Causes of Bullous Pemphigoid
Bullous Pemphigoid Causes: Book Excerpts
Related information on causes of Bullous Pemphigoid:
As with all medical conditions,
there may be many causal factors.
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Vesicular & Bullous Lesions:
Differential Diagnosis
(In a Page: Signs and Symptoms)
Localized
-
Allergic contact dermatitis (e.g. rhus)
–Localized vesicular and bullous eruptions
-
Herpes-zoster or shingles
–Due to reactivation of latent virus
–More common in adults
–Presents as painful vesicles on an
erythematous base in a dermatomal distribution, beginning with fever, dysesthesia, and/or malaise -
Herpes simplex virus
–Herpetic lesions present as painful, recurrent vesicles on an erythematous base
–Type 1 usually affects oral mucosa and vermilion border
–Genital HSV (most commonly HSV-2) may manifest as nonspecific symptoms (e.g., dysuria, urethritis)
-
Bullous impetigo
–Most common in children
–Presents as flaccid vesicles and bullae with honey-colored crust
-
Bites from many insects
-
Many viral infections of childhood can present with focal vesicles, especially hand-foot-andmouth disease
-
Burns and friction blisters
–Common causes of bullae, especially on hands
-
Diabetics can develop bullae on the legs
- Dyshidrotic eczema (pompholyx)
–Causes itching, scaling, and erythema, and minute vesicles and painful fissures
Diffuse
-
Polymorphous light eruption
–Common reaction to ultraviolet light
–Presents as itchy vesicles or erythematous papules on sun-exposed areas
-
Varicella or “chicken pox”
–Presents with vesicles in crops, and in many stages of evolution
-
Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN)
–Most commonly caused by medications
–TEN is life threatening
-
Blistering diseases like bullous pemphigoid, pemphigus vulgaris, and porphyria cutanea tarda present with coalescing vesicles and bullae
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Source: In a Page: Signs and Symptoms, 2004
Atopic dermatitis:
Causes
(Professional Guide to Diseases (Eighth Edition))
The cause of atopic dermatitis is still unknown. However, several theories attempt to explain its pathogenesis. One theory suggests an underlying metabolically or biochemically induced skin disorder that’s genetically linked to elevated serum immunoglobulin (Ig) E levels. Another theory suggests defective T-cell function.
Exacerbating factors of atopic dermatitis include irritants, infections (commonly caused by Staphylococcus aureus), and some allergens. Although no reliable link exists between atopic dermatitis and exposure to inhalant allergens (such as house dust and animal dander), exposure to food allergens (such as soybeans, fish, or nuts) may coincide with flare-ups of atopic dermatitis.
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Source: Professional Guide to Diseases (Eighth Edition), 2005
Dermatitis:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
The cause of atopic dermatitis is unknown, but a genetic predisposition may be exacerbated by such factors as food allergies, infections, irritating chemicals, temperature and humidity, and emotions. Approximately 10% of childhood cases are due to allergy to certain foods, particularly eggs, peanuts, milk, fish, soy, and wheat. Atopic dermatitis tends to flare up in response to extremes in temperature and humidity. Other causes of flare-ups are sweating and psychological stress.
An important secondary cause of atopic dermatitis is irritation, which seems to change the epidermal structure, allowing immunoglobulin (Ig) E activity to increase. Consequently, chronic skin irritation usually continues even after exposure to the allergen has ended or after the irritation has been systemically controlled.
Atopic dermatitis is most common in infants, usually developing between ages 1 month and 1 year, commonly in those with strong family histories of atopic disease. At least half of those cases clear by age 36 months. These children often acquire other atopic disorders as they grow older. Typically, this form of dermatitis flares and subsides repeatedly before finally resolving during adolescence. However, it can persist into adulthood. In adults, it’s generally chronic or recurring.
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Source: Professional Guide to Diseases (Eighth Edition), 2005
Arthritis/Dermatitis:
Differential Overview
(Field Guide to Bedside Diagnosis)
❑Lyme disease
❑Erythema nodosum
❑Rheumatoid arthritis
❑Systemic lupus erythematosus
❑Psoriatic arthritis
❑Disseminated gonococcemia
❑Sarcoidosis
❑Scleroderma
❑Dermatomyositis
❑Reiter syndrome
❑Rheumatic fever
❑Behçet syndrome
❑Still disease
❑Hypersensitivity vasculitis
Clinical Findings
Lyme disease Erythema migrans, a rapidly expanding annular rash with a clearing center, is the key early finding. The site of the ixodid tick bite at the center of the lesion is usually intensely indurated, vesicular, or necrotic. The arthritis is an asymmetric oligoarthritis that usually occurs after the rash has resolved.
Erythema nodosum A prodromal syndrome of fever, chills, malaise, and polyarthralgia is followed by the development of lesions that are discrete, tender, slightly raised subcutaneous nodules on the shins or ankles. They represent a hypersensitivity reaction to group A streptococcal infection, tuberculosis, sarcoidosis, inflammatory bowel disease, or drugs such as oral contraceptives and sulfonamides.
Rheumatoid arthritis Symmetric polyarticular arthritis with synovial proliferation, especially of the wrist, and morning stiffness lasting more than 1 hour characterize the early joint involvement. Rheumatoid nodules appear over extensor surfaces. Vasculitic lesions are frequently found on the digits, appearing as small red or purpuric macules that progress to painful nodules or ulcers.
Systemic lupus erythematosus A classic butterfly rash occurs in 40% and is exacerbated by sun exposure. A diffuse maculopapular rash in areas exposed to the sun heralds disease flares. Discoid lesions and scaling plaques that range in color from red to violaceous, with central atrophy and telangiectasias, occur in 20%. Vasculitis, in the form of painful ulcers on the extremities, palpable purpura, or lupus profundus (firm nodules in the subcutaneous fat on the forehead, cheeks, buttocks, and upper arms) are found. The arthritis is typically one of symmetric fusiform swelling of the proximal interphalangeal and metacarpophalangeal joints, diffuse puffiness of the hands, and tenosynovitis.
Psoriatic arthritis Psoriatic plaques, erythematous with a silvery scale, are critical to diagnosis, but may be hidden in the scalp, umbilicus, or gluteal folds. Nail changes such as pitting or yellow discoloration of the nail plate are other clues. The arthritis typically involves the proximal interphalangeal and distal interphalangeal joints, creating sausage digits. The arthritis may become erosive, leading to telescoping of the hands. One-fourth of patients have axial skeletal arthritis.
Disseminated gonococcemia Acral lesions are typically hemorrhagic pustules, but petechiae, hemorrhagic papules, or hemorrhagic bullae can occur. Fever, rigor, tenosynovitis, and polyarthritis are other findings.
Sarcoidosis Transient maculopapular eruptions of the trunk, face, and extremities are often accompanied by uveitis, adenopathy, and parotid enlargement. Translucent reddish-brown to purple indolent plaques may develop on the face (lupus pernio), buttocks, or extremities. Joint symptoms consist of migratory transient arthralgias.
Scleroderma Early findings are primarily Raynaud phenomenon and puffy fingers. Later findings include sclerodactyly (smooth, shiny, tapered fingers with taut, bound-down skin); contractures with “claw hand” deformity; expressionless face (with thin lips, a beak-like nose, and sunken cheeks); microstomia; mat telangiectasias on the nail folds, face, lips, oral mucosa, or trunk; and calcinosis with leathery crepitation over the joints.
Dermatomyositis The classic skin manifestation is a lilac-colored heliotrope rash on the eyelids and in a butterfly distribution. Gottron papules are violaceous, scaly, flat lesions on the extensor aspect of the interphalangeal joints, elbows, knees, and medial malleoli; these occur as a late manifestation. Proximal muscle aching/weakness, not arthritis, is prominent. The patient is unable to reach overhead or arise from a chair. Neck flexors are more involved than extensors.
Reiter syndrome Arthritis, urethritis, conjunctivitis, and mucocutaneous ulcers are found. The arthritis is asymmetric, usually involving the lower extremity joints. Solitary sausage digits may be seen. Tendinitis and fasciitis are common. The mucocutaneous lesions are eroded red vesicles or papules of the corona and glans, which when confluent are called circinate balanitis. Pustules may change into thick hyperkeratotic plaques on the palms and soles, keratoderma blennorrhagicum.
Rheumatic fever There is an acute migratory polyarthritis with fever. Subcutaneous nodules appear over the bony prominences of the elbows, knuckles, ankles, scapulae, and occiput. They are associated with carditis. Erythema marginatum, appearing as evanescent pink lesions with serpiginous borders, is also associated with carditis.
Behçet syndrome The classic triad is arthritis, iritis, and oral and genital ulcerations. Recurrent aphthous ulcers are a sine qua non. They begin as macular erythema that develops into superficial gray ulcers. Scrotal or labial ulcerations are also found. Hypopyon uveitis, a hallmark, is a rare finding. The arthritis is primarily of the knees and ankles.
Still disease Skin lesions are red, flat, and less than 1 cm in diameter. Lesions are evanescent, occurring with fever spikes. A migratory polyarthralgia occurs.
Hypersensitivity vasculitis After an upper respiratory infection, young adults may develop palpable purpura over the extensor surfaces and buttocks. Arthritis, edema, and colicky abdominal pain, followed by bloody stools, suggests the diagnosis.
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Source: Field Guide to Bedside Diagnosis, 2007
Dermatitis:
Causes
(Handbook of Diseases)
The cause of atopic dermatitis is unknown, but there is a genetic predisposition exacerbated by such factors as food allergies, infections, irritating chemicals, temperature and humidity, and emotions. Approximately 10% of childhood cases are caused by allergy to certain foods, particularly eggs, peanuts, milk, and wheat.
Atopic dermatitis tends to flare up in response to extremes in temperature and humidity. Other causes of flare-ups are sweating and psychological stress.
An important secondary cause of atopic dermatitis is irritation, which seems to change the epidermal structure, allowing immunoglobulin (Ig) E activity to increase. Consequently, chronic skin irritation usually continues even after exposure to the allergen has ended or after the irritation has been systemically controlled.
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Source: Handbook of Diseases, 2003
Cough - Case 4-2: 7-Week-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
The causes of a chronic cough in an infant are diverse, but the most common
causes are viral infections. In infants with a history of conjunctivitis,
C. trachomatis should be considered. B. pertussis can occur in infants and produce a chronic cough. Most often, infants are unable
to generate the force necessary for the classic
“whoop.” Certainly, other bacterial pneumonias should be considered with the lobar
infiltrate noted on chest roentgenogram in this case. Finally, GER must always
be considered a common cause for cough in infancy. Other, less common causes of
cough in this age group include congenital malformations including
tracheoesophageal fistulas, tracheobronchomalacia, vascular rings, lobar
emphysema, bronchogenic cyst, pulmonary sequestration, laryngeal cleft, and
airway hemangiomas.
Congestive heart failure should always be considered, with common etiologies in
infancy being volume overload (patent ductus arteriosus, truncus arteriosus,
ventricular septal defect, common atrioventricular canal, total anomalous
pulmonary venous return), myocardial dysfunction (myocarditis, Kawasaki
syndrome, anomalous left coronary artery), arrhythmias (supraventricular
tachycardia), pressure overload (coarctation of the aorta, aortic stenosis),
and secondary causes (hypertension, sepsis).
The features of this case that prompted additional evaluation were cardiomegaly
and increased vascular markings noted on the chest roentgenogram, presence of a
heart murmur, and biventricular hypertrophy seen on the ECG.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Cough - Case 4-3: 7-Month-Old Girl:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
Viral infections are the most common cause of a cough in infancy, with
respiratory syncytial virus, adenovirus, and influenza and parainfluenza
viruses among the leading agents. In infancy, these viruses also commonly
produce lower airways disease, so that bronchiolitis quite often accompanies
the cough. Infants with bronchiolitis have decreased aeration with diffuse
rales and wheezing appreciated on auscultation. Fever is common, as is profuse
rhinorrhea.
Other infectious etiologies are possible and should always be considered in the
differential diagnosis; they include
C. trachomatis, pertussis, and bacterial pneumonia. Less commonly, infants present with
pulmonary tuberculosis or a fungal infection. Rarely, infectious entities such
as measles or parasitic infections manifest with cough.
Although cough can be the presenting symptom in many cases of congenital
malformations, this case strongly suggests an infectious etiology. The features
of this case that prompted additional evaluation included the rash and the
associated respiratory findings.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Cough - Case 4-6: 4-Month-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
A cough in infancy is most likely related to an infectious process, with viral
processes the leading causes. Respiratory syncytial virus is a common cause of
cough. However, other infectious etiologies should always be considered. Even
with good adherence to vaccine regimens, bacterial infections such as
B. pertussis are possible in infants. M. pneumoniae infections also occur rarely in infants.
Reactive airways disease, most often secondary to viral infection, is also a
common cause of cough in infancy. GER should be considered as well, even if
gastrointestinal symptoms are few.
Less common causes for cough in infancy include congenital malformations such as
tracheoesophageal fistula, tracheobronchomalacia, vascular rings, lobar
emphysema, bronchogenic cysts, pulmonary sequestration, laryngeal cleft, and
cystic adenomatoid malformation. Furthermore, one should attempt to elicit a
history for any possible swallowing disorder that might lead to recurrent
aspiration.
Other, less common causes of cough in infancy include CF, congestive heart
failure, interstitial pneumonitis, and congenital immunodeficiencies.
This patient's history is suggestive of an infectious etiology, because he was in good health
until approximately 1 one week before presentation. However, his history of
prematurity should add one more disease to the differential diagnosis:
bronchopulmonary dysplasia. Such patients are also more likely to develop
reactive airways disease in response to a viral infection.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Diarrhea - Case 17-1: 2-Month-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
In this case, diarrhea was associated with vomiting and a critical physical
finding, that of an inguinal mass. This essential finding directed the
differential diagnosis toward causes of inguinal or scrotal swelling. An
important distinction to make is between a painful and a painless mass. A
hydrocele is a common entity that causes painless inguinal or scrotal swelling.
It is primarily differentiated from an inguinal hernia by the ability to
palpate above the mass, revealing discontinuity between the mass and the
inguinal canal. The mass, as a result, does not change in size with straining
or crying. In addition, a hydrocele is not reducible and usually
transilluminates, although the ability to transilluminate the mass does not
exclude the possibility of an incarcerated hernia.
Another cause of a painful scrotal mass is testicular torsion. There often is no
history of a prior scrotal mass, and in fact there may be a history of
undescended testis. This mass is very tender and does not extend into the
inguinal canal.
Torsion of the appendix testis results in a painful scrotal mass that may appear
as a tender blue nodule on the upper pole of the testis, which itself is not
tender. Inguinal lymphadenopathy may be tender or painless, but the key to
diagnosis is the lateral and inferior location of these nodes in relation to
the inguinal canal. Signs of infection in the area of lymphatic drainage are
also important in making this diagnosis. An inguinal hernia is usually
characterized by a painless swelling in the inguinal area that often increases
in size with crying or straining. Incarceration of the hernia results in
extreme pain and signs of bowel obstruction. If strangulation occurs, bloody
diarrhea may result.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Diarrhea - Case 17-4: 15-Month-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
The chronic nature of his diarrhea for the last 3 months, associated with weight
loss, moved the differential diagnosis away from the diagnosis of acute
infectious diarrhea due to either bacterial or viral causes. A prolonged bout
of postinfectious diarrhea due to disaccharidase deficiency was possible but
unlikely. Chronic diarrhea due to infection with
C. difficile or ova and parasites was a possibility even without a history of antibiotic
use, bloody diarrhea, foreign travel, or use of untreated water sources. The
key observation in making this diagnosis occurred while the patient was in the
hospital: he took nothing by mouth but continued to produce profuse voluminous
watery diarrhea. This finding indicated the presence of secretory, rather than
osmotic, diarrhea. In this differential diagnosis, the list is rather brief and
includes rare congenital and paraneoplastic conditions. Congenital defects in
chloride or sodium transport are more likely to manifest in infancy. Infectious
causes of secretory diarrhea include small-bowel overgrowth or infection with
immuno adherent
E. coli stimulating gastrointestinal secretions. Any cause of villous atrophy, whether
congenital, autoimmune, or secondary to immune deficiency (e.g., HIV infection,
severe combined immunodeficiency) may also result in this presentation.
Neuroblastoma or other tumors of neural crest origin (e.g., ganglioneuroma) may
secrete vasoactive intestinal peptide (VIP), resulting in secretory diarrhea.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Fever - Case 11-1: 18-Month-Old Girl:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
This child presented with fever and seizures. Given her age and the difficult
examination, a lumbar puncture was performed to exclude meningitis as a cause
of seizures. The reassuring CSF findings led to other diagnostic
considerations. The maternal grandmother used an oral hypoglycemic agent,
making an ingestion-induced hypoglycemic seizure possible. However, the child
's serum glucose concentration was normal. The history of a cousin drowning
during a reported seizure raised the possibility of a cardiac condition such as
prolonged QT syndrome, Wolff-Parkinson-White syndrome, or hypertrophic
cardiomyopathy as a possible cause of hypoxic seizures. The electrocardiogram,
performed in light of this history, was normal.
In an 18-month-old girl who presents with a brief (less than 10-minute) seizure
in the context of fever, typical febrile seizure is the most likely diagnosis.
However, it is possible that the fever lowered the seizure threshold in a child
with an underlying seizure disorder. Potentially important clues in this case
were the hyperpigmented macules on this child
's skin. Café-au-lait spots are characteristic for neurofibromatosis type 1 (NF1) but may
also be noted in unaffected children and in children with other disorders. The
critical factor in this case was the number of spots seen; fewer than 0.1% of
normal individuals have more than six caf
é-au-lait spots. Inherited disorders associated with café-au-lait spots are summarized in Table 11-2.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Fever - Case 11-4: 7-Month-Old Girl:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
Neutropenia, defined as an absolute decrease in the number of circulating
neutrophils in the blood, can be caused by decreased production, increased
peripheral utilization, or increased destruction. The ANC is calculated by
multiplying the total WBC count by the total percentage of band forms and
segmented neutrophils: ANC = total WBC
× (percent bands + percent segmented neutrophils). In general, patients may be
characterized as having mild (1,000 to 1,500 cells/mm
3), moderate (500 to 1,000 cells/mm3), or severe (fewer than 500 cells/mm3) neutropenia. Blacks tend to have lower neutrophils counts; therefore, in some
patients an ANC of 900 cells/mm
3 may be considered normal.
The differential diagnosis of neutropenia in infancy includes a wide range of
conditions (Table 11-4). In a child who was previously healthy, the most likely
causes are alloimmune neonatal neutropenia, cyclic neutropenia, autoimmune
neutropenia (AIN) in infancy, and Kostmann syndrome. Alloimmune neutropenia, a
condition occurring in neonates, is analogous to Rh hemolytic disease. Maternal
sensitization to fetal neutrophils results in maternal immunoglobulin G (IgG)
antibodies
' crossing the placenta and causing an immune-mediated destruction of fetal
neutrophils. The neutropenia lasts several weeks but rarely persists beyond 6
months of age, making it an unlikely diagnosis in this 7-month-old patient.
Cyclic neutropenia can be diagnosed by serial WBC counts.
Less likely causes include neutropenia related to infection. Neutropenia
associated with increased peripheral utilization is possible in the context of
a serious cellulitis. Infections such as Epstein-Barr virus and parvovirus B19
can also cause neutropenia, but the normal hemoglobin and platelet count in
this case make these infections less likely. The mother does not have AIN, a
finding that sometimes leads to transient secondary neutropenia in newborn
infants.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-1: 14-Day-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
The differential diagnosis for the systemically ill neonate is quite broad.
Infectious causes are often considered first, especially common bacterial
pathogens (e.g., group B
Streptococcus, staphylococci, Escherichia coli, Listeria monocytogenes) and viruses (e.g., HSV, enterovirus). Less often, fungi (e.g., Candida species) and other classes of organisms (e.g., parasites) are implicated.
Congenital heart disease is another critically important consideration in sick
neonates; ductal-dependent anatomic lesions (e.g., coarctation of the aorta,
hypoplastic left heart syndrome) and tachydysrhythmias may manifest early in
life with profound cardiovascular compromise. Shock can also be seen in
severely anemic infants
—for instance, after a placental catastrophe or even a major intracranial
hemorrhage. Multiorgan dysfunction can also result from perinatal asphyxia,
neonatal surgical emergencies, and a multiplicity of endocrine and metabolic
abnormalities (including congenital adrenal hyperplasia, glucose and
electrolyte derangements, and numerous inborn errors of metabolism).
Conjugated hyperbilirubinemia in the neonate, such as that seen in the patient
described here, also has a multiplicity of causes. Among the possibilities are
idiopathic neonatal hepatitis,
α1-antitrypsin deficiency, hypopituitarism, hypothyroidism, bile acid synthesis
deficiency, exposure to intravenous hyperalimentation, and long lists of
infections and disorders of hepatobiliary anatomy. Similarly, neonatal
hepatomegaly is seen in a wide variety of settings, including infections
—either congenitally acquired (e.g., TORCH) or acute-onset (e.g., sepsis);
neonatal hepatitis; liver or gall bladder disease (e.g.,
α1-antitrypsin deficiency, biliary atresia, choledochal cyst); hydrops or
congestive heart failure; tumors; and metabolic disease (e.g., glycogen storage
diseases, galactosemia, tyrosinemia).
In addition to jaundice and hepatomegaly, the baby in this case study had
elevated liver enzymes and possible liver synthetic dysfunction (as a potential
contributing factor in his coagulopathy). In addition, his hepatobiliary
scintigraphic examination was concerning for its lack of excretion at 4 hours.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-3: 2-Month-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
The susceptibility of neonates to unconjugated hyperbilirubinemia is favored by
a number of factors, including relative increases in bilirubin production and
enterohepatic circulation along with relative decreases in hepatic uptake and
conjugation. Unconjugated hyperbilirubinemia is a very common occurrence in the
newly born and is usually self-limited and benign. However, if the serum
concentration of bilirubin exceeds 17 mg/dL, the jaundice can no longer be
regarded as physiologic.
Given this predisposition of newborns to an imbalance between bilirubin
generation and hepatic excretory capacity, pathologic or prolonged neonatal
hyperbilirubinemia is often attributable to conditions that exacerbate the
imbalance. For instance, hemolytic diseases (e.g., ABO incompatibility),
polycythemia, and extravascular blood collections (e.g., cephalohematoma,
subgaleal blood, ecchymoses) are conditions that favor increased bilirubin
production. Decreased bilirubin clearance can result from inherited bilirubin
metabolism disorders (e.g., Crigler-Najjar syndrome, Gilbert disease),
hypothyroidism, and circumstances that increase enterohepatic reuptake (e.g.,
breast-feeding, delayed meconium passage). As for older children, Gilbert
syndrome (a genetic disorder of the uridine diphosphoglucuronate
glucuronosyltransferase enzyme system that occurs in about 6% of adults) and
hemolytic anemias are the most common causes of unconjugated bilirubinemia.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-4: 6-Week-Old Girl:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
The differential diagnosis for cholestatic jaundice in the infant is quite
broad, and many excellent and detailed reviews exist. General categories of
disease entities to be considered include
infections, such as hepatitis viruses, TORCH infections, and serious bacterial infections; idiopathic neonatal hepatitis; a long list of metabolic and endocrine diseases, including galactosemia, α1-antitrypsin deficiency, cystic fibrosis, hypothyroidism, hypopituitarism, and
bile acid synthesis defects;
genetic cholestatic syndromes, such as Byler disease; obstructions to bile flow, including biliary atresia, Alagille syndrome, choledochal cysts, and
cholelithiasis; and
iatrogenic causes, such as drug-induced cholestasis or cholestasis related to total parenteral
nutrition.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Jaundice - Case 15-6: 5-Week-Old Girl:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
This infant presented with the following signs: a nonobstructive conjugated
hyperbilirubinemia; renal insufficiency with a mild, non
–anion gap metabolic acidosis; poor weight gain; and a heart murmur. Her liver
biopsy revealed cholestasis and bile duct paucity.
Interlobular bile duct paucity is the characteristic, but not unvarying,
pathologic finding in Alagille syndrome; biopsies performed early in the
disease
's course might simply reveal findings of cholestasis, inflammation, or even
ductal proliferation. In addition, bile duct paucity is sometimes seen in
diseases other than Alagille syndrome. So-called nonsyndromic bile duct paucity
can be a feature of congenital infections (e.g., CMV, rubella, syphilis),
metabolic disorders (e.g.,
α 1-antitrypsin deficiency, defects of bile acid synthesis), sclerosing
cholangitis, and idiopathic cholestasis.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-1: 3-Week-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
This male child presented with pallor at a young age. There was no history of
jaundice, which lessened the likelihood of a hemolytic anemia. There was no
issue of dietary causes because of the baby
's young age. No chronic illness was apparent, and there was no history of blood
loss. Therefore, the focus shifted to a congenital defect in RBC production.
There was a remarkable drop in the hemoglobin and a very poor bone marrow
response as far as reticulocyte production. The WBC and platelet concentrations
were normal. The anemia was macrocytic. The sum of these findings indicated a
defect of RBC production. There was also the physical examination finding of a
possible skeletal anomaly at the distal right femur.
RBC aplasia may be congenital or acquired. Most of the acquired forms occur in
adults, but some may be seen in adolescent patients. In childhood, the major
causes are Diamond-Blackfan anemia, transient erythroblastopenia of childhood,
and acquired aplasia of RBCs associated with chronic hemolysis. The aplastic
crisis of a sickle cell disease patient is an example of the latter.
In this case, there was no evidence of acute or chronic hemolysis. The patient
was too young to be considered for transient erythroblastopenia of childhood.
Another possible cause to be considered was Fanconi anemia, an autosomal
recessive disorder associated with aplastic anemia, short stature, skeletal
defects, pigmentation changes, and other abnormalities. Some cases of Fanconi
anemia are diagnosed in the first year of life. The anemia involves all cell
lines; bone marrow analysis and genetic studies establish the diagnosis. In
this case, there was only RBC involvement, and hence Diamond-Blackfan anemia
was the most plausible diagnosis.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-2: 12-Month-Old Girl:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
Table 10-4 lists the differential diagnosis of microcytic anemia in children.
Causes of iron deficiency include poor bioavailability, decreased iron
absorption, disruption of enteric mucosa or loss of functional bowel, blood
loss, and insufficient intake. Alkaline gastric pH reduces the solubility of
inorganic iron, impeding absorption. Chronic use of acid pump blockers,
vagotomy (for severe gastroesophageal reflux), and impaired gastric parietal
cell function in pernicious anemia may compromise iron absorption. Iron
absorption may also be disrupted after surgical bowel resection, often
performed because of volvulus or intussusception. Iron deficiency in such cases
develops slowly and may not become evident for several years. Blood loss is a
leading cause of iron deficiency. Common causes of gastrointestinal blood loss
in children include Meckel diverticulum, cow
's milk protein allergy, and parasitic infestation. Blood loss from hematuria or
pulmonary hemorrhage can also occur. In this case, the dietary history
suggested a likely cause.
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Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Pallor - Case 10-3: 5-Month-Old Boy:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
This child came to the hospital with significant severe pallor of acute onset.
The child had had no medications or unusual exposures. It was clear that he was
critically ill. Despite the severe illness, the WBC and platelet counts were
normal. The most significant finding was the severe anemia. The increased
unconjugated bilirubin and lactate dehydrogenase levels suggested a hemolytic
process. Other causes of hemolytic anemia were considered, including
drug-associated hemolytic anemia, disorders of RBC membrane and cytostructure
(e.g., hereditary spherocytosis), abnormalities of RBC metabolism (e.g., G6PD
deficiency), as well as sepsis with disseminated intravascular coagulation. The
patient was given antibiotics to cover this last possibility. Parvovirus B19
infection can cause severe anemia but usually as a result of bone marrow
suppression rather than hemolysis.
Patients with a microangiopathic hemolytic anemia (e.g., hemolytic-uremic
syndrome, thrombotic thrombocytopenic purpura) usually have schistocytes rather
than spherocytes on peripheral blood smear. Both of these conditions usually
present with severe thrombocytopenia. A child of this age should be closely
examined for physical abnormalities seen with Diamond- Blackfan syndrome or
Fanconi anemia. Laboratory studies allowed differentiation of the diagnostic
possibilities.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
Rash - Case 9-2: 7-Week-Old Girl:
I. Differential Diagnosis
(Pediatric Complaints and Diagnostic Dilemmas)
Bruising caused clinicians to consider hematologic causes primarily. The initial
workup was done to evaluate for von Willebrand
's disease, which causes decreased platelet adhesiveness, impaired agglutination
of platelets in the presence of ristocetin, and prolonged bleeding time. The
usual presentation is mild to moderate bleeding involving mucous membranes,
including easy bruising, epistaxis, and prolonged bleeding after dental
procedures. In boys, hemophilia (factor VIII and IX deficiency) should be
considered. These children have bruising with a firm or nodular consistency
because of deep soft-tissue bleeding. Vitamin K deficiency can be seen in
patients with fat malabsorption syndromes, and hemorrhagic disease of the
newborn may be seen in those not given vitamin K at delivery. In these infants,
signs and symptoms typically occur within the first few days of life and
include diffuse bruising and, rarely, catastrophic central nervous system
bleeding. However, the timing in this case was not consistent with vitamin K
deficiency. ITP, an acute and self-limited illness that causes bruising and
petechiae 2 to 4 weeks after a minor illness, could be considered. This infant
did not have any preceding illness, and her platelet count was normal. The peak
age for presentation with ITP is 2 to 5 years, and infants who are diagnosed
before 1 year of age have a high likelihood of developing chronic symptoms.
Leukemia was considered less likely on the basis of a normal complete blood
count in the context of significant bruising and bleeding. Anticoagulant
ingestions from medications or commercial rat poison have been seen in older
children and in cases of Munchausen syndrome by proxy, but this child had
normal PT and PTT times, which would not have been the case after ingestion of
anticoagulants.
Dermatologic considerations include Mongolian spots, which are rare in Caucasian
children and do not progress through the color changes indicative of a healing
bruise. These slate-blue patches of skin are commonly seen in pigmented skin.
Phytophotodermatitis is a skin reaction to psoralens (a chemical compound in
citrus fruits such as limes). After contact with psoralens and on exposure to
sunlight, this manifests as red marks that appear as bruises or burns. The
locations of the lesions, as well as the child
's age and lack of contact with psoralens, made such a diagnosis unlikely.
Hemangioma was considered. Unlike this child
's lesions, hemangiomas undergo a typical growth pattern of rapid growth for the
first 6 months of life, then a slowing of growth until 3 years. This child
's lesions resolved and then new ones appeared. Approximately 85% of hemangiomas
spontaneously involute or partially regress, but not until later childhood.
Collagen vascular diseases should be considered. Ehlers-Danlos syndrome (EDS) is
a congenital defect in collagen synthesis that may lead to easy bruising. Many
forms have been identified that involve a variety of basic defects and
inheritance patterns. This child did not display the clinical triad seen in
these patients: skin hyperextensibility, joint hypermobility, and skin
fragility. Osteogenesis imperfecta is a congenital abnormality of quality or
quantity of type I collagen synthesis. Of the four subtypes, type I is
associated with easy bruising and fractures as seen in this child, but this
child did not display other signs, such as blue sclera, hearing impairment,
osteopenia, bony deformities, and excessive laxity of joints. Should a question
have persisted, a punch biopsy of skin for analysis of collagen synthesis would
confirm the diagnosis. Infectious causes were unlikely given the timing of the
child
's lesions. Child abuse remains the most alarming cause of unexplained bruising
in children.
» READ BOOK EXCERPT ONLINE »
Source: Pediatric Complaints and Diagnostic Dilemmas, 2003
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