An unusual case of DRESS syndrome.

BACKGROUND: DRESS syndrome (drug reaction with eosinophilia and systemic symptoms) is a rare but severe drug reaction. OBSERVATIONS: A 27-year-old male with paranoid schizophrenia was hospitalized with all three diagnostic criteria of DRESS syndrome: cutaneous drug eruption, hematological abnormalities (presence of atypical lymphocytes on blood smear) and systemic involvement (generalized lymphadenopathy and hepatitis). On hospitalization the patient exhibited an unusual fever pattern of high temperatures in the morning hours and lower temperatures towards evening. In this case of a patient who needs life-long therapy, we demonstrated the value of the IFN-gamma release test, which showed positive reactivity to 3 out of 9 suspicious drugs: paracetamol, phenytoin and dypirone, allowing for more therapeutic options. After therapy, at 6-month follow-up the patient is doing well under haloperidol treatment, laboratory values including liver function tests are normal and his skin condition is good. CONCLUSION: We suggest that clinicians take the fever pattern of high temperatures in the morning hours and lower temperatures towards evening into account in a patient presenting with a severe cutaneous drug eruption. An interferone-gamma release test may facilitate identification of drugs responsible for the drug reaction.

Bedside neuropathy disability score compared to quantitative sensory testing for measurement of diabetic neuropathy in children, adolescents, and young adults with type 1 diabetes.

BACKGROUND: While regular yearly screening for diabetic retinopathy and nephropathy is well established in patients with diabetes mellitus, there are no standardized diagnostic tests for diabetic peripheral neuropathy (DPN). In the present study, we compared the bedside neuropathy disability score (NDS) with quantitative sensory testing (QST) for screening for DPN in youth with type 1 diabetes mellitus. METHODS: One hundred sixty-six patients aged 10 to 34 years (median 21 years) were evaluated for DPN by the NDS and QST. Quantitative sensory testing was also done in 43 healthy, age-matched controls. Diabetic peripheral neuropathy grade by both methods was correlated with disease-related variables. RESULTS: On QST, the diabetic group had significantly higher mean scores for vibration (P<.001) and warm sensation (P<.01) than controls, and lower scores for cold sensation (P<.05); however, there was a great degree of overlap. The NDS significantly correlated with the vibration threshold, but not with the warm and cold thresholds. The NDS significantly correlated with age at testing, diabetes duration, and long-term and current HbA1c levels (P<.001), and with the presence of microalbuminuria and diabetic retinopathy (P<.001). Analysis of the QST variables yielded significant correlations of vibration and warm sensation with age at testing (P<.001, P<.05, respectively) and of vibration with diabetes duration (P<.001) and retinopathy (P=.05); none of the quantitative tests correlated with glycemic control. CONCLUSIONS: The stronger association of the NDS with glycemic control and other microvascular complications compared to the perception thresholds, and its shorter time of performance and lack of costly equipment, may make the NDS the preferred method for measuring DPN in this population.

Ichthyosiform mycosis fungoides: an atypical variant of cutaneous T-cell lymphoma.

BACKGROUND: Acquired ichthyosis is a known paraneoplastic sign of lymphoproliferative malignancies, with histopathologic findings that are nonspecific, revealing no insinuation of the underlying neoplasm. Ichthyosiform eruption as a specific manifestation of mycosis fungoides (MF), ie, ichthyosiform MF, is, however, regarded as rare and to date has been reported in only a few cases. OBJECTIVE: We sought to study the clinical, histopathologic, immunohistochemical, and genotypic features of patients with ichthyosiform MF. METHODS: The files of patients with MF seen during the past 8 years in our department were reviewed to search for cases of ichthyosis-like MF. RESULTS: Seven patients, comprising 3.5% of the patients seen with MF, had an ichthyosiform eruption with histopathologic features characteristic of early MF. In 2 patients it was the sole manifestation of the disease and in 5 patients it appeared either in conjunction with conventional patches and/or plaques or with follicular lesions. Immunohistochemically, all showed a predominance of CD3+ CD4+, except for 1 patient in whom the epidermotropic T cells were predominantly CD8+. In 3 of the 7 patients clonality could be demonstrated by polymerase chain reaction. None had extracutaneous involvement. All had an indolent course of the disease and responded well to skin-targeted therapies. CONCLUSIONS: Ichthyosiform MF is yet another atypical clinical variant of cutaneous T-cell lymphoma that is not as rare as reflected in the literature. It may be the sole manifestation of the disease but also may appear in conjunction with conventional or follicular MF lesions.