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Clinical Pearls in Dermatology Lisa H Scatena, M.D. F.A.A.D. Rocky Mountain Dermatology, Boulder Assistant Clinical Professor Dermatology & Internal Medicine University of Colorado, Denver 6 February 2009. What Can We Glean from the Largest Organ in the Body?.

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Clinical Pearls in DermatologyLisa H Scatena, M.D. F.A.A.D.Rocky Mountain Dermatology, BoulderAssistant Clinical ProfessorDermatology & Internal Medicine University of Colorado, Denver6 February 2009

what can we glean from the largest organ in the body3
What Can We Glean from the Largest Organ in the Body?

Earlier detection, diagnosis and treatment of systemic diseases.

where have we been and where are we going
Where Have We Been and Where Are We Going?
  • 2006- The skin biopsy
  • 2008- Common dermatoses
  • 2009- Systemic diseases and skin findings
  • 2010- ?
learning objectives
Learning Objectives
  • Recognize newly described skin diseases
  • Review newer skin signs of well-established systemic diseases
  • Based upon skin findings, make appropriate diagnostic and therapeutic decisions for systemic diseases
slide7

Case 1: A 54 year old man with diabetes mellitus and chronic renal failure complains of swelling, tightness and darkening of the skin on his arms and legs. His symptoms have developed over the past 2-3 weeks following a failed kidney transplant and imaging. Upon further questioning, this patient is most likely to also complain of:

case 1 upon further questioning this patient is most likely to also complain of

0

Case 1: Upon further questioning, this patient is most likely to also complain of:
  • Burning and itching in his arms and legs
  • Changes in his vision
  • Colicky abdominal pain
  • Difficulty swallowing
  • Oral ulcers
nephrogenic fibrosing dermopathy
Nephrogenic Fibrosing Dermopathy
  • 15 patients with renal disease
  • Extensive thickening and hardening of the skin with brawny hyperpigmentation
  • Nearly all had extremity lesions, tendency to spare the trunk
  • Some had diffuse thickening, others papules or subcutaneous nodules

Cowper et al. Lancet 2000; 356:1000

nephrogenic fibrosing dermopathy14
Nephrogenic Fibrosing Dermopathy
  • Fibrosis may lead to calcification and dermal ossification
  • Extension from skin and subcutaneous tissue into the underlying fascia and muscle
  • Flexion contractures continue to worsen with resultant severe disablility
  • Increased mortality
nephrogenic fibrosing dermopathy16
Nephrogenic Fibrosing Dermopathy
  • The dominant cell is a dual staining CD34-procollagen fibrocyte corresponding to a circulating cell that expresses markers of both connective tissue cells and leukocytes
  • Aberrant fibrocyte recruitment, activation or proliferation the cause? Possibly related to ischemia

Curr Opin Rheumatol 2003, 15: 785

nephrogenic fibrosing dermopathy18
Nephrogenic Fibrosing Dermopathy
  • 33 patients in St Louis with NFD
    • Most had received dialysis
    • Only 1 patient had not been exposed to gadolinium
    • 4/33 had NOT been exposed to gadolinium for more than a year (range: 16-68 months)
  • Gadolinium exposure has some causative role in NFD.

CDC NFD associated with exposure to gadolinium-containing contrast agents-St Louis, Missouri, 2002-2006. MMWR 2007 Feb 23;56;137-41

nephrogenic fibrosing dermopathy19
Nephrogenic Fibrosing Dermopathy
  • Cross sectional study of 186 dialysis patients in Boston
    • 25/186 had NFD skin changes identified (13%)
    • 94% had exposure to Gadolinium
    • Mortality rate among dialysis patients with NFD was statistically greater than “usual” dialysis patients
  • NFD is a predictor of early mortality.

Todd et all. Cutaneous changes of NSF. Predictor of early mortality and associated with gadolinium exposure. Arth Rheum 2007 Sept 28;56:3433.

nephrogenic fibrosing dermopathy21
Nephrogenic Fibrosing Dermopathy
  • Topical steroids, retinoids, and lactic acid, systemic retinoids, and electron beam all tried without effect
  • Individual patients have responded to prednisone,plasmapheresis, IVIG, thalidomide, UV-A1, extracoporeal photopheresis and interferon-alpha, many others have not
  • Improvement of renal function or transplantation may improve NFD, but not always
  • Current favored therapy is PREVENTION

BJD 2005; 152: 531

slide22

PEARL #1Consider alternate imaging processes when possible in patients with renal failure. If a patient must have an MRI, prompt dialysis after MRI is suggested.

slide24

This 63 year old man has been on this medication for years. Not only has he experienced the skin changes below, he has developed arthralgias and a (+) ANCA.

causative agents for his skin changes include

10

Causative agents for his skin changes include:
  • Amiodarone
  • Gold
  • Minocycline
  • Plaquenil
minocycline
Well known agent to result in hyperpigmentation as a result of both melanin and iron deposition

Locations

Blue-black scars

Blue gray legs & forearms

Muddy brown on sun exposed areas

Reported to cause a lupus-like syndrome

(+) ANA, (+) pANCA

Occurs at a greater rate in those patients on minocycline for longer duration of time

Resolves when minocyline is discontinued

Minocycline
slide27

PEARL #2If a positive ROS is elicited for connective tissue disease in minocycline recipients, consider serologic testing including ANCA.

slide30

10

Case 3: A 56 year old woman with arthralgias, malaise, scaling scalp, and intractable itching develops this bizarre eruption. What other findings would you expect on examination?

  • Deep red-violaceous patches on extensor forearms
  • Lichenified, excoriated plaques periumbilical area
  • Scarring alopecia
  • Violaceous tender nodules on shins
dermatomyositis centripetal flagellete erythema
DermatomyositisCentripetal Flagellete Erythema
  • Edematous, erythematous streaks on the trunk and proximal extremities
  • Histology – interface dermatitis, positive direct immunofluorescence
  • Parallels disease activity
  • Only reported in dermatomyositis, not seen in other connective tissue diseases, in a review of 183 patients from one institution

Arch Dermatol 2000; 136: 665

J Rheumatol 1999; 26: 692

Arch Dermatol 2000; 136: 665 J Rheum 1999;26:692

dermatomyositis gingival telangiectases
Dermatomyositis:Gingival Telangiectases
  • Five patients with juvenile DM
  • 1 boy, 4 girls
  • All had similar nail fold telangiectases
  • Other oral findings of DM include edema, erosions, ulcers and white plaques

Arch Dermatol 1999; 135: 1370

what finding is most predictive of an underlying malignancy in dm

10

What finding is most predictive of an underlying malignancy in DM?
  • Cutaneous Necrosis
  • Gottron Papules
  • Itching
  • Mechanics Hands
  • Shawl sign
dermatomyositis cutaneous necrosis
DermatomyositisCutaneous Necrosis
  • Cutaneous necrosis – rare in adults
  • 1990: 5 cases reported in a study of 32 patients with dermatomyositis
    • In 4 of 5 cases, patients had associated malignancy
  • 7 of 10 in a larger series had malignancy
  • Positive predictive value of cutaneous necrosis is 71.4%
  • Cause uncertain, some with antiphospolipid antibodies

Arch Dermatol 2003; 139: 539

dermatomyositis risk of internal malignancy
DermatomyositisRisk of Internal Malignancy
  • Classic dermatomyositis has a definite association with occult malignancy <25%
    • Women: ovarian carcinoma
    • Asian men: nasopharyngeal carcinoma
  • Negative risk factor: Interstitial lung disease
  • Little-to-no increase risk of malignancy in polymyositis

Lancet 2001; 357:85-86

Br J Cancer 2001; 85:41-45

Curr Opin Rheumatol 2000; 12:498-500

dermatomyositis what about cutaneous amyopathic dm
DermatomyositisWhat about cutaneous amyopathic DM?
  • Sontheimer reviewed world literature which reported a total of 300 cases
    • 10% of these were associated with malignancy
  • Mayo Clinic 1976-1994 found 32/746 patients had cutaneous amyopathic dermatomyositis
    • 25% found to have malignancy in 2-10 year follow-up
    • All were women
    • Lung CA, ovarian CA, breast CA, endometrial CA and metastatic adenocarcinoma of unknown primary

Dermatol Clin 2002; 20:387-408

dermatomyositis occult malignancy work up
DermatomyositisOccult Malignancy Work-up
  • Repeat malignancy surveillance measures every 6-12 months for the first 3-5 years following the diagnosis
  • After 5 years, the risk of malignancy returns to that of the general population for that age and sex

Dermatol Clin 2002; 20:387-408

slide43

PEARL #3In the case of a new diagnosis of dermatomyositis in an adult, at a bare minimum, age appropriate screening for malignancy should be performed. Strongly consider imaging if clinically indicated.

slide44
Case 6 month history of this minimally itchy eruption. Started with 1 red patch and spread after application of a prescription medication.
what prescription medication did he put on his skin with this resultant rash

10

What prescription medication did he put on his skin with this resultant rash?
  • Efudex
  • Eucerin Calming Lotion
  • Neosporin ointment
  • Retin-A cream
  • Triamcinolone cream
tinea versicolor
Tinea Versicolor
  • Not really a dermatophyte, caused by Malassezia furfur or pityrosporum ovale
  • As a yeast, considered normal follicular flora
  • As hyphae, results in skin disease
  • Common in summer months
  • Treatment
    • Azoles,selenium sulfide lotions, Zinc pyrithione soap
    • PO Azoles for difficult to treat cases
      • Ketoconazole 400mg doses repeated monthly
      • Itraconazole 200mg qd x7days
      • Fluconazole 400mg once monthly
slide49
64 year old man presents with asymptomatic 1-2mm firm, white-flesh colored bumps all over his face since his 30’s.

5.

  • PMHx: HTN, history of pneumothorax
  • Medications: Lisinopril, ASA
  • Wears sunscreen routinely
  • 5-10 blistering sunburns in his lifetime
our patient and his family members are at risk for

9

Our patient and his family members are at risk for:
  • Adenocarcinoma
  • Anticardiolipin Syndrome
  • Basal Cell Carcinomas
  • Factor V Leiden Deficiency
  • Renal Cell Carcinoma
birt hogg dube syndrome
Birt-Hogg-Dube Syndrome
  • Multiple smooth skin colored to whitish papules
    • Face 60-70%
    • Neck 80%
    • Upper trunk 90%
  • Oral fibromas 25%
  • Acrochordons 30%
  • Lipomas and collagenomas 15%
birt hogg dube syndrome associated abnormalities
Birt-Hogg-Dube SyndromeAssociated Abnormalities
  • Skin papules develop in 30’s
  • Pulmonary cysts and pneumothorax, symptoms begin in late teens or 20s, odds ratio 50.3
  • Renal cancers, onset in 50s, 10% of hereditary renal cancer patients with BHD, odds ratio 6.9, abdominal CT and renal ultrasound
  • Five patients with parotid oncocytomas

JAAD 2000; 43:1120

JAAD 2003; 48:111

birt hogg dube syndrome58
Birt-Hogg-Dube Syndrome
  • Described in 1977 as an autosomal dominant triad of skin findings
    • fibrofolliculomas
    • trichodiscomas
    • acrochordons
  • Penetrance 88% over age 25, gene isolated on 17p, mutation in gene for protein folliculin
  • Understanding of cutaneous tumors, relationship to renal cancer and pulmonary cysts recently understood in the past five years

J Am Acad Dermatol 2003;49:698,717

slide59

PEARL #5If you see a patient with numerous monomorphic firm small papules on his face, ASK about family members. If others in the family have similar bumps, this warrants further evaluation including skin biopsy and renal imaging.

associated disease61

9

Associated disease?
  • Deep fungal infection
  • Diabetes Mellitus
  • Hepatitis C Virus
  • Lupus Erythematosus
  • Sarcoidosis
necrobiosis lipoidica diabeticorum63
Necrobiosis Lipoidica Diabeticorum
  • Women are over represented 3:1 to men
  • <3% diabetics have NLD
    • 80% DM diagnosis preceeds NLD
    • 10% DM FOLLOWS NLD diagnosis
  • Chronic indolent course
  • Complicated by ulceration (35%)
  • Increased rate of microvascular disease in this subset of diabetic patients
slide64

PEARL #6If you see a patient with a waxy yellow atrophic plaque on the anterior tibia, without a diagnosis of diabetes, check for it. If you see a patient with known diabetes and NLD, aggressive management to minimize the risk of microvascular complications is a must.

which of his pre existing conditions has likely contributed to his dermatoses

10

Which of his pre-existing conditions has likely contributed to his dermatoses?
  • Alcohol
  • Cocaine use
  • Diabetes Mellitus
  • Hypertension
  • IVDA
necrolytic acral erythema hepatitis c
Necrolytic Acral ErythemaHepatitis C
  • Ten patients, 4 reports
  • 7 women, 3 men
  • Ages 11-55
  • All hepatitis C positive

Arch Dermatol 2005; 141: 85

necrolytic acral erythema hepatitis c69
Necrolytic Acral ErythemaHepatitis C
  • Tender well-defined, velvety or scaly surfaced dusky red plaques
  • Occasional blisters and erosions
  • Usually involves the dorsal feet, but legs and hands also affected
  • May respond to interferon, ribaviron, or zinc treatment
slide70

PEARL #7A number of cutaneous clues can steer you towards Hepatitis C Virus Infection in the appropriate patients.

slide71

55yo man presents with an indolent onset of thick skin on the back of his neck and shoulders. He also complains of “chipmunk cheeks” which he did not have 6 months ago.

far and away the most common association with his finding is

10

Far and away, the most common association with his finding is:
  • Amyloidosis
  • Dermatomyositis
  • Diabetes Mellitus
  • Hypothyroidism
  • Systemic Lupus Erythematosus
scleredema
Scleredema
  • Characterized by stiffening and hardening of subcutaneous tissues on face, neck, upper back, and chest
  • Variants
    • Type 1:
      • Women primarily
      • Following URI, especially GAS; may involve tongue, pharnyx (self limiting <2y)
    • Type 2:
      • No antecedent illness. MUGAS
    • Type 3:
      • Middle-age men
      • Associated with diabetes mellitus
scleredema74
Scleredema
  • Associated systemic symptoms can include:
    • Serositis, dysarthria, dysphagia, myositis, parotitis, ocular and cardiac abnormalities
  • DDx:
    • Scleroderma, scleromyxedema, cellulitis
  • Treatment:
    • Type 1: self limiting
    • Type 2 and 3: PUVA, cyclophosphamide, corticosteroids
slide76

58 year old man presents with painful genital sores and “thrush” in his mouth. The thrush has not improved with nystatin swish and spit regimen. He recently got new glasses, but he still finds his vision has changed and feels as if something is in his eyes.He wonders if it could be from a lack of Vitamin D because his dermatologist has beaten into him the need for daily sunscreen.

your leading clinical diagnosis is

10

Your Leading Clinical Diagnosis is:
  • Cicatricial Pemphigoid
  • Herpes Simplex Virus
  • Major Apthous Ulcer Disease/Sutton’s Disease
  • Stevens Johnson Syndrome
  • Vitamin D Deficiency
anti epiligrin cicatricial pemphigoid84
Anti-Epiligrin Cicatricial Pemphigoid
  • Mucosal predominant blistering disease
    • Mouth, eye and genital
    • Skin involvement not uncommon
  • Pathogenic IgG antibodies against Laminin 5

Arch Dermatol 1994, 130:1521

cicatricial pemphigoid
Cicatricial Pemphigoid
  • Immunologically distinct immunobullous dieases with scarring
  • Oral (90%), conjunctival (66%),
  • Cutaneous lesions (25%)
  • Circulating autoantibodies targeting hemidesmosomal proteins: BP 180, BP 230 and integrins
    • CP with antibodies directed against Laminin 5 or epiligrin is associated with an increased risk for solid tumors, especially adenocarcinoma.

Vodegel RM, et al JAAD 2003;48:542.

anti laminin 5 cicatricial pemphigoid
Retrospective study of 35 patients, median age 65

10 had solitary solid tumors

lung, stomach, colon, endometrial

Overall relative risk= 6.8; RR 1y after diagnosis = 15.4

8 of 10 developed cancer AFTER blisters

7 of 8, within 14 months of onset

8 of 8diedwithin 21 months of diagnosis

Anti-Laminin 5 Cicatricial Pemphigoid

Lancet 2001; 357: 1850

pearl 8 wear your sunscreen you wouldn t want an autoimmune blistering disorder and a skin cancer

PEARL #8Wear your sunscreen. You wouldn’t want an autoimmune blistering disorder AND a skin cancer.