泛发性脓疱性药疹：通过体外试验确诊

Lazarov A, Livni E, Halevy S

Dermatology Clinic, Sapir Medical Center, Meir General Hospital, Kfar Saba, Israel.

J Eur Acad Dermatol Venereol. 1998 Jan;10(1):36-41.

BACKGROUND

Generalized pustular eruptions are characterized by acute onset of aseptic pustules in febrile patients with leukocytosis after exposure to the offending drug. They have been regarded as uncommon manifestations of adverse drug reactions. Until now few confirmation studies have been carried out.

OBJECTIVE

Our purpose was to describe a series of patients with generalized pustular drug eruption. The diagnosis and etiological role of drugs in these cases was confirmed by two in vitro tests, namely the macrophage migration inhibitory factor (MIF) and the mast cell degranulation (MCD) tests.

METHODS

The clinical, pathological and laboratory findings in six patients with generalized pustular eruption were studied prospectively. The MIF and MCD tests were performed with the drugs taken by the patients.

RESULTS

The dermatological manifestations included numerous pustules on large erythematous areas, papules and erythema multiforme-like lesions. The pustular eruption developed between 12 h and 5 days after the administration of the provoking drug. The histopathological changes were characterized by subcorneal pustules, papillary edema and mixed cell inflammatory infiltrate. In two biopsies the infiltrate displayed numerous eosinophils. Laboratory investigations revealed leukocytosis with neutrophilia (4 of 6 patients) and eosinophilia and hyperimmunoglobulinemia E (2 of 6 patients). MCD tests with the suspected drugs (Ampicillin, Cefazolin, Tetracycline, Griseofulvin, Enalapril Maleate) were positive in all patients. Positive MIF results were seen in five of the six patients. Withdrawal of the drug led to fast recovery.

CONCLUSION

The diagnosis of pustular drug eruptions depends on circumstantial evidence, some characteristic clinicopathological findings and exclusion of alternative diagnoses of other disseminated pustular eruptions. In vitro tests, namely MIF and MCD tests, are a useful diagnostic aid in the identification of the offending drugs.

背景

泛发性脓疱疹的特征为发热且白细胞增多的患者在接触致病药物后急性出现无菌性脓疱。它们一直被视为药物不良反应的罕见表现。迄今为止，很少有验证性研究。

目的

我们的目的是描述一系列泛发性脓疱性药疹患者。通过两项体外试验，即巨噬细胞移动抑制因子（MIF）试验和肥大细胞脱颗粒（MCD）试验，证实了药物在这些病例中的诊断及病因学作用。

方法

对6例泛发性脓疱疹患者的临床、病理和实验室检查结果进行前瞻性研究。用患者服用的药物进行MIF和MCD试验。

结果

皮肤表现包括大片红斑区域上的大量脓疱、丘疹和多形红斑样损害。脓疱疹在使用诱发药物后12小时至5天内出现。组织病理学变化的特征为角层下脓疱、乳头水肿和混合细胞炎性浸润。在两份活检标本中，浸润物显示有大量嗜酸性粒细胞。实验室检查显示白细胞增多伴中性粒细胞增多（6例患者中的4例）以及嗜酸性粒细胞增多和高免疫球蛋白E血症（6例患者中的2例）。对可疑药物（氨苄西林、头孢唑林、四环素、灰黄霉素、马来酸依那普利）进行的MCD试验在所有患者中均为阳性。6例患者中有5例MIF结果为阳性。停用药物后病情迅速恢复。