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Tiêu đề Dermatology Therapy Essentials - Part 9 doc
Tác giả Mallory SB, Stutman HR, Yogalingam G, Hopwood JJ
Trường học University of California, San Francisco
Chuyên ngành Dermatology
Thể loại lecture notes
Năm xuất bản 2023
Thành phố San Francisco
Định dạng
Số trang 64
Dung lượng 4,87 MB

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514 San Joaquin Valley feverPathogenesis Infection caused by salmonellae, gram-neg-ative, rod-shaped bacteria of the family Enterobacteriaceae; most common sources of bacteria: beef, pou

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PART19.MIF Page 513 Friday, October 31, 2003 12:22 PM

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514 San Joaquin Valley fever

Pathogenesis

Infection caused by salmonellae,

gram-neg-ative, rod-shaped bacteria of the family

Enterobacteriaceae; most common sources

of bacteria: beef, poultry, eggs

Clinical manifestation

Skin signs: light red papules (rose spots)

occurring in crops on trunk during second

to fourth week of illness; erythema

nodo-sum, Sweet’s syndrome, pustular

dermati-tis and generalized erythroderma

(ery-thema typhosum)

Gastrointestinal signs: loose stool or watery

diarrhea; abdominal pain; mild

hepat-osplenomegaly

Differential diagnosis

Viral gastroenteritis; shigellosis; ingestion

of preformed toxins (“food poisoning”);

campylobacter infection;

cryptosporidio-sis; cyclospora infection; escherichia coli

infection; listeriosis; vibrio infection

Therapy

Antibiotics only for patients with severe

disease or those at high risk of invasive

dis-ease: ciprofloxacin; amoxicillin

 Typhoid fever

References

Stutman HR (1994) Salmonella, shigella, and

campylobacter: common bacterial causes of

in-fectious diarrhea Pediatric Annals 23(10):538–

mucopoly-saccharidosis type III-C

Autosomal recessive trait; deficiency of

Clinical manifestation

Onset of symptoms from age 2–6 years;organs most involved: bone, viscera, con-nective tissue, and brain; regression of psy-chomotor development and neurologicsigns, including severe mental retardation,hyperactivity, autistic features, and behav-ioral disorders; thickened facial features;coarse hair; hirsutism; genu valgum; shortneck; progressive deterioration and death,usually before age 20 years

Differential diagnosis

Hunter syndrome; Hurler syndrome; Scheiesyndrome; Gaucher’s disease; Niemann-Pick disease

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Say syndrome 515

S

IIIB: Diagnostic, clinical, and biological

impli-cations Human Mutation 18(4):264–281

Sao Paulo fever

 Rocky Mountain spotted fever

Sarcoidosis

Synonym(s)

Definition

Chronic multisystem disease, characterized

by noncaseating epithelioid granulomas

Pathogenesis

May result from exposure of a genetically

susceptible host to specific environmental

agents, such as infectious organisms,

alu-minium, zirconium, talc, pine tree pollen,

and clay, that the immune system is unable

to effectively clear

Clinical manifestation

Skin: asymptomatic, red-brown macules

and papules commonly involving the face,

periorbital, nasolabial folds, extensor

sur-faces of extremities; round-to-oval,

red-brown-to-purple, infiltrated plaques, the

center of which may be atrophic;

non-tender, firm, oval, flesh-colored or

viola-ceous nodules on extremities or trunk

(Darier-Roussy sarcoidosis); inflitration of

scars

Pulmonary system: involvement in most

patients; dyspnea; dry cough; chest

tight-ness or pain

Lymphatic system: palpable lymph nodes

Ocular involvement: anterior uveitis,

asso-ciated with fever and parotid swelling

(uve-oparotid fever)

Neurologic system: central nervous systeminvolvement sometimes fatal; seventh cra-nial nerve palsy most frequent finding; mis-cellaneous findings: myocardial involve-ment, arthritis, proximal muscle weakness,renal failure

Differential diagnosis

Tuberculosis; lymphoma; phoma; foreign body granuloma; drug reac-tion; granuloma annulare; granulomafaciale; lichen planus; lupus erythematosus;leprosy; syphilis; psoriasis; tinea corporis;necrobiosis lipoidica

pseudolym-Therapy

Cutaneous involvement: triamcinolone

3 mg per ml intralesionalSevere, recalcitrant disease: methotrexate;azathioprine; hydroxychloroquine

Symptomatic systemic disease:

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Contagious infestation of the skin by

arach-nid mite Sarcoptes scabiei, var hominis

Pathogenesis

Causative organism is mite, Sarcoptes

sca-biei; disease spreads through direct and

prolonged contact between hosts; possible

transmission through fomites, such as

infected bedding or clothing, but less likely;

delayed type IV hypersensitivity reaction to

mites, eggs, or scybala (packets of feces)

which causes intense pruritus

Clinical manifestation

Intense pruritus, particularly at night;

slightly elevated, pink-white, linear, curved,

or s-shaped line (burrow), located in

webbed spaces of fingers, flexor surfaces of

wrists, elbows, axillae, belt line, feet, and

scrotum in men and areolae in women;

bur-rows on the palms and soles in infants;

vesi-cles; red papules on penile shaft

Nodular variant: pink, tan, brown, or red

nodules lasting for weeks

Crusted (Norwegian) variant: occurs in

immunocompromised and

institutional-ized patients; minimally pruritic,

hyperk-eratotic, crusted plaques over large areas;nail dystrophy; scalp lesions

Differential diagnosis

Atopic dermatitis; dermatitis formis; pityriasis lichenoides; lichen pla-nus; insect bite reaction; contact dermati-tis; psoriasis; ecthyma; impetigo; xeroticeczema; transient acantholytic dermatosis;linear IgA bullous dermatosis; seborrheicdermatitis; erythroderma from othercauses such as Sézary syndrome and pem-phigus foliaceus; Langerhans cell histiocy-tosis; fiberglass dermatitis; dyshidroticeczema; pityriasis rosea; animal scabies;pediculosis; delusions of parasitosis; meta-bolic pruritus

pedicu-Scalded skin syndrome

 Staphylococcal scalded skin drome

syn-Scalp and head syndrome

 Adams-Oliver Syndrome

Scalp cyst

 Pilar cyst

in the finger web spaces

PART19.MIF Page 516 Friday, October 31, 2003 12:22 PM

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Schamberg’s progressive pigmented purpura 517

Bacterial infection caused by

toxin-produc-ing group-A beta hemolytic streptococci

Pathogenesis

Eruption caused by erythemogenic toxin as

consequence of local production of

inflam-matory mediators and alteration of the

cutaneous cytokines

Clinical manifestation

Abrupt onset of fever, headache, vomiting,

malaise, chills, and sore throat, with rash

appearing after 1–4 days; exudative

tonsilli-tis a common site of infection; mucous

membranes usually bright red; scattered

petechiae and small, red papules on soft

palate; during first days of infection, white

membrane coating on tongue through

which edematous, red papillae protrude

(white strawberry tongue); after white

membrane sloughs, tongue red with

promi-nent papillae (red strawberry tongue);

exanthem consisting of fine red, punctatepapules, appearing within 1–4 days follow-ing the onset of illness; first appear onupper trunk and axillae and then general-ize, with accentuation in flexural areas; mayappear more intense at dependent sites andsites of pressure, such as the buttocks; sand-paper feel to affected skin; transverse areas

of hyperpigmentation with petechiae in theaxillary, antecubital, and inguinal areas(Pastia lines); flushed face with circumoralpallor; rash fades with fine desquamationafter 4–5 days

Differential diagnosis

Viral exanthem, including rubella, rubeola,fifth disease; toxic shock syndrome; Kawa-saki syndrome; lupus erythematosus; drugreaction

Therapy

penicillin allergy – cephalexin, cin

erythromy-References

Chiesa C, Pacifico L, Nanni F, Orefici G (1994) current attacks of scarlet fever Archives of Pediatrics & Adolescent Medicine 148(6):656–

 Benign pigmented purpura

papillae

PART19.MIF Page 517 Friday, October 31, 2003 12:22 PM

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518 Scheie syndrome

Scheie syndrome

Synonym(s)

syndrome

Definition

Inherited metabolic storage disease arising

from a deficiency of alpha-L-iduronidase

Pathogenesis

Autosomal recessive trait; deficiency of

alpha-L-iduronidase, which results in

accu-mulation of mucopolysaccharides in the

lysosomes of the cells in the connective

tis-sue

Clinical manifestation

Onset of symptoms from age 2–4 years;

signs and symptoms similar to those of

Hurler syndrome, but milder, with slower

progression; lichenified, dry, thick skin

with diminished elasticity; increased

pig-mentation on the dorsum of the hands;

sclerodermalike changes; hypertrichosis of

the extremities; pale colored hair; mild

skeletal deformation and deformity of the

hands; growth sometimes normal; aortic

stenosis or regurgitation sometimes

present; hepatosplenomegaly; intelligence

usually normal

Differential diagnosis

Hurler syndrome; Hunter syndrome;

Gau-cher’s disease; Niemann-Pick diseae;

osteo-genesis imperfecta

Therapy

None

References

Schiro JA, Mallory SB, Demmer L, Dowton SB,

Luke MC (1996) Grouped papules in

Hurler-Scheie syndrome Journal of the American

urti-Pathogenesis

May be related to deposition of the IgMparaprotein, leading to immune complexdeposition and complement activation

Clinical manifestation

Chronic urticaria; individual episode ally resolves within few hours; fevers per-

arthralgias; bone pain involving tibia,femur, ileum, and vertebral column; myal-gias; fatigue; weight loss

Differential diagnosis

Urticarial vasculitis; lupus erythematosus;

adult Still disease; Waldenström roglobulinemia; chronic hepatitis B infec-tion

mac-Therapy

Acute disease flare: prednisonePART19.MIF Page 518 Friday, October 31, 2003 12:22 PM

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Scleredema adultorum of Buschke 519

S

References

Lipsker D, Veran Y, Grunenberger F, Cribier B,

Heid E, Grosshans E (2001) The Schnitzler

syn-drome Four new cases and review of the

 Granular cell tumor

Schweninger and Buzzi,

idiopathic anetoderma of

 Anetoderma

Scleredema

Synonym(s)

Scleredema adultorum; scleredema

adul-torum of Buschke; scleredema

diabeti-corum; scleredema diabeticorum of

Bus-chke

Definition

Disorder characterized by nonpitting,

indu-rated plaques and histological evidence of

dermal mucin deposition

to 2 yearsGroup 2 subtype: no prior history of febrileillness; insidious onset of skin lesions; atrisk of developing paraproteinemias,including multiple myeloma

Group 3 subtype: prior history of diabetesmellitus, usually adult onset and insulindependent, unremitting course; ill-defined,woody, nonpitting, indurated plaques; ery-thema, hyperpigmentation, and/or a peaud’orange appearance; usually located onface, neck, trunk, or upper extremities

Scleredema adultorum

 Scleredema

Scleredema adultorum of Buschke

 Scleredema

PART19.MIF Page 519 Friday, October 31, 2003 12:22 PM

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Disorder of the subcutaneous fat in

debili-tated neonates, resulting in generalized

sub-cutaneous plaques

Pathogenesis

Prematurity, hypothermia, shock, and

met-abolic abnormalities increases

saturated-to-unsaturated fatty acid ratio, possibly as a

result of enzymatic alteration, allowing

pre-cipitation of fatty acid crystals within

lipocytes; occurs with prematurity,

pneu-monia, septicemia, respiratory distress

syn-drome, congenital heart defects, teritis, and intestinal obstruction

gastroen-Clinical manifestation

Firm, violaceous subcutaneous plaquesappearing suddenly, first on thighs and but-tocks and then spreading; may affect allparts of the body except palms, soles, andgenitalia; temperature instability; restrictedrespiration; difficulty in feeding; decreasedspontaneous movement

insti-References

Fretzin DF, Arias AM (1987) Sclerema rum and subcutaneous fat necrosis of the new- born Pediatric Dermatology 4(2):112–122

 Progressive systemic sclerosus References

Haustein UF (2002) Systemic

sclerosis-scleroder-ma Dermatology Online Journal 8(1):3

Sclerodermoid fasciitis

 Eosinophilia-myalgia syndrome

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Caused by Rickettsia tsutsugamushi ettsia orientalis), acquired when infectedchigger bites and inoculates pathogens

(Rick-Clinical manifestation

High, severe headache, myalgia; ocularpain; wet cough; malaise; injected conjunc-tiva; eruption begins as a red, indurated

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522 Scurvy

papule that eventually enlarges to 8–12 mm,

vesiculates, and ruptures, developing

necro-sis; 5–8 days later, onset of

centrifugal-spreading macular eruption on trunk,

sometimes becoming papular

Differential diagnosis

Tularemia; leptospirosis; typhoid fever;

other rickettsial infections; viral exanthem;

Vitamin C deficiency disease manifested by

gingival lesions, hemorrhage, arthralgia,

loss of appetite, and listlessness

Pathogenesis

Vitamin C deficiency, after at least 3 months

of severe or total lack of vitamin C,

result-ing in defective collagen synthesis and

defective folic acid and iron utilization

Clinical manifestation

Perifollicular hyperkeratotic papules,

sur-rounded by a hemorrhagic halo; hairs are

twisted like corkscrews and may be

frag-mented; submucosal gingival bleeding;

sub-periosteal hemorrhage causes painful bones

of the legs and elsewhere; arthralgia;

ano-rexia; listlessness; conjunctival

hemor-rhage; poor wound healing

Differential diagnosis

Vasculitis; physical abuse; coagulation

abnormalities with leukemia; platelet

abnormalities; deep vein thrombosis;thrombophlebitis

Therapy

Ascorbic acid 800–1000 mg per day PO for

at least 1 week, then 400 mg per day untilrecovery complete

References

Hirschmann JV, Raugi GJ (1999) Adult scurvy Journal of the American Academy of Dermatol- ogy 41(6):895–906

Scutula

Definition

Dense masses of mycelium and epithelialdebris forming yellowish, cup-shapedcrusts, seen in the favus form of tinea capi-tis

References

Qianggiang Z, Limo Q, Jiajun W, Li L (2002) port of two cases of tinea infection with scutu- la-like lesions caused by Microsporum gypseum International Journal of Dermatolo-

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Sebaceous carcinoma 523

S

Definition

Pruritic, papular eruption occurring

under-neath the swimsuit after extended

expo-sure to seawater

Pathogenesis

Hypersensitivity reaction to larval form of

the thimble jellyfish, Linuche unguiculata;

factors promoting larval venom discharge:

wearing of bathing suits for prolonged

peri-ods following swimming, exposure to fresh

water through showering, and mechanical

stimulation

Clinical manifestation

Onset a few hours after ocean bathing;

pru-ritic papules in a bathing suit distribution

pattern; occurence in axilla and on chest in

men with significant chest hair

Differential diagnosis

Cercarial dermatitis; insect bite reaction;

scabies; folliculitis; jellyfish sting; urticaria

Therapy

Corticosteroids, topical, high potency;

anti-histamines, first generation, for sedation

References

Wong DE, Meinking TL, Rosen LB (1994)

Seabath-er's eruption Clinical, histologic, and

immu-nologic features Journal of the American

smooth-sur-Differential diagnosis

Basal cell carcinoma; sebaceous carcinoma;sebaceous gland hyperplasia; nevus seba-ceous; xanthoma; xanthelasma; molluscumcontagiosum; other adnexal neoplasms

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524 Sebaceous cyst

Differential diagnosis

Keratoconjunctivitis;

blepharoconjunctivi-tis; chalazion; squamous cell carcinoma;

basal cell carcinoma; Merkel cell

carci-noma; pyogenic granuloma; melacarci-noma;

metastasis; benign adnexal tumor;

sar-coidosis, ocular pemphigoid

Therapy

References

Snow SN, Larson PO, Lucarelli MJ, Lemke BN,

Madjar DD (2002) Sebaceous carcinoma of the

eyelids treated by mohs micrographic surgery:

report of nine cases with review of the

litera-ture Dermatologic Surgery 28(7):623–631

Benign cutaneous tumor composed of less

than 50 % of cells having sebaceous

Firm, flesh-colored or yellowish, smooth,

sessile, or pedunculated papule on face,

scalp, or eyelid; older lesions may form

plaque and ulcerate

Differential diagnosis

Sebaceous carcinoma; squamous cell

carci-noma; basal cell carcicarci-noma; Merkel cell

car-cinoma; chalazion; pyogenic granuloma;melanoma; metastasis; sarcoidosis

Therapy

References

Brown MD (2000) Recognition and management

of unusual cutaneous tumors Dermatologic Clinics 18(3):543–552

Sebaceous gland carcinoma

 Sebaceous carcinoma

Sebaceous gland hyperplasia

seba-Definition

Hamartomatous enlargement of facial ceous glands, characterized by yellowpapules with central dell

seba-Pathogenesis

Occurs commonly in organ transplantrecipients, suggesting immune mecha-nisms in some cases

Clinical manifestation

Well-demarcated, yellow-to-flesh-colored,delled papules, most commonly on fore-head and cheeks

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Seborrheic dermatitis 525

S

Differential diagnosis

Sebaceous carcinoma; melanocytic nevus;

sebaceous adenoma; sebaceous

epitheli-oma; squamous cell carcinepitheli-oma; basal cell

carcinoma; sarcoidosis; colloid milium;

fibrous papule; granuloma annulare; lipoid

proteinosis; milium; molluscum

contagio-sum; syringoma; trichoepithelioma;

xan-thoma; xanthelasma

Therapy

Light electrodesiccation; liquid nitrogen

cryotherapy; laser ablation; shave removal;

isotretinoin for multiple lesions

References

de Berker DA, Taylor AE, Quinn AG (1996)

Seba-ceous hyperplasia in organ transplant

recipi-ents: shared aspects of hyperplastic and

dysplastic processes? Journal of the American

Definition

Inflammatory dermatosis in areas with highsebum flow and accumulation, such as thescalp, face, intertriginous areas, and chest

Pathogenesis

Abnormal immune response to a normalconstituent of the skin flora, Pityrosporumovale

Clinical manifestation

Scalp: appearance varies from mild, patchyscaling to widespread, thick, adherentcrusts

Face: central facial erythema and scale,most prominent in skin folds

Eyelids: poorly defined, scaly, brown plaques

reddish-Presternal or interscapular area: poorlydefined, red-brown, scaly papules andplaques

Intertriginous areas: fairly sharply cated, red, scaly plaques

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526 Seborrheic eczema

diaper dermatitis; pityriasis rosea;

pityria-sis lichenoides chronica; lupus

erythemato-sus; rosacea; Darier disease; Hailey-Hailey

disease; Grover’s disease; pemphigus

foliaceus; xerotic eczema; chronic

granulo-matous disease; exfoliative erythroderma;

infectious eczematoid dermatitis;

Letterer-Siwe disease; staphylococcal blepharitis;

tinea amiantacea; vitamin B and/or zinc

deficiency; glucagonoma syndrome

Therapy

Anti-seborrheic shampoo, used daily;

corti-costeroids, topical, low potency for face;

corticosteroids, topical, mid potency for

trunk; azole antifungal agents seborrheic

blepharitis: scrubbing of eyelids daily with

baby shampoo diluted 1 : 1 with water

References

Faergemann J (2000) Management of seborrheic

dermatitis and pityriasis versicolor American

Journal of Clinical Dermatology 1(2):75–80

Hereditary component; sunlight may be a

factor in some cases

Clinical manifestation

Non-inflamed, single or multiple, sharply

defined, flesh-colored, light brown, gray,

blue, or black, flat papules with a velvety or

finely verrucous surface; edges raised offskin surface, giving lesion a “stuck-on”appearance

Dermatosis papulosa nigra variant: small,pedunculated, heavily pigmented papule,with minimal keratotic element, on faceStucco keratosis variant: superficial, gray-to-light-brown, flat, keratotic papules onthe dorsa of the feet, ankles, hands, andforearms

Melanoacanthoma variant: deeply mented keratotic plaque with histologic evi-dence of proliferation or activation of den-dritic melanocytes

pig-Differential diagnosis

Melanocytic nevus; melanoma; don; actinic keratosis; basal cell carcinoma;squamous cell carcinoma; psoriasis; pem-phigus foliaceus; wart

acrochor-Therapy

Electrodesiccation and curettage; liquidnitrogen cryotherapy; shave removal; ellip-tical excision

References

Pariser RJ (1998) Benign neoplasms of the skin Medical Clinics of North America 82(6):1285– 1307

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Self-healing squamous cell carcinoma 527

Generic names in parentheses:

Celexa (citalopram); Zoloft (sertraline);

Prozac (fluoxetine); Paxil (paroxetine);

Dermatologic indications and dosage

See table

Common side effects

Cutaneous: skin eruption Gastrointestinal: anorexia, hyperexia Genitourinary: sexual dysfunction Neurologic: insomnia, sedation, headache

Serious side effects

Neurologic: serotonin syndrome

Drug interactions

Buspirone; cimetidine; ergot alkaloids; anol; anti-psychotics, both typical andatypical; lithium; MAO inhibitors; metopro-lol; phenytoin; quinidine; tricyclics; warfa-rin

eth-Contraindications/precautions

Hypersensitivity to drug class or nent; MAO inhibitors within 14 days; avoidrapid withdrawal

compo-References

Gupta MA, Guptat AK (2001) The use of pressant drugs in dermatology Journal of the European Academy of Dermatology & Venere- ology 15(6):512–518

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528 Self-limiting acroderamatitis enteropathica

Senile depigmented spots

 Idiopathic guttate hypomelanosis

Selective serotonin reuptake inhibitor (SSRI) Dermatologic indications and dosage

Burning mouth

syndrome

Celexa: 20–40 mg PO once daily;

Zoloft: 50–100 mg PO once daily;

Prozac 10–60 mg PO once daily;

Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime

Celexa: safety and effectiveness not established; Zoloft: 25 mg PO once daily (6–12 years old); Prozac 5–20 mg PO once daily; Paxil 10–30 mg PO once daily (> 8 years old); Luvox 25–50 mg PO at bedtime Obsessive-compulsive

disorders

Celexa: 20–40 mg PO once daily;

Zoloft: 50–100 mg PO once daily;

Prozac 10–60 mg PO once daily;

Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime

Celexa: safety and effectiveness not established; Zoloft: 25 mg PO once daily (6–12 years old); Prozac 5–20 mg PO once daily; Paxil 10–30 mg PO once daily (> 8 years old); Luvox 25–50 mg PO at bedtime

Zoloft: 50–100 mg PO once daily;

Prozac 10–60 mg PO once daily;

Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime

Celexa: safety and effectiveness not established; Zoloft: 25 mg PO once daily (6–12 years old); Prozac 5–20 mg PO once daily; Paxil 10–30 mg PO once daily (> 8 years old); Luvox 25–50 mg PO at bedtime

Zoloft: 50–100 mg PO once daily;

Prozac 10–60 mg PO once daily;

Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime

Celexa: safety and effectiveness not established; Zoloft: 25 mg PO once daily (6–12 years old); Prozac 5–20 mg PO once daily; Paxil 10–30 mg PO once daily (> 8 years old); Luvox 25–50 mg PO at bedtime

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Self-limited immune complex disease

caused by exposure to foreign proteins or

haptens

Pathogenesis

With slight antigen excess,

intermediate-sized immune complexes deposit in small

vessels and activate complement; increased

adhesion molecule expression in

endothe-lial cells causes cytokine release and lar injury

vascu-Clinical manifestation

Urticarial, morbilliform, or scarlatiniformeruption; palpable purpura; erythema mul-tiforme; facial edema; pruritus and ery-thema at injection site; symmetrical arthri-tis, usually in metacarpophalangeal andknee joints; myalgias; lymphadenopathy;splenomegaly; neurologic complications,including headache, optic neuritis; cranialnerves palsies, Guillain-Barré syndrome;gastrointestinal complaints, includingabdominal pain, nausea, vomiting,diarrhea; clinical recovery after 7–28 days

Differential diagnosis

Urticaria; cryoglobulinemia; hepatitis;mononucleosis; hypersensitivity vasculitis;lupus erythematosus; Henoch-Schönleinpurpura; Still disease

Therapy

Antihistamines, first generation; nisone for patients with multisysteminvolvement and significant symptomatol-ogy

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Short anagen syndrome

 Loose anagen hair syndrome

Sicca syndrome

 Sjögren syndrome

Siemerling-Creutzfeldt syndrome

References

Schwartz RA (1996) Sign of Leser-Trelat Journal

of the American Academy of Dermatology 35(1):88–95

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Chronic disorder characterized by

kerato-conjunctivitis sicca and xerostomia

Pathogenesis

Autoimmune dysregulation, particularly

genetic susceptibility; abnormality in

cellu-lar apoptosis

Clinical manifestation

Glandular symptoms: dry eye syndrome,

characterized by dryness of cornea and

conjunctiva; dry mouth; dry lips; red,

smooth dry tongue; dental caries; recurrent

oral candidiasis; recurrent salivary glandswelling; nasal dryness with recurrentinfections, hoarseness, and aphonia;atrophic changes in the vulva and vagina,resulting in pruritus and vaginitis; anal andrectal mucosal dryness

Skin symptoms: xerosis; decreased ing; dry, sparse hair; annular, red, scalyplaques, especially on face and neck; cuta-neous vasculitis

sweat-Primary variant: no associated connectivetissue or autoimmune disease; extraglandu-lar involvement: lung involvement, nervoussystem dysfunction, renal involvement,Raynaud phenomenon, and lymphoprolif-erative disorders

Secondary variant: associated connectivetissue or autoimmune disease; milder dis-ease with fewer systemic manifestations

Differential diagnosis

HIV infection; drug reaction; lupus thematosus; amyloidosis; environmentaldryness

ery-Therapy

Dry eyes: Artificial tears (e.g., lose, 1 % hyaluronic acid solution, alcoholsolutions) applied 4–6 times daily

methylcellu-Dry mouth: frequent small drinks andmouthwashes; artificial saliva; stimulation

of salivary secretion with sweets, etc

References

Manoussakis MN, Moutsopoulos HM (2001) Sjogren's syndrome: current concepts Advanc-

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 Merkel cell carcinoma

Small plaque parapsoriasis

Synonym(s)

Benign parapsoriasis; digitate dermatitis;

digitate dermatosis; chronic superficial

der-matitis; guttate parapsoriasis; Brocq’s

dis-ease

Definition

Chronic, benign, cutaneous disease,

charac-terized by scaly plaques resembling

Differential diagnosis

Psoriasis; dermatophytosis; lupus thematosus; lichen planus; pityriasis rosea;syphilis; seborrheic dermatitis; mycosisfungoides; xerosis; nummular dermatitis

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South African porphyria 533

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534 South African tick typhus

South African tick typhus

Systemic mycotic infection, endemic to

countries in Central America and South

America, caused by the fungus

Paracoccidi-oides brasiliensis

Pathogenesis

Caused by thermally dimorphic fungus,

Paracoccidioides brasiliensis; acquired by

inhalation of conidia fungus that

trans-forms into yeast cells within alveolar

mac-rophages; fungus may disseminate, causing

granulomatous disease in multiple organs;

alcohol and tobacco use associated with

dissemination

Clinical manifestation

Adult chronic form:

Mucous membranes: slowly progressive,

painful papules or plaques ulcerate in oral,

nasal, pharyngeal, and laryngeal tissue;

gin-gival lesions cause loss of teeth;

conjunctivi-tis and ulcerative lesions of the perianal

area

Skin: occurs most commonly on the face;

may have nodules, ulcerations or

papillo-matous lesions; most often arises from

direct extension of mucous membrane

lesions; hematogenous spread causes widely

scattered subcutaneous abscesses; lymph

nodes: extensive hypertrophic, painful

lym-phadenopathy with visceral and

subcutane-ous nodes; cervical nodes commonly

affected; suppuration causes sinus tracts orskin ulcers

Respiratory: lung involvement in 70-80% ofpatients and often the only organ systeminvolved; frequently resembles tuberculo-sis, with chronic dyspnea, cough, and spu-tum production

Other systemic problems: galy, adrenal insufficiency meningitis,intestinal ulcerations, and osteomyelitisJuvenile subacute form:

hepatosplenome-Mucous membranes: rare mucosal tions

ulcera-Skin: acneiform eruption or subcutaneousabscesses; scrofuloderma as a result oflymph node suppuration

Lymph nodes: prominent thy with suppuration; mesenteric adenopa-thy may produce bowel obstructionRespiratory: occasional pneumoniaOther problems: cachexia, hepatosplenome-galy, adrenal insufficiency, osteomyelitis,gastrointestinal problems

lymphadenopa-Differential diagnosis

Actinomycosis; coccidioidomycosis; maniasis; sporotrichosis; syphilis; tubercu-losis; histoplasmosis; North American blas-tomycosis; Wegener’s granulomatosis; oralcarcinoma; drug eruption; lymphoma;leukemia

leish-Therapy

ketoconazole; itraconazole; for severe ease: amphotericin B – 0.7–1 mg per kg IVdaily for 4–8 weeks, followed by trimetho-prim and sulfamethoxazole for 2–3 years

dis-References

Rivitti EA, Aoki V (1999) Deep fungal infections

in tropical countries Clinics in Dermatology 17(2):171–190

South American pemphigus

 Fogo selvagem

Trang 23

Spanish toxic oil syndrome

 Toxic oil syndrome

Speckled lentiginous nevus

Clinical manifestation

Red macule or papule surrounded by eral distinct radiating vessels, occurringmost commonly on face, below eyes, andover cheekbones; central pressure causeslesion to blanch

vascu-Journal of the American Academy of ogy 37(4):523–549

Trang 24

Benign tumor of sweat gland origin,

pre-senting as a solitary gray-pink papule

Pathogenesis

Unclear whether tumor arises from

apo-crine or ecapo-crine epithelium

Clinical manifestation

Solitary firm, gray-pink papule, usually

arising in the head and neck region or

trunk; occasional pain and tenderness

Differential diagnosis

Cylindroma; basal cell carcinoma;

trichoep-ithelioma; eccrine poroma; angiofibroma;

Spironolactone Dermatologic indications and dosage

Trang 25

Sporotrichosis 537

S

Common side effects

Dermatologic: skin eruption

Gastrointestinal: dyspepsia

Neurologic: sedation, headache

Genitourinary: sexual dysfunction,

ACE inhibitors; cyclosporine; non-steroidal

anti-inflammatory agents; COX-2

inhibi-tors; potassium salts; tacrolimus

Contraindications/precautions

Hypersensitivity to drug class or

compo-nent; renal insufficiency; hyperkalemia;

caution in patients with liver dysfunction

References

Thiboutot D (2001) Hormones and acne:

patho-physiology, clinical evaluation, and therapies

Seminars in Cutaneous Medicine & Surgery

Schenck's disease; Beurmann's disease; rose

gardener's disease; peat moss disease

Definition

Subcutaneous or systemic fungal infection

caused by soil pathogen, Sporothrix

schenckii

Pathogenesis

Caused by Sporothrix schenckii, dimorphic

fungus commonly found on vegetative

mat-ter, particularly in humid climates; may

gain entry through puncture wound,spreading via lymphatic vessels

Clinical manifestation

History of prick injury at site of infection,within 3 weeks of onset of signs and symp-toms

Lymphocutaneous variant: subcutaneousnodule developing at site of inoculation andsometimes ulcerating after central abscessformation; satellite lesions along associatedlymphatic chain with lymphadenopathyFixed cutaneous variant: scaly, acneform,verrucous, or ulcerative nodule remaininglocalized to site of inoculation

Disseminated variant: multiple organinvolvement causing pyelonephritis, orchi-tis, mastitis, arthritis, synovitis, meningi-tis, osseous infection, or (rarely) pulmo-nary disease

Differential diagnosis

Atypical mycobacterial infection; sis; North American blastomycosis; SouthAmerican blastomycosis; leishmaniasis;bacterial pyoderma; anthrax; cutaneoustuberculosis; tularemia; foreign body gran-uloma; herpes zoster

nocardio-Therapy

saturated solution of potassium iodide:300–500 mg PO 3 times daily for 4–8 weeks

3 mg per kg per day IV until significantclinical response; itraconazole

Sporotrichosis Linear, scaly, red papules, one of

which is ulcerated

Trang 26

 Rocky Mountain spotted fever

Spotted leg syndrome

 Diabetic dermopathy

Spun glass hair

 Uncombable hair syndrome

Squamous cell carcinoma

Synonym(s)

Epidermoid carcinoma; prickle cell

carci-noma

Definition

Malignant tumor of keratinocytes, most

often arising in chronically sun-exposed

skin

Pathogenesis

Related closely to chronic sun exposure;

other risk factors: immunosuppression, fair

complexion, history of ionizing radiation or

photochemotherapy, abnormal DNA repair

mechanisms, infection with certain human

papillomavirus virus subtypes, and localsites of chronic inflammation

Clinical manifestation

Elevated, firm, pink to flesh-colored, totic papule or plaque with or without over-lying cutaneous horn or ulceration, oftenarising from pre-existing actinic keratosis;lip lesion: most commonly on vermillionborder of lower lip; shiny, ulcerated papule

kera-or nodule

Differential diagnosis

Actinic keratosis; basal cell carcinoma;benign adnexal neoplasm; melanoma; Mer-kel cell carcinoma; atypical fibroxanthoma;seborrheic keratosis; wart; pyogenic granu-loma; proliferating trichilemmal cyst; gran-ular cell tumor; granulomatous diseasessuch as tuberculosis, leishmaniasis, coccidi-oidomycosis, North American blastomyco-sis, syphilis, and bromoderma

Therapy

Surgical excision; destruction by desiccation and curettage or liquid nitro-gen cryotherapy; superficial orthovoltageradiation therapy; large tumors, lesions inanatomically sensitive areas, or recurrenttumors – Mohs micrographic surgery orother form of microscopically controlledexcision

Trang 27

Toxin-mediated disease of young children,

characterized by acute generalized skin

exfoliation

Pathogenesis

Caused by toxigenic strains of

Staphylococ-cus aureus, usually belonging to phage

group 2 (types 3A, 3B, 3C, 55, or 71); two

exotoxins (ETs), epidermolytic toxin A

(ET-A) and epidermolytic toxin B (ET-B),

responsible for the pathologic changes and

blistering produced by disruption of

epi-dermal granular cell layer

Clinical manifestation

Original focus of infection may be purulent

conjunctivitis, otitis media, or

nasopharyn-geal infection; fever; irritability;

general-ized, faint, orange-red, macular erythema

with cutaneous tenderness and periorificial

and flexural accentuation; early positive

progresses to generalized, superficial

blis-tering eruption, with tissue paper-like

sur-face wrinkling, followed by large, flaccidbullae in axillae, groin, and around thebody orifices, sparing mucous membranes;after epidermal sloughing, moist erythema-tous base present; healing usually completewithin 5–7 days

Differential diagnosis

Toxic shock syndrome; Kawasaki disease;scarlet fever; erythema multiforme; childabuse

antibiot-Staphylococcal toxic shock syndrome

 Toxic shock syndrome

Trang 28

540 Steatoblepharon

release of inflammatory mediators;

leuko-cyte sludging may block dermal capillaries,

leading to tissue ischemia

Clinical manifestation

Erythematous, scaling, eroded plaques of

lower extremity; medial ankle most

fre-quently and severely involved; acute flares

with exudative, weeping plaques;

long-standing lesions with lichenification and

hyperpigmentation; skin induration

some-times progresses to significant scarring

lipodermatosclerosis and violaceous

plaques and nodules on the legs and dorsal

feet (acroangiodermatitis)

Differential diagnosis

Contact dermatitis; cellulitis; Kaposi’s

sar-coma; atopic dermatitis; xerotic eczema;

necrobiosis lipoidica; nummular eczema;

dermatophytosis; benign pigmented

pur-pura; pretibial myxedema

Therapy

compression therapy with Unna boot

dress-ings, controlled gradient compression

device or compression stockings;

pred-nisone for severe acute flares

References

Weingarten MS (2001) State-of-the-art treatment

of chronic venous disease Clinical Infectious Diseases 32(6):949–954

Pathogenesis

Autosomal dominant trait; hamartomatousformation of abortive hair follicles at sitewhere sebaceous glands attach; associatedwith vellus hair cysts and trichostasisspinulosa, and sometimes existing on spec-trum with these entities

Clinical manifestation

Asymptomatic, smooth, colored papules; occasional rupture into thedermis producing inflammation with scar-ring; concentrated over upper torso, proxi-mal extremities; contents of lesion odor-less, creamy or oily fluid; non-hereditaryvariant: solitary lesion morphologicallyidentical to multiple lesions (steatocystomasimplex)

flesh-to-yellow-Stasis dermatitis Scaly, crusted, and eroded

plaque on the lower extremity

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Stevens-Johnson syndrome 541

S

Differential diagnosis

Acne vulgaris; epidermoid cyst;

trichilem-mal cyst; eruptive vellus hair cyst; milia;

syringoma; Gardner syndrome

Therapy

Tetracycline; isotretinoin; surgical excision

of individual inflammatory lesions

References

Rollins T, Levin RM, Heymann WR (2000) Acral

steatocystoma multiplex Journal of the

Ameri-can Academy of Dermatology 43(2 Pt 2):396–

Steely hair syndrome

 Menkes kinky hair syndrome

Cell-mediated immune response, mediated

by CD8 lymphocytes; may involve an DQw3-related, altered immune response;associated with medications, such as sul-fonamides, penicillin, or anti-convulsants,and with infections (most commonly, her-pes simplex virus infection and myco-plasma pneumonia)

HLA-Clinical manifestation

Erythematous papules, vesicles, bullae, andtarget-like papules, mainly on face, trunk,and mucous membranes, including oral,genital mucosa; < 35 % of body surfaceinvolved; lesions may be located on linings

of respiratory and gastrointestinal tracts;conjunctivitis with photophobia; burningsensation in eyes; hepatitis; nephritis; gas-trointestinal bleeding; pneumonia; myal-gia; arthritis; arthralgia

Differential diagnosis

Pemphigus vulgaris, erosive lichen planus;varicella zoster infection; Behcet's disease;Reiter's syndrome; herpes simplex virusinfection; bullous pemphigoid; toxic epider-mal necrolysis; Henoch-Schönlein pur-pura; urticaria; viral exanthem; Kawasakidisease; figurate erythema; fixed drug erup-tion; lupus erythematosus; aphthous stoma-titis

Trang 30

542 Stewart-Bluefarb syndrome

Therapy

Prednisone

References

Prendiville J (2002) Stevens-Johnson syndrome

and toxic epidermal necrolysis Advances in

Malignant vascular tumor arising in an area

of chronic lymphedema, particularly on

upper extremity after radical mastectomy

Pathogenesis

Occurs in the context of chronic

lymphe-dema

Clinical manifestation

Purplish patch, evolving into plaque or

nodule in the area of chronic lymphedema;

palpable subcutaneous mass or poorly

heal-ing eschar with recurrent bleedheal-ing and

ooz-ing; nodules may become polypoid,

develop small satellite papules and become

confluent; overlying epidermis sometimes

ulcerates, producing recurrent episodes of

bleeding and infection; high metastatic

potential

Differential diagnosis

Angioendotheliomatosis; angiolymphoid

hyperplasia with eosinophilia; Kaposi’s

sar-coma; lymphangioma; melanoma;

metasta-sis; hemangioendothelioma;

Lymphangi-Sticker disease

 Erythema infectiosum

Sticker's disease

 Erythema infectiosum

Stomatitis areata migrans

 Benign migratory glossitis

Trang 31

Streptococcal toxic shock-like syndrome 543

 Aplasia cutis congenita

Strep toxic shock syndrome

 Streptococcal toxic shock-like

Strep toxic shock-like syndrome;

strepto-coccal TSS flesh eating disease

Definition

Acute febrile illness, characterized by signs

of localized infection, often in the skin; eralized erythematous eruption accompa-nied by shock and multiple organ dysfunc-tion

gen-Pathogenesis

Caused by strains of Streptococcus genes; superantigen behavior of pyrogenicexotoxin-A (SPE-A); may also producestreptococcal pyrogenic exotoxin-B (SPE-B), streptococcal pyrogenic exotoxin-C(SPE-C), streptococcal superantigen andmitogenic factor, as well as non–group-Astreptococci aureus; release of tumor necro-

(IL-1b), which mediate signs and symptoms ofdisease; predisposing factors: influenza A,soft tissue wounds, varicella, pneumonia,unidentified bacteremia, surgical site infec-tion, septic arthritis, thrombophlebitis,meningitis, pelvic infection, endophthalmi-tis; additional risk factors: HIV, diabetesmellitus, cancer, ethanol abuse, and otherchronic diseases

ery-Differential diagnosis

Toxic shock syndrome; Stevens-Johnsonsyndrome; Kawasaki disease; staphylococ-cal scalded skin syndrome; toxic epidermalnecrolysis; drug reaction; scarlet fever;

Trang 32

544 Streptococcal TSS flesh eating disease

Rocky Mountain spotted fever;

leptospiro-sis; gas gangrene; meningococcemia

Therapy

Nafcillin: 2 gm IV every 4 hours in adults;

100–200 mg per kg per day divided into 4–6

doses per day in children

Clindamycin: 600–900 mg IV every 8 hours

in adults; 20–40 mg per kg per day IV

divided into 3–4 doses in children

Intravenous immunoglobulin (IVIG) 1–

2 gm per kg over 2–3 days

References

Levine N., Kunkel M, Thanh N; Ackerman L

(2002) Emergency department dermatology

Current Problems in Dermatology 14(6):188–

Striae distensae; striae atrophicans; striae

rubra; striae alba; stretch marks

Definition

Linear dermal scars accompanied by

epi-dermal atrophy

Pathogenesis

Results from stress rupture of dermal

con-nective tissue framework; affects skin

sub-jected to continuous and progressive

stretching; skin distension causes excessivemast cell degranulation with subsequentdamage of collagen and elastin; maydevelop more easily in skin with high pro-portion of rigid cross-linked collagen; asso-ciated with increased adrenal cortical hor-mone activity, such as in Cushing’s disease

or with exogenous glucocorticoid therapy

Clinical manifestation

Flattened, atrophic plaques with a pink hue,which enlarge in length and width andbecome violaceous; older striae are white,depressed, irregularly shaped bands withtheir long axis parallel to skin tension lines;

in pregnancy, striae affect abdomen andbreasts; adolescent striae occur on outeraspects of thighs and lumbo-sacral region

in boys, and thighs, buttocks, and breasts ingirls; flexures affected with topical corticos-teroid use, especially if used under occlu-sion

tretin-References

McDaniel DH, Ash K, Zukowski M (1996) ment of stretch marks with the 585-nm flash- lamp-pumped pulsed dye laser Dermatologic Surgery 22(4): 332–337

Treat-Striae Linear, red-brown, atrophic plaques

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