Cutaneous Conditions Associated with Systemic Disease

Transcription

1 Cutaneous Conditions Associated with Systemic Disease Johnnie M Woodson, M.D., F.A.A.D. Assistant Professor of Dermatology University of Nevada School of Medicine Director of J. Woodson Dermatology & Associates, LTD

2 Objectives Identified common dermatologic conditions Addressed medical and surgical options for the management of skin diseases Described how new data and therapeutic techniques can be incorporated in the individualized treatment of patients Described the pathophysiology cutaneous conditions associated with systemic disease Outlined the benefits and limitations of new and emerging treatments for cutaneous manifestations of systemic disease Johnnie M Woodson, M.D.

3 Can You Identify This Condition?

4 Can You Identify This Condition?

5 Can You Identify This Condition?

6 Stevens-Johnson Syndrome Epidemiology Any age, greater than 40 years old, equal sex Risk factors: SLE, HLA-B 12, HLA-B1502-HLA-B5801 in Han Chinese, HIV/AIDS Etiology & Pathogenesis Polyetiologic reaction pattern, Drugs leading factor SJS 50% drug exposure also chemicals, mycoplasma pneumonia, viral infections, immunizations Cytotoxic immune reaction, destruction of keratinocytes expressing foreign drug related antigens FAS and FAS ligand interactions therefore apoptosis Johnnie M Woodson, M.D.

7 Stevens-Johnson Syndrome& Toxic Epidermal Necrolysis Overview TEN is Maximal variant of SJS differing only by body surface involvement 50% of TEN diffuse erythema to immediate necrosis and epidermal detachment SJS less than 10% epidermal detachment TEN greater than 30% epidermal detachment General Finding TEN fever higher, severe pain Mentally alert, severe pain,>120 pulse, low BP Tubular necrosis, ARF, resp and GI erosions Johnnie M Woodson, M.D.

8 Stevens-Johnson Syndrome& Toxic Epidermal Necrolysis Clinical Manifestations 1-3 wks after exposure, fever, malaise, arthragias Tenderness, conjunctival(85%corneal erosions), itching, burning, photophobia, necrotic epidermis, flaccid blisters Medication Allopurinol, phenobarbital, NSAIDS, aminopenicillins Sulfamethoxazole, phenobarbital Course & Prognosis <4 days,related to necrosis extent, transcutaneous fluid loss, electrolyte abnormalities Prerenalazotemia, bacterial colonization, hypermetabolicstate, interstitial pneumonitis Mortality TEN 30%,SJS 5-12% Johnnie M Woodson, M.D.

9 Stevens-Johnson Syndrome& Toxic Epidermal Necrolysis Lab Exams Anemia, lymphopenia, neutropenia (poor prognosis), serum urea Basal keratinocytes necrosis, full thickness epidermal necrosis Differential Diagnosis Drug eruption,scarlet fever,phototoxic eruption,toxic shock synd., Thermal burn,staph scaled skin syndrome,exfoliative Derm. Management Drug withdrawal, intermediate or intensive care unit, IV fluids and electrolytes Systemic glucocorticosteroids, High dose IV immunoglobulins, Oropharyngealinvolvement suction prevent aspiration pneumonitis Treat complicating infections, sepsis Johnnie M Woodson, M.D.

10 Can You Identify This Condition?

11 Can You Identify This Condition?

12 Can You Identify This Condition?

13 Vitiligo Course & Prognosis Rapid onset, stability period, slow progression 30% spontaneous repig. Tx. of associated Ds. no impact Management Sunscreen,coverup Repigmentation-topical glucocorticoids, topical calcineurininhibitors, PUVA, Excimerlaser (308 nm), narrow band UVB(311nm) Depigmentation-monobenzylether of hydroquinone 20% Epidemiology Both sexes equal, any age,1% pop. social stigma darker skinned persons,>30% genetic Thyroid Ds., diabetes, GI (ie.pernicious anemia) Johnnie M Woodson, M.D.

14 Vitiligo Differential Diagnosis Pityriasisalba, P.alba, Leprosy, Mycosisfungoides, chemicals, Nevusanemicus, Nevusdepigmentosus, Tuberous sclerosis Lab Exams Wood lamp, Bx., Electron micron (no melanosomes,t4,tsh,fasting blood glucose,cbc) Pathogenesis & Clinical 3 theories-autoimmune,neurogenic,self-destruct Melanocyte destruction Koebnerization,macules,patches,focaland generalized Associated-white hair, alopecia areata, solar keratoses, SCC Sent from my iphone Johnnie M Woodson, M.D.

15 Can You Identify This Condition?

16 Can You Identify This Condition?

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18 Kaposi Sarcoma HHV-8 Classic or European, African-endemic, Immunosuppression-associated, HIV/AIDS-associated Not true malignancy, reactive polyclonal proliferation, lesions produce growth factors Not known how HHV-8 induces/promotes endothelial cell proliferation Johnnie M Woodson, M.D.

19 Can You Identify This Condition?

20 Can You Identify This Condition?

21 Can You Identify This Condition?

22 Can You Identify This Condition?

23 Diabetic Dermopathy AcanthosisNigerians,drug reaction (hypoglycemic agents,insulin), Calciphylaxis, perforating disorders, Bullae, Dermopathy, Granulare annulare, infections, Necrobiosis lipoidica, Peripheral neuropathy Peripheral vascular Ds.-Small vessel (microangiopathy),large vessel Johnnie M Woodson, M.D.

24 Identify This Unknown!

25 Kaposi Sarcoma

26 Identify This Unknown!

27 Diabetic Dermopathy

28 Identify This Unknown!

29 Stevens-Johnson syndrome

30 Identify This Unknown!

31 Vitiligo

32 Summary Identified common dermatologic conditions Addressed medical and surgical options for the management of skin diseases Described how new data and therapeutic techniques can be incorporated in the individualized treatment of patients Described the pathophysiology cutaneous conditions associated with systemic disease Outlined the benefits and limitations of new and emerging treatments for cutaneous manifestations of systemic disease Johnnie M Woodson, M.D.