Cover

Contents

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Preface

Welcome to the first edition of Handbook of Dermatology: A Practical Manual, a pocket guide designed for practicing dermatologists, dermatology residents, medical students, and physicians in other fields who may be interested in dermatology. Written and edited by former residents and attending physicians in the Division of Dermatology at Washington University School of Medicine, this book is based on an in-house resident handbook which has been used by our department for the past five years. Our goal was to compile and consolidate need-to-know dermatologic information for daily use in patient care and resident and fellow education. As such, it represents the indispensable pocket-sized quick reference which we had wanted during our training and which we now use in our practices.

Currently, there are multiple in-depth dermatology textbooks and atlases, most of which are too bulky to be carried around in the clinic. Our manual concisely presents data in outline, bullet-point, and table formats such that information is manageable and easily retrievable. The compact design is lightweight, allowing information to be accessible in seconds during clinics, facilitating patient care. We have tried to balance space limitations with the need to cover a subject in sufficient detail.

Our manual has three main sections – medical dermatology, surgical dermatology, and pharmacology/treatment. Each section is designed to provide the reader with up-to-date, comprehensive yet concise information for patient care. In addition to core material, we sought to consolidate the information which we found ourselves most often looking up, which our attendings most frequently quizzed us on, and which were emphasized on the dermatology board exam. The manual consolidates the dermatologic algorithms, protocols, guidelines, staging and scoring systems which we find most essential. Each section is designed for easy reference, with tabular and graphic information throughout. The diseases covered are those which we frequently encountered in clinic, on call, during teaching conferences, and on board exams.

We hope you will find this manual helpful to you in providing care to your patients. We welcome your input as this manual continues to evolve.

Margaret W. Mann

David R. Berk

Daniel L. Popkin

Susan J. Bayliss

Dedication

We wish to express our thanks to the many people who have inspired us to write this book and supported us in our careers. Special thanks to the following physicians who contributed to the manuscript: Drs. Paul Klekotka, Alison Klenk, and Neel Patel – who helped make the prototype possible – without you, this manual would never have happened; Drs. Milan Anadkat, Grace Bandow, Amy Cheng, Michael Heffernan, Yadira Hurley, and David Smith for their valuable contributions; Drs. Stacey Tull and Quan Vu for the beautiful drawings; Drs. Senait Dyson, Kristen Kelly, and Anne Lind for their proofreading and comments; and finally Drs. Lynn Cornelius, Arthur Eisen, and all the faculty in the Division of Dermatology at Washington University for their support and encouragement.

Margaret Mann would like to thank her parents and her ever-patient husband, Daniel, for all the love and support over the years.

David Berk wishes to thank his family, especially his wife Melissa and his parents, for their constant support and patience.

Daniel Popkin would like to thank his parents and his wife Margaret.

Susan Bayliss wishes to thank her grandsons Cai and Eli Kenemore, and her daughters Elizabeth Kenemore and Meredith Mallory for all the joy they constantly bring her.

Abbreviations

ACDallergic contact dermatitis
ADautosomal dominant
AFBacid fast bacilli
AKactinic keratoses
ANAanti-nuclear antibody
ANCAanti-neutrophilic cytoplasmic antibody
APSantiphospholipid syndrome
ARautosomal recessive
ASOantistreptolysin O titer
asxasymptomatic
BCCbasal cell carcinoma
BIDtwice daily
BMbone marrow
BMPbasic metabolic panel
BMZbasement membrane zone
BPbullous pemphigoid
BPblood pressure
Bxbiopsy
Ca++calcium
CADcoronary artery disease
CBCcomplete blood count
CCBcalcium channel blocker
CFcystic fibrosis
cGVHDchronic graft-versus-host disease
CH50total hemolytic component
CMPcomplete metabolic panel
CMVcytomegalovirus
CNcranial nerve
CNScentral nervous system
CPcicatricial pemphigoid
CRcreatinine
CRFchronic renal failure
CRPC-reactive protein
Cryocryoglobulinemia
CTcomputed tomography
CTCLcutaneous T-cell lymphoma
CTDconnective tissue disease
CVAcerebral vascular accident
Cxculture
CXRchest X-ray
DCNdoxycycline
DEJdermal–epidermal junction
DFdermatofibroma
DFAdirect fluorescent antibody
DFSPdermatofibrosarcoma protuberans
DHdermatitis herpetiformis
DHEA-Sdehydroepiandrosterone sulfate
DIdiabetes insipidus
DIFdirect immunofluorescence
DMdermatomyositis
DM2diabetes mellitus type II
Dsgdesmoglein
Dzdisease
EBAepidermolysis bullosa acquisita
EBVEpstein–Barr virus
EDSEhlers–Danlos syndrome
EEDerythema elevatum diutinum
EKGelectrocardiogram
EMelectromicroscopy
EMGelectromyogram
ENAextractable nuclear antigen
eoseosinophils
ESRerythrocyte sedimentation rate
ETOHalcohol
Ffever
FLPfasting lipid panel
FMFFamilial Mediterranean fever
G6PDglucose-6-phosphate dehydrogenase
GAgranuloma annulare
GFgranuloma faciale
GIgastroenterology
GVHDgraft-versus-host disease
h/ohistory of
HAheadache
HBVhepatitis B virus
HCVhepatitis C virus
HDLhigh density lipoprotein
Hephepatitis
HSMhepatosplenomegaly
HSVherpes simplex virus
HTNhypertension
IBDinflammatory bowel disease
IIFindirect immunofluorescence
ILintralesional
IMintramuscular
IVintravenous
IVIGintravenous immunoglobulin
KOHpotassium hydroxide
LANlymphadenopathy
LCHLangerhans Cell Histiocytosis
LCVleukocytoclastic vasculitis
LDHlactate dehydrogenase
LDLlow density lipoprotein
LElupus erythematosus
LFTliver function test
LNlymph nodes
LPlichen planus
MCNminocycline
MCTDmixed connective tissue disease
MENmultiple endocrine neoplasia
MFmycosis fungoides
MMmalignant melanoma
MRmental retardation
MRImagnetic resonance imaging
MTXmetrotrexate
nlnormal
NLDnecrobiosis lipoidica diabeticorum
NSAIDsnon-steroidal anti-inflammatory drugs
NXGnecrobiosis xanthogranuloma
OCPoral contraceptive pill
OTCover the counter
PANpolyarteritis nodosa
PCNpenicillin
PCRpolymerase chain reaction
PCTporphyria cutaneous tarde
PETpositron emission tomography
PFTspulmonary function tests
PIHpost inflammatory hyperpigmentation
PMLEpolymorphous light eruption
PMNspolymorphonuclear leukocytes
poper oral
PPDtuberculosis skin test
PT/PTTprothrombin time/ partial thromboplastin time
PUVApsoralen + ultraviolet A
PVpemphigus vulgaris
QDonce a day
QHSevery night
QODevery other day
RArheumatoid arthritis
RFrheumatoid factor
ROSreview of systems
RPRrapid plasma reagin (screening test for syphilis)
Rxnreaction
SCCsquamous cell carcinoma
SCMsternocleidomastoid
SJSStevens–Johnson syndrome
SLNsentinal lymph node
SPEPserum protein electrophoresis
SQsubcutaneous
SSsystemic sclerosis
SSRIselective serotonin reuptake inhibitor
SSSSstaphylococcal scalded skin syndrome
Sxssymptoms
szsseizures
TBtuberculosis
TBSAtotal body surface area
TCAtricyclic antidepressant
TCNtetracycline
TENtoxic epidermal necrolysis
TGtriglycerides
TIBCtotal iron binding capacity
TIDthree times a day
TNFtumor necrosis factor
TSHthyroid stimulating hormone
Txtreatment
UAurinalysis
UPEPurine protein electrophoresis
VLDLvery low density lipoprotein
WBCwhite blood cell count
WLEwide local excision
XDx-linked dominant
XRx-linked recessive
X-RXNcross reaction
XPxeroderma pigmentosa
yoyear old

Part 1

General Dermatology

Work-up Quick Reference

CTCLCBC, LDH, Sezary prep, flow cytometry, CXR
VasculitisCBC, ESR, BMP, UA, consider drug-induced vasculitis, further testing guided by ROS and type of vasculitis suspected (CRP, SPEP, UPEP, cryo, LFT, HBV, HCV, RF, C3, C4, CH50, ANA, ANCA, ASO, CXR, guaiac, cancer screening, HIV, ENA, echo, electromyogram, nerve conduction, biopsy (nerve, respiratory tract, kidney))
UrticariaIn children, often due to Strep Consider ASO, Rapid Strep
Urticarial vasculitisCBC, UA, ANA, C1, C3, C4, CH50, anti-C1q, ESR
LupusANA, ENA (Ro/La), CBC, BMP, ESR, C3, C4, UA, G6PD
SarcoidBMP, Ca++, CXR, PFTs, G6PD, EKG, ophtho consult
AngioedemaCBC, C1 est inhib, C1,C2,C4; Hereditary: C1-nl; C2,C4 and C1 est inhib-↓ (C1est inhib levels may be nl but non-functional); Acquired: C1--↓; C2,C4 and C1 est inhib-↓
PhotosensitivityENA (Ro/La)
HypercoagulableCBC, PT/PTT, Factor V Leiden,
Anti-phospholipid Ab, protein C&S, prothrombin G20210A, anti-thrombin III activity, homocysteine
TENTx: IVIG 2–4 gm/kg (total dose, divided over 2–5 days) use GammaGard if possible (low IgA) Check for IgA deficiency. See TEN protocol p. 282–283

Direct immunofluorescence – where to biopsy?

Source:

DiseasesWhere to biopsy
LE, MCTD, PCT, LP, VasculitisErythematous border of active lesion/involved skin (avoid old lesions, facial lesions, ulcers)
Pemphigus group, Pemphigoid group, Linear IgAErythematous perilesional skin (avoid bullae, ulcers, erosions)
DHNormal-looking perilesional skin (0.5–1 cm away)
Lupus bandUninvolved, non-photoexposed skin (buttock)

False positive/negative DIFs

False negative in BP: (1) low yield of biopsy on distal extremity (esp. legs) (controversial), (2) predominantly IgG4 subclass of auto-antibody (poorly recognized on DIF)

False positive in LE: chronically sun-exposed skin of young adults

To increase DIF yield: transport in saline (reduces dermal background) – cannot do DIF on formalin-fixed specimen

Biopsy for GVHD

Biopsy for GVHD vs. lymphocyte recovery vs. drug eruption

Marra DE et al. Tissue eosinophils and the perils of using skin biopsy specimens to distinguish between drug hypersensitivity and cutaneous graft-versus-host disease. JAAD. 2004; 51(4):543–545.

Zhou Y et al. Clinical significance of skin biopsies in the diagnosis and management of graft vs host disease in early postallogeneic bone marrow transplantation. Arch Derm. 2000; 136(6):717 –721.

The Dermatologic Differential Algorithm

1. Is it a rash or growth?

2. If it is a rash, is it mainly epidermal, dermal, subcutaneous, or a combination?

3. If the rash is epidermal or a combination, try to define the characteristics of the rash. Is it mainly papulosquamous? Papulopustular? Blistering?

4. After defining the characteristics, then think about causes of that type of rash (CITES MVA PITA):
Congenital, Infections, Tumor, Endocrinologic, Solar related, Metabolic, Vascular, Allergic, Psychiatric, Iatrogenic, Trauma, Autoimmune. When generating the differential, take the history and location of the rash into account.

5. If the rash is dermal or subcutaneous, then think of cells and substances that infiltrate and associated diseases (histiocytes, lymphocytes, mast cells, neutrophils, metastatic tumors, mucin, amyloid, immunoglobulin, etc.).

6. If the lesion is a growth, is it benign or malignant in appearance? Think of cells in the skin and their associated diseases (keratinocytes, fibroblasts, neurons, adipocytes, melanocytes, histiocytes, pericytes, endothelial cells, smooth muscle cells, follicular cells, sebocytes, eccrine cells, apocrine cells, etc.).

Alopecia Work-Up

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Associations

1. Medications? Telogen effluvium-associated meds: anticonvulsants, anticoagulants, chemotherapy, psychiatric meds, antigout, antibiotics, beta-blockers

2. Hormones (pregnancy, menstruation, OCPs)?

3. Hair care/products?

4. Diet (iron or protein deficiency)?

5. Systemic illness/stress?

Cicatricial or non-cicatricial?

1. Non-cicatricial: Is hair breaking off or coming out at the roots? Is hair loss focal or diffuse?

BreakageComing out at roots
Hair shaft defects, trichorrhexis nodosa, hair care (products, traction, friction), tinea capitis, trichotillomania, anagen arrest/chemotherapyTelogen effluvium, alopecia areata, androgenetic, syphilis, loose anagen, OCPs
Focal lossDiffuse loss
Hair care (traction), tinea capitis, trichotillomania, alopecia areata, syphilis, hair shaft defectsTelogen effluvium, anagen effluvium, androgenetic alopecia, hair shaft defects

2. Cicatricial: Is biopsy predominantly lymphocytic, neutrophic, or mixed?

Classification of cicatricial alopecia

LymphocyticNeutrophilicMixed
• LPP (including classic, frontal fibrosing, Graham-Little)• Folliculitis decalvans• Folliculitis/acne keloidalis
• Dissecting cellulitis/folliculitis• Folliculitis/acne necrotica
• Erosive pustular dermatosis
• Central centrifugal
• Alopecia mucinosa
• Keratosis follicularis spinulosa decalvans
• Chronic cutaneous LE
• Pseudopelade (Brocq)

Adapted from Olsen EA et al. North American hair research Society Summary of sponsored Workshop on Cicatricial Alopecia. J Am Acad Dermatol 2003; 48:103–10.

Structural hair abnormalities classified by hair fragility

Increased fragilityNo increased fragility
Trichorrhexis invaginata (bamboo)Loose anagen
MonilethrixPili annulati
Trichorrhexis nodosaUncombable hair (spun-glass)
TrichothiodystrophyWoolly hair
Pili tortiPili bifurcati
Pili multigemini
Acquired progressive kinking

Adapted from Hordinsky MK. Alopecias. In: Bolognia JL, Jorizzo JL, Rapini RP. Dermatology Vol. 1, Mosby; London. 2003, p. 1042.

Pull test and hair mount

1. Pull test – reveals telogen hairs in telogen effluvium, and anagen hairs in loose anagen syndrome. Helpful to identify active areas in cicatricial alopecia or alopecia areata.

2. Hair mount

DisorderHair mount findings
MonilethrixBeaded, pearl necklace, knots
Trichorrhexis nodosaFractures, paint brushes
Trichorrhexis invaginataBamboo/golf tee hair
TrichothiodystrophyTrichoschisis, tiger-tail on polarization
Loose anagenAnagen hairs with ruffled cuticles and curled ends and lacking root sheaths
Pili tortiFlattened, 180º irregularly spaced twists
Uncombable hairPili canaliculi et trianguli, triangular in cross section
Pili annulatiAbnormal dark bands on polarization, air bubbles in cortex
ElejaldePigment inclusions
GriscelliPigment clumping
MenkesMultiple – pili torti, trichorrhexis nodosa, trichoptilosis

Hair count – helpful in quantifying hair loss

1. Daily hair count: collect all hairs before shampooing (Normal is <100)

2. 60 second hair count: comb for 60 seconds (Normally yields 10–15 hairs).

Biopsy – helpful in persistent alopecia, may help determine if an alopecia is cicatricial

1. 4 mm punch biopsy for horizontal sectioning

a. Hair count: Caucasians should have ~40 total hairs (20–35 terminal, 5–10 vellus) while African Americans should have fewer (18 terminal, 3 vellus) – assess catagen vs. telogen at isthmus level and terminal vs. vellus at infundibular level.

b. Look at terminal to vellus* hair ratio:

Normal>4 (~7–10T: 1V)
Androgenic<2–4T: 1V

c. Look for characteristic findings:

Alopecia areata: lymphocytes around anagen bulbs

Trichotillomania: pigment casts, trichomalacia, catagen hairs, dermal hemorrhage

Androgenetic alopecia: miniaturized follicles.

Labs – TSH, CBC, iron, TIBC, ferritin; consider RPR, ANA; check hormones (testosterone, DHEAS, prolactin) if irregular menses, infertility, hirsutism, severe acne, galactorrhea, or virilization.

figure

* Vellus hairs – true vellus hairs (small and lack melanin) and miniaturized terminal hairs are histologically identical.

Management of Acne

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