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Common Themes in Hospital Dermatology:
From Morphology to Management
Lindy P. Fox MD Associate Professor of Clinical Dermatology Director, Hospital Consulta�on Service University of California, San Francisco
DISCLOSURE OF CONFLICTS OF INTEREST
Lindy P. Fox, MD
I have no relevant conflicts of interest to disclose.
I will be discussing off-‐label uses of medica�ons.
Goals of this talk
Present common morphologies that arise during inpa�ent consulta�ons
Generate a working differen�al diagnosis
Use cases to demonstrate key teaching points about diagnosis or management
Morphologies 1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Morbilliform
Drug erup�on Viral exanthem Acute Gra� vs. Host Disease Erup�on of lymphocyte recovery Engra�ment syndrome Toxic erythema of chemotherapy Toxin mediated erythema RARE: disseminated histoplasmosis, cryptococcosis, coccidioidomycosis
Acute GVHD
Timing (average 25d) – Hyperacute (within first 14 d post HSCT)
Morbilliform erup�on – Perifollicular accentua�on – Acral distribu�on
Ears, hands, periungual areas
Associated nausea, vomi�ng, diarrhea, hyperbilirubinemia
Viral Reac�va�on and GVHD
HHV6 appears to play a role in poten�a�ng acute and chronic GVHD – Associated with poorer outcome
CMV seroposi�vity in donor or recipient is associated with cGVHD
EBV, HHV7 may occur in the post transplant se�ng, but has NOT been shown to be associated with GVHD
de Pagter et al Biol Blood Marrow Transplant. 2008; 14:831-9 Zerr et al. Clin Infect Dis 2005; 40: 932-40 Kitamura et al. JAAD. 2008; 58: 802-9
Papules/ Papulonodules
Deep fungal (o�en purpuric, necro�c) Sep�c emboli Sweets syndrome Leukemia/lymphoma cu�s Kaposis sarcoma Cutaneous metastasis Sarcoidosis
Purple Plumb
Lymphoma Kaposi Sarcoma Bacillary angiomatosis Melanoma Cutaneous metastasis
Celluli�c Plaques
Celluli�s Stasis derma��s Deep fungal infec�on (especially Cryptococcus) Carcinoma erysipeloides Neutrophilic disease (Sweets, NEH) Acute inflammatory edema (ICU)
Cryptococcal celluli�s s/p liver transplant for HCV
Celluli�s-‐ most common presenta�on in OTR
Bilateral disease is common
Look for extracutaneous infec�on
Palpable purpura
Small or mixed (small and medium) vessel vasculi�s
Secondary hemorrhage into papular process Leukemia/lymphoma cu�s Sweet’s syndrome Cutaneous reac�on to cytarabine
Intravascular Vascular
Thrombotic Embolic
Re�form Purpura DDX
Re�form Purpura Vascular-‐ infiltra�on in vessel wall
Infectious – Bacterial
Meningococcemia Gonococcemia Staphylococcus E. coli Klebsiella Pseudomonas
– Fungal Mucor/ Rhizopus Aspergillus Candida Fusarium
– Other Strongyloidiasis Lucio (leprosy)
Vasculitis – IgA vasculitis – Connective tissue disease
vasculitis – Mixed cryoglobulinemia – Microscopic polyangiitis – Wegener granulomatosis – Churg-Strauss syndrome – Polyarteritis nodosa – Levamisole/cocaine
Calciphylaxis Oxalosis
Re�form Purpura Thrombo�c
Abnormal coagulation Thrombotic vasculopathy Platelet Plugging Cold-related Red cell occlusion Phlegmasia Levamisole/cocaine Calciphylaxis!!
Re�form Purpura Emboli
Emboli- DDX – Cholesterol – Cardiac
Marantic endocarditis Septic endocarditis Libman-Sachs endocarditis Atrial myxoma
– Air – Fat
Clinical – Few lesions – Acral/distal – Cholesterol
Post procedure – Air/Fat
Upper extrem> lower extrem
Concomitant Thrombosis and Vasculi�s
Cyroglubulinemia Sep�c vasculi�s Levamisole exposure
Sclerodermoid
Carcinoma en cuirasse Radia�on derma��s (chronic) GVHD (chronic) Nephrogenic systemic fibrosis Scleroderma Paraffinoma Lipodermatosclerosis Scleredema Scleromyxedema
Subcutaneous nodules
Sweets syndrome Panniculi�s Deep fungal infec�on Nodular tuberculid/erythema induratum Polyarteri�s nodosa
JAAD 2005; 53:S154-‐6
cPAN Papulonodules on the legs
Histopathology -‐dermohypodermal junc�on – Muscular walled artery
Evaluate for associated infec�ons/medica�ons
Evaluate for APLA
Treat with immunosuppression – ? Role for an�coagula�on
Papulonodules on the legs
Histopathology -‐dermohypodermal junc�on – Muscular walled artery OR vein
PPD or quan�FERON® Gold posi�ve
Treat with 3-‐4 drug therapy
Nodular Tuberculid vs.
Necro�c papules/plaques
Deep fungal infec�on Sep�c emboli Pyoderma gangrenosum Ecthyma gangrenosum Calciphylaxis Malignant syphilis Malignancy
Calciphylaxis analagous to a “myocardial infarction” occurring in the skin
Vascular stenosis (medial arterial calcification and subintimal thrombosis) develops slowly
+ Thrombosis (acute event)
= Calciphylaxis
(tissue ischemia stellate purpuric plaques)
Weenig. J Am Acad Dermatol 2008;58:458-71
Calciphylaxis = cutaneous “MI” Implications for treatment
Ca2+, PO4, PTH- sodium thiosulfate, cinacalcet, lanthanum, bisphosphonates, parathyroidectomy, etc NEED TO ADDRESS THROMBUS- tPA Warfarin with caution, if at all
Inhibits activation of matrix G1a protein (Vitamin K dependent protein that inhibits vascular calcification) Reduced functional protein C levels in ESRD patients with calciphylaxis (normal in ESRD without calciphylaxis) Cases of warfarin induced calciphylaxis reported
Ulcers
Venous insufficiency ulcers Pyoderma gangrenosum Deep fungal infec�on Chronic viral infec�ons (HSV, CMV)
Vesiculobullous
Autoimmune bullous disorder Drug induced bullous disorder (esp SJS, linear IgA to vancomycin)
Herpe�c (HSV or VZV, localized or disseminated) Contact derma��s Miliaria crystallina Coma bulla Edema bulla
Drug-Induced Linear IgA Disease� Common causes
– Vancomycin�– Penicillins�– Cephalosporins�– Captopril�
Others – Amiodarone – Sulfamethoxazole – Diclofenac – Furosemide – Glyburide – GCSF – IFN – Lithium – Phenytoin – Piroxicam – Rifampin
Pustules
Bacterial infec�on Sep�c emboli Acute generalized exanthematous pustulosis Drug induced hypersensi�vity reac�on Pustular psoriasis Steroid acne Folliculi�s-‐ non-‐bacterial (Demodex, Pityrosporum, eosinophilic) Candidiasis-‐ localized and disseminated
Candidemia Purpura with pale, sometimes pustular, centers
Image courtesy of Peter Heald, MD
Erythema, macular (acute)
Toxin mediated erythema Drug erup�on (esp toxic epidermal necrolysis, acute generalized exanthematous pustulosis)
GVHD (acute) Viral exanthem Kawasaki disease
Erythroderma, subacute to chronic
CTCL Atopic derma��s Drug erup�on (Drug induced hypersensi�vity) Psoriasis Hyperkerato�c scabies
Hypersensi�vity Reac�ons “DRESS”-‐ Drug reac�on w/ eosinophilia and systemic symptoms “DIHS”= Drug induced hypersensi�vity syndrome Begins 2-‐ 6 weeks a�er medica�on started Viral reac�va�on (esp HHV6) appears to play a real role
Long term toxici�es include: – Thyroid-‐ check TSH monthly for 6 months – Cardiac-‐ low EF, give pa�ents ER warnings
Hypersensi�vity Reac�ons Each class of drug causes a slightly different clinical picture Facial edema characteris�c of all An�convulsants:
– 3 weeks – Atypical lymphocytosis, hepa�c failure
Dapsone: – 6 weeks – No eosinophilia
Allopurinol: – 7 weeks – Elderly pa�ent on thiazide diure�c – Renal failure – Requires steroid sparing agent to treat (avoid azathioprine)
Erythroderma, erosive
Toxic epidermal necrolysis GVHD (TEN-‐like) Linear IgA bullous dermatosis (drug induced) Acute generalized exanthematous pustulosis Staphylococcal scalded skin syndrome Toxic erythema of chemotherapy
Ulcers, oral and genital
HSV CMV EBV Aphthae Post transplant lymphoprolifera�ve disease (rare) Sweets syndrome (rare) Malignancy
Case
28 M with advanced HIV Found down in his home with facial necrosis
4-‐6 weeks with slowly increasing, painful lesion on face
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
HHV6 plays a role in GVHD and hypersensi�vity drug erup�ons
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
“Purple Plumb”: Lymphoma Kaposi Sarcoma Bacillary angiomatosis Melanoma Cutaneous metastasis
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Celluli�s is a common presenta�on of cryptococcosis in the immunosuppressed pa�ent
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
The cutaneous reac�on to cytarabine may mimic vasculi�s
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Levamisole exposure classically presents with concomitant vasculi�s and thrombosis
Inpa�ent Dermatology From Morphologies to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Paraffinomas may develop many years a�er ini�al exposure and may travel to non-‐injected areas
Inpa�ent Dermatology From Morphologies to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Rule out TB before making a defini�ve diagnosis of PAN
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
When trea�ng calciphylaxis address Ca2+/PO4 and thrombus
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Chronic HSV is common in immunosuppressed, bedridden pa�ents
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Drug-‐induced linear IgA disease may be mucosal predominant
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Disseminated candidiasis presents as purpura with pale, pustular centers
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Eosinophilia is a common finding in toxin mediated erythemas
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Watch for late cardiac involvement in pa�ents with drug hypersensi�vity
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
Toxic erythema of chemotherapy is a rare cause of widespread cutaneous necrosis
Inpa�ent Dermatology From Morphology to Management
1. Morbilliform 2. Papules/Papulonodules 3. Celluli�c plaques 4. Palpable purpura 5. Re�form Purpura 6. Sclerodermoid 7. Subcutaneous nodules 8. Necro�c papules/plaques 9. Ulcers 10. Vesiculobullous 11. Pustules 12. Erythema, macular (acute) 13. Erythroderma, subacute to chronic 14. Erythroderma, erosive 15. Ulcers, oral and genital
More than one infec�on or e�ology may account for the clinical picture, especially in immunocompromised pa�ents
Inpa�ent Dermatology Rules to Live By
If more than one morphology is present, work up each one separately
Most cases require some degree of clinicopathologic correla�on
Think of the zebras-‐ this is how you will make the rare diagnosis