Dermatologic Manifestations of Chronic Disease

1. Dermatologic Manifestations of Chronic Disease Shelbi Hayes. M.D. Saints Dermatology October, 25 2013

2. I. Creating a Framework for Evaluating Skin Lesions II. Application of the framework to the most common manifestations of chronic disease

3. I have no financial disclosures.

4. Creating a Framework Question #1 Is this a primary or secondary lesion?

5. Macule Patch Papule Plaque

6. Pustule Nodule Pustule Nodule Vesicle Bulla

7. Pustule

8. Vesicle

9. Bulla

10. Wheals

11. Wheals

12. Macular-Patch Papular Papulosquamous (scaly papules) Nodular Pustular Vesicular-bullous Urticarial Petechial Telangiectasia Burrow Poikiloderma Hyperkeratotic/scale Atrophic Morphologic categories

13. Secondary Lesions • Crust • Erosions and ulcers • Excoriations • Fissures • Scars • Lichenification • Atrophy

14. Creating a Framework Question #2 Is there scale?

15. Scale or No Scale? • Scale indicates the disease process involves the epidermis. • Lack of scale indicated the disease process affects the dermis or subcutaneous fat. • Exception: Tinea Incognito, Early Vesiculobullous Lesions

16. Creating a Framework Question #3 What is the configuration?

17. Annular Arcuate Geographic Discrete Confluent Serpiginous Linear Reticulated Configuration

18. Creating a Framework Question #4 What is the color?

19. Color • Pink • Violet • Orange • Blue • Green • Yellow • Black • Brown

20. Color

21. Color • Pink—Pityriasis rosea • Violet—Lichen planus • Orange—Juvenile xanthogranuloma • Blue—Amiodarone skin pigmentation • Green—Pseudomonas • Yellow—Xanthomas • Black—Eschar • Brown—Café au lait spots

22. Creating a Framework Question #5 What is the distribution?

23. Immunosuppression

24. Herpes Simplex

25. Herpes Simplex • Caused by HSV-1 and HSV-2 • Infections occurs at the primary site, transported via neurons to dorsal root ganglion where latency is established • Pain, tenderness or tingling occur often before reactivation. • Grouped vesicles on erythematous base, however you may not see the primary lesion when the patient presents!!

29. Herpes Simplex VirusEczema Herpeticum

30. Herpes Simplex VirusEczema Herpeticum

31. Herpes Zoster

32. EM-SJS-TEN • Spectrum of epidermal damage +/- mucosal involvement • EM minor = no mucous membrane • EM in kids usually secondary to HSV, drugs in adults • SJS-TEN constitute one of the few derm emergencies • Treat in burn unit, frozen section of bx to check for necrosis, little inflammation • Fluids, infection prophylaxis, consult ophtho and uro as indicated

33. Erythema Multiforme

35. Erythema Multiforme Major • Also thought to be a hypersensitivity reaction • As with EM minor, but with involvement of ≥2 mucosal surfaces (precedes rash by 1-2 days) • Pronounced constitutional symptoms common

36. Stevens-Johnson Syndrome • Is SJS separate entity from EM major? • Some feel SJS is a distinct entity as the rash is more erythematous and less acral than EM major • EM major is more commonly triggered by infections and SJS by drugs.

37. Stevens-Johnson Syndrome

38. Stevens-Johnson Syndrome

39. Stevens-Johnson Syndrome

40. Toxic Epidermal Necrolysis Nikolski’s Sign = separation of the epidermis from the dermis by rubbing skin between the lesions

41. Toxic Epidermal Necrolysis (TEN) • A life-threatening, exfoliating disease of the skin and mucous membranes • Hallmark is full-thickness necrosis of the epidermis with separation at the dermoepidermal junction.

42. SJS vs TEN • Some use %BSA to define with: <10% = SJS >30% = TEN • Histologically SJS has a much higher density cell infiltrate (T-lymphocytes) vs TEN (low density macrophages and dendrocytes)

43. TEN - Pathogenesis • Majority of cases are likely adverse drug reactions (foreign antigen response). • Mean time from drug to onset = 13.6 days • Higher risk drugs • NSAIDS [38%] • Antibiotics [36%] (sulfonamides) • Anticonvulsants [24%] (phenobarb, lamotrigene) • Corticosteroids [14%]

44. Use Trimethoprim-SulfamethoxazoleJudiciously. Up to 17% of patients can have an adverse cutaneous reaction. Occurs within the first 3 weeks. Warn Patients to alert you immediately. Do not prescribe if the patient has a family history of sulfa allergy.

45. TEN - Clinical Features • Initial symptoms (1-3 days) • Fever (100%) • Conjuctivitis (32%) • Pharyngitis (25%) • Pruritis (28%) • Headache, myalgias, arthralgias, vomiting, and diarrhea may occur

46. TEN - Clinical Features: Mucosal Involvement • Erosive mucosal lesions (1-3 days before skin eruption) occur in 97% • Oral (93%) • Ocular (78%) • Genital (63%) • Anal

47. TEN - Clinical Features:Skin Eruption • Burning / painful skin rash • Usually begins on face / upper trunk • Begins as one of: • Diffuse erythema • Irregular bullae • Poorly defined dusky or erythematous macules • Scalp usually spared

48. Multisystem Involvement • GI - Mucosal sloughing in esophagus (dysphagia, GI bleeding) • Resp - Tracheal/bronchial erosions (Respiratory decompensation) • Renal – Glomerulonephritis • Profound fluid and electrolyte disturbances

49. Dermatophytes • Named for area involved: tinea capitis, corporis, manum, facei, pedis, cruris, etc. • If there is scale, do KOH exam. • Words of a famous dermatologist: “If it is scaly, SCRAPE it!”