Dermatology therapy essentials - part 5 ppsx

Goltz-Gorlin syndrome 259G erythema, and diabetes mellitus; hypoami-noacidemia; cheilosis; normochromic, nor-mocytic anemia; venous thrombosis; weight loss; neuropsychiatric signs and sy

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Glanders and melioidosis 257

Kanik AB, Lee J, Wax F, Bhawan J (1997) Penile

verrucous carcinoma in a 37-year-old

circum-cised man Journal of the American Academy of

Giant malignant condyloma

Giant condyloma of Buschke and

Related diseases produced by bacteria of the

Burkholderia species, which are tive rods

Clinical manifestation

Similar clinical syndrome in both diseases

Localized form: bacteria enter the skinthrough a laceration or abrasion; localinfection with ulceration and regional lym-phadenopathy; incubation period 1–5 days;bacteria that enter the host throughmucous membranes sometimes causeincreased mucus production in the affectedareas

Pulmonary form: occurs when bacteria areaerosolized and enter respiratory tract viainhalation or hematogenous spread; withinhalational melioidosis, cutaneousabscesses may develop; septicemia: whenbacteria disseminated in the bloodstream

in glanders, usually fatal within 7–10 daysChronic form: multiple abscesses affectingthe liver, spleen, skin, or muscles

Differential diagnosis

Anthrax; plague; tuberculosis; atypicalmycobacterial infection; brucellosis; NorthAmerican blastomycosis; coccidioidomyco-sis; nocardia infection

PART7.MIF Page 257 Friday, October 31, 2003 10:22 AM

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258 Glomangioma

Therapy

Amoxicillin; tetracycline

References

Rosenbloom M, Leikin JB, Vogel SN, Chaudry ZA

(2002) Biological and chemical agents: a brief

synopsis American Journal of Therapeutics

Benign neoplasm of modified smooth

mus-cle cells (glomus cells)

Pathogenesis

Unknown cause for solitary lesion;

multi-ple glomus tumors, especially those of the

disseminated form, inherited as

autosomal-dominant trait with incomplete

pene-trance; tumors arise from the arterial

por-tion of the glomus body

Solitary glomus tumor: paroxysmal pain,

which can be severe and exacerbated by

pressure or temperature changes, especially

cold; blanchable blue or purple papule,

located most commonly in acral areas,

especially subungual areas of fingers and

toes

Multiple glomus tumors: pain relatively

uncommon

• Regional variant: blue-to-purple,

com-pressible papules or nodules that are

grouped and limited to a specific area, most

commonly an extremity

• Disseminated variant: multiple lesions

distributed over the body with no specific

grouping; congenital plaquelike glomustumors: grouped papules coalescing intoindurated plaques or clusters of discretenodules

Angioleiomyoma; angiolipoma; enous malformation; blue nevus; hemangi-oma; melanoma; spiradenoma; tufted angi-oma; Kaposi’s sarcoma; blue rubber blebnevus; neurilemmoma

arteriov-Therapy

Solitary glomus tumor: surgical excision;multiple glomus tumors: surgical removalfor cosmetic reasons only

References

Alam M, Scher RK (1999) Current topics in nail surgery Journal of Cutaneous Medicine & Sur- gery 3(6):324–335

Parsons ME, Russo G, Fucich L, Millikan LE, Kim

R (1997) Multiple glomus tumors International Journal of Dermatology 36(12):894-900

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Goltz-Gorlin syndrome 259

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erythema, and diabetes mellitus;

hypoami-noacidemia; cheilosis; normochromic,

nor-mocytic anemia; venous thrombosis;

weight loss; neuropsychiatric signs and

symptoms; pseudoglucagonoma syndrome:

necrolytic migratory erythema without a

glucogon-secreting tumor, but with another

underlying cause such as cirrhosis, celiac

sprue, or pancreatitis

Pathogenesis

Unclear relation between glucagonoma and

skin findings; levels of glucagon not well

correlated with the episodic course of the

skin manifestations; possible role of

rela-tive zinc deficiency; theories of causation:

related to glucagon-induced

hypoalbumine-mia; zinc-dependent delta-6 desaturation of

linoleic acid; poor hepatic breakdown of

glucagon contributing to an excessive

pros-taglandin-mediated inflammatory response

Presents with nonspecific complaints, such

as weight loss, diabetes mellitus, diarrhea,

and stomatitis; necrolytic migratory

ery-thema: found anywhere on the body, but

most common in the perineum, buttocks,

groin, lower abdomen, and lower

extremi-ties; eruption starts as a pruritic or painful,

erythematous patch that blisters centrally,

erodes, crusts over, and heals with

hyper-pigmentation; annular lesions with

conflu-ence into plaques; confluconflu-ence in severely

affected areas; associated mucocutaneous

findings, including atrophic glossitis,

cheilosis, dystrophic nails, and buccal

mucosal inflammation

Acrodermatitis enteropathica; candidiasis;

paraneoplastic pemphigus; Hailey-Hailey

disease; Darier disease; pellagra;

kwash-iorkor

Therapy

Surgical resection of the tumor, if

local-ized; in the absence of tumor, treat

under-lying cause

References

Chastain MA (2001) The glucagonoma syndrome:

a review of its features and discussion of new perspectives American Journal of the Medical Sciences 321(5):306–320

Glucosyl cerebroside lipidosis

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Gonococcal dermatitis-arthritis syndrome;

disseminated gonococcal infection

Definition

Sexually transmitted disease caused by the

bacterium Neisseria gonorrhoeae, which

spreads from the initial site of infection

through the bloodstream to other parts ofthe body

Pathogenesis

Neisseria gonorrhoeae transmitted throughvaginal, oral, and anal intercourse; infec-tion also transmitted by a woman to hernewborn during childbirth; disseminationoften occurs during menses

More common in women, often withasymptomatic infection; disseminated dis-ease generally follows the primary genitalinfection by several days to 2 weeks; fever;myalgias; tenosynovitis; monoarticular sep-tic arthritis, affecting large, weight-bearingjoints; acral palpable purpuric papules andpustules, usually relatively few in number

Meningococcemia or other infectiouscauses of septic vasculitis; lupus erythema-tosus; cryoglobulinemia; Reiter syndrome;infective endocarditis

500 mg PO twice daily or cefixime 400 mg

PO twice daily or azithromycin 500 mg POper day; concurrent therapy for presumedchlamydia with doxycycline 100 mg POtwice daily for 7 days

Basal cell nevus syndrome

Gonococcemia Violaceous papule on the toe

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Graft versus host disease 261

Pathogenesis

Three criteria for development – (1) graftcontaining immunologically competentcells, (2) host appearing foreign to the graft,(3) host incapable of reacting sufficientlyagainst the graft; recognition of epithelialtarget tissues as foreign by the immuno-competent cells, with subsequent induction

of an inflammatory response and eventualapoptotic death of the target tissue; reac-tion against the host's keratinocytes, result-ing in the clinical syndrome

Incidence higher in recipients of allogeneichematopoietic cells than in patients receiv-ing syngeneic or autologous hematopoietic

Graft versus host disease Sclerotic,

hyperpigmented and hypopigmented plaques

on the upper trunk

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262 Granular bacteriosis

cells; greatest incidence in patients in whom

bone marrow is used as the source of

hematopoietic cells

Acute graft versus host disease: observed

10–30 days posttransplant; eruptions

usu-ally begin as faint, tender, erythematous

macules, often centered around hair

folli-cles; as disease progresses, macules

some-times coalesce to form confluent plaques or

papules; subepidermal bullae may occur

Chronic graft versus host disease: evolves

from acute form in 70–90% of patients; risk

increases with the severity of acute

reac-tion; violaceous lichenified papules, often

on the ventral skin surfaces, very similar to

those of lichen planus; lacy white plaques

on the buccal mucosa; scattered

scleroder-matous plaques; widespread disease

result-ing in ulcerations, joint contractures, and

esophageal dysmotility

Acute graft versus host disease: erythema

multiforme; drug eruption;

Stevens-John-son syndrome/toxic epidermal necrolysis;

eruption of lymphocyte recovery

Chronic graft versus host disease:

sclero-derma; lichen planus; lichenoid drug

erup-tion; lupus erythematosus

Therapy

Acute graft versus host disease:

pred-nisone; extracorporeal photochemotherapy

Chronic graft versus host disease:

photo-chemotherapy; methotrexate;

extracorpor-eal photochemotherapy;

hydroxychloro-quine; etretinate

References

Jacobsohn DA, Vogelsang GB (2002) Novel

phar-macotherapeutic approaches to prevention and

treatment of GVHD Drugs 62(6):879–889

Granular bacteriosis

Botryomycosis

Granular cell myoblastoma

Granular cell tumor

Granular cell neurofibroma

Granular cell neuroma

Granular cell schwannoma

Synonym(s)

Granular cell myoblastoma; granular cellschwannoma; granular cell neuroma;granular cell neurofibroma; Abrikossof’stumor

Definition

Acquired tumor of neural crest origin, acterized by cells with eosinophilic cyto-plasmic granules

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Fibroma; squamous cell carcinoma; wart;

dermatofibroma; neurofibroma;

epider-moid cyst

Therapy

Surgical excision

References

Becelli R, Perugini M, Gasparini G, Cassoni A,

Fa-biani F (2001) Abrikossoff 's tumor Journal of

Inflammatory skin disease characterized by

annular plaques consisting of small papules

Erythema annulare centrifugum; tinea poris; lichen planus; lupus erythematosus;insect bite reaction; sarcoidosis; Lyme dis-ease; necrobiosis lipoidica; rheumatoidnodules; acquired perforating disease;lichen myxedematosus; cutaneous T-celllymphoma; erythema multiforme

cor-Therapy

Localized disease: intralesional nolone; corticosteroids, topical, superpo-tent

triamci-Generalized disease: photochemotherapy

References

Smith MD, Downie JB, DiCostanzo D (1997) Granuloma annulare International Journal of Dermatology 36(5):326–333

Granuloma faciale

Synonym(s)

Facial granuloma; granuloma faciale nophilicum, granuloma faciale with eosi-nophilia

eosi-Granuloma annulare Annular red-brown

plaques on the dorsal aspect of the hand

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264 Granuloma faciale eosinophilicum

Definition

Benign chronic skin disease of unknown

origin, characterized by single or multiple

cutaneous nodules, usually occurring over

Solitary or multiple, sharply marginated,

red or violaceous papules or nodules;

sur-face sometimes has telangiectasias and/or

enlarged follicular orifices; usually occurs

on the face, but also on the upper

extremi-ties or trunk

Sarcoidosis, granuloma annulare; discoid

lupus erythematosus; mycosis fungoides;

fixed drug eruption; Jessner’s lymphocytic

infiltrate; lymphoma; leprosy; lupus

vul-garis; foreign body granuloma

Therapy

Triamcinolone 3–4 mg per ml intralesional;

dapsone

References

Inanir I, Alvur Y (2001) Granuloma faciale with

extrafacial lesions British Journal of

Synonym(s)

Kaposi’s sarcoma-like granuloma;granuloma intertriginosum infantum;infantile vegetating halogenosis; vegetatingpotassium bromide toxic dermatitis;vegetating bromidism

Definition

Disease characterized by oval, tous nodules on the gluteal surfaces andgroin areas of infants

granuloma-Pathogenesis

Unclear; unusual cutaneous response tolocal inflammation, maceration, and sec-ondary infection; contact occlusion proba-bly predisposing factor

Solitary or mulptiple, purple to brown, firm-to-hard, discrete dermal nod-ules with smooth or slightly lichenified sur-faces; aligned with the long axis parallel tothe skin folds; located on the gluteal sur-faces, in the groin area, upper thighs, lowerabdomen, or rarely the neck and face

red-Differential diagnosis

Langerhans cell histiocytosis; candidiasis;contact dermatitis; lymphoma; mastocyto-sis; scabies; syphilis; juvenile xanthogranu-loma; pyogenic granuloma; sarcoma; for-eign body granuloma

Therapy

Spontaneous resolution; no therapy cated

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indi-Granulomatosis disciformis chronica et progressiva 265

G

References

Bluestein J, Furner BB, Phillips D (1990)

Granulo-ma gluteale infantum: case report and review of

the literature Pediatric Dermatology 7(3):196–

Sexually transmitted disease characterized

by genital lesions presenting as indolent,

progressive ulcerations with a

granuloma-tous appearance

Pathogenesis

Infection caused by a gram-negative

pleo-morphic bacillus, Calymmatobacterium

granulomatis; mode of transmission

prima-rily through sexual contact; mildly

conta-gious

Occurs on glans penis and scrotum in men,

and labia minora, mons veneris, and

four-chette in women; rare cervical

involve-ment; soft, red papules or nodules arising at

the site of inoculation; lesions eventually

ulcerate and produce red, friable,

granulo-matous plaques and nodules; ulcers with

clean, friable bases and distinct, raised,

rolled margins; autoinoculation results in

lesions on adjacent skin; occasional

hyper-trophic or verrucous plaques, with

forma-tion of large, vegetating masses resembling

genital warts; swelling of the external

geni-talia in later-stage lesions

Syphilis; lymphogranuloma venereum;

chronic herpes simplex virus infection;

squamous cell carcinoma; lichen sclerosus

Granuloma gluteale infantum

Granuloma pyogenicum

Pyogenic granuloma

Granuloma telangiectaticum

Actinic granuloma

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Caused by two genes: MYA5 and RAB27A;gene MYA5 produces severe neurologicalproblems; gene RAB27A causes acceleratedphase sometimes lethal within a shortperiod of time

Silvery blond hair; occasional subtle mentary dilution of the skin and iris andhyperpigmentation in sun-exposed areas;accelerated phase of the disease with fever,jaundice, hepatosplenomegaly, lymphaden-opathy, pancytopenia and generalized lym-phohistiocytic infiltrates of various organsincluding the central nervous system; neu-rologic manifestations: hyperreflexia, sei-zures, signs of intracranial hypertension,regression of developmental milestones,

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pig-Griseofulvin 267

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hypertonia, nystagmus, and ataxia; variety

of immunological abnormalities, restricted

to the patients with RAB27A defect

Hematophagic lymphohistiocytosis;

famil-ial lymphohistiocytosis; Chediak-Higashi

syndrome; X-linked lymphoproliferative

syndrome

Therapy

Bone marrow transplantation;

chemother-apy for accelerated phase

References

Klein C, Philippe N, Le Deist F, Fraitag S, Prost C,

Durandy A, Fischer A, Griscelli C (1994) Partial

albinism with immunodeficiency (Griscelli

syndrome) Journal of Pediatrics 125(6):886–

Common side effects

Cutaneous: photosensitivity, vascular

Serious side effects

Bone marrow: granulocytopenia Gastrointestinal: hepatotoxicity

Drug interactions

Amiodarone; barbiturates; carbamazepine;clarithromycin; oral contraceptives;cyclosporine; erythromycin; itraconazole;ketoconazole; protease inhibitors; tac-rolimus; warfarin

Griseofulvin Dermatologic indications and dosage

Onychomycosis 500 mg PO twice daily for

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268 Groin dermatophytosis

Other interactions

Ethanol

Contraindications/precautions

Hypersensitivity to drug class or

compo-nent; acute intermittent porphyria;

preg-nancy; caution in patients with penicillin

allergy or impaired liver function

References

Bennett ML, Fleischer AB Loveless JW, Feldman

SR (2000) Oral griseofulvin remains the

treat-ment of choice for tinea capitis in children

Enlargement of the nodes above and below

the inguinal ligament in patients with

lym-phogranuloma venereum

References

Brown TJ, Yen-Moore A, Tyring SK (1999) An

overview of sexually transmitted diseases Part

I Journal of the American Academy of

in the liver but also occurring in the brain,testis, heart, skin, and bone

References

Quinn P, Weisberg L (1997) Cerebral syphilitic gumma New England Journal of Medicine 336(14):1027–1028

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McCormack CJ, Cowen P (1997) Haber's drome Australasian Journal of Dermatology 38(2):82–84

syn-Hailey-Hailey disease

Familial benign chronic pemphigus

Hair follicle nevus

Pathogenesis

Caused by Epstein-Barr virus; unclearwhether a development following superin-fection with EBV or activation of latentinfection due to reduced immune surveil-lance

Asymptomatic, white plaque along the eral tongue borders, with accentuation ofvertical folds; occasionally spreads to thePART8.MIF Page 271 Friday, October 31, 2003 10:31 AM

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lat-272 Hairy tongue

mouth floor, tonsillar pillars, ventral

tongue, and pharynx; occurs almost

exclu-sively in immunocompromised patients,

particularly those infected with HIV

Wart; syphilis; premalignant leukoplakia

(“smoker’s leukoplakia”); traumatic

leuko-plakia; squamous cell carcinoma;

candidia-sis; geographic tongue; lichen planus

Therapy

None

References

Itin PH, Lautenschlager S, Fluckiger R, Rufli T

(1993) Oral manifestations in HIV-infected

pa-tients: diagnosis and management Journal of

the American Academy of Dermatology 29(5 Pt

1):749–760

Hairy tongue

Synonym(s)

Black hairy tongue; lingua nigra; lingua

vil-losa; lingua villosa nigra

Definition

Condition of defective desquamation of the

filiform papillae of the tongue that results

in an irregular, discolored plaque, with

elongation of filiform papillae and a lack of

normal desquamation

Pathogenesis

Inadequate hygiene or microbial growth stimulates elongation of filiformpapillae; lack of mechanical stimulationand debridement

over-Clinical manifestation

Elongation of the filiform papillae on thedorsal surface of the tongue, which retainpigments from food, beverages, andtobacco, resulting in brown, black or red-dish discoloration

Candidiasis; lichen planus; oral hairy plakia

leuko-Therapy

Mechanical removal of elongated papillae

by brushing the tongue with a toothbrush

or using a tongue scraper; destruction byelectrodesiccation and curettage or CO2laser vaporization; tretinoin; acitretin

References

Sarti GM, Haddy RI, Schaffer D, Kihm J (1990) Black hairy tongue American Family Physician 41(6):1751–1755

the tongue

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Halo nevus 273

H

References

Mazuryk HA, Brodkin RH (1991) Cutaneous clues

to renal disease Cutis 47(4):241–248

oculomandibulodyscephaly with

hypotri-chosis; oculomandibulofacial syndrome

Definition

Genetic disorder characterized by

malfor-mations of the skull and facial region,

sparse hair, ocular abnormalities, dental

defects, degenerative skin changes, and

short stature

Pathogenesis

Unknown

Skin findings: sparse hair; atrophy,

particu-larly in the scalp and nasal regions

Craniofacial features: brachycephaly with

frontal and/or parietal bossing; small,

underdeveloped lower jaw; narrow, highly

arched palate; thin, pinched, tapering nose

Ocular findings: congenital cataracts;

microphthalmia; other ocular

abnormali-ties

Dental defects: presence of natal teeth;

hypodontia or partial adontia

malforma-tion; and/or improper alignment of teeth

Skeletal findings: short stature

Cohen MM Jr (1991) Hallermann-Streiff

syn-drome: a review American Journal of Medical

Genetics 41(4):488–499

Hallermann-Streiff-Francois syndrome

Hallermann-Streiff syndrome

Hallopeau, acrodermatitis continua

Acrodermatitis continua of peau

melano-Pathogenesis

Unclear; apparently an immunologic tion against melanocyte; cells predomi-nantly T lymphocytes; precipitating causeand exact role of lymphocytes unknown

hypopig-Differential diagnosis

Vitiligo; atypical mole; melanoma; tineaversicolor; lichen sclerosus; morphea; post-traumatic hypopigmentation

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274 Halobetasol propionate

Therapy

None indicated for childhood lesions;

surgi-cal excision for adult-onset lesions,

although considered controversial

References

Zeff RA, Freitag A, Grin CM, Grant-Kels JM (1997)

The immune response in halo nevi Journal of

the American Academy of Dermatology

Skin eruption resulting from exposure to

bromide-containing drugs or substances

such as potassium bromide (bromoderma),

iodide-containing drugs or substances such

as water-soluble contrast media

(iodo-derma), or fluoride-containing drugs or

substances such as fluoride teeth gels

ulcerat-Iododerma: vesicular, pustular, rhagic, suppurative, and/or ulcerativepapules and plaques occurring on the areas

hemor-of skin with the highest concentration hemor-ofsebaceous glands, such as the face

Fluoroderma: resembles iododerma, withnumerous and scattered papules and nod-ules

Tuberculosis; sarcoidosis; North Americanblastomycosis; rosacea; pyoderma gan-grenosum; acute febrile neutrophilic der-matosis; syphilitic gumma; pemphigus veg-etans

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Disorder caused by defective transport of

neutral amino acids in the small intestine

and kidney, resulting in a pellagra-like skin

eruption, cerebellar ataxia, and

aminoaci-duria

Pathogenesis

Failure of the transport of tryptophan andother neutral alpha-amino acids in thesmall intestine and renal tubules; abnor-mality in tryptophan transport, leading toniacin deficiency that is responsible for pel-lagra-like eruption and photosensitivity

Gingivitis, stomatitis, glossitis; tivity; multiple sun exposures leading todry, scaly, well-marginated plaques, resem-bling chronic eczema, affecting preferen-tially the forehead, cheeks, periorbitalregions, dorsal surface of the hands, andother light-exposed areas; vesiculobullouseruption with exudation sometimes occurs;hypopigmentation and/or hyperpigmenta-tion that is intensified with further sunlightexposure; intermittent cerebellar ataxiawith wide-based gait, spasticity, delayedmotor development, and tremulousness, allreversible with niacin therapy; diarrhea;attacks sometimes provoked by a febrile ill-ness, poor nutrition, sulfonamides, andpossibly emotional stress

photosensi-Differential diagnosis

Polymorphous light eruption; lupus thematosus; atopic dermatitis; seborrheicdermatitis; nutritional pellagra; Cockaynesyndrome; carcinoid syndrome; ataxia tel-angiectasia; xeroderma pigmentosum

ery-Therapy

Niacin 50–100 mg PO 3 times per day;avoidance of sun exposure; high proteindiet

References

Kahn G (1986) Photosensitivity and titis in childhood Dermatologic Clinics 4(1):107–116

photoderma-Hartnup disorder

Hartnup disease

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solia-Spindle cell hemangioendothelioma: firmblue papules or nodules, often multifocalwithin given anatomic sites, occurring overthe distal extremities

Kaposiform hemangioendothelioma: ally in retroperitoneum, but sometimesPART8.MIF Page 276 Friday, October 31, 2003 10:31 AM

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usu-Hemangiopericytoma 277

H

occurring in the skin; bluish papule or

nod-ule; associated with consumption

coagulop-athy and lymphangiomatosis

Retiform hemangioendothelioma:

slow-growing plaque with ill-defined borders,

usually on the distal extremities

Grezard P, Balme B, Ceruse P, Bailly C, Dujardin T,

Perrot H (1999) Ulcerated cutaneous

epithelio-id hemangioendothelioma European Journal

Dense collections of dilated vessels

occur-ring in the skin or internal organs

References

Dinehart SM, Kincannon J, Geronemus R (2001)

Hemangiomas: evaluation and treatment

Fibrous histiocytoma; malignant fibroushistiocytoma; synovial sarcoma; juxta-glomerular tumor; vascular leiomyoma;juvenile hemangioma; myxoid lipoma;myxoid liposarcoma; mesenchymal chond-rosarcoma

Therapy

Bland lesions with minimal mitotic ity: wide local excision; active and dys-plastic lesions: radical surgical excision,with or without adjunctive radiotherapy

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activ-278 Hematoma

References

Pandey M, Kothari KC, Patel DD (1997)

Haeman-giopericytoma: current status, diagnosis and

management European Journal of Surgical

McGillis ST, Ratner D, Clark R, Madani S, et al

(1998) Atlas of excision and repair

Dermato-logic Clinics 16(1):181–194

Hemochromatosis

Synonym(s)

Bronze diabetes, iron deposition disease,

hereditary hemochromatosis; genetic

hemochromatosis; primary

hemochroma-tosis

Definition

Abnormal accumulation of iron in

paren-chymal organs, leading to organ toxicity

Pathogenesis

Autosomal recessive trait; associated with

two mutations in the HFE gene; error of

iron metabolism characterized by excess

dietary iron absorption and iron

deposi-tion in tissues; presence of free iron in

bio-logical systems leads to rapid formation of

damaging reactive oxygen metabolites,

which can produce DNA cleavage, impaired

protein synthesis, and impairment of cell

integrity and cell proliferation, resulting in

cell injury and fibrosis

Generalized hyperpigmentation; sis; skin atrophy; koilonychia; partial alo-pecia; diabetes mellitus; cirrhosis; conges-tive heart failure; hepatomegaly; splenome-galy; arthritis; amenorrhea; loss of libido;impotence; symptoms of hypothyroidism

ichthyo-Differential diagnosis

Addison’s disease; polymorphous lighteruption; post-inflammatory hyperpigmen-tation; sun-induced tanning; drug-inducedhyperpigmentation; actinic reticuloid;poikiloderma of Civatte; argyria; iron over-load associated with chronic anemia; multi-ple blood transfusions; hyperplastic eryth-roid marrow from diseases such as heredi-tary sideroblastic anemias, severe alpha andbeta thalassemia; myelodysplastic syn-drome variants

Therapy

Phlebotomy; limiting of alcohol tion; avoidance of iron supplements andraw oysters

consump-References

Powell LW (2002) Hereditary hemochromatosis and iron overload diseases Journal of Gastro- enterology & Hepatology 17 Suppl:S191–195

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small-ves-Hereditary angioedema 279

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gastrointestinal tract, joints, and kidneys,

occurring primarily in children

Pathogenesis

Vascular deposition of IgA immune

com-plexes, which activate complement

compo-nents, which mediate tissue injury

Prodrome of fever, anorexia, and headache;

erythematous macules and papules on

but-tocks and extremities, which become

pur-puric; colic, vomiting, and diarrhea;

polyar-thralgia; proteinuria and hematuria

Urticaria; lupus erythematosus;

Churg-Strauss syndrome; essential mixed

cry-oglobulinemia; polyarteritis nodosa;

rheu-matoid arthritis; benign pigmented

pur-pura; child abuse; bacterial endocarditis;

meningococcemia; Rocky Mountain

spot-ted fever

Therapy

Prednisone; dapsone; azathioprine;

intrave-nous immunoglobulin (IVIG)

References

Saulsbury FT (2001) Henoch-Schonlein purpura

Current Opinion in Rheumatology 13(1):35–40

Heparin necrosis

Synonym(s)

None

Definition

Necrotic areas of skin, usually at the site of

heparin injection, characterizing a

local-ized hypersensitivity reaction

Pathogenesis

Possible immunologic basis

Begins as localized erythema, typically at

heparin injection sites, usually in women;

burning pain; progression to bulla tion and necrosis over a few days; morecommon in obese or diabetic patients

forma-Differential diagnosis

Pyoderma gangrenosum; calciphylaxis; der bite reaction; factitial disease; bacterialpyoderma; herpes simplex virus infection;fixed drug eruption

spi-Therapy

Discontinuance of heparin therapy;hydrocolloid dressings to ulcerated area;ulcer excision and skin grafting if ulcera-tion persists

References

Levine LE, Bernstein JE, Soltani K, Medenica MM, Yung CW (1983) Heparin-induced cutaneous necrosis unrelated to injection sites Archives

of Dermatology 119(5):400–403

Hepatic porphyria

Porphyria cutanea tarda

Hepatolenticular degeneration

Mutations in the C1-INH gene, transmitted

as an autosomal dominant trait; two

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vari-280 Hereditary baldness

ants: type I – low antigenic and functional

plasma levels of C1-INH protein; type II –

presence of normal or elevated antigenic

levels of a dysfunctional mutant protein

together with reduced levels of the

func-tional protein; C1-INH deficiency permits

autoactivation of the first component of

complement (C1) with consumption of C4

and C2

Recurrent, noninflammatory swelling of the

skin and mucous membranes; erythema or

mild urticarial eruption occasionally

pre-ceding edema; sometimes precipitated by

trauma, anxiety, or stress; associated with

lupus erythematosus and other

autoim-mune diseases

Chronic urticaria; pressure-induced

urti-caria; acquired angioedema; ACE

inhibitor-induced angioedema

Therapy

Acute episodes: replacement with C1-INH

concentrates; fresh-frozen plasma;

proph-ylaxis: danazol 400–600 mg PO per day

References

Nzeako UC, Frigas E, Tremaine WJ (2001)

Hered-itary angioedema: a broad review for

clini-cians Archives of Internal Medicine

Pathogenesis

Autosomal dominant disease, resultingfrom defects in coproporphyrinogen oxi-dase; related to deposition of formed por-phyrins in the skin which become photoac-tive after sunlight exposure

Skin changes: blisters forming in exposed areas; skin fragility; scarring;hypertrichosis in sun-exposed areasNeurologic changes: central nervous sys-tem signs, including seizures, mental statuschanges, cortical blindness, and coma;peripheral neuropathies predominantlymotor neuropathies; diffuse pain, espe-cially in the upper body; autonomic neu-ropathies, including hypertension and tach-ycardia; psychiatric abnormalities

sun-Differential diagnosis

Porphyria cutanea tarda; acute intermittentporphyria; adrenal crisis; biliary disease;fibromyalgia; Addison’s disease; acute abdo-men from diverse causes; psychosis; leadintoxication

Therapy

Glucose 400 mg IV per day for mild attacks;hematin 4 mg per kg per day for 4 days foracute attacks

References

Lim HW, Cohen JL (1999) The cutaneous rias Seminars in Cutaneous Medicine & Sur- gery 18(4):285–292

porphy-Hereditary hemochromatosis

Hemochromatosis

Trang 25

Acrokeratoelastoidosis

Hereditary symmetrical aplastic nevi of the temples

Brauer’s syndrome

Heredofamilial angiomatosis

Osler-Weber-Rendu syndrome

Heredopathia atactica polyneuritiformis

Refsum disease

Herlitz syndrome

Epidermolysis bullosa

Hermansky-Pudlak syndrome

Synonym(s)

None

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282 Herpes gestationis

Definition

Oculocutaneous albinism associated with a

mild hemorrhagic diathesis

Pathogenesis

Autosomal recessive inheritance, many with

a mutation of the HPS1 gene; storage pool

platelet defect with poor platelet

aggrega-tion; accumulation of a ceroid lipofuscin in

the lysosomes of a variety of tissues

Variable degrees of hypopigmentation;

pig-mented nevi and freckles common; mild

bleeding disorder with epistaxis, easy

bruis-ing, hemoptysis, gingival bleedbruis-ing, and

postpartum bleeding; interstitial lung

fibro-sis; restrictive lung disease; granulomatous

colitis

Albinism; Chediak-Higashi syndrome

Therapy

Avoidance of aspirin; low vision evaluation

and rehabilitation; sun avoidance

References

Toro J, Turner M, Gahl, WA (1999) Dermatologic

manifestations of Hermansky-Pudlak

syn-drome in patients with and without a 16-base

pair duplication in the HPS1 gene Archives of

Dermatology 135(7)774–780

Herpes gestationis

Synonym(s)

Pemphigoid gestationis; autoimmune

der-matosis of pregnancy;

pregnancy-associ-ated autoimmune disease

Definition

Autoimmune bullous eruption developing

in association with pregnancy

Pathogenesis

Immunoglobulin G (IgG) autoantibodies

produced against bullous pemphigoid (BP)

antigen 2 (BPAG2) (also known as BP 180),which is component of the hemidesmo-some; trigger for autoantibody productionunknown

Eruption develops during the second andthird trimesters; in 25% of patients, lesionsappear immediately after delivery, begin asintensely pruritic erythematous urticarialpatches and plaques, often periumbilical;lesions progress to tense vesicles and bul-lae, spreading peripherally, often sparingthe face, palms, soles, and mucous mem-branes; disease activity usually remitswithin days after parturition; some patientshave persistent disease activity that lastsmonths or years; sometimes recurs with theresumption of menses, use of oral contra-ceptives, and with subsequent pregnancies

Bullous pemphigoid; linear IgA bullous matosis; dermatitis herpetiformis; herpessimplex virus infection; drug-induced bul-lous disorder; papular dermatitis of preg-nancy; prurigo gestationis of Besnier; pru-ritic urticarial papules and plaques of preg-nancy (PUPPP)

der-Therapy

Mild disease: corticosteroids, topical, highpotency

Severe disease: prednisone

Herpes gestationis Multiple vesicles and bullae

on the upper extremities in a pregnant woman

Trang 27

Herpes simplex virus infection 283

H

References

Scott JE, Ahmed AR (1998) The blistering

diseas-es Medical Clinics of North America

82(6):1239–1283

Herpes gladiatorum

Herpes simplex virus infection

Herpes simplex virus

infection

Synonym(s)

None

Definition

Viral infection caused by Herpesvirus

hom-inis (herpes simplex virus)

Pathogenesis

Transmitted through close personal

con-tact; two viral subtypes: HSV-1 transmitted

primarily by contact with infected saliva;

HSV-2 mainly transmitted sexually; after

direct exposure to infectious material (i.e.,

saliva, genital secretions), initial viral

repli-cation occurs at either the skin or mucous

membrane entry site; after retrograde

axonal flow from neurons at viral point of

entry and local replication, viral genome

becomes latent and no viral particles are

produced; stimulus (e.g., physical or

emo-tional stress, fever, ultraviolet light) causes

reactivation of the virus

Neonatal infection: onset of illness within

24 hours of birth; most often, symptoms of

illness within the first week of life; rash

noted after symptoms begin;

manifesta-tions of illness representative of the organ

systems involved (i.e., CNS, lungs,

gastroin-testinal tract, heart, kidneys); skin vesicles

develops on an erythematous base, which

may coalesce into playues; localized eye

infection with conjunctival injection and awatery discharge; dendritic lesions on fluo-rescein staining of the cornea; acute gingi-vostomatitis: most frequent clinical presen-tation of first-episode, primary HSV infec-tion, although most patients haveasymptomatic first infection; fever (102–104°F); listlessness or irritability; inability

to eat and/or drink; gingivitis with edly swollen, erythematous, bleeding gums;occasional increased drooling noted ininfants; vesicular lesions develop on thetongue, buccal mucosa, and palate, withextension to lips and face; tender sub-mandibular or cervical adenopathy; dis-ease lasting from 3–7 days; recurrent orola-bial herpetic infection (herpes labialis):heralded by a prodrome of pain, tingling,burning, or itching, usually lasts up to

mark-6 hours; vesicular rash in crops of 3–5 cles, frequently arising near the vermillionborder; recurrences often associated withfebrile illnesses, local trauma, sun expo-sure, or menstruation; primary genitalinfections: most infections asymptomatic;severe constitutional symptoms: fever,malaise, myalgias, and occasional head-ache; vesicular rash; lesions sometimes per-sist for up to 3 weeks; painful inguinal lym-phadenopathy; dysuria; vaginal discharge;recurrent genital infections: vulvar irrita-tion and/or ulcerating or vesicular lesions;symptoms more severe in females; recur-rent infections in males sometimes presentwith vesicular lesions on the shaft of thepenis; local symptoms of recurrence: pain,itching, and dysuria; CNS infection:encephalitis possible manifestation of pri-mary or recurrent infection; other seque-lae: aseptic meningitis, transverse myelitis;herpetic whitlow (infection of a digit):presents with acute onset of edema, ery-thema, and localized pain and tenderness

vesi-in the fvesi-inger; associated fever and enlargedregional adenopathy; herpes gladiatorum:begins with painful vesicular lesions, fre-quently over the shoulders and neck inwrestlers (sites of skin-to-skin contact);Kaposi’s varicelliform eruption (eczemaherpeticum): clusters of umbilicated vesicu-lopustules in areas of a pre-existent derma-

Trang 28

284 Herpes zoster

titis; transmission occurs through contact

with an infected person or by

dissemina-tion of primary or recurrent herpes;

recur-rent episodes sometimes occur, but milder

and not usually associated with systemic

symptoms; severe cases sometimes cause

scarring

Impetigo; candidiasis; varicella; herpes

zoster; vesicular dermatophytosis; bullous

pemphigoid; pemphigus vulgaris; aphthous

stomatitis; Behçet’s disease; contact

derma-titis

Therapy

Neonatal infection, CNS infection:

acyclo-vir; first episode mucocutaneous

infec-tion, recurrent mucocutaneous infecinfec-tion,

herpetic whitlow, herpes gladiatorum:

vala-cyclovir, famciclovir; chronic suppression:

valacyclovir; famciclovir

References

Simmons A (2002) Clinical manifestations and

treatment considerations of herpes simplex

vi-rus infection Journal of Infectious Diseases 186

Neurocutaneous infection caused by the

varicella-zoster virus, which occurs in

peo-ple who have had chickenpox; represents a

reactivation of the dormant varicella-zoster

virus

Pathogenesis

Reactivation of dormant varicella-zoster

virus (VZV); results most often from a

fail-ure of the immune system to contain latent

VZV replication; most commonly occurs in

one or more posterior spinal ganglia or

cra-nial sensory ganglia; trigger of reactivation

unclear, but some cases possibly related toexternal re-exposure to the virus, acute orchronic disease processes such as malig-nancies and other infections, medications,and emotional stress

May begin with non-specific constitutionalsymptoms and signs; prodromal pain orparathesias along one or more der-matomes, lasting 1–10 days, followed bypatchy erythema in the dermatomal area ofinvolvement and regional lymphadenopa-thy; unilateral, grouped vesicles on ery-thematous base, with severe local pain; ves-icles initially clear, but eventually becom-ing pustular, rupturing, crusting, andinvoluting; scarring ensues if deeper epider-mal and dermal layers compromised byscratching, secondary infection, or othercomplications

Zoster oticus (geniculate zoster, zosterauris, Ramsay-Hunt syndrome, Hunt syn-drome): Ménière disease, Bell palsy, cer-brovascular accident or abscess of the ear;beginning with otalgia and herpetiformvesicles on the external ear canal, with orwithout features of facial paralysis, result-ing from facial nerve involvement, auditorysymptoms (e.g., deafness), and vestibularsymptoms

Disseminated zoster: generalized eruption

of more than 15–25 extradermatomal cles, occurring 7–14 days after the onset ofdermatomal disease; occurs rarely in thegeneral population, but commonly in eld-erly, hospitalized, or immunocompromisedpatients; often an indication of depressedcell-mediated immunity caused by variousunderlying clinical situations, includingmalignancies, radiation therapy, cancerchemotherapy, organ transplants, andchronic use of systemic corticosteroids; dis-semination sometimes includes involve-ment of the lungs and central nervous sys-tem

vesi-Differential diagnosis

Varicella; herpes simplex virus infection;impetigo; candidiasis; erysipelas; cellulitis;bullous pemphigoid; pemphigus; contact

Trang 29

Hidradenitis suppurativa 285

H

dermatitis; urticaria; photoallergic

reac-tion; folliculitis; insect bite reacreac-tion;

bra-chioradial pruritus

Therapy

Famciclovir; valacyclovir; post-herpetic

neuralgia prophylaxis: prednisone;

post-herpetic neuralgia: capsaicin; tricyclic

anti-depressants, such as amitriptyline: 25–

100 mg PO daily; gabapentin: 300–2400 mg

PO daily

References

Chen TM, George S, Woodruff CA, Hsu S (2002)

Clinical manifestations of varicella-zoster virus

infection Dermatologic Clinics 20(2):267–282

Disorder of the terminal follicular

epithe-lium in the apocrine gland–bearing skin,

characterized by comedone-like follicular

occlusion, chronic relapsing inflammation,

mucopurulent discharge, and progressive

scarring

Pathogenesis

Unknown disorder of follicular occlusion;

earliest change: follicular plugging which

obstructs apocrine gland ducts; earliest

inflammatory event: rupture of the

follicu-lar epithelium: friction in intertriginous

locations considered possible contributing

factor; rupture followed by spillage of

for-eign-body material into the dermis,

initiat-ing an inflammatory response resultinitiat-ing in

foreign-body granuloma; bacterial tion a risk factor for destructive scarring,but not a primary cause of the disease;genetic factors may be operative

infec-Clinical manifestation

Hirsutism and obesity common findingsamong affected women; early symptoms ofpruritus, erythema, and local hyperhidro-sis; lesions occur in the axillae, groin area,nipples, and buttocks; painful and/or ten-der red papules and nodules; lesion healwith fibrosis and eventual recurrence in theadjacent area; painful or tender abscessesand inflamed, discharging papules or nod-ules; nodules coalesce and sometimesbecome infected, resulting in acuteabscesses; dermal contractures and rope-like elevation of the skin; multiple abscessesand sinus tracts form a subcutaneous hon-eycomb; double-ended comedones; associ-ated arthropathy sometimes presentingwith asymmetric pauciarticular arthritis,symmetric polyarthritis, or polyarthralgiasyndrome

Granuloma inguinale; lymphogranulomavenereum; actinomycosis; staphylococcalabscesses; Bartholin cyst; carbuncle; Crohndisease; infected or inflamed epidermoidcyst; tuberculosis; tularemia; ulcerative col-itis

Therapy

Wide surgical excision, preferably taking asmuch apocrine gland-bearing skin as possi-ble; localized disease: surgical techniquesincluding incision and drainage; exteriori-zation; curettage; electrocoagulation of thesinus tracts; simple excision; triamcinolone3–5 mg per kg intralesionally to inflamednodules; tetracycline; erythromycin;isotretinoin; acitretin; dapsone

References

Brown TJ, Rosen T, Orengo IF (1998) Hidradenitis suppurativa Southern Medical Association Journal 91(12):1107–1114

Trang 30

286 Hidradenoma, clear cell

Hidradenoma, clear cell

Eccrine hidradenoma

Hidradenoma papilliferum

Synonym(s)

Papillary hidradenoma; hidradenoma

vul-vae; apocrine adenoma; adenoma

hidrade-noides

Definition

Benign tumor with apocrine

differentia-tion, most commonly seen in the genital

area of women

Pathogenesis

Unknown

Solitary, well-circumscribed, firm-to-cystic,

bluish papule or nodule, with occasional

ulceration, usually noted in the vulvar area

of middle-aged women

Leiomyoma; epidermoid cyst; squamous

cell carcinoma; hemangioma; pyogenic

granuloma; melanoma; Bartholin cyst

Therapy

Surgical excision

References

Vang R, Cohen PR (1999) Ectopic hidradenoma

papilliferum: a case report and review of the

lit-erature Journal of the American Academy of

dys-Pathogenesis

Autosomal dominant trait; abnormal

α-proteins in hair and nails

Dystrophic nails; sparse, thin, fragile hair;thickening of the palms and soles; normalsweat function; skin dryness

Anhidrotic ectodermal dysplasia; chia congenita; Basan syndrome; chondr-oectodermal dysplasia; dyskeratosis con-genita

Trang 31

Chitty LS, Dennis N, Baraitser M (1996) Hidrotic

ectodermal dysplasia of hair, teeth, and nails:

case reports and review Journal of Medical

Ge-netics 33(8):707–710

Hirsutism

Definition

Development of androgen-dependent

ter-minal body hair in a woman at sites where

terminal hair not normally found

References

Marshburn PB, Carr BR (1995) Hirsutism and

vi-rilization A systematic approach to benign and

potentially serious causes Postgraduate

Langerhans cell histiocytosis

Histiocytosis, regressing atypical

Cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma

Trang 32

288 HIV-associated eosinophilic folliculitis

Definition

Pulmonary and systemic infection caused

by the fungus Histoplasma capsulatum

Pathogenesis

Alveolar deposition caused by

aerosoliza-tion of conidia and mycelial fragments from

contaminated soil; susceptibility to

dissemi-nation increased with impaired cellular

host defenses; intracellular conversion from

mycelial to pathogenic yeast form after

macrophage phagocytosis; clinical

manifes-tations occur with continued exposure to

large inocula; pulmonary infection may

dis-seminate, with hematogenous spread

Acute pulmonary infection usually

asymp-tomatic; with symptomatic disease, fever,

headache, malaise, myalgia, abdominal

pain, and chills; with exposure to large

inoculum, severe dyspnea may occur;

non-specific signs of infection: erythema

nodo-sum and erythema multiforme; occsional

joint pain and infiltrated papules in the

skin

Chronic pulmonary disease mostly in

patients with underlying pulmonary

dis-ease; associated with cough, weight loss,

fevers, and malaise; if cavitations present,

hemoptysis, sputum production, and

increasing dyspnea

Progressive disseminated disease occurs

mostly in immunocompromised patients;

skin lesions begins as small papules and

ulcerations; oropharyngeal ulcers

some-times involve buccal mucosa, tongue,

gin-giva, and larynx

Bacterial or mycoplasma pneumonia; North

American blastomycosis;

coccidioidomyco-sis; tuberculococcidioidomyco-sis; sarcoidococcidioidomyco-sis; aspergillococcidioidomyco-sis;

squamous cell carcinoma; lymphoma

Therapy

None for asymptomatic disease or for

cuta-neous disease as sole sign of dissemination;

progressive disease, particularly with

men-ingitis – amphotericin B – 0.7–1 mg per kg

per day IV to a total dose of 35 mg per kg;

mildly symptomatic or prolonged acutepulmonary disease – ketoconazole; itraco-nazole

References

Mocherla S, Wheat LJ (2001) Treatment of plasmosis Seminars in Respiratory Infections 16(2):141–148

histo-HIV-associated eosinophilic folliculitis

Eosinophilic pustular folliculitis

HIV-related eosinophilic folliculitis

Eosinophilic pustular folliculitis

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