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Drug Eruptions, Author Unknown

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Drug Eruptions, Author Unknown

  1. 1. Rotator Lecture VI Drug Eruptions, CTD, NF, MF
  2. 2. Exanthematous Drug Eruptions • Most common manifestation of drug reactions • Presents as erythematous macules and papules coalescing into diffuse erythema • Skin may peel as rash is resolving • Most common causes include sulfa, PCN, and PCN derivatives
  3. 3. Figure 23.1 Exanthematous drug eruptions. Numerous pink papules on the trunk due to a cephalosporin (A). Confluence of lesions on the trunk (B) and annular plaques on the forehead (C) secondary to phenobarbital. Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  4. 4. Figure 23.1 Exanthematous drug eruptions. Numerous pink papules on the trunk due to a cephalosporin (A). Confluence of lesions on the trunk (B) and annular plaques on the forehead (C) secondary to phenobarbital. Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  5. 5. Usually worst on LE due to orthostatic pressure.
  6. 6. Fixed Drug Eruption • Occurs in the same place each time a patient is challenged with a particular drug • Round or oval lesion with an ash gray to slate blue colored center, may have bullae • Can have 1 lesion or multiple • Common causes include laxatives, NSAIDs, sulfa drugs, tetracyclines
  7. 7. Figure 23.11 Fixed drug eruptions. Well-demarcated erythematous (A) to violet-brown plaques that can develop a detached epidermis Responsible drug was phenophthalein (A) Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  8. 8. Erythema Multiforme (EM) • This has many causes, drug eruption is one etiology • Erythematous target-like lesions • Minor variant does not require hospitalization – Usually a reaction to HSV infection • Major variant (Stevens-Johnson) requires hospitalization and immediate treatment
  9. 9. Figure 21.1 Phenotypic variety in EM. D classic target lesions on the palms. Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  10. 10. Stevens Johnsons Syndrome (SJS) and Toxic Epidermal Necrosis (TEN) • SJS and TEN always caused by a drug • More severe than EM • Involves mucosa • SJS arbitrarily defined as involving at least 2 mucous membranes and having at least 10% body surface area involved • TEN is worst end of spectrum with more severe cutaneous and mucosal manifestations and can be fatal
  11. 11. SJS and TEN • Common causes include antiepileptics, sulfa drugs • More severe than EM • SJS: Rapid course, large sheets of necrotic skin, can involve mucosa, evolves within days; can rapidly turn into TEN (hrs) • TEN: Extremely rapid course. Evolves within hours. Can be fatal! Treat patient in burn unit, administer IV Ig, plasma pheresis • Oral/systemic steroids have not been able improve mortality (can increase susceptibility to infection)
  12. 12. Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  13. 13. Figure 21.6 Denuded and crusted lesions of the lips with minimal cutaneous lesions in a child with SJS secondary to antibiotic therapy. Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  14. 14. Figure 21.7 Severe conjunctival erosions and exudate in SJS secondary to trimethoprim-sulfamethoxazole therapy. Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  15. 15. Figure 22.6 Stevens-Johnson syndrome (SJS) versus SJS-TEN overlap. In addition to mucosal involvement and widespread erythematous papules, there are small areas of epidermal detachment (arrows). Because the latter involve 10% body surface area, the patient is classified as having SJS.Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  16. 16. Figure 22.5 Clinical features of toxic epidermal necrolysis (TEN). A Detachment of large sheets of necrolytic epidermis (>30% body surface area), leading to extensive areas of denuded skin. B Hemorrhagic crusts with mucosal involvement. C Epidermal detachment of palmar skin.Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  17. 17. Figure 22.4 Cutaneous features of toxic epidermal necrolysis (TEN). A Characteristic dusky-red color of the early macular eruption in TEN. Lesions with this color often progress to full-blown necrolytic lesions with dermo-epidermal detachment. B Positive Nikolsky sign: epidermal detachment reproduced by mechanical pressure on an area of erythematous skin. Downloaded from: Dermatology (on 29 June 2006 07:55 PM) © 2005 Elsevier
  18. 18. TEN
  19. 19. Urticaria • Has many causes! Pressure, exercise, food, temperature, and drug • Transient lesion (<24 hours), new ones can form with continued contact • Resolve without scarring • Remove offending agent(s), administer anti- histamines
  20. 20. Angioedema • severe form of urticaria involving dermis and subcutaneous swelling which can affect airway, mucosa, and bowels. • Can be from food, medication, latex allergy
  21. 21. Angioedema • Can occur simultaneously with urticaria • Angioedema without urticaria can also occur – Drug reaction – C1 esterase inhibitor deficiency JAAD 2002; 46: 645-57.
  22. 22. Angioedema: Drug Reactions ACE Inhibitors • Often orofacial angioedema • Urticaria/angioedema is believed to result from the inhibition of endogenous kininase • Can occur up to 1 yr after starting med NSAIDs Aspirin
  23. 23. Angioedema: Drug Reactions Management • Withdrawal of drug • Antihistamines, corticosteroids • Epinephrine may be needed if airway in trouble or very severe • Report of FFP used in refractory case (to above and IVIg, CSA)
  24. 24. Hereditary Angioedema • Autosomal dominant; with incomplete penetrance • 1:150,000 • Mutations in one copy of the gene for C1 inhibitor – Type 1: reduced levels of C1 inh (85% of cases) – Type 2: functionally deficient C1 inh (15% of cases) • C1inh deficiency allows activation of the C1 – generation of bradykinin – subsequent consumption of complement leads to low levels of C4 in the serum
  25. 25. Hereditary Angioedema • Trauma can precipitate attacks • Attacks last 48-72 hrs – Laryngeal edema – Abdominal pain • NO WHEALS (ie NO URTICARIA)
  26. 26. Hereditary Angioedema Fitz 6th ed p 1132
  27. 27. Acquired Angioedema Type 1 • C1 fixed by anti-idiotypic antibodies causes consumption of C1 inh – Seen in lymphoproliferative disease, esp. multiple myeloma, Waldenstroms, B cell lymphoma, CLL Type 2 • autoantibodies (IgG1) against C1 inh – Associated with SLE, RA, Sjogren’s
  28. 28. Angioedema (only) work-up • Medications: – Avoid estrogens • FH • C4 : BEST SCREEN – Screens both hereditary and acquired C1 inhibitory deficiency • C1q – Low in acquired C1 inh def. • C1 inh assay – Amount: type 1 deficiency – Function: type 2 deficiency
  29. 29. Hereditary Angioedema: Rx • C1 inh concentrate of FFP – For emergencies • Tranexamic acid (IV) – FDA approved for hemophilia – antifibrinolytic agent: competitively inhibits the activation of plasminogen to plasmin • Danazol, Stanozolol: treatment of choice
  30. 30. Acquired Angioedema: Rx • Secondary to lymphoproliferative disease – Stanozolol – Danazol • Secondary to anti-C1Inh autoantibodies – Respond to glucocorticoids
  31. 31. Dermatomyositis • Combination of skin and striated muscle inflammation • Characteristic Skin Manifestations: – Heliotrope Rash – Gottron’s papules/sign – Poikiloderma (atrophy, telangectasia, and pigmentary alteration) • Muscle Manifestations – Symmetric proximal muscle weakness – Elevation of skeletal muscle enzymes (CPK, aldolase) – EMG, muscle biopsy, and MRI can also be used to confirm myositis
  32. 32. Heliotrope: periorbital edema with a lightly violaceous hue
  33. 33. Gottron’s Papules • Pathogmonic for Dermatomyositis • Erythematous-violaceous scaly papules over dorsal IP joints, elbows, or knees • Gottron’s sign are patches or plaques of the same color in the same distribution • Photosensitivity is the proposed etiology • Associated with proximal nail fold atrophy and telangiectasia
  34. 34. Gottron’s papules
  35. 35. Figure 44.4 Gottron's sign with violaceous poikiloderma over the knuckles. Downloaded from: Dermatology (on 29 June 2006 08:28 PM) © 2005 Elsevier
  36. 36. Figure 44.2 Violaceous poikiloderma of the face, plus thin plaques on the elbows (Gottron’s sign) that are sometimes misdiagnosed as psoriasis. Downloaded from: Dermatology (on 29 June 2006 09:20 PM) © 2005 Elsevier
  37. 37. Figure 44.1 Violaceous poikiloderma of the face. Heliotrope also noted. Downloaded from: Dermatology (on 29 June 2006 09:20 PM) © 2005 Elsevier
  38. 38. Dermatomyositis • Up to 25% are associated with underlying malignancy • Ovarian cancer is overrepresented in women • Patient’s with new diagnosis need cancer screening at diagnosis and yearly
  39. 39. Scleroderma • Disorder characterized by fibrosis of connective tissue, increased collagen deposition, and vascular alterations. • Localized disease without systemic manifestations is called “morphea” or “localized scleroderma” • Systemic disease with skin manifestations is called “systemic scleroderma” – Subsets of this are CREST and systemic sclerosis • ANA is not helpful in diagnosis
  40. 40. Localized form of morphea: ‘en coup de sabre’
  41. 41. Limited Systemic Sclerosis: CREST
  42. 42. Sclerodactyly seen in CREST
  43. 43. Figure 45.3 Mat telangiectasias in a patient with systemic sclerosis (scleroderma). Downloaded from: Dermatology (on 29 June 2006 08:28 PM) © 2005 Elsevier
  44. 44. Progressive Systemic Sclerosis • Skin thickening is more widespread and proximal • Can be severe and rapidly fatal • Both CREST and PSS can causes renal disease and pulmonary sclerosis
  45. 45. Cutaneous Lupus • ACUTE: Typical photosensitive malar rash when acute – Highly associated with systemic LE (almost 100%) • SUBACUTE: This variant is psoriasiform or papulosquamous – ~50% of these patients will meet criteria for SLE • CHRONIC: ie Discoid Lupus – Most patients (85-90% never develop systemic lupus)
  46. 46. Figure 43.4 Acute cutaneous lupus (ACLE) lesions in a butterfly distribution on the face of a young woman. Note sparing of nasolabial folds. Downloaded from: Dermatology (on 29 June 2006 08:28 PM) © 2005 Elsevier
  47. 47. Figure 43.5 Acute cutaneous lupus (ACLE). The patient shown in this photo had ACLE lesions on the arms as well as the face. Downloaded from: Dermatology (on 29 June 2006 08:28 PM) © 2005 Elsevier
  48. 48. Figure 43.7 Subacute cutaneous lupus (SCLE) lesions of the sun- exposed aspects of the upper arm. Note the annular configuration and crusting of the borders. Downloaded from: Dermatology (on 29 June 2006 08:28 PM) © 2005 Elsevier
  49. 49. Discoid Lupus Erythematous (DLE) • Most scarring and chronic form of cutaneous lupus • Discoid shaped plaques with white scale, with time, lesions become atrophic • Can lead to scarring alopecia • Few patients meet criteria for SLE • Treat with intralesional or topical steroids, sun avoidance, plaquenil if severe or large areas involved
  50. 50. Figure 43.9 Chronic cutaneous lupus erythematosus (CCLE) with discoid lesions. The ear is a common site of involvement. Note the central depigmentation, scarring and peripheral hyperpigmentation. Downloaded from: Dermatology (on 29 June 2006 08:28 PM) © 2005 Elsevier
  51. 51. Figure 43.10 Chronic cutaneous lupus erythematosus (CCLE) discoid lesion. Downloaded from: Dermatology (on 29 June 2006 08:28 PM) © 2005 Elsevier
  52. 52. Neurofibromatosis • Type 1 (vonRecklinghousen) is more common that Type 2 • Autosomal dominant inheritance in half of cases, other half are spontaneous • Hereditary form has variable penetrance; can be associated with mental retardation • Some Criteria: 2 or more neurofibromas, 6 or more café au lait macules, Lisch nodules in the eyes, Axillary or inguinal freckles (Crows sign)
  53. 53. Mycosis Fungoides (MF) • Most common type of cutaneous T-cell lymphoma • NOT A FUNGAL DISEASE! • Most common in middle-aged white men • Occurs in patch and plaque stages • Asymmetrical distribution of lesions • Treat with steroids, UVB, nitrogen mustard, PUVA, methotrexate, retinoids, interferons
  54. 54. Patch vs. Plaque Stages of MF • Flat • Fine white scale • Erythematous • ‘Cigarette paper’ wrinkling • Serpiginous or annular • Non-specific rash, can resemble psoriasis or eczema • Localized • Red to violaceous nodules • Associated with lymphadenopathy • Alopecia • Hyperkeratosis of palms and soles • Widespread • Lesions can become ulcerated
  55. 55. Thank You!

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