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rare in pregnancy, there are few data upon which to make specific to temporarily elevate serum levels of C1-INH, although this
recommendations. The treatment often balances the risk of certain exposes mother and fetus to transfusion risks. C1-esterase inhi-
agents to the fetus versus the maternal benefit. Fetal morbidity and bitor concentrate can be given for acute angioedema and before
mortality arise from both the diseases and their treatments. These major surgery, and should be available in the operating room.
autoimmune diseases are discussed more fully in Chapter 23.

Autoimmune progesterone dermatitis
Biologic agents
Biologic agents are drugs that enhance or diminish immune This disease is characterized by exacerbations during the luteal
system function to effect a specific action. The term biologic is phase of the menstrual cycle and presents with recurrent angio-
used as a descriptive term for therapeutic agents with biologic edema, skin changes (e.g. erythema multiforme, eczema, and
urticaria) and possible anaphylaxis.163,164 O™Rourke et al. reported
properties, including monoclonal antibodies and soluble cytokine
receptors. The first two biologic agents approved for use were the a case of autoimmune progesterone dermatitis during pregnancy.
tumor necrosis factor (TNF)-a inhibiting agents etanercept and Delivery by C/S with hysterectomy and bilateral oophorectomy
infliximab, both developed for the treatment of rheumatoid arthri- was planned since oophorectomy provides prolonged relief from
tis (RA). Biologic agents currently used in dermatology include the disease. Angioedema developed during delivery and was trea-
etanercept, alefacept, efalizumab, adalimumab, and natalizumab. ted with fluids, ephedrine, phenylephrine, and epinephrine. The
Additional biologic agents are being studied in order to achieve authors suggested spinal anesthesia to avoid airway
manipulation.165
greater clinical success with fewer adverse side effects. The more
severe clinical cases of both rheumatoid arthritis and psoriasis are
increasingly being managed with parenteral biological therapy.
Mastocytosis
Bensen recently reviewed immunologic manipulation during
pregnancy and suggests roles for intravenous immunoglobulin, Mastocytosis comprises several diseases characterized by an
plasma exchange, immunosuppressive drugs, and biologics abnormal increase in tissue mast cells. Cutaneous mastocytosis
including C1 esterase inhibitor protein, cell surface complement (CM) or urticaria pigmentosa is the most common form and
regulator proteins, or interleukin-3.156 The biologics have efficacy presents as a mast cell hyperplasia limited to the skin. In the
in models of antibody-induced cell injury but clinical trials are USA, of new patients visiting dermatology clinics, 0.1“0.8% have



353
Table 19.9 Viral diseases with dermatologic manifestations (see also Chapter 18)

Virus Lesion Symptoms and course Maternal“fetal transmission Newborn pathology Medical therapy and effects Anesthetic implications

Herpes simplex Erythematous papules, Primary: fever, malaise, Close muco-cutaneous When infected, newborn Acyclovir and its analogs Primary: viremia
virus (HSV) vesicles and pustules. lymphadenopathy, contact at birth. mortality > 50%. Half of decrease symptoms and associated with risk of
Type 1: Usually Crusting by 7“10 days. myalgia associated with Primary: 50% survivors have neurologic viral shedding. spread to CNS. Regional
oral Recurrences less viremia. Secondary: < 1% or ophthalmologic Excreted by kidney. block avoided. Viremia
involvement symptomatic. Secondary: latency weeks morbidity. Minimal maternal and fetal usually an indication for
Type 2: Usually to years. No viremia. toxicity. C/S. Epidural morphine
genital may reactivate latent
involvement HSV.
Secondary: regional block
acceptable.
Human Lesions due to immuno- Irreversible immune Occurs in 15“40%, probably Affected babies appear Zidovudine may cause Strict transmission
immuno- suppression. suppression with via placenta. disease-free for about maternal anemia, precautions.
deficiency Common outbreaks include quiescent periods. Transmission significantly eight months. neutropenia, rash, and Multiorgan pathology
Median survival ¼ three
virus (HIV) herpes-zoster (face, torso, decreased by antiretroviral proximal myopathy. common.
extremities); molluscum therapy. years. Oral ketoconazole has Regional anesthesia likely
contagiosum (widespread potential hepatotoxicity. acceptable (viral entry
firm, translucent into CNS occurs early in
papules); candidiasis (oral disease; therefore
and vulvar); regional anesthesia
staphylococcus folliculitis unlikely to ˜˜spread™™ the
(face, thorax, back); infection).
Kaposi™s sarcoma (any
region).
Human Genital papules and Swelling, pain, and itch in Transmitted vaginally at Latent infection associated Podophyllin, fluorouracil Transmission hazard to
papillomavirus verrucous friable growths. birth canal. birth on exposure to with laryngeal cream, and alpha- health workers during
laser vaporization.
(HPV) Possible obstruction by lesions. papillomatosis in interferon are
May need C/S due to mass
lesions. Patient may be childhood. contraindicated during
effect of lesions.
immunocompromised Latency period is up to five pregnancy.
or have coexisting years. Acceptable therapies
genital infections. include laser vaporization
and topical
trichloroacetic acid.
Transplacental. May cause stillbirth, None. Infection precautions.
Parvovirus B19 Bright red ˜˜slapped cheeks™™ Concurrent upper
miscarriage, or fetal May be associated
erythema. Pink, lacy, respiratory symptoms,
hydrops with fetal with aplastic crisis
reticulate rash on torso fever, myalgia,
complications in up to and chronic hemolytic
and extremities. fatigue, lymph node
132
Arthritis of 20% of infected anemia.
Papular pruritic ˜˜gloves and swelling.
136,137,138
hands, wrists, and knees. mothers.
socks™™ syndrome.
May act as trigger for
Maculopapular eruptions,
autoimmune diseases
petechiae, and purpura.
e.g. systemic lupus
erythematosus.133,134,135
Rubella (German Generalized macular rash Mild fever and malaise; Transplacental early Many congenital anomalies No effective therapy Anesthesiologists should
measles) lasting three days. arthralgia, neuritis, and transmission leads to may result involving although mother™s disease be vaccinated to prevent
thrombocytopenia are more significant virtually any organ generally requires no spread.
rare. 30% asymptomatic. pathology including system. therapy. Immunization
demise. recommended before first
pregnancy.
Rubeola Koplik spots on buccal Prodrome of fever and Transplacental maternal Transplacental Symptomatic. Oral pharynx, respiratory
(measles) mucosa are first malaise followed by infection may lead to transmission within a few Aggressive treatment of system, and neurologic
pathognomonic sign conjunctivitis and oral preterm labor. No known days of birth leads to secondary infections. systems may be
(1-mm white dot encircled pharyngeal involvement. related congenital congenital measles, which Exposed pregnant women involved.
by rosy red ring). Cough persists for malformations. may be mild but are fatal should receive immune
Followed by total body ten days along with to 32%. Best avoided by serum globulin.
maculopapular rash lymphadenopathy and immunizations.
beginning on head and splenomegaly.
spreading caudally. Complications
include pneumonia,
laryngotracheo-
bronchitis, myocarditis,
encephalitis, and
thrombocytopenia
purpura.
Transplacental first and Neonatal varicella occurs if Varicella-zoster immune Due to risk of pneumonia,
Varicella Primary: chickenpox “ leads Rash and symptoms last one
second trimester infection mother becomes infected globulin (VZIG) should be airway manipulation
to erythematous rash week; Infectivity starts
leads to congenital and fetus is born before administered within 96 h should be avoided in
followed by punctate two days before rash and
varicella syndrome in maternal antibody of exposure to prevent mothers with varicella.
vesicles that become continues until all lesions
about 1% (limb formation (within five maternal infection. Regional anesthesia is
purulent and crust. crusted.
acceptable.140
hypoplasia and days of infection). Neonatal varicella is more
Significant itching. Incidence: 1 in 5“10 000
psychomotor Neonatal varicella has 30% severe if maternal rash
Secondary: herpes zoster pregnancies. In pregnant
retardation). neonatal mortality so at appears five days before or
(shingles). women, disease much
risk infants should receive two days after delivery. The
worse: may get fulminant
immune globulin. newborn should be given
varicellar pneumonia.
VZIG immediately.
Women at particular risk
Intravenous acyclovir is
for varicellar pneumonia
indicated for maternal
are smokers and those
139
with !100 skin lesions. pneumonia and severely
affected neonate.
Acyclovir and valacyclovir
have not yet been
adequately studied for
postexposure prophylaxis
to pregnant women or
neonates.

C/S ¼ cesarean section
Table 19.10 Bacterial diseases with dermatologic manifestations (see also Chapter 18)

Bacteria Lesion Symptoms and course Maternal“fetal transmission Newborn pathology Medical therapy and effects Anesthetic implications

Neisseria Small vesiculopustules and Mother may be Transmission during fetal Gonococcal ophthalmia can Penicillin or ceftriaxone Infection precautions.
a
gonorrhoeae purpuric macules up to asymptomatic or have passage through cause blindness. preferred.
2 cm in diameter. fever, arthralgia, and infected birth canal Urethritis in male Newborn treated with
Lesions common around malaise. or transplacental. offspring. ophthalmic silver nitrate,
joints and on soles and Complications included May have preterm labor Dissemination leads to tetracycline, or
palms. Usually have meningitis, myocarditis, and/or fetal loss. meningitis and arthritis. erythromycin.
purulent cervical and and pericarditis.
urethral discharges in
early infections.
Streptococcus Cutaneous infection may be Fever, prodromal flu-like Direct contact in birth canal. Fetal outcome related to Plasma pheresis to remove Group B streptococcus
spp. e.g. noted. symptoms. maternal condition and toxin, IV immuno- prophylaxis is often
S. pyogenes May lead to necrotizing exposure at birth. globulin, antibiotics. given in labor or during
fasciitis and toxic shock May need pressor support C/S. Anaphylaxis may
occur.141
like syndrome. and ventilatory support.
Bacterial endocarditis may
occur.
b 159
Lyme disease Erythema chronicum Flu-like symptoms Spirochete may spread to Cardiac abnormality, Doxycycline is usual therapy Depends on organs
(caused by the migrans is an expanding common. fetus transplacentally. syndactyly, cortical but is avoided during involved.
spirochete erythematous patch with May develop into chronic Adverse outcomes in 32% of blindness, rash. pregnancy (fetal effects).
Borrelia central clearing. Lesions systemic syndrome pregnancies (preterm Penicillin, amoxicillin,
burgdorferi) common on thigh, groin, marked by neurologic labor, fetal demise, ceftriaxone, erythromycin
and axilla. Smaller, changes (meningitis, cardiac abnormality). all used. Prophylactic
annular secondary cranial nerve palsies), Should examine placenta antibodies after tick bites
lesions. cardiac abnormalities at birth for spirochetes. are controversial.
(myocarditis, heart
block), and arthritis.

a
Differential diagnosis includes meningococcemia, bacterial endocarditis, Rocky Mountain spotted fever, and vasculitis.
b
The accurate diagnosis of Lyme disease is difficult. The disease occurs in three stages: early localized infection, early disseminated infection, late persistent infection. The clinical features of the three stages evolve,
as do the laboratory findings. Laboratory confirmation requires the finding of specific antibodies to B. burgdorferi in serum. Numerous immunologic assays have been used. Extreme caution is necessary with
regards to the interpretation of both the clinical and laboratory findings as there have been many false negatives and also false positives. Fortunately, treatment is generally successful.142
Table 19.11 Autoimmune diseases with dermatologic manifestations

Maternal“fetal transmission
Disorder Lesion/etiology Symptoms and course Effects of pregnancy and newborn pathology Medical therapy and effects Anesthetic implications

Heliotope a rash, Gottron
Idiopathic Progressive proximal Pregnancy exacerbates High-risk pregnancy with 50% During periods of activity, Weakness of pharyngeal and
b
inflammatory papules. symmetrical muscle maternal disease and fetal loss but no congenital negative nitrogen balance intercostal muscles and
myopathies Necrotizing inflammatory weakness; marked by exacerbation may continue disease among from muscle wasting. diaphragm lead to airway
148,149
(polymyositis myopathy of striated relapses and exacerbations. postpartum. survivors. Steroids and immune sup- and ventilatory problems
[PM], dermato- muscle; unknown etiol- 20% associated with No newborn pathology. pressants (e.g. methotrex- including pneumonia;
myositis [DM], ogy but autoimmune malignancy; elevated CPK. ate /azathioprine). myocardial fibrosis may
and inclusion responses to various Mortality reduced from 33% lead to heart block and LV
body myositis environmental and to 8% by steroid use. dysfunction.
144,145
[IBM]) genetic factors have been Avoid malignant hyperther-
146,147
proposed. mia triggering agents.
May have altered neuromus-
cular blocker responses.150
Pemphigus Flaccid blisters and erosions 1st or 2nd trimester or Precipitated or aggravated by Antibodies cross placenta. Plasmapheresis favored over Secondary infection may be
vulgaris on skin and oral mucosa. postpartum. pregnancy. Transient lesions in new- immunosuppressive present.
151
IgG autoantibodies Very rare in childbearing born (neonatal therapy. Some patients have coexisting
against desmosomal years. pemphigus). Mortality about 5%. myasthenia gravis and thy-
proteins in serum and High fetal mortality/ moma. Airway considera-
epidermis. morbidity with severe tions similar to those of
disease. epidermolysis bullosa (see
above).
Polyarteritis Tender nodules, ulcerations, Affects medium-sized arteries Uncertain. Antibodies may cross to fetus. Immunosuppressive therapy. Depends on organ
152
nodosa erythema. of all organs except lung, Few cases reported. Previously reported mater- involvement.
May occur after hepatitis B especially kidney, liver, Manifested by livedo reti- nal mortality was high.
( > 80%).153,154
or b-hemolytic heart, and GI tract. cularis, cutaneous
Streptococcus infection. nodules, and acral necro-
sis. Self-limiting in
neonate.
Scleroderma Thick, taut, sclerotic skin, ˜˜salt Excessive collagen production Pregnancy exacerbates No newborn effects but high Vasodilators for Raynaud Consider renal, cardiac, and
and pepper™™ hyper- and leads to diffuse fibrosis, disease in half of cases. rate of prematurity/SGA phenomenon. pulmonary involvement.
155
hypopigmentation. especially of skin, lung, and Renal crisis is major risk. infants due to placental Steroids, May be restrictive lung
Cutaneous ulcer of finger- kidneys. May have micro- vascular abnormalities. immunosuppressants. disease.
tips and joints. May develop pulmonary chimerism wherein fetal No increase in miscarriages Vasoconstriction and skin
c
Limited disease: CREST hypertension, renal fail- cells enter or infertility. changes make intravenous
syndrome. ure, esophageal dysmo- maternal block and and monitoring access
Diffuse/systemic disease: tility, pericarditis, invoke the autoimmune difficult.
internal organ cardiac fibrosis with response and systemic Need to maintain warm room
involvement. conduction defects. sclerosis. to prevent Raynaud™s.
Airway compromise from
esophageal involvement.
Regional analgesia acceptable.
Table 19.11 (cont.)

Maternal“fetal transmission
Disorder Lesion/etiology Symptoms and course Effects of pregnancy and newborn pathology Medical therapy and effects Anesthetic implications

Systemic lupus Butterfly-like malar rash; Fluctuating course with May be exacerbated by A 40% incidence of pregnancy NSAIDs, aspirin, steroids, and Consider systemic problems,
erythematosus generalized morbilliform multiorgan involvement. pregnancy. However, loss up to 28 weeks is not heparin as needed. particularly renal disease.
(SLE) eruption with edema. May have nonerosive arthri- pregnancy does not affect correlated with disease Immuno-modulators. Airway may be compromised
Progression to elevated tis, Raynaud phenom- long-term prognosis. severity but likely related to by edema and lesions.
plaques, dense scaling, enon, pericarditis, Risk of maternal death is presence of lupus Need to maintain warm room
severe edema, epidermal pleuritis, glomerulone- highest in the peripartum anticoagulant and to prevent Raynaud dis-
necrosis. phritis, renal failure, sei- period, due to pulmonary anticardiolipin antibodies, ease. Vasoconstriction
zures, thrombocytope- hemorrhage or lupus which cross placenta. from Raynaud disease may
nia, leukopenia, and pneumonitis. Also increased fetal risk from make arterial line insertion
hemolytic anemia. Increased risk of pre- maternal disease. more difficult and make
eclampsia. May be hard to Neonatal lupus syndrome monitoring of arterial
differentiate from lupus consists of hematologic, blood pressure and pulse
nephritis. dermatologic, and car- oximetry less accurate.
diac abnormalities (e.g. Should document neuro-
complete heart block). pathies preoperatively.
Two-thirds of neonates are At risk for thromboses in
normal. brain, placenta, and lower
extremities.
Regional anesthesia must take
into account any coagulo-
pathy or thrombo-
prophylaxis therapies.

CPK ¼ creatine phosphokinase; IgG ¼ immunoglobulin G; GI ¼ gastrointestinal; NSAID ¼ nonsteroidal anti-inflammatory drug; LV ¼ left ventricle; SGA ¼ small for gestational age
a
Heliotrope: periorbital and eyelid violaceous erythema and edema that may involve entire face.
b
Gottron papules: similar to heliotrope but found on metacarpophalangeal and more distal interphalangeal joints and extensor aspects of knees and elbows.
c
CREST syndrome: calcinosis, Raynaud phenomenon, esophageal strictures, sclerosis, telangiectasis.
Chapter 19


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