Тема: Index of Suspicion
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Старый 09.10.2005, 08:25
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Case 12 (см. также Case 9)

A 17-year-old Caucasian girl is admitted to the hospital because of pain, swelling, and redness of her left leg for 6 months, with occasional low-grade fevers.

Previously, cellulitis had been diagnosed and antistaphylococcal antibiotics prescribed on more than one occasion, with temporary improvement. Cultures of the cellulitis had yielded Staphylococcus aureus sensitive to all antistaphylococcal antibiotics. CBC, ESR, liver and renal function tests, calcium, phosphorous, antinuclear antibody (ANA), rheumatoid factor (RF), and blood cultures resulted in normal or negative findings. Radiographs of the pelvis, hip, femur, knee, tibia, fibula, ankle, and lumbosacral spine, as well as a venogram and bone scan, were interpreted as normal. Arthrocentesis yielded normal results. Additionally, she had been admitted repeatedly for strict bed rest, elevation of the extremity, and intravenous antibiotics, with no sustained resolution of swelling, redness, or pain.

She had been traveling to various islands of the western Pacific as a member of a soccer team prior to the onset of her symptoms.

Physical findings today are normal, with the exception of redness, pain, and warmth over the left calf and anterior portion of the lower left leg, with pitting edema involving the calf, pretibial region, ankle, foot, and knee area, although results of the knee examination are normal. Pulses and strength are normal.
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This patient had experienced numerous episodes of disease in her left lower leg that involved variable degrees of swelling, erythema, warmth, and pain. Her clinical picture suggested both inflammation of unknown cause and secondary infection. To complicate the picture, she demonstrated features of chondromalacia of the left patella, which the clinicians thought might be related to the lower extremity disease, perhaps through infection. The differential diagnosis included cellulitis, septic arthritis, osteomyelitis, thrombophlebitis, collagen vascular disease, necrotizing fasciitis, pyomyositis, compartment syndrome, immunodeficiency, venomous insect or snake bite, and lymphedema.

By the time of admission to this hospital, an extensive evaluation had been completed, eliminating many of the diagnoses on the list, including septic arthritis (negative arthrocentesis), chronic osteomyelitis (negative radiograph, bone scan, and blood culture), thrombophlebitis (negative venogram), and collagen vascular disease (negative RF and ANA and normal ESR).

With necrotizing fasciitis, progression should be more acute. Signs and symptoms of pyomyositis should progress and by 6 months would require surgical management for resolution. With compartment syndrome, there should be a history of pain on exercising. Immunodeficiency is unlikely given the onset of symptoms at age 17 years and absence of risk factors for human immunodeficiency virus. There was no history of snake or insect bite.

One other important diagnostic consideration is lymphedema. This finding can occur in lymphedema praecox (Meige disease), which is a primary lymphedema with onset in adolescence and usually is a diagnosis of exclusion. In addition, several secondary causes of lymphedema include tumors; postradiation fibrosis; retroperitoneal fibrosis; infection (including filariasis); and postinflammatory scarring from trauma, surgery, or burns. Lymphoscintigraphy can be used to differentiate primary from secondary lymphedema.

In this patient, filariasis was an intriguing possibility given her travel history. Filariasis is a roundworm infestation that can lead to massive lymphedema of the legs and genitalia. Diagnosis is made by reviewing blood smears for microfilariae or by antigen immunoassay. In this case, blood smears were negative.

The Condition
This patient’s diagnostic dilemma was solved dramatically one day on rounds when she was discovered to be applying a constrictive tourniquet around her leg just above the knee. When she was confronted, she became withdrawn and denied she was doing anything. With one-to-one nursing supervision to ensure no further placement of the tourniquet, the redness and swelling resolved rapidly. A psychiatrist was consulted, and factitious illness was diagnosed.

This unusual presentation is a striking example of how physicians can be deceived by patients who have factitious disorders and can be compelled into performing extensive and invasive diagnostic procedures. In retrospect, one diagnostic clue could have been a zone of demarcation between the normal and edematous areas caused by the tourniquet. Careful re-examination after the true cause had been revealed did show that finding, which had not been evident on previous examinations.

Psychosomatic illnesses have a wide spectrum of presentations and are common in adolescents. Frequently, they are manifestations of underlying psychological disease, but they also can be seen in healthy adolescents during stressful situations. Examples include adjustment disorders, somatization disorders, conversion disorders, hypochondriasis, malingering, and factitious disorder.

According to the American Psychiatric Association Diagnostic and Statistical Manual of Mental Disorders, Edition 4 (DSM IV), adjustment disorders relate to a difficult adjustment to a situation that is out of proportion to the circumstances. In these disorders, stress can lead to physical symptoms. Examples include tension headaches, palpitations, neurodermatitis, and tremor.

In somatization disorders such as conversion disorder and hypochondriasis, a variety of symptoms can present vaguely and imprecisely over a period of several years. There must be a significant impairment in functioning or a history of no explanation of symptoms after an appropriate assessment. The patient often goes to many different physicians and frequently has underlying anxiety and depression.

Conversion disorder is characterized by neurologic or other somatic symptoms that do not fit biologic explanations. The patient benefits from these symptoms by avoiding stressful conflict (primary gain) and evokes attention and sympathy for being stricken with often dramatic symptoms (secondary gain). For patients who have hypochondriasis, there is an exaggerated preoccupation or belief that they have a serious disease.

There are three mental health disorders in which an individual intentionally produces symptoms: malingering, factitious illness, and factitious illness by proxy. In malingering, the patient voluntarily, deliberately, and purposefully produces his or her symptoms to achieve some goal, such as gaining insurance money, avoiding military service, obtaining drugs, or avoiding work.

In factitious illness, or M?nchhausen syndrome, the patient produces signs or symptoms to assume the sick role. Examples of clinical presentations include factitious fever, induced infections, and hypoglycemia caused by exogenous insulin injections. There was one reported case of a tourniquet placed around the wrist, which mimicked reflex sympathetic dystrophy. "M?nchhausen syndrome by proxy" is a term used when a caregiver induces signs or symptoms in a child.

Patients who have factitious illness often have a vague and inconsistent history. When questioned in detail, they frequently have an extensive knowledge of diseases and may have a medical background. Patients commonly become hostile about not being diagnosed correctly and demand and request medications and procedures that are medically unnecessary. The cause of factitious illness is unknown. The diagnosis requires a high degree of suspicion and often is difficult to prove. The patient frequently uses the symptoms to gain attention and recognition. Prolonged psychotherapy is recommended.

Lessons for the Clinician
This case reminds clinicians always to consider the possibility of factitious illness in patients who present with atypical clinical manifestations. Careful observation of the patient in the hospital, including video surveillance, may be necessary to confirm the diagnosis, although clinicians contemplating video surveillance should consult the hospital attorney and risk management office to be sure they are acting legally. Once the diagnosis is considered, clinicians should avoid harmful invasive procedures or treatment with potentially toxic medications.


James J. Burns, MD
Baystate Medical Center, Springfield, Mass

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Light одобрил(а): Очень поучительно. В "загадочных" случаях всегда надо помнить о возможности такого варианта.
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