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
Trang 1PART19.MIF Page 513 Friday, October 31, 2003 12:22 PM
Trang 2514 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
Trang 3Say 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:
Trang 4Contagious 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
Trang 5Schamberg’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
Trang 6518 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
Trang 7Scleredema 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
Trang 8Disorder 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
Trang 9Caused 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
Trang 10522 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-
Trang 11Sebaceous 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
Trang 12524 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
Trang 13Seborrheic 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
Trang 14526 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
Trang 15Self-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
Trang 16528 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
Trang 17Self-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
Trang 18Short 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
Trang 19Chronic 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-
Trang 20Merkel 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
Trang 21South African porphyria 533
Trang 22534 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 23Spanish 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 24Benign 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 25Sporotrichosis 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 26Rocky 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 27Toxin-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 28540 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
Trang 29Stevens-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 30542 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 31Streptococcal 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 32544 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