Dermatology therapy essentials - part 3 pps

Chronic granulomatous disease of childhood 131 Linear IgA dermatosis Chronic bullous disease of childhood Linear IgA dermatosis Chronic cutaneous lupus erythematosus Lupus erythemato

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Chromoblastomycosis 129

C

Therapy

Cryotherapy; triamcinolone 3–5 mg per ml

intralesional; surgical excision; CNH pillow

to relieve pressure

References

Beck MH (1985) Treatment of chondrodermatitis

nodularis helicis and conventional wisdom?

British Journal of Dermatology 113(4):504–505

Pathogenesis

Elevated levels of lipofuscins possiblyinvolved; substance P possibly an impor-tant neurotransmitter; extrinsic contribut-ing factors include dyes, chromogenic bac-teria, and chemical contactants

Pathogenesis

Inoculation by one of the following: modendrum pedrosoi, H compactum, or

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Hor-130 Chromomycosis

Phialophora verrucosa; organisms isolated

from wood and soil

Clinical manifestation

Asymptomatic, verrucous papule, slowly

enlarging to large plaque or thick nodule;

lesions often ulcerate; satellite lesions

pro-duced by autoinoculation

Differential diagnosis

North American blastomycosis; South

American blastomycosis; tuberculosis;

leishmaniasis; syphilis; yaws; squamous cell

carcinoma; atypical mycobacterial

infec-tion; sporotrichosis; nocardiosis

Therapy

Itraconazole; terbinafine; flucytosine with

or without localized hyperthermia;

cryo-therapy; surgical excision for small lesions

References

Rivitti EA, Aoki V (1999) Deep fungal infections

in tropical countries Clinics in Dermatology

Actinic reticuloid; persistent light

reactiv-ity; photosensitive eczema; photosensitivity

dermatitis; persistent light reaction

Definition

Persistent eczematous eruption in the exposed areas of greater than 3 months'duration, with abnormal sensitivity toeither ultraviolet or visible light

Polymorphous light eruption; allergic tact dermatitis; photocontact dermatitis;solar urticaria; actinic prurigo; atopic der-matitis; lupus erythematosus; cutaneous Tcell lymphoma

con-Therapy

Protection from sunlight; apy; azathioprine; hydroxychloroquine sul-fate; cyclosporine

photochemother-References

Lim HW, Morison WL, Kamide R, Buchness MR, Harris R, Soter NA (1994) Chronic actinic dermatitis An analysis of 51 patients evaluated in the United States and Japan Archives of Der- matology 130(10):1284–1289

Chronic adrenal insufficiency

Addison’s disease

Chronic atrophic acrodermatitis

Acrodermatitis chronica atrophicans

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Chronic granulomatous disease of childhood 131

Linear IgA dermatosis

Chronic bullous disease of

childhood

Linear IgA dermatosis

Chronic cutaneous lupus

erythematosus

Lupus erythematosus, discoid

Chronic erythema nodosum

Subacute nodular migratory

pan-niculitis

Chronic granulomatous

disease

Synonym(s)

Chronic granulomatous disease of

child-hood; fatal granulomatosis of childchild-hood;

progressive septic granulomatosis; X-linkedchronic granulomatous disease

Definition

Inherited disorder of phagocytic cells, ing to recurrent, life-threatening bacterialand fungal infections

lead-Pathogenesis

Failure of phagocytes to generate sufficientquantities of reactive oxygen species;molecular defect represents a mutation in

the gene encoding the b subunit of chrome b558 (CYBB), located on the X chro-

cyto-mosome

Early onset of severe recurrent bacterialand fungal infections, often involving theskin; lungs most common site of infection;other involved sites include gastrointesti-nal tract, lymph nodes, liver, and spleen

Bruton agammaglobulinemia; common iable immunodeficiency; severe combinedimmunodeficiency; HIV infection; comple-ment deficiency; leukocyte adhesion defi-ciency; Wiskott-Aldrich syndrome

var-Therapy

Prophylaxis of bacterial infections with methoprim-sulfamethoxazole 5 mg per kgper day PO divided into 2 doses; bone mar-row transplantation

tri-References

Goldblatt D, Thrasher AJ, Chronic granulomatous disease Clinical & Experimental Immunology 122(1):1–9

Chronic granulomatous disease of childhood

Chronic granulomatous disease

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132 Chronic hair pulling

Chronic hair pulling

Development of a blue-gray pigmentation

in skin and mucous membranes, caused by

exposure to gold compounds

Pathogenesis

Deposition of gold salts in the dermis;

increased melanin production in the

epi-dermis

Blue-gray or violaceous hue to sun-exposed

skin and sclerae; mucous membranes

spared; pigmentation usually permanent;

occurs only after a cumulative dose of at

least 50 mg per kg

Argyria; other drug-induced pigmentation

(e.g minocycline; amiodarone); Addison’s

disease; hemosiderosis; jaundice; mia; hemochromatosis

Definition

Disorder characterized by asthma, sient pulmonary infiltrates, eosinophilia,and systemic vasculitis

tran-Pathogenesis

Activated eosinophils possibly pathogenic

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Ciclopirox 133

C

Cutaneous findings: red papules and

mac-ules; palpable purpuric papules and

plaques; cutaneous and subcutaneous

papules and nodules

Respiratory tract findings: allergic rhinitis;

asthma; transient pulmonary infiltrates

Vasculitis target organs: kidney, heart,

cen-tral nervous system, gastrointestinal tract

Henoch-Schönlein purpura; lupus

ery-thematosus; bronchopulmonary

aspergillo-sis; lymphoma; Loeffler syndrome;

lympho-matoid granulomatosis; polyarteritis

nodosa; rheumatoid arthritis

Therapy

Prednisone; steroid-sparing agents:

meth-otrexate; azathioprine 100–150 mg PO per

day; cyclosporine; cyclophosamide pulse

therapy 0.6 gm per m2 IV monthly for up to

1 year

References

Gross WL (2002) Churg-Strauss syndrome:

up-date on recent developments Current Opinion

in Rheumatology 14(1):11–14

Cicatricial pemphigoid

Synonym(s)

Benign mucous membrane pemphigoid;

scarring pemphigoid; mucosal pemphigoid

Definition

Autoimmune vesiculobullous disease

pre-dominately affecting mucous membranes

Pathogenesis

IgG antibodies against antigens in

base-ment zone; major antigens associated are

BPAG2 and epiligrin (laminin 5); immune

reaction causes loss of adhesion at the

der-mal-epidermal junction and blisters

Persistent, painful erosions on mucousmembranes, often healing with scarring;ocular involvement: pain or the sensation ofgrittiness in the eye; conjunctival inflam-mation and erosions; keratinization of theconjunctiva and shortening of the fornices;entropion with subsequent trichiasis; skin:tense vesicles or bullae, sometimes hemor-rhagic, sometimes healing with scarring ormilia; scalp involvement leads to alopecia

Bullous pemphigoid; linear IgA dermatosis;erythema multiforme; Stevens-Johnsonsyndrome; epidermolysis bullosa; epider-molysis bullosa acquisita; dermatitis herpe-tiformis; impetigo; pemphigus foliaceus;pemphigus vulgaris; herpes simplex virusinfection; herpes zoster

Therapy

Limited disease: mid potency topical costeroid gel for mucous membranesExtensive disease: prednisone, dapsone;cyclophosphamide; azathioprine

corti-References

Fleming TE, Korman NJ (2000) Cicatricial phigoid Journal of the American Academy of Dermatology 43(4):571–591

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134 Ciprofloxacin

Dosage form

0.77% cream, gel, lotion; 8% nail lacquer

Dermatologic indications and dosage

See table

Common side effects

Cutaneous: burning, itching, redness,

Gupta AK, Baran R (2000) Ciclopirox nail lacquer

solution 8% in the 21st century Journal of the

American Academy of Dermatology 43(4

500 mg per 5 ml for intravenous infusion

See table

Cutaneous: photosensitivity, urticaria, or

other vascular reaction

Gastrointestinal: nausea and vomiting,

diarrhea, abdominal pain

Neurologic: agitation, confusion, insomnia,

headache, dizziness, restlessness

Serious side effects

Gastrointestinal: pseudomembranous

Ciclopirox Dermatologic indications and dosage

Onychomycosis Apply Penlac once daily for up to

48 weeks

Apply Penlac once daily for up to

48 weeks Tinea corporis Apply Loprox twice daily Apply Loprox twice daily

Tinea cruris Apply Loprox twice daily Apply Loprox twice daily

Tinea faciei Apply Loprox twice daily Apply Loprox twice daily

Tinea nigra Apply Loprox twice daily Apply Loprox twice daily

Tinea pedis Apply Loprox twice daily Apply Loprox twice daily

White piedra Apply Loprox twice daily Apply Loprox twice daily

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Clarithromycin 135

C

Contraindications/precautions

Hypersensitivity to drug class or

compo-nent; safety not established for patients < 18

years old; caution in those with impaired

renal or liver function; caution in those

Cutaneous: skin eruption, vaginitis Gastrointestinal: nausea, vomiting, abdom-

inal pain, diarrhea, anorexia

Cutaneous: anaphylaxis, Stevens-Johnson

syndrome, toxic epidermal necrolysis

Gastrointestinal: pseudomembranous

coli-tis, cholestatic jaundice

Ciprofloxacin Dermatologic indications and dosage

Cellulitis 250–500 mg PO twice daily for

7–21 days, depending on response

Not indicated Chancroid 500 mg PO twice daily for 3 days Not indicated

Malakoplakia 250–500 mg PO for 7–14 days Not indicated

months to years

Not indicated Rickettsialpox 250–500 mg PO daily for 5 days Not indicated

Salmonellosis 500 mg IV twice daily, then switch to

PO when tolerated for a total course

of 10–14 days

Not indicated

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136 Clark’s nevus

Drug interactions

Amiodarone; antacids; budesonide;

bus-pirone; carbamazepine; clozapine; oral

con-traceptives; cyclosporine; digoxin; ergot

alkaloids; methadone; phenytoin;

pimoz-ide; protease inhibitors; quinidine; statins;

tacrolimus; theophylline; valproic acid;

vinca alkaloids; warfarin

compo-nent; caution in those with impaired liver

function; do not use concomitantly with

terfenadine or astemizole

References

Alvarez-Elcoro S, Enzler MJ (1999) The

mac-rolides: erythromycin, clarithromycin, and

azi-thromycin Mayo Clinic Proceedings 74(6):613–

Inappropriate distribution of pressure onto

a specific site, producing increased tional forces and reactive skin thickening

fric-Clinical manifestation

Thickened skin, with retained skin toglyphics, most commonly on the foot;occasional secondary maceration and fun-gal or bacterial infection

derma-Clarithromycin Dermatologic indications and dosage

Bacillary angiomatosis 250 mg PO twice daily for 3 weeks > 45 kg weight; 7.5 mg per kg PO

twice daily for 3 weeks Bartonellosis 250 mg PO twice daily for 3 weeks > 45 kg weight; 7.5 mg per kg PO

twice daily for 3 weeks Cellulitis 250 mg PO twice daily for 5–7 days > 45 kg weight; 7.5 mg per kg PO

twice daily for 5–7 days Ecthyma 250 mg PO twice daily for 5–7 days > 45 kg weight; 7.5 mg per kg PO

twice daily for 5–7 days Erythrasma 1 gm PO for 1 dose > 45 kg weight; 7.5 mg per kg PO for

1 dose Impetigo 250 mg PO twice daily for 5–7 days > 45 kg weight; 7.5 mg per kg PO

twice daily for 5–7 days Mycobacterium

marinum infection

500 mg PO twice daily for 6–12 weeks

15 mg per kg PO divided into

2 doses daily for 6–12 weeks

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Clindamycin, systemic 137

C

Wart; gout; lichen planus; interdigital

neu-roma; lichen simplex chronicus;

palmo-plantar keratoderma; keratosis punctata;

porokeratosis plantaris

Therapy

Mechanical pressure redistribution:

orthot-ics; well-fitted shoes; protective pads on

pressure points; skin-surface paring for

symptomatic lesions

References

Freeman DB (2002) Corns and calluses resulting

from mechanical hyperkeratosis American

Family Physician 65(11):2277–2280

Clear cell acanthoma

Synonym(s)

Clear cell acanthoma of Degos; Degos’

acanthoma; acanthome à cellules claires

Solitary, dome-shaped papule or nodule,

with a peripheral scale; occurring most

commonly on the lower extremities

Histiocytoma; seborrheic keratosis;

lichenoid keratosis; pyogenic granuloma;

amelanotic melanoma

Therapy

Surgical excision

References

Degos R, Civatte J (1970) Clear-cell acanthoma

Experience of 8 years British Journal of

Der-matology 83(2):248–254

Clear cell acanthoma of Degos

Clear cell acanthoma

Clear cell adenoma

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138 Clostridial myonecrosis

Dosage form

75 mg, 150 mg tablet; intramuscular

prepa-ration; solution for intravenous injection

See table

Cutaneous: skin eruption, pruritus

Gastrointestinal: nausea, vomiting,

diarrhea, abdominal pain, jaundice

Serious side effects,

Bone marrow: thrombocytopenia;

compo-nent; history of ulcerative colitis; caution

with renal or hepatic impairment

References

Weingarten-Arams J, Adam HM (2002)

Clin-damycin Pediatrics in Review 23(4):149–150

Clostridial myonecrosis

Gas gangrene

Clouston’s disease

Hidrotic ectodermal dysplasia

Clubbing of the nails

Definition

A broadening and thickening of the fingers

or toes, with increased lengthwise ture and curvature of the tip of the nail, andflattening of the angle between the cuticleand nail

curva-References

Collins KP, Burkhart CG (1985) Clubbing of the fingers International Journal of Dermatology 24(5):296–297

Cobb syndrome

Synonym(s)

Cutaneomeningospinal angiomatosis

Clindamycin, systemic Dermatologic indications and dosage

Acne vulgaris 150 mg PO 2–3 times daily Not indicated

Gas gangrene 15 mg per kg IV daily divided into

3 doses

10 mg per kg daily IV divided into

3 doses Necrotizing fasciitis 600–900 mg IV every 6–12 hours 25–40 mg per kg IV divided into

3–4 doses daily Paronychia, acute 150 mg PO 3 times daily for

7–10 days

Not indicated Streptococcal toxic

shock-like syndrome

600–900 mg IV every 6–12 hours 25–40 mg per kg IV divided into

3–4 doses daily

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Coccidiosis 139

C

Definition

Association of spinal angiomas or

arteriov-enous malformations with congenital

cuta-neous vascular lesions in the same

der-matome

Pathogenesis

Apparently a developmental abnormality of

the vessels of the spinal cord and skin

Vascular abnormalities, including

asympto-matic port wine stain, angiokeratoma, or

hemangioma; various neurologic findings

depending on level of the vascular

abnor-mality; associated scoliosis or

kyphoscolio-sis

Nevus flammeus; infantile hemangioma;

Sturge-Weber syndrome; Wyburn-Mason

syndrome; Klippel-Trenaunay-Weber

syn-drome; angiokeratoma corporis diffusum

Therapy

Neurosurgical evaluation

References

Shim JH, Lee DW, Cho BK (1996) A case of Cobb

syndrome associated with lymphangioma

cir-cumscriptum Dermatology 193(1):45–47

Coccidioidomycosis

Synonym(s)

Valley fever, San Joaquin Valley fever;

desert rheumatism; coccidiosis

Definition

Disease caused by the spores of the fungus,

Coccidioides immitis

Pathogenesis

Inhalation of arthroconidia from the

organ-ism C immitis; sometimes spreading

within the lungs or via the bloodstream;

rare direct skin inoculation of C immitis

Prodrome of fever, weight loss, malaise, andheadache; acute or subacute pneumonic ill-ness most common clinical presentation,with cough and inspiratory chest painNon-specific skin findings: erythema nodo-sum; erythema multiforme

Specific skin findings: superficial papules;keratotic nodules; verrucous ulcers; subcu-taneous fluctuant abscesses

Other organs of dissemination: bones andjoints; adrenal glands; central nervous sys-tem; liver

Rosacea; tuberculosis; sarcoidosis; mycosis; leishmaniasis; Wegener’s granulo-matosis; vasculitis; syphilis; tinea faciei;sporotrichosis; chromoblastomycosis; par-apsoriasis; mycosis fungoides; lichen planus

actino-Therapy

Disseminated disease: Amphotericin B 0.3–

1 mg per kg per day IV; start with 0.25 mgper kg per day and increased by 5–10 mgper day; fluconazole; itraconazole

plaque on the upper nasal bridge

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140 Cochin China diarrhea

Cochin China diarrhea

Cockayne’s syndrome; dwarfism with

reti-nal atrophy and deafness

Definition

Disorder characterized by sunlight

sensitiv-ity, short stature, neurologic abnormalities,

cataracts, and the appearance of premature

aging

Pathogenesis

Defective DNA repair, specifically

tran-scription-coupled repair; two defective

genes, CSA and CSB, coding for proteins

that interact with components of the

tran-scriptional machinery and with DNA repair

proteins

Growth failure; aged appearance; extreme

photosensitivity; dental abnormalities;

pro-gressive neurologic abnormalities,

includ-ing mental retardation and deafness;

degen-erative retinal pigmentary abnormalities

Xeroderma pigmentosum, particularly the

DeSanctis-Cacchione variant; Bloom’s

syn-drome; progeria; Werner’s synsyn-drome;

Roth-mund-Thompson syndrome;

Cutaneous: skin eruption, alopecia Gastrointestinal: diarrhea, nausea, vomit-

ing, abdominal pain

Hematologic: anemia, thrombophlebitis

Cutaneous: cellulitis Hematologic: agranulocytosis, aplastic ane-

mia, neutropenia

Neurologic: myoneuropathy

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compo-nent; blood dyscrasias; pregnancy; caution

in serious gastrointestinal disorders

References

Ritter S, George R, Serwatka LM, Elston DM

(2002) Long-term suppression of chronic

Sweet's syndrome with colchicine Journal of

the American Academy of Dermatology

Acute, nodular, erythematous eruption at

skin sites exposed to the cold

Pathogenesis

Localized cold injury leading to

inflamma-tion of the subcutaneous adipose tissue;

infants possibly at greater risk because ofincreased fatty acid content in adipose tis-sue

Beginning 1–3 days after a cold injury toexposed or poorly protected areas; painful,firm, red or cyanotic, indurated noduleswith ill-defined margins; in obese patients,buttocks, thighs, arms, and area under thechin are most commonly affected; in smallchildren, is the site of involvement oftencheeks

Subcutaneous fat necrosis of the newborn;sclerema neonatorum; poststeroid pannicu-litis; erythema infectiosum; atopic dermati-tis; cellulitis

385

Colchicine Dermatologic indications and dosage

Acute neutrophilic

dermatosis

0.6–1.8 mg PO daily Not established Aphthous stomatitis 0.6–1.8 mg PO daily Not established

Behçet’s disease 0.6–1.8 mg PO daily Not established

Calcinosis cutis 0.6–1.8 mg PO daily Not established

Dermatomyositis 0.6–1.8 mg PO daily Not established

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Physical urticaria characterized by

ery-thematous papules and plaques arising

when the body temperature cools

Pruritus, erythema, and urticaria

precipi-tated by exposure to cold objects, cold air,

or cold water; sometimes associated with

constitutional signs and symptoms, such as

fever, chills, headache, myalgia, loss of

con-sciousness; symptoms often disappear in a

few months in the acquired type

Aquagenic urticaria; dermatographism;

anaphylaxis from foods, medications, etc.;

Claudy A (2001) Cold urticaria Journal of

Investi-gative Dermatology Symposium Proceedings

6(2):141–142

Collagenoma

Connective tissue nevus

Collagenoma perforant verruciforme

Reactive perforating collagenosis

Collodion baby

Definition

Newborn infant enveloped in a shiny,smooth collodion-like membrane, whichmay deform the facial features and distalextremities

References

Frenk E, de Techtermann F (1992) Self-healing collodion baby: evidence for autosomal reces- sive inheritance Pediatric Dermatology 9(2):95–97

Colloid degeneration

Synonym(s)

Colloid milium; colloid pseudomilium;colloid degeneration of the skin; elastosiscolloidalis conglomerata

Colloid degeneration Multiple translucent

papules on the ear

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Coma blister 143

C

Definition

Deposition of amorphous material

(col-loid) in the dermis

Pathogenesis

Related to excessive sun exposure; juvenile

form inherited; origin of colloid unclear;

possibly formed from degeneration of

elas-tic fibers or synthesized from ultraviolet

light-transformed keratinocytes

Adult type: multiple, discrete, shiny,

trans-lucent papules in sun-exposed areas of face

and ears; sometimes gelatinous material

extruded

Juvenile type: onset before puberty;

numer-ous, yellow-to-brown, waxy papules, mainly

on the face; possibly related to severe

sun-burn

Nodular type: one or a few large,

pink-to-brown, smooth nodules on the face

Nodular amyloidosis; sarcoidosis;

epider-moid cyst; syndrome of Favre-Racouchot;

sebaceous hyperplasia; xanthoma;

tuber-ous sclerosis; porphyria cutanea tarda

Therapy

Cryotherapy; dermabrasion

References

Touart DM, Sau P (1998) Cutaneous deposition

diseases Part I Journal of the American

pres-Clinical manifestation

One or a few vesicles or bullae over sure points, such as fingers, heels, or knees,may involve two limbs apposing oneanother for long periods during an uncon-scious state

pres-Differential diagnosis

Fixed drug eruption; insect bite reaction;localized bullous pemphigoid; herpes sim-plex virus infection; bullous impetigo; por-phyria cutanea tarda; epidermolysis bul-losa acquisita

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Small, flesh-colored, white, or dark

concre-tion found at the opening of a sebaceous

follicle; also known as whitehead or

black-head

References

Thiboutot DM (1996) An overview of acne and its

treatment Cutis 57(1 Suppl):8–12

Pink-to-brown, verrucous, soft papules ornodules of the genitalia, perineum, cruralfolds, and anus, often forming large, exo-phytic, cauliflower-like tumors

Syphilis; verrucous carcinoma of genitalia(giant condyloma of Buschke-Löwenstein);bowenoid papulosis; seborrheic keratosis;anogenital carcinoma; erythroplasia ofQueyrat; lichen planus; Reiter syndrome;pearly penile papules

Therapy

Cryotherapy; imiquimod; podofilox; tolytic agents, such as salicylic acid;destruction by electrodesiccation andcurettage or laser ablation; surgical exci-sion of large tumors

kera-References

Krogh G von (2001) Management of anogenital warts (condylomata acuminata) European Journal of Dermatology 11(6):598–603

Condyloma lata

Definition

Skin lesions associated with secondarysyphilis, characterized by flat-topped,

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Confluent and reticulated papillomatosis 145

C

necrotic papules clustering in

intertrigi-nous sites and secreting a seropurulent

fluid

References

Rosen T, Hwong H (2001) Pedal interdigital

con-dylomata lata: A rare sign of secondary

syphi-lis Sexually Transmitted Diseases 28(3):184–

Cutaneous papillomatosis;

Gougerot-Car-teaud papillomatosis; Gougerot-CarGougerot-Car-teaud

syndrome; atrophie brilliante; confluent

and reticular papillomatosis; confluent and

reticulate papillomatosis;

erythrokeratoder-mia papillaris et reticularis; parakeratose

brilliante; pigmented reticular dermatosis

of the flexures

Definition

Disorder characterized by chronic,

persist-ent, verrucous papules, with a reticulated

pattern and a tendency to become confluent

Tinea versicolor; erythrokeratoderma abilis; epidermodysplasia verruciformis;pityriasis rubra pilaris; acanthosis nigri-cans; dermatopathic pigmentosa reticula-ris; dyschromatosis universalis; epidermalnevus; Naegeli-Franceschetti-Jadassohnsyndrome; flat warts

vari-Therapy

Minocycline; isotretinoin; keratolytics;vitamin A; sodium thiosulphate; oral con-traceptives; tretinoin; ultraviolet light; pro-pylene glycol; calcipotriene

References

Jang HS, Oh CK, Cha JH, Cho SH, Kwon KS (2001) Six cases of confluent and reticulated papillomatosis alleviated by various antibiotics Jour- nal of the American Academy of Dermatology 44(4):652–655

Confluent and reticulated papillomatosis

Reddish-brown, scaly papules coalescing into reticulated plaques

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146 Congenital absence of skin

Congenital absence of skin

Aplasia cutis congenita

Gunther's disease; erythropoietic

porphy-ria; congenital porphyporphy-ria; porphyria

eryth-ropoietica; congenital hematoporphyria;

erythropoietic uroporphyria

Definition

Inborn error of porphyrin-heme synthesis

involving mutation of a gene encoding the

enzyme uroporphyrinogen III synthase,

which leads to accumulation of porphyrins

of the isomer I type, that causes cutaneous

photosensitivity

Pathogenesis

Disorder of bone marrow heme synthesis;

deficient uroporphyrinogen III synthase

activity in erythrocyte precursor cells

causes a shift away from the isomer III

por-phyrinogen production that affects the

end-product heme; isomer I porphyrinogens areoverproduced; interaction of excess por-phyrins in the skin and light radiationcauses photo-oxidative damage of biomo-lecular targets, manifested as mechanicalfragility and blistering

Blistering and fragility of light-exposedskin; hypertrichosis; teeth have a reddishcolor; blepharitis, cicatricial ectropion, andconjunctivitis; hemolytic anemia can causesecondary hypersplenism

Erythropoietic protoporphyria; porphyriacutanea tarda; variegate porphyria; pseu-doporphyria; polymorphous light erup-tion; xeroderma pigmentosum; Bloom’ssyndrome

Therapy

Strict sun avoidance; erythrocyte sion; bone marrow transplantation; beta-carotene 120–300 mg PO per day in divideddoses; activated charcoal; cholestyramine;alpha-tocopherol; ascorbic acid

transfu-References

Desnick RJ, Astrin KH (2002) Congenital poietic porphyria: Advances in pathogenesis and treatment British Journal of Haematology 117(4):779–795

erythro-Congenital erythropoietic protoporphyria

Erythropoietic protoporphyria

Congenital hematoporphyria

Congenital erythropoietic porphyria

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Congenital self-healing Langerhans cell histiocytosis 147

Excess hair growth present in the newborn

period which persists beyond the neonatal

period

References

Schnur RE (1996) Congenital hypertrichosis In:

demis DJ (ed) Clinical Dermatology Lippincott

Williams & Wilkins, Philadelphia, Volume 1

Section 2–26

Congenital keratoma of the

palms and soles

Unna-Thost palmoplantar

Conradi disease

Congenital self-healing Langerhans cell histiocytosis

Synonym(s)

Congenital self-healing Langerhans cellreticulohistiocytosis; congenital histiocyto-sis X; Hashimoto-Pritzker disease

Definition

Heterogeneous eruption, with the cal appearance of Langerhans cell histiocy-tosis, occurring at birth or in infancy andhealing spontaneously

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148 Congenital self-healing Langerhans cell reticulohistiocytosis

Other forms of Langerhans cell

histiocyto-sis; mastocytohistiocyto-sis; lymphoma; juvenile

xan-thogranuloma; benign cephalic

histiocyto-sis

Therapy

None indicated

References

Larralde M, Rositto A, Giardelli M, Gatti CF,

San-tos Munoz A (1999) Congenital self-healing

his-tiocytosis (Hashimoto-Pritzker) International

Shields SR (2000) Managing eye disease in

prima-ry care Part 2 How to recognize and treat mon eye problems Postgraduate Medicine 108(5):83–86, 91–96

com-Connective tissue nevus

Milia; morphea; scar; athlete’s nodules(knuckle pads, etc.); Cowden disease

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Sears JK, Stone MS, Argenyi Z (1988) Papular

elas-torrhexis: A variant of connective tissue nevus

Case reports and review of the literature

Jour-nal of the American Academy of Dermatology

19(2 Pt 2):409–414

Conradi disease

Synonym(s)

Conradi Hunermann syndrome; congenital

punctate chondrodystrophy;

chondrodys-trophia calcificans congenita; dysplasia

epi-physialis punctata; chondrodysplasia

punc-tata, X-linked dominant type

Definition

Form of chondrodysplasia punctata,

char-acterized by punctate opacities within the

growing ends of long bones and other

regions, dysmorphic facial features,

cata-racts, sparse, coarse scalp hair, and/or

abnormal thickening, dryness, and scaling

of the skin

Pathogenesis

Unknown; X-linked dominant trait

Sparse, coarse scalp hair; thickening,

dry-ness, and scaling of the skin;

mild-to-mod-erate growth deficiency; disproportionate

shortening of long bones, particularly those

of the humeri and the femora; short

stat-ure; kyphoscoliosis; prominent forehead

with midfacial hypoplasia and a low nasal

bridge; cataracts

Epidermal nevus; incontinentia pigmenti;

ichthyosis vulgaris; X-linked ichthyosis

Conradi Hunermann syndrome

Pathogenesis

Irritant variant: caused by direct injury ofthe skin by an agent capable of producing

Trang 22

150 Contact eczema

cell damage in any individual if applied for

sufficient time and in sufficient

concentra-tion

Allergic variant: type IV hypersensitivity

reaction only affecting previously

sensi-tized individuals

Contact urticaria variant: possibly

immu-nologic in some cases

Photocontact variant: irradiation of certain

substances by light resulting in the

transfor-mation of the substance into full antigens

(photoallergic) or irritants (phototoxic)

Acute contact stage: red and edematous

skin; vesicles or bullae sometimes develop;

weeping and oozing as vesicles rupture

Subacute stage: less edematous and

ery-thematous; scaling and punctate crusts

from scratching (excoriations) often

present

Chronic stage: scaling, fissuring, and

lichenification with minimal edema

Contact urticaria variant: urticarial wheals

at site of contact

Phototoxic variant: appearance of an

exag-gerated sunburn

Atopic dermatitis; dyshidrotic eczema;

sun-burn; chemical sun-burn; seborrheic

dermati-tis; insect bites; erysipelas; erythema

multi-forme; nummular eczema; lichen simplex

chronicus; asteatotic eczema; bullous

pem-phigoid; pemphigus vulgaris;

epidermoly-sis bullosa; dermatophyte infection; diasis; impetigo; scabies

candi-Therapy

Removal of source of dermatitisMild-to-moderate disease: corticosteroids,topical, mid potency or high potency; alu-minium acetate 5% compresses applied 15–

30 minutes 2–4 times dailySevere disease: prednisone; antihista-mines, first generation, for sedation

Wakelin SH (2001) Contact urticaria Clinical & Experimental Dermatology 26(2):132–136

Orf

Contact dermatitis Erythematous, edematous

plaques around the eyes in a patient with an

allergic contact dermatitis to a topical eye

medication

Trang 23

Corticosteroids, topical, high potency 151

Brachmann-de Lange syndrome; de Lange

syndrome; Amsterdam syndrome; typus

degenerativus amstelodamensis

Definition

Syndrome characterized by a distinctive

facial appearance, prenatal and postnatal

growth deficiency, feeding difficulties,

psy-chomotor delay, behavioral problems;

mal-formations mainly involve the upper

extremities

Pathogenesis

Unknown; few cases transmitted in

auto-somal dominant pattern

Short stature; microcephaly; facial features:

confluent eyebrows, long curly eyelashes,

low anterior and posterior hairline,

under-developed orbital arches, anteverted nares,

down-turned angles of the mouth, thin lips,

low-set ears, depressed nasal bridge,

micro-gnathia; hypertrichosis; micromelia;

Opitz JM, Brachmann-de Lange syndrome (1994)

A continuing enigma Archives of Pediatrics &

Adolescent Medicine 148(11):1206–1208

Corporis circumscriptum naeviforme

Angiokeratoma circumscriptum

Corpus callosum facial anomalies-Robin sequence syndrome

agenesis- Toriello-Carey syndrome

Corrugated skin

Cutis verticis gyrata

Corticosteroids, topical, high potency

Trade name(s)

Generic in parentheses:

Cyclocort (amcinonide); Lidex, Lidex-E,Licon (fluocinonide); Topicort (desoximeta-sone); Diprosone, Maxivate, Alphatrex (bet-amethasone dipropionate); Halog, Halog-E(halcinonide)

Trang 24

152 Corticosteroids, topical, high potency

See table

Cutaneous: skin atrophy; steroid addiction

(rebound flare after discontinuing the

med-ication); tachyphylaxis; increased

suscepti-bility to local infection; perioral dermatitis;

delayed wound healing; hypopigmentation;acneform eruption; striae

Miscellaneous: adrenal insufficiency

Drug interactions

None

Corticosteroids, topical, high potency Dermatologic indications and dosage

Atopic dermatitis Apply twice daily Apply twice daily

Bullous pemphigoid Apply twice daily Apply twice daily

Contact dermatitis Apply twice daily Apply twice daily

Dyshidrotic eczema; Apply twice daily Apply twice daily

Pemphigus vulgaris Apply twice daily Apply twice daily

Pityriasis lichenoides Apply twice daily Apply twice daily

Apply twice daily Apply twice daily

Psoriasis Apply twice daily Apply twice daily

Seabather’s eruption Apply twice daily Apply twice daily

Seborrheic dermatitis Apply twice daily Apply twice daily

Subcorneal pustular

dermatosis

Apply twice daily Apply twice daily

T cell lymphoma Apply twice daily Apply twice daily

Xerotic dermatitis Apply twice daily Apply twice daily

Trang 25

Corticosteroids, topical, low potency 153

C

compo-nent; avoid use on the face for more than 14

days; avoid getting in the eye; do not apply

in intertriginous areas for more than 1 week

at a time

References

Brazzini B, Pimpinelli N (2002) New and

estab-lished topical corticosteroids in dermatology:

clinical pharmacology and therapeutic use

American Journal of Clinical Dermatology

Hydrocortisone 1% (Hytone; Cortef;

Cor-taid; Texacort); alclometasone 0.05%

(Aclovate); desonide 0.05% (Tridesilon;

Cream; ointment; lotion; gel

See table

(rebound flare after discontinuing the ication); tachyphylaxis; increased suscepti-bility to local infection; perioral dermatitis;delayed wound healing; hypopigmentation;acneform eruption; striae

med-Serious side effects

estab-American Journal of Clinical Dermatology 3(1):47–58

Corticosteroid, topical, low potency Dermatologic indications and dosage

Dyshidrosis Apply twice daily Apply twice daily

Netherton syndrome Apply twice daily Apply twice daily

Nummular eczema Apply twice daily Apply twice daily

Pityriasis alba Apply twice daily Apply twice daily

Xerotic eczema Apply twice daily Apply twice daily

Trang 26

154 Corticosteroids, topical, medium potency

Corticosteroids, topical,

medium potency

Trade name(s)

Generic in parentheses:

Kenalog, Aristocort (triamcinolone);

Valisone, Betatrex, Luxiq (betamethasone

valerate); Cloderm (clocortolone); Cordran

(flurandrenolide); Cutivate (fluticasone);

Dermatop (prednicarbate); Synalar,

Derma-Smoothe (fluocinolone); Elocon

(mometa-sone); Locoid (hydrocortisone butyrate);

Uticort (betamethasone benzoate);

West-cort (hydroWest-cortisone valerate)

Cream; ointment; lotion; gel; foam

See table

(rebound flare after discontinuing the ication); tachyphylaxis; increased suscepti-bility to local infection; perioral dermatitis;delayed wound healing; hypopigmentation;acneform eruption; striae

med-Serious side effects

Corticosteroids, topical, medium potency Dermatologic indications and dosage

Benign pigmented

purpura;

Apply twice daily Apply twice daily Cercarial dermatitis Apply twice daily Apply twice daily

Dyshidrosis Apply twice daily Apply twice daily

Id reaction Apply twice daily Apply twice daily

Idiopathic guttate

hypomelanosis

Apply twice daily Apply twice daily Keratosis pilaris Apply twice daily Apply twice daily

Nummular eczema Apply twice daily Apply twice daily

Pityriasis lichenoides Apply twice daily Apply twice daily

Prurigo of pregnancy Apply twice daily Apply twice daily

Psoriasis Apply twice daily Apply twice daily

Stasis dermatitis Apply twice daily Apply twice daily

Wiskott-Aldrich

syndrome

Apply twice daily Apply twice daily Xerotic eczema Apply twice daily Apply twice daily

Trang 27

compo-nent; avoid use on the face for more than 14

days; avoid getting in the eye; do not apply

in intertriginous areas for more than 2

weeks at a time

References

Brazzini B, Pimpinelli N (2002) New and

estab-lished topical corticosteroids in dermatology:

clinical pharmacology and therapeutic use

American Journal of Clinical Dermatology

Temovate, Olux, Cormax, Embeline

(clobetasol); Ultravate (halobetasol);

Dipro-lene AF (augmented betamethasone

dipro-pionate); Psorcon, Maxiflor, Florone

(diflo-rasone diacetate); Cordran Tape

Cream; ointment; lotion; gel; foam; tape

See table

(rebound flare after discontinuing the

med-ication); tachyphylaxis; increased bility to local infection; perioral dermatitis;delayed wound healing; hypopigmentation;acneform eruption; striae

suscepti-Serious side effects

compo-at a time

References

Brazzini B, Pimpinelli N (2002) New and lished topical corticosteroids in dermatology: clinical pharmacology and therapeutic use

estab-American Journal of Clinical Dermatology 3(1):47–58

asso-Pathogenesis

Low constitutive levels of protein C; in thepresence of coumarin, levels of protein Cfall more rapidly than do procoagulant fac-tors IX, X and prothrombin, producing atransient hypercoagulable state and localthrombosis of dermal vessels

Signs and symptoms beginning 3–5 daysafter initiation of coumarin; single or multi-ple areas of painful erythema rapidly ulcer-

Trang 28

156 Coumarin necrosis

ating and developing a blue-black eschar;

most common areas of involvement: thighs,

breasts, and buttocks; most likely

occur-ring in patients in whom large initial doses

of coumarin initiated in the absence ofheparin anticoagulation

Corticosteroids, topical, super potency Dermatologic indications and dosage

Alopecia areata Apply twice daily for up to 2 weeks;

1 week rest period before reuse

Apply twice daily for up to 2 weeks;

1 week rest period before reuse Atopic dermatitis Apply twice daily for up to 2 weeks;

1 week rest period before reuse Contact dermatitis Apply twice daily for up to 2 weeks;

1 week rest period before reuse Dyshidrosis Apply twice daily for up to 2 weeks;

1 week rest period before reuse Eosinophilic pustular

folliculitis

1 week rest period before reuse Inflammatory

epidermal nevus

1 week rest period before reuse Lichen nitidus Apply twice daily for up to 2 weeks;

1 week rest period before reuse Lichen planus Apply twice daily for up to 2 weeks;

1 week rest period before reuse Lichen simplex

chronicus

1 week rest period before reuse Lupus erythematosus,

discoid

1 week rest period before reuse Lupus erythematosus,

subacute systemic

1 week rest period before reuse Mastocytosis Apply twice daily for up to 2 weeks;

1 week rest period before reuse Nummular eczema Apply twice daily for up to 2 weeks;

1 week rest period before reuse Pemphigus foliaceus Apply twice daily for up to 2 weeks;

1 week rest period before reuse Pityriasis lichenoides Apply twice daily for up to 2 weeks;

1 week rest period before reuse Psoriasis Apply twice daily for up to 2 weeks;

1 week rest period before reuse Reiter syndrome Apply twice daily for up to 2 weeks;

1 week rest period before reuse Seborrheic dermatitis Apply twice daily for up to 2 weeks;

1 week rest period before reuse Vitiligo Apply twice daily Apply twice daily

Xerotic eczema Apply twice daily for up to 2 weeks;

Trang 29

Cowden/Bannayan-Riley-Ruvalcaba overlap syndrome 157

C

Other coagulopathies; heparin necrosis;

spider bite reaction; pyoderma

gangreno-sum; vasculitis; cutaneous anthrax;

trau-matic ulceration; calciphylaxis; necrotizing

soft tissue infection

Therapy

Medical therapy: continued coumarin

ther-apy

Surgical therapy: hydrocolloid dressings;

skin grafting if healing markedly delayed

References

Cole MS, Minifee PK, Wolma FJ (1988) Coumarin

necrosis – A review of the literature Surgery

Cowden’s syndrome; Cowden syndrome;

multiple hamartoma syndrome

Definition

Hamartomatous neoplasms of the skin and

mucosa, gastrointestinal tract, bones,

cen-tral nervous system, eyes, and

genitouri-nary tract

Pathogenesis

Mutation in the PTEN tumor suppressor

gene on chromosome 10q23 regulating the

function of other proteins by removing

phosphate groups from those molecules;

mutation causing loss of the protein's

func-tion and allowing over-proliferafunc-tion of cells,

resulting in hamartomatous growths

Flesh-colored, flat-topped, lichenoid or

elongated, verrucous papules of the face;

oral cavity papules with a smooth surfaceand a whitish color; sometimes coalescinginto cobblestone-like plaques; acral kera-totic papules, including palmoplantar kera-totic papules; thyroid abnormalities; fibro-cystic disease and fibroadenomas of thebreast; increased incidence of breast carci-noma; gastrointestinal polyps; ovariancysts; uterine leiomyomas

Wart; sebaceous hyperplasia; milia; thoma; trichilemmoma; trichoepithelioma;Darier disease; syringoma; fibrofollicu-loma; multiple benign fibromas; multipleendocrine neoplasia; tuberous sclerosis;lipoid proteinosis; Goltz syndrome; floridoral papillomatosis

Cowden/Bannayan-Riley-syndrome

Bannayan-Riley-Ruvalcaba drome

Trang 30

Gastrointestinal polyposis syndrome,

gen-eralized, associated with

hyperpigmenta-tion, alopecia, and nail atrophy

Definition

Association of generalized gastrointestinal

polyps, cutaneous pigmentation, alopecia,

and onychodystrophy

Pathogenesis

Unknown

Onset of constant or episodic pain in the

lower or upper abdomen, with weight loss;

alopecia simultaneously from the scalp,

eye-brows, face, axillae, pubic areas, and

extremities; lentigo-like macules and/or

dif-fuse hyperpigmentation, including the cal mucosa; nail dystrophy; peripheral orgeneralized edema; multiple gastrointesti-nal polyps, with increased incidence ofcolon carcinoma

buc-Differential diagnosis

Gardner’s syndrome; Peutz-Jeghers drome; Bandler syndrome; Ménétrier dis-ease; familial polyposis

syn-Therapy

No therapy for cutaneous manifestations;close follow-up for gastrointestinal prob-lems

References

Finan MC, Ray MK (1989) Gastrointestinal posis syndromes Dermatologic Clinics 7(3):419–434

cryofibrino-Pathogenesis

Unknown

Trang 31

Cryptococcosis 159

C

Primary (essential) form: unassociated with

underlying disease; secondary form:

associ-ated most commonly with internal

malig-nancies and thromboembolic disease, but

also with rheumatic diseases, diabetes

mel-litus, and pregnancy; purpura;

ecchy-moses; cutaneous gangrene; persistent,

painful ulcerations, with surrounding

livedo reticularis

Cryoglobulinemia; benign pigmented

pur-pura; antiphospholipid antibody

syn-drome; Churg-Strauss synsyn-drome;

polyar-teritis nodosa; serum sickness;

Walden-ström hyperglobulinemia; septic vasculitis;

systemic lupus erythematosus; sarcoidosis

Therapy

Stanozolol: 4–8 mg PO daily;

plasmapher-esis

References

Helfman T, Falanga V (1995) Stanozolol as a novel

therapeutic agent in dermatology Journal of

the American Academy of Dermatology 33(2 Pt

Presence of abnormal proteins in the

blood-stream, which thicken or gel on exposure to

cold

Pathogenesis

Some of the sequelae of cryoglobulinemia

related to immune-complex disease; other

sequelae related to cryoprecipitation in

vivo, including plugging and thrombosis of

small arteries and capillaries; some cases in

otherwise normal patients (essential mixed

cryoglobulinemia)

Skin findings: palpable purpura; distalnecrosis; urticaria, and ischemic necrosisleading to ulceration; cold-induced urti-caria; acrocyanosis

Internal manifestations: pulmonary; renal;joints; central nervous system; sometimespresent in mycoplasma pneumonia, viralhepatitis, multiple myeloma, certain leuke-mias, primary macroglobulinemia, andsome autoimmune diseases, such as sys-temic lupus erythematosus and rheuma-toid arthritis

Antiphospholipid antibody syndrome;Churg-Strauss syndrome; polyarteritisnodosa; serum sickness; Waldenströmhyperglobulinemia; septic vasculitis; sys-temic lupus erythematosus; sarcoidosis

Trang 32

blasto-160 Cushing syndrome

Definition

Fungal infection caused by the inhalation of

the fungus, Cryptococcus neoformans

Pathogenesis

Human disease associated only with

Cryp-tococcus neoformans; following inhalation

of the organism, alveolar macrophages

ingest the yeast; cryptococcal

polysaccha-ride capsule has antiphagocytic properties

and may be immunosuppressive;

anti-phagocytic properties of the capsule block

recognition of the yeast by phagocytes and

inhibit leukocyte migration into the area of

fungal replication; decreased host

immu-nity main element in susceptibility to

clini-cal infection; organ damage primarily from

tissue distortion secondary to increasing

fungal burden

Pre-existing medical problems, such as

sys-temic steroid use, malignant disease, organ

transplantation, or HIV infection

Skin findings: papules, sometimes

umbili-cated; pustules; nodules; ulcers; draining

sinuses; rarely occurs as a primary

inocula-tion disease

Internal organ involvement: pulmonary –

variable, ranging from asymptomatic

air-way colonization to acute respiratory

dis-tress syndrome

Central nervous system: usually meningitis

or meningoencephalitis

Pyogenic abscess; nocardia, aspergillosis;

lymphoma; meningeal metastases;

tubercu-losis; histoplasmosis; acne; molluscum

con-tagiosum; syphilis; toxoplasmosis

Therapy

Non-AIDS-related: amphotericin B 0.5–

1 mg per kg per day IV; total cumulative

dose of 3 gm; fluconazole

AIDS-related infection: initially,

amphoter-icin B for 2 weeks, with or without 2 weeks

of flucytosine, followed by fluconazole for a

minimum of 10 weeks

References

Thomas I, Schwartz RA (2001) Cutaneous festations of systemic cryptococcosis in immu- nosuppressed patients Journal of Medicine 32(5-6):259–266

adminis-Clinical manifestation

Skin changes: facial plethora; striae; moses and purpura; telangiectasias; skinatrophy; hirsutism and male pattern bald-ing in women; increased lanugo facial hair;steroid acne; acanthosis nigricans

ecchy-Central obesity; increased adipose tissue inthe face (moon facies), upper back at thebase of neck (buffalo hump), and above theclavicles

Endocrine abnormalities: hypothyroidism;galactorrhea; polyuria and nocturia fromdiabetes insipidus

Menstrual irregularities, amenorrhea, andinfertility

Other organ system abnormalities: vascular; musculoskeletal; gastroentero-logic; neuropsychological

cardio-Differential diagnosis

Exogenous obesity; anorexia nervosa; holism; drug effects from phenobarbitalphenytoin or rifampin; psychiatric illness

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