitis, Pernicious Anemia, Addison’s disease)

17

Vitiligo – Treatment

Topical steroids

Topical calcineuron inhibitor (protopic ointment)

Narrow-band UVB (311nm) or Excrimer Laser (308 nm) --> repigmentation

Psoralens plus UVA (PUVA)

Minigrafting

Depigmentation (monobenzylether of hydroquinone cream)

18

Excimer laser

UVB ray source (308nm)
Xenon-Chloride lamp emitting non-coherent, monochromatic 308nm light

19

Erythema Nodosum – clinical manifestations

Painful, erythematous subQ nodules (subQ inflammation)

Usually symmetically over pretibial lower extremities

Develop bruiselike appearance in later stages

20

Erythema nodosum – M vs. F, Associated symptoms, Histopathology

More common in women

May also have fever, arthralgias, malaise

Histopathology shows septal panniculitis wit neutrophils (inflame. in subQ fat)

21

Erythemia nodosum - etiology

Delayed hypersensitivity to various antigen stimuli (usually infections)

Most common cause: Strep infections, esp. URI

Other infections (1/3 of the cases) – viral URIs, Mycoplasma, TB

Coccidioidomycosis

22

Erythema nodosum – other more common associations

Idiopathic (35-55%)
Drugs – estrogens, oral contraceptives, sulfonamides, penicillin, bromides, iodides
Sarcoidosis (11-22%)
IBD (esp. Crohn’s)

23

Treatment of Erythema nodosom

Bed rest
Treat underlying condition
NSAIDs – Naproxen, Indomethacin

24

Pyroderma Gangrenosum - clinical manifestations of lesions

Initial lesion often pustule on erythematous base or erythematous nodule

Characteristic lesion is an ulcer w/necrotic, undermined (rolled) border

Painful

Usually on lower extremities

Often begins in sites of minor trauma (pathergy)

25

Pyoderma Gangrenosum – associations

- 50-70% have underying associated condition
- IBD (20-30%)
- Arthritis (seronegative arthritis, RA – 20%)
- Monoclonal gammopathy (often IgA, up to 15%)
- Other hematologic disorders (Myelogenous leukemia, hair cell leukemia, myelofibrosis – 10%)

26

Treatment of Pyoderma Gangrenosum

- Conservative wound care
- AVOID SURGERY/DEBRIDEMENT (causes more injury, more inflammation, never heals)
- Oral / topical anti-inflammatory agents like steroids

27

Lichen Planus – clinical manifestations

- Purple polygonal pruritic papules
- Wickham’s striae = overlying lace-like pattern of white lines on the surface
- Mucosal lesions w/out skin findings in 15-25% of pts
-Nail involvement in 10% (may be only involvement)

28

Lichen Planus – Clinical Variants

Annular or Linear (Koebner)
Hypertrophic or Atrophic
Erosive/ulcerative
Actinic
Bullous

29

Lichen Planus – Associations

*Hepatits C – screen for*

Contact allergy to dental metals (amalgams [mercury], copper, gold)

30

Lichen Planus – Treatment

Nothing works very well :(
- Corticosteroids (Topical, Intralesional, Oral
- Phototherapy
- Systemic retinoids (isotretinoin, acetretin)
- Griseofulvin
- Dapsone
- Plaquenil
- Cyclosporine

*treatment of underlying HepC won’t necessarily make lichen planus go away

31

Acanthosis Nigricans – clinical manifestations

Velvety hyperpigmentation of intertriginous surfaces & extensor surfaces (less commonly)

Most often neck, axillae, dorsal hands

32

Acanthosis Nigricans – due to…

Due to factors stimulating epidermal keratinocytes & dermal fibroblast proliferations

33

Acanthosis Nigricans – Associations

- Rarely Familial : AD, onset in childhood
- *Obesity
- *Diabetes mellitus & Insulin resistance
- Endocrinopathies (hyperandrogenemia, Cushing’s, polycystic ovary, total lipodystrophy)
- Drugs (rare) – nicotinic acids, systemic steroids
- Malignancy

34

Acanthosis Nigricans –Malignancy

- May precede or accompany or follow onset of internal cancer
- Malignancies include gastric adenocarcinoma (also, lung & breast cancer, others)
- Rapid onset w/ weight loss (to distinguish from other reasons for acanthosis nigricans)

35

Acanthosis Nigricans – Treatment

Treat underlying disorder

Keratolytics like ammonium lactate or urea cream (exfoliate)

36

Dermatomyositis – clinical manifestations

- Photodistributed violaceous poikiloderma
- favors scalp, periocular & extensor skin sites
- Heliotrope = eruption on upper eyelids +/- periorbital edema
- Samitz sign
- Gottron’s papules
- Gottron’s sign
-Shawl sign

37

Dermatomyositis – Samitz sign

ragged cuticles in Dermatomyositis

38

Dermatomyositis – Gottron’s papules

Lichenoid papules overlying knuckles, elbows, knees in Dermatomyositis

39

Dermatomyositis – Gottron’s sign

Poikiloderma over knuckles, elbows, knees in Dermatomyositis

40

Dermatomyositis – shawl sign

Poikiloderma across back & shoulders in Dermatomyositis

41

Dermatomyositis – Malignancy prevalence

- Internal malignancy in adults from 10-50%
- More often in females
- May be higher in pts w/dermatomyositis sine myositis
- With new onset dermatomyositis in older adult (usually not kids), think about malignancy

42

Dermatomyositis – Malignancy types

Most common: GU malignancies, esp. ovarian
Others: breast, lung, gastric carcinoms
Nasopharyngeal carcinoma in Asian men

43

Dermatomyositis – Malignancy work-up

Complete Hx & PE

Age appropriate malignancy screening (mammo, CXR, colonoscopy, Pap, PSA, CBC)

Repeat malignancy screen ever 6-12 mo for 1st 2 yrs or more

Risk of malignancy declines after 1st 2 years & reaches baseline after 5yrs

44

Dermatomyositis – Treatment of Skin disease

Sunscreen
Topical steroids
Hydroxychloroquine
Quinacrine
Methotrexate
Retinoids

45

Dermatomyositis – Treatment of Systemic disease

- Oral prednisone (tapered over 2-3 years)
- Methotrexate
- Azathiprine
- High dose IVIG

46

Acute urticaria – rxn type

Immediate type I hypersensitivity rxn by IgE antibodies

47

Urticaria – Acute vs. Chronic

Acute: 6 week duration, women
- most often in women 20-40 yo
- may be related to circulating autoAbs against Ig-epsilon-R1 or IgE

48

Causes of Urticaria

Usually idiopathic/autoimmune

Infection, Drugs, Foods, Vasculitis, Contactants, Inhalants, Pregnancy, Meds

49

Drugs & Urticaria

Cause <10% of all urticaria

Commmon drugs : Penicillins, Cephalosporins, NSAIDs, mAbs, Contrast media, Latex

50

Eczematous eruptions

Most common type of drug rxn in skin

Usually cell-mediated type IV hypersensitivity

Begins 7-14 days after new med (sooner if receiving “old” med)

51

Exanthematous eruptions - causes

Occur all over the body – systemic drug rxn or infection, usually

More commonly drug-induced in adults

Aminopenicillins, sulfonamides, cephalosporins, anticonvulsants, allopurinol

52

Exanthematous drug eruption - treatment

- Discontinue offending med & check of results in three weeks (educated guesswork)
- Supportive topical steroids & anti-histamines
- Generally resolves spontaneously after 1-2 weeks (but can take up to 3 mo to resolve completely)

53

Stevens-Johnson Syndrome vs. Toxic Epidermal Necrolysis

30% = Toxic epidermal necrolysis

In between = overlap syndrome

54

Stevens-Johnson Syndrome – clinical manifestations

- Eruption on face/upper trunk --> may become confluent
- Evolves to skin necrosis & flaccid bullae
- Involves mucous membrane

55

Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis –Cause

Due to abnormal drug metabolism & immune-complex mediated hypdersensitivity

56

Stevens-Johnson Syndrome – characterized by

Epidermal detachment

57

Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis – Long term sequelae

Ocular problems including conjunctival synechiae & blindness

58

Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis – Med causes

- Antibiotics (sulfur, ampicillin)
- Seizure meds
- NSAIDS
- Allopurinol
- Antiretroviral (Nevirapine, Abacavir)

59

Stevens-Johnson Syndrome – Treatment

- Early withdrawal of all possible offending drugs
- Symptomatic (dressing, IVF, nutrition)
- Steroids / High dose IVIG = controversial

60

Toxic Epidermal Necrolysis – SCORTEN

Score based on Prognostic factors to determine Mortality:
- Age >40yrs
- HR >120 bpm
- Malignancy
- Body Surface Area (BSA) >10% at day 1
- high BUN & Glucose, low Bicarb

61

Skin signs of Thyroid disease

Vitiligo
Alopecia areata
Exophthalmos
Pretibial myxedema

62

Signs of immunosuppression

Kaposi’s sarcoma
Proximal subungual onychomycosis

63

Skin & GI disorders

Hep C virus = Lichen planus

IBD = Erythema nodosum, Pyoderma grangrenosum

64

Skin signs of internal malignancy

Acanthosis Nigricans
Dermatomyositis
Clubbing

65

Drug eruptions

Urticaria
Morbilliform eruptions
Steven Johnson syndrome
Toxic Epidermal Necrolysis