It's Not So Sweet
It's Not So Sweet
Sixteen-year-old Lucy initially was seen in mid October with symptoms of fatigue, anorexia, and upper respiratory symptoms including a sore throat. Results of laboratory tests, including a complete blood cell count (CBC), were unremarkable, and a mononucleosis screen and throat culture were negative. She returned 2 weeks later and had been in bed since she was last seen with increasing symptoms of fever, headache, total body aches, and 5 days of dysphagia. She had noted a lesion on her labia that day. She denied urinary frequency, urgency, and dysuria and stated unequivocally that she was not sexually active. Lucy has been an excellent student and normally is very active in school sports and extracurricular activities. On examination she appeared to be ill, was adequately hydrated, and was significantly fatigued. Her examination was within normal limits with the exception of shotty bilateral anterior cervical and inguinal nodes and a small, approximately one-half centimeter, eroded lesion on the left labia majora. A mononucleosis screen and bacterial and viral cultures of the labial lesions were negative. A CBC, Epstein-Barr virus screen, and immunoglobulin G and A were within reference range. Her bacterial culture of the labial lesion was negative for any growth.
Her medical history was noncontributory. She was taking no regular medications and had no known drug allergies. She lives with both parents and a sister and is a junior at a private high school. Her family history reveals no history of rheumatologic disease, and her grandmother had breast cancer.
Her progress was followed closely by telephone contact, and her symptoms cleared after approximately 5 weeks. She did well for about 3 weeks; then a fever up to 104°F developed, along with night sweats, myalgias, arthralgias, and multiple blister-type lesions on her labia. She had no oral ulcers.
She was hospitalized and underwent an extensive evaluation, including computed tomography (CT) scans of her abdomen and pelvis, which were normal. Her rheumatologic workup included a normal antinuclear antibody, rheumatoid factor, and Complement 3 and 4. A colonoscopy showed no sign of inflammatory bowel disease. Her labial lesions were biopsied and found to be consistent with Sweet's syndrome or acute febrile neutrophilic dermatosis, a skin disorder characterized by fever and painful skin lesions that occurs most frequently in women 30 to 50 years of age (Sweet's Syndrome, 2006). Lucy was treated with a tapering dose of steroids and did well. All her symptoms resolved following her steroid course.
Two weeks later a cough and sore throat developed, which was thought by her primary care provider to be due to influenza, even though an influenza test was negative. Tamiflu was prescribed. She began having fevers and night sweats and a new labial lesion developed, which was extremely painful. She described her throat as hurting very low in her neck, feeling different than most sore throats. She again was admitted to the hospital because of concerns that the Sweet's syndrome could be a marker of lymphoma. A pediatric oncology consult was done.
Her admission examination showed some weight loss, a 1-cm erosive lesion at the base of the vulva, and a small punctate lesion on the right mid labia. Results of her examination were otherwise normal. Findings of laboratory tests included a normal CBC and differential, an erythrocyte sedimentation rate of 6 mm/hour, and an elevated C-reactive protein of 4.1 mg/L. CT scans of her neck, chest, abdomen, and pelvis were done to rule out adenopathy that might be associated with lymphoma and showed a subglottic soft tissue density compatible with secretions, a right upper lobe infiltrate, and a small amount of free fluid in the right pelvis. After admission, Cefotaxime and subsequently Indomethacin were prescribed, and she had marked improvement, with regression of her labial lesions and improved appetite.
A rheumatology consult was obtained, and the diagnosis remained Sweet's syndrome/acute febrile neutrophilic dermatosis. She had no occult malignancy associated with Sweet's syndrome, nor did she have any other rheumatologic disorders such as inflammatory bowel disease, systemic lupus erythematosus, Sjögren's syndrome, or rheumatoid arthritis. Lucy was referred to a pediatric rheumatologist at a tertiary children's hospital where her diagnosis was changed.
Sixteen-year-old Lucy initially was seen in mid October with symptoms of fatigue, anorexia, and upper respiratory symptoms including a sore throat. Results of laboratory tests, including a complete blood cell count (CBC), were unremarkable, and a mononucleosis screen and throat culture were negative. She returned 2 weeks later and had been in bed since she was last seen with increasing symptoms of fever, headache, total body aches, and 5 days of dysphagia. She had noted a lesion on her labia that day. She denied urinary frequency, urgency, and dysuria and stated unequivocally that she was not sexually active. Lucy has been an excellent student and normally is very active in school sports and extracurricular activities. On examination she appeared to be ill, was adequately hydrated, and was significantly fatigued. Her examination was within normal limits with the exception of shotty bilateral anterior cervical and inguinal nodes and a small, approximately one-half centimeter, eroded lesion on the left labia majora. A mononucleosis screen and bacterial and viral cultures of the labial lesions were negative. A CBC, Epstein-Barr virus screen, and immunoglobulin G and A were within reference range. Her bacterial culture of the labial lesion was negative for any growth.
Her medical history was noncontributory. She was taking no regular medications and had no known drug allergies. She lives with both parents and a sister and is a junior at a private high school. Her family history reveals no history of rheumatologic disease, and her grandmother had breast cancer.
Her progress was followed closely by telephone contact, and her symptoms cleared after approximately 5 weeks. She did well for about 3 weeks; then a fever up to 104°F developed, along with night sweats, myalgias, arthralgias, and multiple blister-type lesions on her labia. She had no oral ulcers.
She was hospitalized and underwent an extensive evaluation, including computed tomography (CT) scans of her abdomen and pelvis, which were normal. Her rheumatologic workup included a normal antinuclear antibody, rheumatoid factor, and Complement 3 and 4. A colonoscopy showed no sign of inflammatory bowel disease. Her labial lesions were biopsied and found to be consistent with Sweet's syndrome or acute febrile neutrophilic dermatosis, a skin disorder characterized by fever and painful skin lesions that occurs most frequently in women 30 to 50 years of age (Sweet's Syndrome, 2006). Lucy was treated with a tapering dose of steroids and did well. All her symptoms resolved following her steroid course.
Two weeks later a cough and sore throat developed, which was thought by her primary care provider to be due to influenza, even though an influenza test was negative. Tamiflu was prescribed. She began having fevers and night sweats and a new labial lesion developed, which was extremely painful. She described her throat as hurting very low in her neck, feeling different than most sore throats. She again was admitted to the hospital because of concerns that the Sweet's syndrome could be a marker of lymphoma. A pediatric oncology consult was done.
Her admission examination showed some weight loss, a 1-cm erosive lesion at the base of the vulva, and a small punctate lesion on the right mid labia. Results of her examination were otherwise normal. Findings of laboratory tests included a normal CBC and differential, an erythrocyte sedimentation rate of 6 mm/hour, and an elevated C-reactive protein of 4.1 mg/L. CT scans of her neck, chest, abdomen, and pelvis were done to rule out adenopathy that might be associated with lymphoma and showed a subglottic soft tissue density compatible with secretions, a right upper lobe infiltrate, and a small amount of free fluid in the right pelvis. After admission, Cefotaxime and subsequently Indomethacin were prescribed, and she had marked improvement, with regression of her labial lesions and improved appetite.
A rheumatology consult was obtained, and the diagnosis remained Sweet's syndrome/acute febrile neutrophilic dermatosis. She had no occult malignancy associated with Sweet's syndrome, nor did she have any other rheumatologic disorders such as inflammatory bowel disease, systemic lupus erythematosus, Sjögren's syndrome, or rheumatoid arthritis. Lucy was referred to a pediatric rheumatologist at a tertiary children's hospital where her diagnosis was changed.
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