Longitudinal analysis of antibody profiles against plakins in severe drug eruptions: emphasis on correlation with tissue damage in drug-induced hypersensitivity syndrome and drug reaction with eosinophilia and systemic symptoms.

BACKGROUND: The evidence for severe drug eruption as a trigger for autoimmune disease has recently increased. No information is available on how tissue damage in severe drug eruptions can induce autoimmune responses. OBJECTIVES: To investigate whether the generation of autoantibodies (autoAbs) against plakin family proteins could be the cause or result of tissue damage in patients with severe drug eruptions and whether the generation of autoAbs could be prevented by systemic corticosteroids during the acute stage. METHODS: We retrospectively analysed alterations of serum levels of autoAbs against plakin family proteins in patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) and drug-induced hypersensitivity syndrome (DiHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) during the acute stage and long after resolution over a period of more than 10 years. RESULTS: AutoAbs against plakin family proteins were detected in patients with either SJS/TEN or DiHS/DRESS regardless of the epidermal damage in the acute stage, and were sustained even long after resolution in DiHS/DRESS, indicating that those autoAbs are neither the cause nor the consequence of epidermal damage, at least in DiHS/DRESS. Severe liver damage and noncorticosteroid therapy during the early and acute stages of DiHS/DRESS were associated with the subsequent generation of these autoAbs. CONCLUSIONS: These autoAbs are neither necessarily the cause nor the result of epidermal damage in DiHS/DRESS, because the presence of these autoAbs was not restricted to patients with SJS/TEN but was also observed in those with DiHS/DRESS, which is characterized by lack of epidermal damage. Severe liver damage and/or immune responses that could be prevented by corticosteroids in the acute stage of DiHS/DRESS are among the causal factors contributing to the generation of autoimmune responses.

Bone atrophy in complex regional pain syndrome patients measured by microdensitometry.

PURPOSE: To determine the usefulness of quantitative measurement of bone atrophy in the diagnosis and the long-term follow-up of patients with complex regional pain syndrome (CRPS). The bone-sparing effect of a 5-hydroxytriptamine (5-HT2) antagonist was also studied. METHODS: Bone mass was measured by computerized micro-densitometry at the middle position of the second metacarpal. The effect of repeated stellate ganglion blocks (SGBs) three times per week with mepivacaine (n = 11), administration of a 5-HT2 antagonist (sarpogrelate hydrochloride, 300 mg a day po) (n = 12), and combined therapy (n = 10) were compared by micro-densitometry and conventional visual analogue scale (VAS) for analgesia after three months of treatment. RESULTS: In CRPS patients, metacarpal index (cortical bone thickness), maximum bone density (cortical bone density), minimum bone density (trabecular bone density), and average bone density were reduced on the affected side (14.1%, 12.1%, 25.0% and 19.3% respectively). The rate of reduction in bone mass correlated with the duration of the disease (P < 0.05). Therapy with the 5-HT2 receptor antagonist (with or without repeated SGBs) decreased pain intensity (from 6.10 to 3.81 with SGB, from 6.30 to 2.91 without SGB, respectively; P < 0.01) and bone atrophy evaluated by micro-densitometry (P < 0.05). In contrast, repeated SGBs alone reduced pain intensity (from 6.30 to 2.91; P < 0.01) but did not ameliorate bone atrophy. CONCLUSION: Bone micro-densitometry is useful in the assessment and follow-up of CRPS and for evaluation of treatment. The 5-HT2 antagonist, sarpogrelate hydrochloride, is a promising treatment for CRPS patients.

[A case report of postoperative thyroid crisis accompanied with struma ovarii].

A 39-year-old female without any specific past history was scheduled to receive an operation for myoma uteri and ovarian cyst. She was premedicated with atropine. Anesthesia was induced with thiopental and was maintained with nitrous oxide and enflurane. Tachycardia shortly after premedication with atropine and remarkable sweat during the operation were observed. On the 1st postoperative day an outbreak of thyroid crisis as well tachycardia of 180.min-1 and fever (39.3 degrees C) were observed. Such outbreak of thyroid crisis indicated that the patient had been suffering from Grave disease. Pathological diagnosis of extirpated ovarian cyst was struma ovarii. It is, however, still uncertain whether struma ovarii induced thyroid crisis in this case. It might be concluded that screening of hyperthyroidism at preoperative rounds is essential for prevention of thyroid crisis.