Dermatology therapy essentials - part 4 ppsx

Genodermatosis characterized by reticu-lated hyperpigmentation, nail dystrophy, premalignant leukoplakia of the oral mucosa, and progressive pancytopenia Pathogenesis Mutations in DKC1 c

Trang 1

Dryness of skin 193

D

Doxycycline Dermatologic indications and dosage

Disease Adult dosage Child dosage

Acne vulgaris 50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice

daily Anthrax 100 mg PO twice daily for 60 days in

bioterrorism situation

> 8 years old – 50 mg PO twice daily for 60 days in bioterrorism situation Atrophoderma of

bedtime, followed by 100 mg PO twice daily for 3 days

> 8 years old – 2–5 mg per kg PO daily for 7–10 days

Bullous pemphigoid 50–100 mg PO twice daily > 8 years old – 50 mg PO twice daily

> 8 years old – 200 mg PO or IV twice daily for 3 days, then maintenance dose 100 mg PO or IV twice daily until 48–72 hours after patient becomes afebrile Folliculitis 50–100 mg PO twice daily > 8 years old – 50 mg PO twice daily

Leptospirosis 100 mg PO twice daily for 3 weeks > 8 years old – 50 mg PO twice daily

for 3 weeks Linear IgA bullous

for 5 days Rocky Mountain

spotted fever

100 mg PO twice daily for 7–10 days > 8 years old – 2 mg per kg PO or IV

loading dose, followed by 1 mg per

kg PO or IV every 12 hours for 7 days and for at least 48 hours after defervescence

Rosacea 100 mg PO twice daily for at least

30 days

> 8 years old – 50–100 mg PO twice daily for at least 30 days

Trang 2

Palmoplantar fibromatosis; Dupuytren'sdisease, palmar fasciitis; Viking disease

Definition

Disorder characterized by subcutaneousfascia thickening and shortening, causingthe fingers to retract down towards thepalm of the hand

Pathogenesis

Unclear; dominant genetic inheritance;often involves individuals of northern Euro-pean descent; trauma sometimes initiates

or accelerates the process; associated withalcoholism, diabetes mellitus, smoking, epi-lepsy, pulmonary disease

Clinical manifestation

Asymptomatic, palmar skin nodule, ally within the distal aspect of the palm,often with puckering of the skin above thenodularity; overlying skin sometimesadherent to the fascia, and fibrous cordsometimes extending into the finger; ringfinger most commonly involved site, fol-lowed by the small finger

gener-Differential diagnosis

Trigger finger

Scrub typhus 100 mg PO twice daily for 7–14 days > 8 years old – 50 mg PO twice daily

for 14 days Trench fever 100 mg PO twice daily for 4 weeks > 8 years old – 50 mg PO twice daily

for 4 weeks Tularemia 100 mg PO twice daily for 7–14 days

or until patient is afebrile for 5–7 days

> 8 years old – 50 mg PO twice daily for 7–14 days or until patient is afebrile for 5–7 days

Yaws 100 mg PO twice daily for 15 days > 8 years old – 2–5 mg per kg PO

divided into 2 doses daily for 15 days

Doxycycline Dermatologic indications and dosage (Continued)

Trang 3

Dyshidrotic eczema 195

D

Therapy

Physical therapy in early stages;

intrale-sional triamcinolone 3–5 mg per ml; partial

surgical fasciectomy for a patient with

sig-nificant functional disability

References

Saar JD, Grothaus PC (2000) Dupuytren's disease:

An overview Plastic & Reconstructive Surgery

106(1):125–134

Dupuytren's disease

Dupuytren’s contracture

Dwarfism with retinal

atrophy and deafness

Dyshidrosis; pompholyx; vesicular

pal-moplantar eczema; vesicular eczema of

palms and soles

Symmetric crops of clear vesicles and/orbullae on the palms and lateral aspects offingers and feet; vesicles deep seated, with atapioca-like appearance, and sometimesbecoming confluent to form bullae; maydevelop crusting, scaling, and fissuringafter persistent scratching

Differential diagnosis

Contact dermatitis; vesicular tinea pedis;tinea manus; palmoplantar pustular psoria-sis; autosensitization reaction (id reaction)

References

Landow K (1998) Hand dermatitis The perennial scourge Postgraduate Medicine 103(1):141–142, 145–148, 151–152

Dyshidrotic eczema Multiple vesicles on the

hands, with concentration along the sides of the digits

Trang 4

Genodermatosis characterized by

reticu-lated hyperpigmentation, nail dystrophy,

premalignant leukoplakia of the oral

mucosa, and progressive pancytopenia

Pathogenesis

Mutations in DKC1 cause X-linked recessive

form; involved in the regulation of the

pro-liferative capacity of the cell; defect in

maintenance of telomeres results in

chro-mosomal instability, telomeric

rearrange-ments, and cancer progression; etiology of

autosomal dominant and autosomal

reces-sive forms unknown

Cutaneous manifestations developing

between 5 and 15 years of age; tan-to-gray,

hyperpigmented or hypopigmented

mac-ules and patches in a mottled, or

reticu-lated pattern, sometimes with

poikilo-derma; located on the upper trunk, neck,

and face, often with involvement of

sun-exposed areas; scalp alopecia; mucosal

leu-koplakia on the buccal mucosa, tongue,

oropharynx, esophagus, urethral meatus,

glans penis, lacrimal duct, conjunctiva,

vagina, anus; dental caries; progressive nail

dystrophy; increased incidence of

malig-nant neoplasms, particularly squamous cell

carcinoma of the skin, mouth,

nasophar-ynx, esophagus, rectum, vagina, and

cer-vix; late bone marrow failure; pulmonarycomplications

Graft versus host disease; Fanconi drome; Rothmund-Thompson syndrome;ataxia telangiectasia

Dysplasia epiphysialis punctata

Epidermolysis bullosa

Trang 5

Early-onset prurigo of pregnancy

flesh-tendency for central ulceration; occasionalmalignant degeneration

Basal cell carcinoma; lymphangioma;

hemangioma; squamous cell carcinoma

Therapy

Surgical excision

References

Ishikawa M, Nakanishi Y, Yamazaki N, Yamamoto

A (2001) Malignant eccrine spiradenoma: A case report and review of the literature Derma- tologic Surgery 27(1):67–70

PART5.MIF Page 197 Friday, October 31, 2003 10:08 AM

Trang 6

198 Eccrine chromhidrosis

Pathogenesis

Derived from any portion of the eccrine

apparatus or resulting from the malignant

transformation of an existing benign

eccrine tumor

Non-specific solitary nodule or plaque with

occasional ulceration, on the head,

extremi-ties, or trunk

Basal cell carcinoma; squamous cell

carci-noma; Merkel cell carcicarci-noma; cutaneous

metastasis; eccrine acrospiroma;

micro-cystic adnexal carcinoma; eccrine

porocar-cinoma; cutaneous adenoid cystic

carci-noma

Therapy

Wide local excision; Mohs micrographic

surgery; radiation therapy

References

Katzman BM, Caligiuri DA, Klein DM, DiMaio

TM, Gorup JM (1997) Eccrine carcinoma of the

hand: a case report Journal of Hand Surgery –

Clear cell hidradenoma; clear cell

myoepi-thelioma; solid cystic hidradenoma

ma Journal of the American Academy of matology 12:15–20

Possibly adenomatous cystic proliferations

of the eccrine glands or retention cysts ofthe eccrine sweat apparatus

Asymptomatic, solitary, ish papule, with a predilection for the peri-orbital area

translucent-to-blu-PART5.MIF Page 198 Friday, October 31, 2003 10:08 AM

Trang 7

Ecthyma 199

E

Apocrine hidrocystoma; basal cell

carci-noma; epidermoid cyst; mucous cyst;

syrin-goma; milium; steatocystoma multiplex

Therapy

Incision and drainage, followed by surgical

destruction of the cyst wall by light

electro-desiccation and curettage; punch, shave, or

elliptical excision

References

Alfadley A, Al Aboud K, Tulba A, Mourad MM

(2001) Multiple eccrine hidrocystomas of the

face International Journal of Dermatology

Pathogenesis

Caused by bacterial infection, usually tococcus but sometimes Staphylococcus;predisposing factors: previous tissue injury,immunocompromised state; environmentalfactors: high temperature and humidity,crowded living conditions, poor hygiene

Strep-Clinical manifestation

Begins as a vesicle or pustule, ulceratingand producing a yellowish crust with ery-thematous, indurated borders

Herpes simplex virus infection; atypicalmycobacterial infection; nocardia infec-tion; sporotrichosis; trauma; insect or spi-der bite reaction; pyoderma gangrenosum

Therapy

Mupirocin ointment applied 3 times dailyfor 7–10 days; dicloxacillin; cephalexin;known Streptococcal infection: penicillin

Trang 8

200 Ecthyma contagiosum

References

Mancini AJ (2000) Bacterial skin infections in

children: the common and the not so common

Cutaneous manifestation of Pseudomonas

aeruginosa bacteremia, usually occurring

in patients who are critically ill and/or

immunocompromised

Pathogenesis

Caused by Pseudomonas aeruginosa, a

gram negative bacterial pathogen which

disseminates in patients with impaired

cel-lular or humoral immunity or those with

severe underlying illnesses such as severe

burns, malnutrition, recent chemotherapy,

immunosuppressive therapy, or diabetes

mellitus

Appears as edematous, well-circumscribed

plaques, rapidly evolving into hemorrhagic

bullae, spreading peripherally, and

eventu-ally turning into a black necrotic ulcer with

an erythematous rim; commonly occurs in

the gluteal or perineal region or

extremi-ties; sign of widespread dissemination of

infection

Ecthyma; herpes simplex virus infection;

atypical tuberculosis; nocardiosis;

sporotri-chosis; trauma; gram negative folliculitis;pyoderma gangrenosum; septicemia fromother infectious agents; cryoglobulinemia;polyarteritis nodosa; necrotizing fasciitis;vasculitis

Therapy

Initial therapy: antipseudomonal penicillin(piperacillin) with an aminoglycoside (gen-tamicin)

Subsequent therapy based on culture tivity

sensi-References

Khan MO, Montecalvo MA, Davis I, Wormser GP (2000) Ecthyma gangrenosum in patients with acquired immunodeficiency syndrome Cutis 66(2):121–123

Anhidrotic ectodermal dysplasia

Ectodermal dysplasia, hidrotic

Hidrotic ectodermal dysplasia

Trang 9

Eczematidlike purpura of Doucas and Kapetanakis 201

Eruption caused by herpes simplex virus

(HSV)-1, herpes simplex virus (HSV)-2,

Coxsackie A16 virus, or vaccinia virus that

infects a preexisting dermatosis, most

com-monly atopic dermatitis

Pathogenesis

Caused by herpes simplex virus (HSV)-1,

herpes simplex virus (HSV)-2, Coxsackie

A16 virus, or vaccinia virus infecting a

pre-existing dermatosis; possibly associated

with local T-cell immune defect, low NK

cells, and/or a low antibody titer against the

infective organism

Presents as clusters of umbilicated pustules in areas where the skin has beenaffected by a preexistent dermatitis; umbili-cated vesiculopustules progress to ero-sions, usually over the upper trunk andhead; vesicles often become hemorrhagicand crusted, coalescing to form large,denuded plaques that bleed and sometimesbecome secondarily infected with bacteria

Benign pigmented purpura

Trang 10

202 Eczematoid epitheliomatous dermatosis

Possibly related to ornithine decarboxylase

inhibition, which decreases hair growth

Dosage form

13.9% cream

Dermatologic indications and dosage

See table

Common side effects

Cutaneous: stinging; burning sensation,

irritant contact dermatitis, acneform tion, pseudofolliculitis barbae

erup-Serious side effects

Hu-Ehlers Danlos syndrome

connec-Pathogenesis

Specific collagen defect has been identified

in 6 of the 11 types: Type IV – decreased

Eflornithine Dermatologic indications and dosage

Hypertrichosis Apply twice daily Apply twice daily

Trang 11

Elastolysis 203

E

type III collagen; types V and VI –

deficien-cies in hydroxylase and lysyl oxidase; type

VII – amino-terminal procollagen

pepti-dase deficiency; type IX – abnormal copper

metabolism; type X – nonfunctioning

plasma fibronectin

Findings common to all subtypes: skin

hyperextensible, doughy, white, and soft,

with underlying vessels sometimes visible;

small, spongy tumors (molluscoid

pseudo-tumors) over scars and pressure points;

smaller palpable, and movable calcified

nodules in subcutaneous tissue; nodules in

arms and over tibias; skin fragility, with

fre-quent bruises, lacerations, and poor wound

healing; hyperextensible joints, with

fre-quent dislocations

Pseudoxanthoma elasticum; cartilage-hair

syndrome; cutis laxa; Turner’s syndrome;

Marfan syndrome

Therapy

None; avoidance of surgery, if possible,

because of poor-healing wounds

References

Germain DP (2002) Clinical and genetic features

of vascular Ehlers-Danlos syndrome Annals of

Pathogenesis

Possibly related to trauma by mechanicalfriction of the scapula against the ribs insome cases

Well-circumscribed, painless or slightlytender tumor in the subscapular area in eld-erly women

Elastofibroma dorsi

Elastofibroma

Elastolysis

Cutis laxa Ehlers Danlos syndrome Marked joint

hypermobility of digits

Trang 12

204 Elastolysis cutis laxa

Elastolysis cutis laxa

Synonym(s)

Elastosis perforans serpiginosum; elastosisintrapapillare; elastoma intrapapillare per-forans; elastoma intrapapillare perforansverruciformis; elastosis perforans; elastomaverruciform perforans; keratosis follicu-laris et parafollicularis serpiginosa;keratosis follicularis serpiginosa; reactiveperforating elastosis

Definition

Skin condition with abnormal dermal tic tissue fibers and other connective tissueelements expelled via trans-epidermal elim-ination

elas-Pathogenesis

Granulomatous inflammation displaying anatypical method for removing elastic tissuefrom the area of involvement

Three subtypes:

Reactive form: associated with other eases such as Down syndrome, Ehlers-Dan-los syndrome, Marfan syndrome, osteogen-esis imperfecta, scleroderma, acrogeria,pseudoxanthoma elasticum

dis-Drug-induced form: associated with cillamine use

peni-Idiopathic form (most common variety):flesh-colored or pale red, umbilicatedpapules grouped in linear, arciform, circu-lar, or serpiginous patterns; most com-monly occurring over the nape of the neck

Trang 13

Enchondromatosis with multiple cavernous hemangiomas 205

E

Reactive perforating collagenosis;

perforat-ing folliculitis; Kyrle’s disease; folliculitis;

prurigo nodularis; granuloma annulare;

tinea corporis; lupus erythematosus

Therapy

Tretinoin; isotretinoin; cryotherapy;

elec-trodessication and curettage

References

Mehta RK, Burrows NP, Payne CM, Mendelsohn

SS, Pope FM, Rytina E (2001) Elastosis

per-forans serpiginosa and associated disorders

Clinical & Experimental Dermatology

Visible enlargement of the arms, legs, or

genitals to elephantoid size, usually

second-ary to chronic lymphedema

References

McGuinness CL, Burnand KG (2001)

Lymphoede-ma Tropical Doctor 31(1):2–7

Elephantiasis nostras verrucosa

Definition

In later stages of chronic lymphedema,affected skin becomes indurated and devel-ops verrucous papules and plaques withscale

References

Brantley D, Thompson EC, Brown MF (1995) ephantiasis nostras verrucosa Journal of the Louisiana State Medical Society 147(7):325–327

El-Enchondromatosis

Maffucci syndrome

Enchondromatosis with multiple cavernous hemangiomas

Maffucci syndrome

Elephantiasis nostras verrucosa Plaque

consisting of multiple nodules on the distal lower extremity

Trang 14

206 Endemic pemphigus foliaceus

Dabska tumor; malignant endovascular

papillary angioendothelioma; papillary

intralymphatic angioendothelioma

Definition

Low-grade angiosarcoma of the skin of

chil-dren, with a distinctive histologic

architec-ture of anastomosing vascular channels

with intravascular papillary outpouchings

Pathogenesis

Unclear cell of origin, but tumor marker

studies suggest resemblance to

Enlarged solitary comedone

Dilated pore

Eosinophilia-myalgia syndrome

Synonym(s)

L-tryptophan-induced gia syndrome; sclerodermoid myalgia;sclerodermoid fasciitis

eosinophilia-myal-Definition

Multisystem disease with prominent nophilia and generalized myalgia, usuallyassociated with L-tryptophan ingestion

eosi-Pathogenesis

Cell-mediated immune response causingwidespread tissue injury; skin and connec-tive tissue fibrosis pervading muscles,nerves, and other organs; L-tryptophaninvolvement in process, but mechanismunclear

Trang 15

Eosinophilic granulomatous vasculitis 207

E

Acute episode: shortness of breath, cough,

fever; fatigue, arthralgias, paresthesias,

severe weakness, muscle cramps,

perior-bital and peripheral edema, generalized

erythematous eruption

Chronic signs and symptoms: generalized

myalgias, skin tightening; fingers and toes

usually spared; Raynaud phenomenon

usu-ally absent; scalp alopecia; cutaneous

hyperesthesia

Progressive systemic sclerosis; toxic oil

syn-drome; dermatomyositis; polymyositis;

eosinophilic fasciitis; mixed connective

Blackburn WD Jr (1997) Eosinophilia myalgia

syndrome Seminars in Arthritis &

Rheuma-tism 26(6):788–793

Eosinophilic cellulitis

Synonym(s)

Wells syndrome; recurrent granulomatous

dermatitis with eosinophilia, Wells’

syn-drome, Well’s syndrome

Definition

Cellulitis-like eruption with typical

histol-ogy, including flame figures and marked

dermal infiltrate of eosinophils

Pathogenesis

Association with insect bites in some cases

Pruritus and burning sensation, followed by

cellulitis-like eruption; large, indurated

plaques of edema and erythema, with

viola-ceous edges; occasionally also annular

plaques, papules, and urticarial-like wheals;recurrent episodes common

Cellulitis; erysipelas; urticaria; insect bitereaction; Lyme disease; hypereosinophilicsyndrome; inflammatory metastasis; granu-loma annulare; Churg-Strauss syndrome

Kimura’s disease

Eosinophilic granulomatous vasculitis

Churg-Strauss syndrome

Trang 16

208 Eosinophilic hyperplastic lymphogranuloma

Ofuji's disease; Ofuji disease; eosinophilic

folliculitis; HIV-associated eosinophilic

fol-liculitis; HIV-related eosinophilic tis, sterile eosinophilic pustulosis;eosinophilic pustular dermatosis; infantile/childhood eosinophilic pustulosis of thescalp

folliculi-Definition

Recurrent follicular and non-follicularpapules associated with tissue and periph-eral eosinophilia

in children; increased incidence in infected patients; peripheral eosinophiliaoften present

HIV-Differential diagnosis

Other forms of folliculitis, including rial and fungal varieties; pustular psoriasis;acne; rosacea; perioral dermatitis; scabies;candidiasis; folliculitis decalvans; insectbite reaction; Langerhans cell histiocytosis;follicular mucinosis; superficial pemphigus

bacte-Therapy

Dapsone; super potent topical oids; prednisone; isotretinoin; itracona-zole; UVB phototherapy; photochemother-apy

corticoster-References

Lazarov A, Wolach B, Cordoba M, Abraham D, Vardy D (1996) Eosinophilic pustular folliculitis (Ofuji disease) in a child Cutis 58(2):135–138

EphelidesSynonym(s)

Freckles

Trang 17

Epidemic typhus 209

E

Definition

Tan macules which darken after sun

expo-sure and fade in the winter months

Pathogenesis

Autosomal dominant trait; possibly somatic

mutations in epidermal melanocytes that

promote increased melanogenesis

Multiple, small, uniformly tan macules on

sun-exposed skin; sometimes coalescing

into patches; most common in individuals

with fair skin and/or blond or red hair

Lentigo; seborrheic keratosis; nevus; café au

lait spot; tinea versicolor

Therapy

Sun avoidance

References

Ortonne JP (1990) The effects of ultraviolet

expo-sure on skin melanin pigmentation Journal of

International Medical Research 18 Suppl 3:8C–

Pathogenesis

Caused by Rickettsia prowazekii; louseinfected after feeding on rickettsemic per-son with typhus or during a recrudescentcase; bites human to engage in blood mealand causes pruritic reaction on host skin;scratching by host causes crushing of lice

and Rickettsia-laden excrement inoculated

into wound

Painless papule at site of chigger bite; sequently undergoes central necrosis withformation of eschar; fever; headache;regional or generalized lymphadenopathy;rigors; myalgias; malaise; CNS symptoms;recrudescent form (Brill-Zinser disease):months to decades after treatment, organ-isms reemerge and cause recurrence oftyphus

sub-Differential diagnosis

Tularemia; leptospirosis; typhoid fever;other rickettsial infections; viral exanthem;dengue fever; anthrax; ehrlichiosis; infec-tious mononucleosis; Kawasaki disease;malaria; meningococcemia; relapsing fever;toxic shock syndrome; rubella; rubeola

Therapy

Doxycycline; chloramphenicol – 0.5–

1 gm IV every 6 hours until 48–72 hoursafter patient becomes afebrile; pediatricdose – 80–100 mg per kg per day IV dividedinto 4 doses until 48–72 hours after patientbecomes afebrile

Trang 18

Congenital hamartoma of embryonal

ecto-dermal origin, classified on the basis of its

main component, which may be

keratinoc-ytic, sebaceous, sweat gland, or follicular

Pathogenesis

Probable somatic mutation, which may

reflect genetic mosaicism; arises from

pluripotential germinative cells of the basal

layer of the embryonic epidermis; possibledermal effect on growth

Nevus verrucosus (verucous epidermalnevus): usually present at birth or earlychildhood; solitary or multiple, linear or S-shaped, verrucous or velvety plaques, nevercrossing the midline; flexural lesions some-times macerated and foul-smelling; lesionswith sebaceous or apocrine elements mayenlarge at puberty

Inflammatory epidermal nevus (ILVN):usually present in the first 5 years of life;pruritic, linear, erythematous, scalyplaques, most commonly on the leg; nevuscomedonicus (comedo nevus): confluentclusters of dilated follicular orifices pluggedwith keratin, giving the appearance ofaggregated open comedones; oftenarranged in a linear, arcuate, or zosteri-form pattern; occasionally paralleling thelines of Voigt or the lines of BlaschkoNevus unius lateris (linear epidermalnevus): solitary linear verrucous plaque,present at birth or in early infancy

Nevus sebaceous (sebaceous nevus): ally present at birth; well-circumscribed,pink-to-yellow, smooth or velvety plaques,almost always on the head and neck area;enlarges and thickens at puberty; small risk

usu-of malignant degeneration to basal cell cinoma

car-Epidermal nevus syndrome: one or moreepidermal nevi and involvement of thenervous, ophthalmologic, and/or skeletalsystems; mental retardation, seizures,movement disorders; intracranial and/orintraspinal lipomas

Proteus syndrome; CHILD syndrome; wart;Darier disease; lichen striatus; incontinen-tia pigmenti; psoriasis; syndrome of Favre-Racouchot; acne vulgaris; mastocytoma;juvenile xanthogranuloma; xanthoma

Therapy

Nevus verrucosus: surgical excision;tretinoin; acetretin; inflammatory epider-mal nevus: super potent topical corticoster-

Epidermal nevus Flesh-colored verrucous

nodule on the scalp

Trang 19

Epidermoid cyst 211

E

oids; cryotherapy; surgical excision; nevus

comedonicus: tretinoin; surgical excision;

nevus sebaceous: surgical excision;

epi-dermal nevus syndrome: as above for

indi-vidual variants

References

Losee JE, Serletti JM, Pennino RP (1999)

Epider-mal nevus syndrome: a review and case report

Annals of Plastic Surgery 43(2):211-214

Inherited disorder characterized by

wide-spread and persistent human papilloma

virus (HPV) infection and malignant

degeneration of the virally induced tumors

Pathogenesis

Autosomal recessive trait; impaired cellular

immunity to specific wart virus subtypes;

co-factors: ultraviolet light and X-rays

Polymorphic, verrucous or flat-topped

papules resembling flat warts; macules and

reddish-brown plaques with slightly scaly

surfaces and irregular borders; localized

mostly on sun-exposed regions, palms,

soles, in the axillae, and on external

genita-lia; mucous membranes rarely affected;

malignant tumors typically appears during

the fourth and fifth decades of life

Verruca plana; squamous cell carcinoma;

tinea versicolor; trichoepithelioma; basal

cell carcinoma; papular mucinosis; solar

White or pale yellow, deep dermal or cutaneous, medium-firm papule or nodule,often with a central pore; cheesy, foul-smelling material sometimes exuded withlateral pressure

sub-Differential diagnosis

Lipoma; trichilemmoma; steatocystomamultiplex; granuloma annulare; sarcoido-sis; lymphocytic infiltrates; insect bite reac-tion; acquired perforating disease; metasta-sis

Trang 20

212 Epidermolysis bullosa

Therapy

Simple excision by sharp dissection;

ellip-tical excision; marsupialization of large

lesions; inflamed lesion: incision and

drain-age of purulent material; triamcinolone (3–

5 mg per ml) injected intralesionally

References

Pariser RJ (1998) Benign neoplasms of the skin

Medical Clinics of North America 82(6):1285–

Group of inherited disorders characterized

by blister formation in response to

mechan-ical trauma

Pathogenesis

Epidermolysis bullosa simplex: associated

with mutations of the genes coding for

keratins 5 and 14; level of skin separation at

the mid basal cell associated with variable

intermediate filament clumping

Junctional epidermolysis bullosa:

muta-tions in genes coding for laminin 5

subu-nits (α3 chain, laminin β3 chain, laminin γ2

chain), collagen XVII (BP180), α6 integrin,

Dystrophic epidermolysis bullosa: tions of the gene coding for type VII colla-

muta-gen (COL7A1); anchoring fibrils affected;

degree of involvement ranging from subtlechanges to complete absence

Epidermolysis bullosa simplex:

• Weber-Cockayne variant: most commonform; blisters usually precipitated by trau-matic event; most frequently occurring onthe palms and soles, often with hyperhidro-sis

• Severe variant: generalized onset of ters occurring at or shortly after birth;hands, feet, and extremities most commonsites of involvement

blis-• Koebner variant: sometimes has plantar hyperkeratosis and erosions

palmo-• Dowling-Meara variant: involves oral mu–cosa with grouped herpetiform blisters.Junctional epidermolysis bullosa:

• Letalis (Herlitz) variant: generalized tering at birth; orificial erosions around themouth, eyes, and nares; often accompanied

blis-by significant hypertrophic granulation sue; involvement of the corneal, conjuncti-val, tracheobronchial, oral, pharyngeal,esophageal, rectal, and genitourinarymucosal surfaces; internal complications:hoarse cry, cough, and other respiratorydifficulties; poor prognosis

tis-• Nonlethal junctional variant (mitis form):usually survives infancy; generalized blis-tering; improves with age; scalp, nail, andtooth abnormalities; periorificial erosionsand hypertrophic granulation tissue;mucous membranes erosions, resulting instrictures

Dystrophic epidermolysis bullosa:

• Dominantly inherited variant; onset ofdisease usually at birth or during infancy;generalized blistering is common presenta-tion; evolution to localized blistering withage

Epidermolysis bullosa Bullae, erosions, and

scarring of the hands

Trang 21

Epidermolysis bullosa acquisita 213

E

• Cockayne-Touraine variant: acral

distri-bution and minimal oral or tooth

involve-ment

• Pasini variant: more extensive blistering,

scarlike papules on the trunk

(albopapu-loid lesions); involvement of the oral

mucosa and teeth; dystrophic or absent

nails common

• Mitis variant: involves acral areas and

nails with little mucosal involvement;

clini-cal manifestations similar to the

domi-nantly inherited forms

• Severe recessive variant

(Hallopeau-Sie-mens): generalized blistering at birth;

sub-sequent extensive dystrophic scarring, most

prominent on the acral surfaces,

some-times resulting in pseudosyndactyly

(mit-ten-hand deformity) of the hands and feet;

flexion contractures of the extremities

increasingly common with age; dystrophy

of nails and teeth; involvement of internal

mucosa sometimes resulting in esophageal

strictures and webs, urethral and anal

sten-osis, phimsten-osis, and corneal scarring;

intes-tinal malabsorption leading to a mixed

ane-mia resulting from a lack of iron

absorp-tion and failure to thrive; significant risk of

developing aggressive squamous cell

carci-nomas in areas of chronic erosions

Linear IgA bullous disease; bullous

pemphi-goid; epidermolysis bullosa acquisita;

fric-tion blisters; pemphigus vulgaris; burn

Therapy

Avoidance of frictional trauma; careful

attention to skin and dental hygiene;

severe disease: soft diet to prevent

esopha-geal trauma and blistering; skin equivalent

dressings to promote epithelialization

References

Fine JD, Eady RA, Bauer EA, Briggaman RA,

Bruckner-Tuderman L, et al (2000) Revised

classification system for inherited

epidermoly-sis bullosa: report of the Second International

Consensus Meeting on diagnosis and

classifi-cation of epidermolysis bullosa Journal of the

American Academy of Dermatology

42(6):1051–1066

Epidermolysis bullosa acquisita

Pathogenesis

IgG autoantibodies specific for anchoringfibrils (type VII collagen) of the skin base-ment membrane causes an inflammatoryprocess which is a contributing factor toblister formation; skin trauma a contribut-ing factor; genetic factors possibly impor-tant, since HLA-DR2 is overrepresented inthose with this condition

Non-inflammatory bullae at sites of minorskin trauma, which heal with scars and/ormilia; widespread inflammatory bullae notrelated to trauma; mucous membrane blis-ters and erosions, leading to scarring

Epidermolysis bullosa; bullous oid; cicatricial pemphigoid; linear IgA bul-lous dermatosis; bullous lupus erythemato-sus; porphyria cutanea tarda; bullous dis-ease of diabetes mellitus; erythemamultiforme

pemphig-Therapy

Prednisone 1 mg per kg PO daily; costeroid sparing agents – azathioprine;methotrexate; mycophenolate mofteil;cyclophosphamide; dapsone

corti-References

Kirtschig G, Murrell D, Wojnarowska F, et al

(2002) Interventions for mucous membrane pemphigoid/cicatricial pemphigoid and epidermolysis bullosa acquisita: A systematic lit-

Trang 22

214 Epidermolysis bullosa dystrophica

erature review Archives of Dermatology

Bullous congenital ichthyosiform

erythro-derma; bullous ichthyotic erythroerythro-derma;

ichthyosis bullosa of Siemens; ichthyosishystrix of Curth-Macklin

Definition

Congenital ichthyosis with characteristichistologic finding of epidermolytic hyperk-eratosis

Non-bullous ichthyosiform erythroderma;lamellar ichthyosis; X-linked ichthyosis;epidermolysis bullosa; incontinentia pig-menti; bullous impetigo; staphylococcalscalded skin syndrome

Epi-Epiloia

Tuberous sclerosis

Trang 23

unilat-Differential diagnosis

Paget’s disease of the breast; contact titis; basal cell carcinoma; apocrine glandtumors; hidradenitis suppurativa

Super-of the American Academy Super-of Dermatology 33(5

Pt 2):871–875

ErysipelasSynonym(s)

None

Trang 24

216 Erysipeloid

Definition

Skin infection involving the dermis and

local lymphatics, usually caused by group A

Abrupt onset of illness with fever and chills,

muscle and joint pain, nausea, headache;

skin change begins as small erythematous

patch and progresses to red, indurated,

shiny plaque; raised, sharply demarcated,

advancing margins, with skin warmth,

edema, and tenderness; lymphatic

involve-ment with overlying skin streaking and

regional lymphadenopathy

Contact dermatitis; seborrheic dermatitis;

lupus erythematosus; angioedema; herpes

zoster; erysipeloid; necrotizing fasciitis

Therapy

Penicillin G procaine; Penicillin VK;

dicola-cillin if staphyloccocal infection present;

cephalexin if patient is allergic to penicillin

References

Chartier C, Grosshans E (1996) Erysipelas: an

up-date International Journal of Dermatology

Pathogenesis

Causative organism, E rhusiopathiae,enters the skin through scratches or pricks;organism produces enzymes that help itdissect through the tissues; inflammationproduced when immune system activatedagainst foreign antigen

Food handlers (home makers, farmers, ermen, and butchers) at increased risk ofacquiring the infection

fish-Localized form: well demarcated, red-to-purple, warm, tender plaques with asmooth, shiny surface, most commonly onthe hands

bright-Diffuse cutaneous form: multiple, welldemarcated, violaceous plaques with anadvancing border and central clearingSystemic form: localized areas of swellingsurrounding a necrotic center; sometimespresenting as follicular, erythematouspapules; endocarditis as complication ofsepticemia

rhusi-Erysipelas Erythematous, edematous plaque on

the central face

Trang 25

Erythema annulare centrifugum 217

Erythema ab igne elastosis; ephelis ab igne;

erythema à calore; toasted skin syndrome

Definition

Changes in the skin caused by chronic and

repeated exposure to infrared radiation

Pathogenesis

Unclear mechanism; repeated external heat

exposure in the range of 43–47°C resulting

in histopathologic changes similar to those

seen in solar-damaged skin

Reticulated violaceous and

hyperpig-mented plaques, most common on the legs

of women; poikiloderma occurs with severe

long-standing disease

Livedo reticularis; poikiloderma of Civatte;

poikiloderma atrophicans vasculare;

mor-phea; livedo vasculitis

Therapy

Nd:YAG, ruby, or alexandrite laser

References

Page EH, Shear NH (1988)

Temperature-depend-ent skin disorders Journal of the American

Academy of Dermatology 18(5 Pt 1):1003–1019

Erythema ab igne elastosis

Erythema ab igne

Erythema annulare centrifugum

Synonym(s)

Erythema gyratum perstans; erythema dativum perstans; erythema marginatumperstans; erythema perstans; erythema fig-uratum perstans; erythema microgyratumperstans; erythema simplex gyratum;erythema perstans

exu-Definition

Figurate erythema with a characteristicadvancing, scaly margin and central clear-ing

Pathogenesis

Probably represents hypersensitivity tion to a variety of agents, including drugs,arthropod bites, infections (bacterial,mycobacterial, viral, fungal, filarial), ingest-ants (blue cheese penicillium), and malig-nancy

reac-Clinical manifestation

Begins as asymptomatic, erythematouspapules which spread peripherally whileclearing centrally; often a trailing scale onthe inner aspect of the advancing edge;appears on any skin surface other than thepalms and soles; may be associated with anunderlying disease (e.g., infection, malig-nancy, or other systemic illness)

Erythema marginatum rheumaticum; thema migrans; erythema gyratum repens;urticaria; granuloma annulare; sarcoidosis;tinea corporis; seborrheic dermatitis; lupuserythematosus; benign lymphocytic infil-trate; rheumatoid arthritis; psoriatic arthri-

Trang 26

ery-218 Erythema areata migrans

tis; lupus erythematosus; Reiter syndrome;

Tyring SK (1993) Reactive erythemas: erythema

annulare centrifugum and erythema gyratum

repens Clinics in Dermatology 11(1):135–139

Erythema areata migrans

Benign migratory glossitis

Manifestation of dry skin with large dry

scales and fine fissures giving a cracked-pot

appearance

References

Beacham BE (1993) Common dermatoses in the elderly American Family Physician 47(6):1445– 1450

Erythema dyschromicum perstans

Ashy dermatosis

Erythema dyschronicum perstans

Ashy dermatosis

Erythema dyspepsicum

Erythema toxicum

Erythema elevatum diutinum

Pathogenesis

Possibly involves immune complex tion with subsequent inflammatory cas-cade; associated with IgA monoclonal gam-mopathy, recurrent bacterial infections,(especially streptococcal), viral infections(including Hepatitis B or HIV), and rheu-matologic disease

Trang 27

deposi-Erythema induratum 219

E

Red, violaceous, smooth, brown or yellow

papules, plaques, or nodules over extensor

surfaces, especially over the joints;

occa-sional crusting or bleeding

Acute bebrile neutrophilic dermatosis;

granuloma annulare; insect bite reaction;

sarcoidosis; rheumatoid nodules; gouty

tophi; multicentric reticulohistiocytosis;

xanthomas; erythema multiforme

Therapy

Dapsone

References

Gibson LE, el-Azhary RA (2000) Erythema

eleva-tum diutinum Clinics in Dermatology

Erythema gyratum perstans

Erythema gyratum repens

Erythema annulare centrifugum; loma annulare; tinea corporis; sarcoidosis;lupus erythematosus; glucagonoma syn-drome; urticaria

Ery-Erythema induratum

Nodular vasculitis

Trang 28

220 Erythema infectiosum

Erythema infectiosum

Synonym(s)

Fifth disease, slapped-cheek disease,

academy rash, Sticker's disease, Sticker

dis-ease

Definition

Childhood exanthem caused by human

Par-vovirus B19, in which a 3-phased cutaneous

eruption follows a mild prodrome

Pathogenesis

Parvovirus B19 viremia; production of

spe-cific immunoglobulin M (IgM) antibodies

and subsequent formation of immune

com-plexes; clinical findings probably result

from the deposition of the immune

com-plexes in the skin and joints

4–14 day incubation period; virus spreads

primarily via aerosolized respiratory

drop-lets

Mild prodromal phase, including

head-ache, coryza, low-grade fever, pharyngitis,

and malaise

First stage: erythema of the cheeks, with

nasal, perioral, and periorbital sparing

(slapped-cheek appearance) and fades over

2–4 days

Second stage: within 1–4 days of the facial

rash, erythematous

macular-to-morbilli-form eruption occurs primarily on the

extremities

Third stage: after several days, most of the

second stage eruption fades into a lacy

pat-tern, particularly on the proximal

extremi-ties; lasts from 3 days to 3 weeks; after

start-ing to fade, exanthem sometimes recurs

over several weeks following exercise, sun

exposure, friction, bathing in hot water, or

stress; adults sometimes develop

polyar-thropathy

Other viral exanthems; medication

reac-tion; Lyme disease; lupus erythematosus;

acute rheumatic fever; allergic tivity reaction

Erythema marginatum

Definition

Superficial, often asymptomatic, form ofgyrate erythema, characterized by a tran-sient eruption of macular to slightly palpa-ble, non-scaling plaques on the trunk andextensor surfaces of the extremities; associ-ated with rheumatic fever

References

Rullan E, Sigal LH (2001) Rheumatic fever rent Rheumatology Reports 3(5):445–452

Cur-Erythema marginatum perstans

Erythema microgyratum perstans

Erythema migrans

Lyme disease

Trang 29

Acute inflammatory disorder related to

numerous factors, characterized by

distinc-tive clinical eruption, with hallmark of iris

or target lesion

Pathogenesis

Unclear; herpes-associated disease appears

to represent the result of a cell-mediated

immune reaction associated with herpes

simplex virus (HSV) antigen

Most commonly associated with herpes

simplex virus infection; also associated

with other infections, drug ingestion,

rheu-matic diseases, vasculitides, non-Hodgkin’s

lymphoma, leukemia, multiple myeloma,

myeloid metaplasia, polycythemia

Erythema multiforme minor variant:

occa-sional mild flu-like prodrome; initial lesion

dull red macule or urticarial plaque in the

center, with small papule, vesicle, or bulla

sometimes developing; raised, pale ring

with edematous; periphery gradually

becoming violaceous and forming tric target lesion; lesions appear predomi-nantly on the extensor surfaces of acralextremities and spread centripetally; milderosions of one mucosal surface; palms,neck, and face frequently involved

concen-Erythema multiforme major variant: drome of moderate fever, general discom-fort, cough, sore throat, vomiting, chestpain, and diarrhea, usually for 1–14 dayspreceding the eruption; skin lesions same

pro-as with erythema multiforme minor; severeerosions of at least 2 mucosal surfaces; gen-eralized lymphadenopathy

Stevens-Johnson syndrome; toxic mal necrolysis; Henoch-Schönlein pur-pura; urticaria; viral exanthem; Kawasakidisease; figurate erythema; fixed drug erup-tion; lupus erythematosus; primary her-petic gingivostomatitis; Behçet’s disease;aphthous stomatitis

epider-Therapy

Antihistamines, first generation; nisone; herpes simplex virus prophylaxiswith valacyclovir, if more than 4–5 episodesper year

palm

Trang 30

222 Erythema neonatorum allergicum

Dermatitis contusiformis; erythema

con-tusiformis; focal septal panniculitis; nodose

fever

Definition

Inflammatory vascular reaction pattern to

multiple causes; characterized by tender

subcutaneous nodules, usually on the

ante-rior legs

Pathogenesis

Probably is delayed hypersensitivity

reac-tion to a variety of antigens; most common

associations with streptococcal infections

in children and sarcoidosis in adults; other

associations include tuberculosis,

myco-plasma pneumonia, leprosy,

coccidioid-omycosis, North American blastcoccidioid-omycosis,

histoplasmosis, inflammatory bowel

dis-ease, pregnancy, and Behçet’s disease;

asso-ciated medications include oral

contracep-tives and sulfonamides

Prodrome of flulike symptoms of fever and

generalized aching; lesions begin as

poorly-defined, red, tender nodules; become firm

and painful during the second week;

some-times becoming fluctuant; not suppurating

or ulcerating; individual lesions last

approximately 2 weeks; associated leg

edema and pain

Nodular vasculitis; insect bite reaction;

ery-sipelas; cellulitis; superficial

thrombophle-bitis; Weber-Christian disease; pancreaticpanniculitis; lupus profundus; traumaticpanniculitis; polyarteritis nodosa; rheuma-toid nodules

nodo-Erythema nodosum migrans

Subacute nodular migratory niculitis

Trang 31

Erythrasma 223

E

Erythema simplex gyratum

Erythema solare

Sunburn

Synonym(s)

Erythema toxicum neonatorum; erythema

neonatorum; toxic erythema; erythema

neonatorum allergicum; erythema

papulo-sum; urticaria neonatorum; erythema

dys-pepsicum

Definition

Benign, self-limited eruption occurring

pri-marily in healthy newborns in the early

neonatal period

Pathogenesis

Unknown

Usual onset within the first 4 days of life in

full-term infants, with peak onset

occur-ring within the first 48 hours following

birth; presents with a blotchy, evanescent,

macular erythema, often on the face or

trunk; sites of predilection include the

fore-head, face, trunk, and proximal

extremi-ties; mucous membranes usually spared

Candidiasis; miliaria; pyoderma; insect bite

reaction; varicella; herpes simplex virus

infection; urticaria; folliculitis; transient

neonatal pustular melanosis

Erythema toxicum neonatorum

Erythemato-papulous acrodermatitis

Gianotti-Crosti syndrome

papulous eruptive syndrome

Trang 32

inter-224 Erythroderma

Pathogenesis

Under favorable conditions, such as heat

and humidity, Corynebacteria organisms

proliferate and cause clinical signs

Well demarcated, brown-red, minimally

scaly plaques, commonly occurring over

inner thighs, crural region, scrotum, and

toe webs; other intertriginous sites such as

axillae, submammary area, periumbilical

region, and intergluteal fold less commonly

involved; toe web lesions appear

macer-ated; predisposing factors: excessive

sweat-ing and hyperhidrosis, disrupted

cutane-ous barrier, obesity, diabetes mellitus, and

immunocompromised state

Tinea pedis; tinea corporis; tinea cruris;

contact dermatitis; dyshidrotic eczema;

intertrigo; contact dermatitis

Therapy

Erythromycin base; clarithromycin; drying

powder applied twice daily

Erythropoietic protoporphyria

Erythrokeratoderma

Erythrokeratodermia variabilis

Erythrokeratodermia figurata variabilis

Erythrokeratodermia papillaris et reticularis

Confluent and reticulated matosis

papillo-Erythrokeratodermia progressiva symmetrica

Progressive symmetric keratoderma

Synonym(s)

Erythrokeratoderma; keratosis rubra rata; erythrokeratodermia figurata variabi-lis

Tiêu đề	Dermatology therapy essentials - part 4 ppsx
Trường học	University of Medical Sciences
Chuyên ngành	Dermatology
Thể loại	lecture notes
Năm xuất bản	2023
Thành phố	Unknown

Định dạng
Số trang	64
Dung lượng	3,37 MB