Dermatology Cases December 7, 2013

1. Dermatology CasesDecember 7, 2013 Frank Morocco D.O. FAOCD

2. Case 1 51 y/o white male with 4 year history of silvery scaling thickened erythematous plaques on his knees, elbows and scalp. The patient has 25% BSA. Denies any joint pain.

3. Psoriasis : • A) Involves only the skin and should be managed exclusively by a dermatologist • B) Is associated with multiple comorbidities including diabetes, hyperlipidemia, and arthritis • C) Statins have been shown to worsen psoriasis • D) Topical therapy should be continued as monotherapy for multiple years before switching to a different treatment modality

4. Psoriasis • Psoriasis is a hereditary, papulosquamous skin disorder that affects 3 to5 million people in the United States. • Affects men and women equally. • Psoriasis is a chronic and recurring disease that is best characterized by well-demarcated erythematous plaques • Most commonly plaques are seen on the elbows, knees, and the scalp

5. Psoriasis • Psoriasis is an immune mediated inflammatory skin disorder. • TH1 driven with increase of IL-6, TNF-alpha and Interferon • Unpredictable • Age of onset bimodal 23 y/o and 55 y/o • Aggravated by emotional stress • In addition to its cutaneous manifestations, psoriasis has been associated with arthritis

6. Psoriatic Arthritis Psoriatic arthritis affects an estimated 25% to 34% of patients with psoriasis Most commonly appears 10 years after the onset of psoriasis Psoriatic Arthritis: 5 patterns Asymmetric DIP with nail damage (16%) Arthritis mutilans with osteolysis of phalanges/metacarpals (5%) Symmetric polyarthritis-like RA with claw hands (15%) Oligoarthritis with swelling and tenosynovitis of one or a few hand joints (70%) Ankylosing spondylitis alone or with peripheral arthritis (5%)

8. Psoriasis: Psoriatic Arthritis • Psoriatic Arthritis (cont’d) • X-rays resemble RA except: • Erosion of terminal phalangeal tuft • Tapering or whittling of phalanges or metacarpals • Cupping of proximal phalanges • Bony ankylosis • Osteolysis of metatarsals • Prediliction for DIP and PIP • Sparing of MCP and MTP • Paravertebral ossification • Asymmetric sacroilitis; rarity of bamboo spine

9. Burden of Psoriasis • Quality of life • A population-based survey looking at the association between quality of life and extent of disease. • 60% of patients report psoriasis affects their everyday life and 26% report a change or discontinuation of daily activities. • This was higher than cancer and diabetes!!

10. Burden of Psoriasis • Economic • The estimated total direct and indirect health care cost of psoriasis in the United States is 11.25 billion dollars annually • A two-year study by the National Psoriasis Foundation (NPF) found that having psoriasis is associated with decreased household incomes and reduced employment opportunities. • Psoriasis patients have lower income and are less likely to work full-time. • Psoriasis was reported as the primary reason for unemployment in 17% of patients with severe psoriasis. • Psoriasis patients missed an average of 26 days of work each year due to complications of psoriasis

11. CVD Risk Factors Associated with Psoriasis • Psoriasis may predispose patients to increased risk of atherosclerotic disease. • A chronic, proinflammatory state that fosters the development of the metabolic syndrome. • 40 % of psoriatic patients develop metabolic syndrome • Chronic inflammation  to increased oxidative modifications of lipoproteins, which are more atherogenic than native lipoproteins

12. CVD Risk Factors Associated with Psoriasis • Diabetes • The studies suggest that psoriasis is associated with a 59% increased prevalence of diabetes and a 27% increased risk of developing diabetes among patients with psoriasis • The altered immune pathways may also predispose to impaired glucose tolerance and diabetes • Aggressive therapy ?

13. CVD Risk Factors Associated with Psoriasis • Hyperlipidemia • 19 of 19 studies showed an association with hyperlipidemia • Study subjects had higher levels of very low density lipoproteins,total cholesterol and LDL,and lower levels of cardioprotective high density lipoproteins (HDL)

14. Anti-lipid lowering agents for psoriasis treatment. • 63 pts • 32 pts on Atorvastatin 20mg/day • 31 pts on placebo • 80% reached PASI 75 in 6 months • 78% reach PASI 75 in 3 months with ustekinumab • Also showed significant reduction of CRP and TNF alpha after 3 weeks

15. Psoriasis: Treatment • Topicals or localized tx (laser/pulsed light) for limited plaques • Phototherapy • MTX • Cyclosporin • Biologics • Rotating therapies and combination therapies

16. Psoriasis: Topical therapies • Corticosteroids: Class I for 2 weeks, then weekend pulses • Occlusion for thick keratotic scale • Low-mid strength intertriginous and face • Can give ILK for refractory plaques and nail matrix and lateral nailfold monthly • Tazarotene: modulates keratinocyte differentiation and hyperproliferation and suppresses inflammation • Calcipotriene: keratinocyte differentiation

17. Psoriasis: Light Therapy • Burning can cause Koebner’s phenomenon • Artificial UVB broad or narrow band • 254, 280, 290 are ineffective • 296, 300, 304, 313 give clearing • Narrow band = 311 and is more effective than broad band

18. Psoriasis • Psoriasis is associated with comorbid conditions, including depression, arthritis, diabetes, hypertension, metabolic syndrome, and cardiovascular events • A systemic inflammatory processes may underlie these disease processes. • Treatment must involve a multidisciplinary approach between Dermatologists, PCP cardiologists and rheumatologists

19. Case # 2 -Patient S.S. is a 16 month old boy brought in to see you by his parents for an itchy rash. -“rashy skin” intermittently since birth. -worst during the winter months of the year. -Mom reports that he scratches in his sleep. The parents tell you that S.S. was hospitalized at age 6 months for reactive airway disease for which he required nebulizers. -His mom has strong allergies to pollens in the summer. -Dad has asthma. -S.S. uses Suave Berry Blast soap and shampoo in the bathtub. He takes one bath every other day. They have tried Vaseline intensive care lotion when his skin seems dry.

20. Atopic Dermatitis The Itch that Rashes

21. Atopic Dermatitis

22. Atopic Dermatitis • Eptheilial barrier disruption • 50% of atopic dermatitis caused by Filaggrin gene (FLG) disorder • FLG encodes profilaggrin which is the major component of granular layer. • Filaggrin makes up the the major scaffolding that forms the lipid cell envelope.

23. Atopic Dermatitis

24. Normal Epidermis

25. Atopic Dermatitis

26. Filaggrin • Filaggrin is degraded and forms “natural moisturizing factor” • Decreases pH which helps inhibit Staphylococcus aureus growth • Activate enzymes in ceramide metabolism • Modulating the activity of serine proteases • Epidermal barrier repair is aimed at replacing ceramides, inhibition of elevated protease activity and decreasing skin pH

27. Atopic Dermatitis • In vitro studies have shown immunity still has major effect. • IL-22 and IL-25 are involved in decreasing filaggrin expression. • Decrease of active copies of fillagran by 5-10% can increase severity of atopic dermatitis.

28. Atopic Dermatitis • Other Factors • Delayed introduction of solid foods, early life exposure to antibiotics, exposure to farm animals or ingestion of fish oils have no effect on development of AD • Breast-feeding has been shown to have no effect • Physiologic and psychological benefits of breast feeding makes it preferred feeding modality even in children with risk of developing AD

29. Highlights From NIAID Guidelines • Children < 5 y/o with moderate to severe AD should be considered for food allegies to milk, egg, peanut wheat, and soy IF persistant atopic dermatitis with optimal treatment or if there is a history of immediate reaction after the ingestion of a specific food

30. Highlights From NIAID Guidelines • Fifty percent to 90% of presumed allergies are not allergic in nature • Solid foods should not be delayed beyond 4 to 6 months, because this may paradoxically increase the incidence of food allergies • Individuals without documented or proven food allergies shouldnot avoid potential allergenic foods

31. AD and ADHD • Proposed Mechanisms • Negative effect of inflammatory cytokines on central nervous system • Increased stress and sleep disturbances due to pruritis. • Lack of impulse control and use of stimulatory medication makes ADHD a risk factor for more severe AD

32. Features Associated with Atopy • Dennie-Morgan fold: linear transverse fold just below the lower eyelid • “Normal” skin is subclinically inflamed, dry, scaly • Pityriasis alba: • hypopigmentation with sclight scale on cheeks, upper arms, trunk in young children. Responsive to emollients and topical steroids • Keratosispilaris: • horny follicular lesions of outer aspects of upper arms, legs, cheeks, and buttocks; refractory to treatment

33. Features Associated with Atopy • Increased susceptibility of infection; • Patients heavily colonized with Staph. Treatment of lesional skin reduces colonization even w/o ABX • Eczema herpeticum: generalized herpes simplex, sudden vesicular, pustular, crusted or eroded lesions. Become secondarily infected. • Eczema vaccinatum: widespread vaccinia infxn • Extensive flat wart or molluscum; poor tolerance to Tx

35. Management of Atopy • Infants and children: • Avoid hot baths, alkaline soaps, vigorous rubbing and scrubbing. • Short, once-a-day, tepid baths followed by a barrier cream using soak and smear; ointment bases are preferred. • Immediate change of wet or soiled diapers. • Nighttime sedating antihistamines for itch • Dietary restriction for a specific known antigen

36. Management of Atopy • Adults • Avoid temperature extremes • Hydrate dry skin especially in winter • Avoid overbathing and hot water • Avoid wool • Biofeedback techniques for emotional stress

37. Topicals for Atopy • Topical corticosteroids are the mainstay • 1-2.5% hydrocortisone in infants. Monitor growth in infants and young children. • Mid-potency (TAC) in older children and adults except on the face • 1-2x a day is enough to saturate receptors; more provides only emollient effect • Occlusion increases penetration and receptor saturation • Must be strong enough to control pruritus and remove inflammation • Regular emollients: petrolatum, hydrophilic creams with ceremides • Anti-Staph therapy for acute flares • Topical calcineurin inhibitors

38. Systemics for Atopy • Antihistamines for sedation: hydroxyzine, diphenhydramine, or clopheniramine. • The nonsedating antihistamines do not relieve pruritus • Short courses of anti-Staph ABX, topical mupirocin for nasal carriage • Immunosuppressives and antiproliferatives (Immuran, Cellcept, MTX) can be effective for unresponsive dz • Phototherapy: PUVA, UVA, narrow-band UVB, or Goeckerman with tar may be helpful

39. Atopic Dermatitis • Increased risk of S. Aureus infection and colonization which leads to inflammation • Decreased Human Beta-defensin-2 leads to increased MSSA. • A recent study showed that diluted bleach baths plus intranasal mupirocin led to significant improvement in eczema severity scores. • Typical recipe is ¼ cup of bleach for ½ tub of water and ½ cup of bleach for tub full of water • Mupirocin was administered 5 consecutive days a month

40. Atopic Dermatitis • Wet dressing therapy was has been used in past for severe AD. • Recent study from Mayo Clinic described their institution’s wet dressing therapy for inpatient hospitalizations. • 45% had 75%-100% clearance • 38% had 50%-75% clearance

41. Atopic dermatitis • Traditional AD management dogma consisted of application of anti-inflammatory medication to areas of “active”disease. • Recent research shows a paradigm shift. • After twice daily active treatment of AD flare, patients were given “proactive” twice weekly treatment with topical tacrolimus and had significant fewer AD flares

42. Case 3 • An 18-year-old patient presents with a sore throat and signs of streptococcal pharyngitis. you start treatment with amoxicillin-clavulanate, and results of a rapid strep test are positive. The next day the patient calls regarding a new rash that has erupted all over his body. The palms and soles remain uninvolved

44. Which one of the following is the most likely cause of this patient’s rash? • a. Drug rash • b. Pityriasis rosea • c. Streptococcal scalded skin syndrome • d. Mycoplasma pneumonia • e. Psoriasis

45. Guttate Psoriasis • Guttate psoriasis classically presents after infection with streptococcus in children or young adults. • Guttate psoriasis presents with scaly, “droplike” papules on the trunk and extremities. • It is often mistaken for a drug rash because antibiotics may have been initiated for the streptococcal infection. • Throat cultures for streptococcal pharyngitis should be obtained. • Guttate psoriasis has a good prognosis and may disappear spontaneously or may benefit from phototherapy.

46. Case 4 • A 28-year-old man presents with a rash and pruritus that has been present for 3 weeks. He has no history of skin or other health problems and is not receiving any medications. He has used no new products on the skin and has not frequented wooded areas. On examination, he has red papules and excoriations on the wrists, groin, and axillae and nodular areas on his scrotum

47. Case 7

48. To identify the cause of this patient’s rashand pruritus, which one of the following isthe best laboratory test? • A) Mineral oil preparation of a skin scraping • B). Tissue transglutaminase measurement • C). Skin biopsy • D). Lyme disease serology • E). Skin biopsy for tissue culture

49. Scabies • Scabies is caused by infestation of the epidermis with the mite. • Infestation occurs as a result of direct skin-to-skin contact; fomite transmission is uncommon. • Scabies causes epidemics in schools, nursing homes, and hospitals. • Pruritus is the major complaint, prominently at night, and there is often a history of itching or rash in family members • The rash is due to hypersensitivity reaction to the mite protein. • It can take 4 to 6 weeks after initial mite exposure to develop signs or symptoms of scabies infestation

50. Scabies • Clinical features include inflammatory, excoriated papules in the web spaces of the hands and feet, the axillae, groin, wrists, and areolae, and submammary sites in women. • Facial or scalp involvement is uncommon, except in children and elderly persons. • Nodules or thickened areas in the scrotum are also a helpful clue