Childhood cicatricial pemphigoid with exclusive gingival involvement

A case of cicatricial pemphigoid in a 13-year-old boy presenting in the form of desquamative gingivitis is described. There are only 7 documented cases of childhood cicatricial pemphigoid reported in the literature.     

Gingival manifestations of Wegener's granulomatosis

Localized Wegener's granulomatosis is characterized by necrotizing granulomatous disease affecting the upper and lower respiratory tracts, mucosa, and skin. Without treatment, disseminated necrotizing vasculitis and focal necrotizing glomerulonephritis usually follow. Although oral lesions are relatively common, they have been infrequently described and have rarely been reported as the sole manifestation of this disease. In this report, we present two cases of early Wegener's granulomatosis which featured hyperplastic gingival lesions as the initial presenting lesion. Clinical findings and histological evaluation of tissue obtained from the gingival lesion following incisional and excisional biopsies supported the diagnosis of Wegener's granulomatosis. These cases illustrate that less-frequently occurring entities should be considered in the differential diagnosis of localized gingival lesions which fail to respond to conventional therapy.     

Benign mucous membrane pemphigoid (cicatricial mucosal pemphigoid): a reconsideration.

The name cicatricial mucosal pemphigoid is suggested in an attempt to clarify several synonymous terms and to omit the prefix "benign." The clinical features are reviewed and seven case reports illustrating different aspects are presented. The severe effects of ocular and pharyngeal involvement are highlighted, immunologic findings and treatment are discussed, and the link with neoplasia is emphasized.     

1例瘢痕性类天疱疮的循证治疗

目的：借助循证医学的方法为1例确诊为瘢痕性类天疱疮患者制定循证治疗方案。方法：在充分评估患者情况后,提出临床问题,从Cochrane图书馆（2010年第3期）、Medline（PubMed网站1990年1月～2010年12月）、和http：//sumsearch.uthsca.edu/searchf orm4.htm进行检索。检索词为cicatrical pemphigus;meta analysis,randomizedcontrolled trial,clinicalcontrolled trial,（瘢痕性类天疱疮,系统评价,Meta分析,随机对照试验和临床对照试验）,并手工检索《中华皮肤科杂志》等相关的4种中国科技论文统计源期刊（创刊～2010年）。结果：未检索到系统评价文献,检索到随机对照试验论著6篇,运用了循证医学方法的高质量综述2篇,以及英国皮肤病医师协会拟订的治疗指南。通过对检索结果进行分析,为患者制定合理的治疗方案。皮质类固醇激素是治疗类天疱疮的首选药物,治疗口腔瘢痕性类天疱疮时建议选用中等剂量激素,经6个月的治疗随访,证实该方案适合此患者。结论：循证治疗口腔瘢痕性类天疱疮患者有助于提高临床疗效。     

Cytokine profile and supposed contribution to scarring in cicatricial pemphigoid

BACKGROUND: The progressive scarring observed in cicatricial pemphigoid (CP) is still partially unexplained but recently the release of soluble fibrogenic factors by inflammatory infiltrating cells has been considered as pathogenically relevant. In the present study we evaluated the expression of mRNA for IL-4, IL-5, TGF-beta1, IFN-gamma in CP in comparison to bullous pemphigoid (BP) patients, investigating the role of cytokine profile as possible cause of the different disease evolution. METHODS: Biopsies from patients with oral (n = 10), preputial (n = 3) and cutaneous (n = 1) CP were studied by in situ hybridisation performing a new amplification system based on biotinyl-tyramide. As control, four patients affected by BP were also examined, together with healthy tissue from two CP and two BP patients, respectively. RESULTS: In CP IL-4 mRNA expression was present in 4 out of 14 cases analysed. IL-5 was detected in 12 CP biopsies. TGF-beta1 and IFN-gamma mRNAs were identified in 9 and 11 CP cases, respectively. In BP, IL-4 hybridisation signal could not be observed in any of the cases. By contrast IL-5, TGF-beta1 and IFN-gamma mRNA analyses were positive in all BP cases. Healthy specimens did not show any expression for IL-4, IL-5 and IFN-gamma, while a poor staining for TGF-beta was found in epithelium and subjunctional areas. CONCLUSIONS: Our results highlight the presence of a mixed cytokine pattern in the cellular infiltrate of both blistering diseases, with a corresponding increase of Th2-like activity in fully developed lesions, irrespective of the different sites involved. In addition, the constant presence of TGF-beta1 mRNA in the different lesional phases of CP, and its overlapping expression in BP suggest that the involvement of additional factors is responsible for the scarring course typical of CP.     

瘢痕性类天疱疮与OLP共存1例报道及文献回顾

瘢痕性类天疱疮（CP）和口腔扁平苔藓（OLP）均为与免疫因素密切相关的慢性皮肤黏膜疾病,但尚未见有关两者共存的报道。本文对1例兼有CP和OLP损害的病例进行报道,并结合文献对其临床特点及诊断进行讨论,并提出是否是一种皮肤黏膜大疱性疾病新类型的假设。     

Esthetic management of multiple recession defects in a patient with cicatricial pemphigoid

Cicatricial pemphigoid is one of a number of mucocutaneous disorders that can present in the oral cavity with desquamation, pain, and bleeding of the gingiva and oral mucosa. This case report describes the management of cicatricial pemphigoid in a patient with multiple sites of gingival recession using connective tissue grafting to alleviate root surface sensitivity and improve esthetics. The treatment rationale is presented and discussed in terms of timing of therapy and implications for wound healing in patients who present with desquamative gingivitis.     

Immunopathological diagnosis of cicatricial pemphigoid with desquamative gingivitis. A case report

Cicatricial pemphigoid (CP) is a chronic subepidermal bullous dermatosis which primarily involves the mucous membranes. The oral cavity and the eye are most frequently involved. Since extension of the lesion into the pharynx and esophagus causes sore throat and dysphagia and progressive ocular lesions may cause blindness, early and valid diagnosis is very important. Here we present a case of cicatricial pemphigoid with onset at age 45 in a patient who manifested severe periodontal disease and showed the lesion on the mucous membranes of the mouth (desquamative gingivitis), skin, and eyes. Since definite diagnosis is very important, we describe how we made a differential diagnosis from other diseases which also accompany desquamative gingivitis. We examined the clinical manifestations, blood test results, HLA-genotype, histopathologic findings of the affected tissue, and immunological findings in relation to autoimmunity. Since many of the CP cases are first referred to periodontists or dentists, we believe that the diagnostic strategy described in the present study will be quite informative for making rapid and definite diagnoses of similar cases.     

Gingival and other oral manifestations in measles virus infection

BACKGROUND: Measles is a highly contagious, viral infectious disease affecting mainly children and young adults. It is characterized by high fever, maculopapular rash, keratoconjunctivitis and pathognomonic oral Koplik's spots. METHODS: During an outbreak of measles among soldiers in the Israeli military, patients were referred to one medical center where they were also examined for oral signs and symptoms of their illness. RESULTS: We present a case of measles infection with distinct oral findings. These included Koplik's spots, various forms of ulcerations of the free mucosa, -like gingivitis and pericoronitis. All oral lesions resolved after cessation of systemic illness. CONCLUSIONS: The measles virus is associated with a transient inhibition of the host response. Immunosuppression may create the necessary condition for the proliferation of pathobacteria associated with the observed pericoronitis and the other gingival lesions.     

Direct immunofluorescence of detached gingival epithelium for diagnosis of cicatricial pemphigoid: Report of five cases

The diagnosis of mucosal cicatricial pemphigoid is often problematic because of frequent false negative or equivocal biopsy results. We describe an immunofluorescent technique that uses detached gingival epithelium for diagnostic purposes when oral manifestations of cicatricial pemphigoid are suspected. Erythematous mucosa that is gently rubbed produces detached epithelium that is then submitted for direct immunofluorescence studies along with tissue acquired via conventional techniques. In the five cases reported, the detached gingival epithelium stained intensely for immunoglobulins and complement on the basilar pole of the basal epithelial cells. The donor sites healed without a resultant periodontal defect. This adjunctive biopsy technique offers the advantage of easily procuring gingival tissue with deposition of immunoreactants that are quite specific for the diagnosis of cicatricial pemphigoid.     

Plurimucosal cicatricial pemphigoid. A case with involvement of the gingival, vulval and vaginal mucosa

We have studied a 62 years old woman with the infrequent involvement of the gingiva (desquamative gingivitis), vulvar and vaginal mucouses in benign mucosal pemphigoid. The clinical features and pathology are exposed and we debate differential diagnosis principally with pemphigus and lichen planus.     

The dental manifestations and orthodontic implications of hypoparathyroidism in childhood

The presence of short dental roots can present challenges to the orthodontist both in terms of identifying its aetiology and in subsequent treatment planning. Uncommon causes include hypoparathyroidism and pseudohypoparathyroidism, where short roots may be seen in combination with other oral manifestations including enamel hypoplasia secondary to low calcium levels. This case report highlights these features and the orthodontic treatment proposed.     

Gingival recession

This paper reviews the factors, anatomical, pathological and physiological, which have been implicated in the aetiology of gingival recession. Changes in the root surface when exposed to the oral fluids are also reviewed, a possible mechanism for the perpetuation of recession with increase in age is suggested.