common themes in hospital dermatology: from morphology ... pda- syllabus common...dermatology from...

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

foxli@derm.ucsf.edu

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

  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

HHV6 plays a role in GVHD and hypersensi�vity drug erup�ons

“Purple Plumb”:   Lymphoma   Kaposi Sarcoma   Bacillary angiomatosis   Melanoma   Cutaneous metastasis

Celluli�s is a common presenta�on of cryptococcosis in the immunosuppressed pa�ent

The cutaneous reac�on to cytarabine may mimic vasculi�s

Levamisole exposure classically presents with concomitant vasculi�s and thrombosis

Inpa�ent Dermatology From Morphologies to Management

Paraffinomas may develop many years a�er ini�al exposure and may travel to non-‐injected areas

Inpa�ent Dermatology From Morphologies to Management

Rule out TB before making a defini�ve diagnosis of PAN

When trea�ng calciphylaxis address Ca2+/PO4 and thrombus

Chronic HSV is common in immunosuppressed, bedridden pa�ents

Drug-‐induced linear IgA disease may be mucosal predominant

Disseminated candidiasis presents as purpura with pale, pustular centers

Eosinophilia is a common finding in toxin mediated erythemas

Watch for late cardiac involvement in pa�ents with drug hypersensi�vity

Toxic erythema of chemotherapy is a rare cause of widespread cutaneous necrosis

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

common themes in hospital dermatology: from morphology ... pda- syllabus common...dermatology from...

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