大疱性扁平苔藓1例

患者男 ,5 5岁 ,躯干及四肢紫红色扁平丘疹及张力性水疱 ,伴瘙痒。组织病理显示 ,表皮颗粒层及棘层增厚 ,基底细胞液化 ,真皮浅层淋巴细胞浸润 ;直接免疫荧光检查IgG ,IgM ,IgA和C3均为阴性。     

Childhood Lichen Planus (LP)

In adults, lichen planus (LP) is relatively more common than in children. Among 222 cases of LP, there were 25 (11.2%) children in our study. The majority of the cases were females in the age group of 8–14; the youngest child was 3 years old. Papular and linear types of LP were common in children. There was no familial history of LP in any of the cases. The patients with classic LP lesions responded well to dapsone therapy. This study supports the suggestion of Ramsay and Hurley that childhood LP is more common in the tropics.     

Childhood Lichen Planus with Palmoplantar Involvement

Lichen planus (LP) commonly involves the flexor aspects of the wrists, legs, and oral and genital mucous membranes. But it rarely occurs on the palms and/or soles. It mainly affects people in the age range 30~60 years. Childhood LP is reported to constitute only 1~4% of total cases of LP. In the literature, a few cases of LP on the palms and soles of pediatric patients have been reported. Here we report an interesting case of childhood LP with palmoplantar involvement. The patient was a 7-year-old boy who for 6 months had variously sized and shaped, pruritic, violaceous, polygonal papules and plaques on the whole body. The skin biopsy specimens taken from four sites of the patient (dorsum of the hand and foot, sole and chest) showed typical features of LP on histopathology. A diagnosis of generalized LP with palmoplantar involvement was made.     

家族性与非家族性大疱性扁平苔藓临床分析

目的　比较家族性与非家族性大疱性扁平苔藓 (BLP)临床特点的异同。方法　对 3 6例家族性BLP和 2 1例非家族性BLP的临床资料进行对比分析。结果　家族性与非家族性BLP在初发年龄、病程及皮损累及范围等方面均存在明显差异 (P均 <0 .0 1)。结论　与非家族性BLP相比 ,家族性BLP患者发病年龄更小、病程更长、范围更广、更易累及甲。遗传因素在BLP的发病中可能起重要作用。     

Lichen Planus in Childhood: A Series of 316 Patients

Lichen planus (LP) is infrequently seen in children and the clinical presentation is often atypical. We conducted a retrospective analysis of clinical features and treatment response in childhood LP to date. The clinical profile and treatment response data of patients younger than 14 years old with LP (entered in a predesigned pro forma study) from January 1997 to June 2011 were analyzed. The treatment was administered according to a predetermined departmental protocol and was comprised of topical steroids with or without oral dapsone or corticosteroids. Patients were evaluated for response, adverse effects, and relapse. The study population consisted of 316 children (166 boys, 150 girls), or 18.7% of the total registered patients in the LP clinic. The mean age was 10.28 years (range 2–14 years). Cutaneous lesions were seen in 96.2%. Involvement of the oral mucosa was detected in 18%, nails in 13.9%, scalp in 8.2%, and genitalia in 4.4%. Classic LP was most prevalent (53.8%), followed by eruptive (16.5%), hypertrophic (8.2%), linear (6.9%), and lichen planopilaris (6.3%). LP pigmentosus, annular, and atrophic variants were encountered infrequently. Topical corticosteroids were the most common treatment used in 69.5% of patients, 28.8% of whom had excellent response at 6 months, although 38.8% failed to follow up. Dapsone was prescribed in 20% and systemic steroids in 9.8% of patients. We report the largest series to date of LP in childhood, with a more varied clinical presentation than in previous series. The course and response to treatment were similar to those in adults.     

Lichen Planus in Childhood: A Report of 17 Patients

Lichen planus is a dermatosis of unknown origin with a very limited frequency in children. Over a period of one and a half years we observed 17 cases of childhood lichen planus. The classic form of the disease as seen in adults was the most common in these children; unlike adults, however, mucosal and nail involvement was uncommon. The natural history of the disease was similar to that in adults.     

Familial Bullous Lichen Planus (FBLP): Pedigree Analysis and Clinical Characteristics

BACKGROUND: Familial bullous lichen planus (FBLP) is a rare condition. The clinical features dearly have been described. OBJECTIVE: We report the largest patient series of FBLP and describe its clinical characteristics and inheritance pattern. METHODS: In this retrospective chart review, we analyzed nine consecutive familial pedigrees of FBLP with 36 affected individuals who presented to the Department of Dermatology at the Wuhan Union Hospital, a tertiary referral hospital in central China. Parameters analyzed include age of onset, gender predilection, lesional distribution, nail and mucosal involvement, clinical course, and inheritance pattern. RESULTS: Thirty-six of 85 individuals in the nine families were affected (42.4%). Females were more likely to be affected than males (58.3% vs 35.7%, G(chi 2 = 3.99. P < 0.05). A bimodal disease onset was found, with one peak at 1-3 years and another at 13-17 years. The shin is the most commonly affected area (97%) followed by the upper limbs and the thighs. Involvement of the torso is relatively rare. Only a minority of cases involves the oral mucosa. The disease tends to follow a chronic and progressive course. The inheritance pattern is autosomal dominant with variable penetrance. CONCLUSION: Familial bullous lichen planus is a chronic, progressive bullous eruption of the lower and upper extremities. Compared with non familial bullous lichen planus, it has an earlier onset and wider disease distribution. It may be inherited as an autosomal dominant condition with variable penetrance.     

Ulcerative Lichen Planus: A Rare Variant of Lichen Planus

A 54-year-old Japanese man with ulcerative lichen planus is reported. He had a painful erythema with ulcers on his soles and nail dystrophy. Mucous involvement was present on his lip. His hairs were intact. Histopathological findings confirmed the diagnosis of lichen planus.     

Annular Lichen Planus

Background Annular lichen planus is a clinical variant of lichen planus, and its characteristic lesions are annular plaques consisting of a narrow rim of activity and a depressed center.Objective This article presents two cases of annular lichen planus, a 39-year-old woman and a 45-year-old woman.Methods and Results In both cases, lesions developed mainly on the femoral regions. Histopathological specimens from the margin of the lesion demonstrated liquefaction degeneration of the epidermis and band-like infiltrate of lymphocytes in the upper dermis, whereas there was very mild infiltrate in the upper dermis from the center. In the center, elastic fibers were more fragmented and less numerous than in the margin. Although topical corticosteroid tape was effective for one case, new lesions developed several times over two years in the other.Conclusion Our findings suggest that this clinical behavior is from elastolysis as a result of inflammation.

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SommaireAntécédents Le lichen plan annulaire est une variante du lichen plan qui se caractérise par un groupe annulaire de lésions à bord étroit et affaissées au centre.Objectif Larticle présente deux cas de lichen plan annulaire chez une femme de 39 ans et une autre de 45 ans.Méthodes et résultants Dans les deux cas, les lésions se sont développées principalement dans les régions fémorales. Les spécimens histopathologiques prélevés sur les périphéries des lésions indiquent une dégénération de lépiderme par liquéfaction ainsi que des infiltrats leucocytaires dans la partie supérieure de lépiderme, à partir du centre. Au centre, les fibres élastiques sont plus fragmentées et moins nombreuses que dans les périphéries. Bien que les bandes de corticostéroïdes aient été efficaces dans un cas, dans lautre cas, de nouvelles lésions se sont développées plusieurs fois au cours dun intervalle de deux ans.Conclusion Nos résultats suggèrent que ce comportement clinique proviendrait de lélastolyse résultant de linflammation.

     

肥厚性扁平苔癣

<正>患者男,43岁。主诉:手足背紫红色斑块伴痒3月余。现病史:患者3月前无明显诱因于手足背出现绿豆大小红色丘疹,后渐增大融合,变为紫红色,伴轻度瘙痒,曾于外院诊治(具体不详),效果不佳,近日自觉瘙痒加重来诊。     

Twenty-Nail Dystrophy of Childhood: A Sign of Localized Lichen Planus

Abstract: A 9½-year-old girl was seen with 20-nail dystrophy, Lichen planus was suspected and further examination revealed other lesions localized to the oral mucosa. The diagnosis was confirmed as lichen planus by biopsy of tissue from the oral lesions. Careful examination of all mucocutaneous surfaces is recommended before accepting the diagnosis of so-called isolated 20-nail dystrophy of childhood.     

Childhood Lichen Planus: Demographics of a U.S. Population

Abstract:  Lichen planus is an inflammatory dermatosis of unknown origin that is relatively uncommon in children. Demographic data for lichen planus of children in the United States are lacking, with most large case reports originating from India, Kuwait, Mexico, and the United Kingdom. We hypothesized that a greater proportion of our pediatric lichen planus patients were African American, an observation not previously documented. A retrospective chart review was performed to investigate characteristics of our pediatric lichen planus patients. The ethnicity of the lichen planus patients was compared with the data for our general patient population. The proportion of African American patients in each group was compared using the chi-squared test. We report 36 children (female to male ratio 2:1) who presented with lichen planus to the pediatric dermatology clinic at Children's Hospital of Wisconsin. Twenty-six (72%) of these patients were African American (OR 9.63, p < 0.0001). A personal or family history of autoimmune disease was present in six (17%) patients. Although there has been no reported racial predominance of lichen planus, we observed lichen planus to occur more commonly in African American children. Interestingly, the incidence of autoimmune disease was higher than has previously been reported. Future studies will confirm or refute these observations and advance our understanding of potential genetic or environmental risk factors for the development of lichen planus.     

扁平苔藓中细胞凋亡研究

目的探讨细胞凋亡与扁平苔藓的关系.方法采用原位末端标记检测扁平苔藓中细胞凋亡,采用免疫组化ABC法、SABC法测凋亡相关Bcl-2、Fas和Fas-L、穿孔素.结果扁平苔藓中细胞凋亡较正常人增多.Bcl-2、Fas均阳性表达.结论细胞凋亡在扁平苔藓发病机理中有十分重要的意义.