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融合性网状乳头瘤病(古热罗-卡托综合征):一种对米诺环素敏感的皮肤病,发病机制中无酵母感染证据。39例患者的研究及诊断标准建议

Confluent and reticulate papillomatosis (Gougerot-Carteaud syndrome): a minocycline-responsive dermatosis without evidence for yeast in pathogenesis. A study of 39 patients and a proposal of diagnostic criteria.

作者信息

Davis M D P, Weenig R H, Camilleri M J

机构信息

Department of Dermatology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.

出版信息

Br J Dermatol. 2006 Feb;154(2):287-93. doi: 10.1111/j.1365-2133.2005.06955.x.

DOI:10.1111/j.1365-2133.2005.06955.x
PMID:16433798
Abstract

BACKGROUND

Confluent and reticulate papillomatosis (CRP) (Gougerot-Carteaud syndrome) is a disorder that has been characterized in only small cohorts of patients.

OBJECTIVES

Better to characterize the clinical and pathological findings of the disorder.

METHODS

We retrospectively reviewed the clinical presentation, response to treatment and histological findings of patients presenting to Mayo Clinic (Rochester, MN, U.S.A.) with CRP.

RESULTS

The disorder was diagnosed in 39 patients between 1972 and 2003. Mean age at onset of the skin eruption was 15 years (range 8-32); 21 patients (54%) were male; most were white; most (33) presented for reasons of cosmesis; and eight described the rash as mildly pruritic. At presentation, the skin eruption had been present for a mean of 3.1 years (range 3 months-20 years) and had been recalcitrant to treatment, including antifungal treatment. Typical objective findings were scaling brown macules and patches and velvety papules and plaques, reticulated and papillomatous at least in part, involving the upper trunk, axillae and neck. The most frequent initial diagnostic impressions were tinea versicolor, acanthosis nigricans and CRP. Scales in 32 cases were examined with potassium hydroxide: eight (25%) showed hyphae, and 24 (75%) did not. Skin biopsy specimens from 21 patients showed variable degrees of hyperkeratosis, acanthosis and papillomatosis. Minocycline was prescribed for 22 patients, of whom 14 of 18 (78%) had complete clearing of the skin eruption and four (22%) a partial response. The skin eruptions recurred after stopping treatment in six patients.

CONCLUSIONS

CRP occurs predominantly in young adults and teenagers, with cosmetically displeasing brown scaling patches and plaques affecting the neck, upper trunk and axillae. Frequently, the diagnosis is delayed and the disorder not recognized by physicians, including dermatologists. Clinically, the eruption is most often confused with tinea versicolor. Potassium hydroxide staining of the scale is negative in the majority of cases, implying that fungi are not involved in the pathogenesis of this condition, as has been previously proposed. It is important to recognize this disorder, because minocycline therapy is highly effective in most patients. Criteria for the diagnosis are proposed.

摘要

背景

融合性网状乳头瘤病(CRP)(古热罗 - 卡托综合征)是一种仅在少数患者队列中得到描述的疾病。

目的

更好地描述该疾病的临床和病理表现。

方法

我们回顾性分析了美国明尼苏达州罗切斯特市梅奥诊所诊断为CRP的患者的临床表现、治疗反应和组织学表现。

结果

1972年至2003年间,共诊断出39例该疾病患者。皮疹发作的平均年龄为15岁(范围8 - 32岁);21例(54%)为男性;大多数为白人;大多数患者(33例)因美容原因就诊;8例患者称皮疹有轻度瘙痒。就诊时,皮疹已出现平均3.1年(范围3个月至20年),且对包括抗真菌治疗在内的治疗反应不佳。典型的客观表现为鳞屑性褐色斑片和斑块以及天鹅绒样丘疹和斑块,至少部分呈网状和乳头瘤样,累及上胸部、腋窝和颈部。最常见的初始诊断印象为花斑癣、黑棘皮病和CRP。对32例患者的鳞屑进行了氢氧化钾检查:8例(25%)显示有菌丝,24例(75%)未显示。21例患者的皮肤活检标本显示不同程度的角化过度、棘层肥厚和乳头瘤样增生。22例患者使用了米诺环素,其中18例中的14例(78%)皮疹完全消退,4例(22%)部分缓解。6例患者停药后皮疹复发。

结论

CRP主要发生于年轻人和青少年,颈部、上胸部和腋窝出现影响美观的褐色鳞屑性斑片和斑块。诊断常常延迟,包括皮肤科医生在内的医生往往未能识别该疾病。临床上,皮疹最常与花斑癣混淆。大多数病例中鳞屑的氢氧化钾染色为阴性,这意味着真菌并不像之前所认为的那样参与该疾病的发病机制。认识到这种疾病很重要,因为米诺环素治疗对大多数患者非常有效。本文提出了诊断标准。

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