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Severe panniculitis PDF Print E-mail
AIBS teaching cases
Thursday, 31 March 2005

A 36-year-old woman was admitted to the hospital because of fever and abdominal pain of one-week duration. Fever was up to 39 degrees Celsius and was accompanied by chills. The pain was located in the right lower quadrant of the abdomen. She did not complain for other symptoms.

The patient was married to a shipman and had five healthy children, three of whom were delivered by cesarean section. Her past medical history included recurrent cellulitis-like episodes after a glass related injury, which was located in her left forearm.

Physical examination showed elevated temperature (38,8 degrees Celsius). The pulse rate was 90/min and the blood pressure 120/70 mmHg. There were three areas (2X2 cm), on her left forearm, with atrophy and hyperpigmentation. Inflammation was obvious on the right lower quadrant of the abdominal wall, where swelling, tenderness, heat and redness were found in an area of 25X15 cm. Liver, spleen and peripheral lymph nodes were not palpable. The rest of the physical examination did not show any abnormal findings.

Laboratory work-up revealed: Ht=36%, Hb=12,9 g/dl, WBC=9980/mm3 (neutrophils=75%, lymphocytes=18%, monocytes=6%, eosinophils=1%), platelets=210,000/mm3, erythrocyte sedimentation rate (ESR) = 57mm/first hour. The level of C-reactive protein was normal. The biochemical (glucose, renal and liver function, electrolytes, lipid profile, uric acid, amylase, lactate dehydrogenase, creatine kinase, and proteins) and immunological tests of the serum (rheumatoid factor test, antinuclear antibodies, antimitochondrial antibodies, anti-smooth muscle antibodies, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibodies, serum angioconverting enzyme and complement), were normal, except of the slight elevation of IgM, slight reduction of IgA and low value of C4. Urinalysis did not show abnormal findings. Chest X-rays, ECG, U/S of the abdomen and C/T of the thorax, upper and lower abdomen were normal.

 

Diagnosis
The patient was initially managed with intravenous ticarcillin-clavulanic acid plus clindamycin but there was no improvement. In the meanwhile, she developed new lesions, identical to the initial ones, on the right lower and the left upper quadrants of the abdominal wall. Thus, she underwent an operation for diagnostic and therapeutic purposes. Histological examination of the lesions showed acute panniculitis with hemorrhagic infiltration of the subcutaneous fat. Methylprednisolone intravenously, cyclosporine per os and intravenous immunoglobulin therapy (considering the remote possibility of an autoimmune disease) were given to the patient after the recommendations of variant consultants.

Facing a major diagnostic problem in a patient with no apparent or occult reason for acute panniculitis, we thought of the possibility of a disorder of factitious origin. Therefore, we watched her closely and soon we had valuable information. First, the patient was observed to move her arms in a strange way that did not remind her gestures during her temperature measurements. Specifically, she only moved the arm ipsilateral to the axillary fossa where the thermometer was placed. Second, we investigated a complaint about "shortness of breath during immunoglobulin therapy" reported by her. This "side- effect", which caused a lot of anxiety and concern to the nursing staff, was reproduced in a dramatic way when she took the solvent without the immunoglobulin powder.

At last, our patient had a diagnosis, i.e. Munchausen syndrome, but she deliberately stopped her hospitalization without the recommended follow up. The exact mechanism of production panniculitis was not clarified. Two years later, she was again admitted to our hospital, in order to remove needles, which apparently she herself had put under her skin in the forearm. The patient denied initially any trauma in the area but when she was told about the operative findings she "remembered" an injury several months prior to her last admission to the hospital.

Teaching points
Although panniculitis is rarely reported in the literature as a feature of factitious disorders, it is still one of its presentations. The diagnosis of this disorder is handicapped by the long time required not only to exclude organic disease but also get necessary evidence to prove the diagnosis of Munchausen's syndrome. The prevalence of factitious disorders is unknown because they mimic a variety of syndromes, the patients are usually of high intelligence quotient and the doctor is always afraid of missing a disease of organic origin.

In 1951 the term Munchausen's syndrome was established to describe patients with factitious disorders and exclusively physical symptoms. These patients have experience in causing body injury, produce a wide variety of symptoms and signs and have hospital and polysurgical addiction. Their prognosis is worse comparing to those with only psychiatric symptoms. Munchausen's syndrome by proxy is a particular form of the syndrome; the target is another person-and the maltreatment usually takes place from mother to child or from health care workers to patients. The syndrome mimics an amazing variety of illnesses. Fever, abdominal pain, hemoptysis, hypoglycemia, bacteremia, even systemic lupus erythematosus and sickle-cell crisis are reported to the literature and the invented mechanisms are impressive. For example, patients produced bacteremia by injecting themselves contaminated materials, including stool.

Despite the fact that the Munchausen's syndrome is always a possibility, a scrutinizing clinical and laboratory examination is usually necessary in order to exclude any possible existing cause of the disease. In our patient, a very careful examination was performed which included even surgical procedures (was it what the patient wanted?).

Until recently, extensive and severe fat necrosis was synonymous to Weber Christian disease, but that term tends to be abandoned, since acute panniculitis is included in the clinical spectrum of different syndromes and should always be an indication for further evaluation. Thus, after histological confirmation of acute panniculitis, our patient underwent a complete checkout for a variety of local or systematic causes associated with fat necrosis. We excluded trauma, cold, drugs and lipodystrophy and we performed tests for autoimmune, collagen and vascular diseases, such as systemic lupus erythematosus and scleroderma, lymphoproliferative disorders, a1 antithrypsin deficiency, pancreatic disease, bacterial or virus infections, and protein and complement abnormalities. Cesarean section is not considered to be a predisposing factor for fat necrosis. Our major diagnostic problem was enlightened when we seriously suspected the factitious origin of the syndrome. The case completed the criteria and took the name of Munchausen's syndrome. The diagnosis was confirmed two years later when she was again hospitalised for surgical removal of self-inserted needles.

Skin, mucosa and subcutaneous tissue are the most easily approachable areas for the patients who want to harm themselves. This is concluded by reviewing the literature, where artifact dermatitis and acute panniculitis by skin stigmatization are frequently reported, especially in young females.

Impressively, patients have appeared with a great variety of symptoms and signs, extending from pyoderma gangrenosum, to recurrent herpes zoster. Only a few diagnostic clues can help the physician to escape from the deceit. For example, lesions sparing certain areas of the body such as tattoos could hide a factitious disorder. Extensive hemorrhagic subcutaneous infiltration seen in the microscope may imply hypodermatitis due to deliberate trauma. The way in which our patient created her disease remained unknown. Local trauma, cold, drugs and injections containing toxic or allergic materials such as collagen and silicone, may produce local or systemic panniculitis.

The approach of Munchausen's syndrome is not easy. Differential diagnosis starts from the initial symptom, and then spreads to a cycle of expensive and time consuming examinations, often hazardous for a patient who in fact is undiagnosed. The doctor is troubled despite the detailed exploration and he or she continues to be troubled even when the syndrome is suspected because he or she deals with a difficult patient, familiar with medicine, who is constantly protesting about lack of diagnosis but is eventually satisfied by hospitalization here and there. Physicians must move near the patient gently, patiently and persistently in order not to lose the diagnosis of the syndrome.

References
1. Falagas ME, Vergidis PI. Diseases that masquerade as infectious cellulitis. Annals of Internal Medicine 2005;142:47-55

Acknowledgements
1. This case was prepared for our website by Dr. Evangelos Rosmarakis.
2. A modified version of this case was published in the International Journal of Clinical Practice.

 
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