A 64-year-old Japanese woman had been in good health before she developed intermittent fever (up to 38.5 ° C), cervical lymphadenopathy, myalgia of the thighs, and polyarthralgia in association with evanescent macular erythema on the dorsa hands. Within a week the constitutional symptoms progressively aggravated, the intermittent fever rose to 40 ° C, and she became lethargic. The erythema became persistent and widespread, and coalesced into a diffuse edematous plaque lesion that involved the face, limbs, and trunk. The erythema was most extensive on the back, and was of linear distribution ( Fig. 1a). The mucous membrane was not affected. Skin biopsy specimens showed a moderate degree of mononuclear cell infiltration with a few neutrophils around dilated blood vessels in the edematous upper-mid-dermis (not shown). The epidermis was intact and there was no evidence of vasculitis. Laboratory examinations revealed neutrophilic leukocytosis (25,600/ µ L, 81% neutrophil with a left shit), an increase in the serum CRP and LDH levels (26.5 mg /mL and 1927 IU/mL, respectively; LDH normal range: 200 -450 IU/ mL), and hyperferritinaemia (42,870 ng /mL; normal range: 10-100 ng /mL). Radiological examinations denied lymphoproliferative disorders. Serological tests for collagen vascular diseases and those for various viral diseases were all negative. The results of repeated blood, urine, and throat cultures were not diagnostic, and systemic antibiotics or discontinuation of all medications did not improve the symptoms or the abnormal laboratory data. Adult-onset Still's disease (AOSD) was suspected, and all the constitutional symptoms and the abnormal laboratory data improved in response to the systemic administration of methylprednisolone (1000 mg / day for 3 days). The skin rash disappeared without any sequels. Figure 1 (a) Coalescent linear erythema on the back in Case 1. (b) Coalescent macular erythema on the forearm in Case 2
Histamine released from dermal mast cells plays a central role in the increased vascular permeability in acute urticaria, and administration of anti-histamines usually suppresses development of wheals. Acute idiopathic urticaria, particularly a severe case, occasionally presents with acute inflammatory reactions such as low-grade fever and leukocytosis and is resistant to anti-histamines. Considering the wide spectrum of proinflammatory cytokines and chemokines that can be released from activated mast cells, some of them might be involved in the pathogenesis of urticaria. We measured plasma levels of interleukin-6 (IL-6), interleukin-8 (IL-8), and tumor necrosis factor-alpha (TNF-alpha) in 16 cases of severe acute urticaria. None of them showed elevated plasma levels of IL-8 or TNF-alpha. Nine out of 16 acute urticaria patients showed elevated circulating IL-6 with concomitant increases in serum CRP levels. All such patients were resistant to conventional anti-histamine treatment and required systemic steroids for complete suppression of wheal development. After subsidence of the urticaria, their elevated IL-6 and CRP levels dropped to their normal ranges. In contrast, all but one patient without elevated circulating IL-6 was successfully treated with a H1 receptor antagonist, cetirizine. The data suggest involvement of IL-6 in the pathogenesis of severe acute urticaria that is resistant to anti-histamines.
Prurigo chronica multiformis is an intensely pruritic, chronic cutaneous disorder of unknown etiology without any effective treatment. This is a report on the results of using etretinate therapy to treat prurigo chronica multiformis. Thirty-seven patients (average age; 69.1+/-11.5 year-old) were treated with 30 mg/day etretinate along with topical steroids (very strong classes) and oral antihistamines. Etretinate was discontinued as soon as remission was achieved. Thirty-six patients were followed up for at least four weeks. The number of patients who achieved remission increased progressively after the initiation of etretinate therapy; 18 patients were totally and 14 were partially free of active skin lesions within four weeks. Eventually, 27 patients achieved remission with an average duration of 4.4+/-3.1 weeks etretinate treatment (range; 1-14 weeks), and five achieved partial remission. Four patients discontinued etretinate within two weeks because of the absence of clinical response (two cases) or exacerbation of the skin lesion (two cases). Among the 27 patients who had achieved remission, 23 had recurrence after the cessation of etretinate. The remission period ranged from 1 to 32 weeks with an average duration of 5.7+/-6.7 weeks. Combined treatment with topical steroids and oral antihistamines did not achieve remission in the recurrent cases but re-administration of 30 mg/day etretinate did. Our observation suggests that a moderate dose of etretinate is a safe and effective therapy for prurigo chronica multiformis, which is often resistant to conventional treatment using topical steroids and oral antihistamines.
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