Cicatricial pemphigoid with severe gingival and laryngeal involvement in an 18-year-old female

Cicatricial pemphigoid (CP), also known as mucous membrane pemphigoid, is an autoimmune vesiculobullous disease occurring mostly in elderly people and seldom occurring in individuals under the age of 20 years. It predominantly affects the mucosal surfaces, primarily the oral and conjunctival mucosa. Uncommonly, the upper aerodigestive tract is involved, which can lead to life-threatening complications. We present the case of an 18-year-old girl with desquamative gingivitis and severe laryngeal webbing and stenosis, caused by cicatricial pemphigoid. Airway management necessitated a tracheostomy and additional surgical procedure. The clinical, diagnostic, and therapeutic features of this disease are discussed, focusing on the oral and unique laryngeal manifestations.     

Mucous membrane pemphigoid: a disease of the elderly

Mucous membrane pemphigoid (MMP) is a vesiculo-bullous, mucocu-taneous disease that generally affects elderly people. The diagnosis of mucous membrane pemphigoid may be overlooked since the disease is often confined to the mouth and desquamative gingivitis may be the only symptom. Gerodontic patients with this condition are often diagnosed as having "desquamative gingivitis" without obtaining the proper therapy to control chronic pain and effect disease remission. The discomfort associated with eating may prevent many elderly patients from obtaining adequate nutrition. Topical and/or systemic corticosteroid therapy are the only available methods of managing this condition. Immu-nologic studies support the autoimmune nature of MMP and infer that it may be a variant of bullous pemphigoid .     

Oral mucous membrane pemphigoid in a 6-year-old boy: diagnosis, treatment and 4 years follow-up

Background.  Childhood oral pemphigoid is extremely rare and usually takes the form of desquamative gingivitis.
Case report.  We describe a 6-year-old boy who presented with gingival bleeding, pain, eating difficulty, and peeling of the gums. Clinical examination revealed desquamative gingivitis with no extra-oral involvement. The diagnosis was established as oral pemphigoid based on the clinical, histological, and immunofluorescence findings. Symptoms resolved on treatment with occlusive topical corticosteroids. The patient was a carrier of the HLA-DQB₁*0301 allele.
Conclusion.  Mucous membrane pemphigoid should be considered in the differential diagnosis of chronic desquamative gingivitis in childhood. Occlusive therapy with topical fluocinonide may alleviate the symptoms.     

Desquamative gingivitis: immunologic findings.

A case of desquamative gingivitis is presented that was a gingival manifestation of cicatricial pemphigoid. Immunologic studies of serum and biopsies were of diagnostic significance. The application of immunofluorescence for the diagnosis of desquamative gingival lesions was discussed. If a definite diagnosis can not be made on the basis of light microscopy, immunofluorescence studies should be performed.     

Frequency of desquamative gingivitis in skin diseases

In an attempt to determine the frequency of desquamative gingivitis (DG) in pemphigus vulgaris (PV), bullous pemphigoid (BP), cicatricial pemphigoid (CP), and lichen planus (LP), a large series of patients examined and classified in each group during the years 1972 to 1981 were included in this study. Analysis of the clinical data revealed that, of the four skin diseases, CP manifests as DG in 63.6 percent of the cases. Desquamative gingival lesions are less frequent in LP (25 percent) and in PV (18.4 percent). The great majority of DG patients were females (72.9 percent). Identification of the underlying causes of desquamative gingivitis is of utmost importance and is dependent upon clinical, histologic, and immunologic criteria.     

Microorganism presence in desquamative gingivitis

Histologic tissue analysis was retrospectively performed on 19 cases which met the diagnostic criteria of desquamative gingivitis (DG) or benign mucous membrane pemphigoid (BMMP) to determine whether microorganisms were present. Original paraffin blocks were resectioned and tissue specimens were stained with hematoxylin and eosin and with Brown and Brenn solutions. The presence of microorganisms at specific sites was recorded and identification of gram type and histomorphology was made. Bacteria were seen in hematoxylin and eosin and Brown and Brenn stained specimens on the epithelial surface in 31.6% and 68.4% of cases, within the epithelium in 0% and 57.9% of cases, within the subbasilar cleft in 52.6% and 94.7% of cases, and within the submucosa in 5.3% and 57.9% of cases respectively. The epithelial surface was colonized primarily by gram positive cocci (47.4%) and gram negative bacilli (42.1%), which also were the most common types of bacteria seen within the epithelium (26.3% each). 94.7% of specimens examined demonstrated gram negative bacilli within the subepithelial cleft. The predominate form of bacteria found in the submucosa was gram negative cocci (42.1%). Results of this study show that colonization of desquamative gingivitis/benign mucous membrane pemphigoid tissue samples occurs.     

Successful treatment of mucous membrane pemphigoid with tacrolimus

Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune, chronic inflammatory subepithelial vesiculobullous disorders. It predominantly affects the mucous membranes of the oral cavity and eyes of the elderly population. Oral manifestations of pemphigoid include desquamative gingivitis, ulcers, erythematous patches, erosions, vesicles and bullae located on the attached gingiva, palate, buccal mucosa, labial mucosa, and tongue. Diagnosis is based on history, clinical features and a biopsy stained with hematoxylin and eosin (H&E), and also direct and indirect immunofluorescence. Topical and systemic corticosteroids are the most commonly used medications for managing pemphigoid. Recently, topical tacrolimus has been successfully used in the treatment of ocular and skin pemphigoid. In this report we present a patient with longterm recalcitrant MMP that did not respond to conventional treatment but was treated successfully with tacrolimus ointment.     

Desquamative gingivitis

The desquamative gingivitis is the clinical term given to the gingival manifestation of mucocutaneous diseases. It is characterized by an erythematous, glazed, friable and hemorrhagic gingiva, which can be accompanied by pains. Except the gingiva, the lesions can be localised on other oral mucous membranes or on the skin. The three principal diseases at the origin of the desquamative gingivitis are by order of frequency: the cicatricial pemphigo?d, erosive lichen planus and pemphigus. The knowledge of the clinical, histological and immunohistochemical characteristics of these three affections is essential for the diagnosis and the suitable treatment of the desquamative gingivitis.     

The management of intransigent desquamative gingivitis with Dapsone

Fifteen patients suffering from intransigent desquamative gingivitis due to lichen planus or benign mucous membrane pemphigoid were treated with dapsone (diphenylsulphapyridine) over a 3-month period. Twelve patients completed the trial. Therapeutic benefit was estimated clinically with the aid of 35mm color transparencies taken at 4-week intervals; and subjectively by the patients estimating their progress as worse, no change, some improvement or complete recovery. Taken as a whole, some 58% of patients had some benefit from therapy. Of the 7 patients with lichen planus, 1 showed complete recovery and 3 showed some improvement. In the 5 patients with desquamative gingivitis due to mucous membrane pemphigoid, 3 showed some improvement and 2 received no benefit. Three patients withdrew from the trial due to side effects of the dapsone such as headaches and nausea. It is concluded that dapsone therapy may be of some use in the management of desquamative gingivitis when traditional treatments have failed. Patients should be warned of the possibility of side effects.     

Adult linear immunoglobulin A disease manifesting as desquamative gingivitis

Desquamative gingivitis is a manifestation of various dermatoses, particularly lichen planus and mucous membrane pemphigoid. A rare example of adult linear immunoglobulin A disease manifesting as desquamative gingivitis is presented. Although the initial clinical features were typical of desquamative gingivitis, the persistence of ulceration after dental extractions was unusual, and the management of the oral lesions proved difficult. The clinical, immunopathologic, and therapeutic aspects of linear immunoglobulin A dermatoses are reviewed.     

Desquamative gingivitis and extragingival collapsed bulla

Mucous membrane pemphigoid is a relatively rare, chronic scarring vesiculobullous disease that frequently affects the oral and ocular mucosa. Other mucous membranes and skin are less frequently affected. Characteristic oral lesions include desquamative gingivitis, and extragingival ulcers and collapsed vesicles and bullae. Ocular involvement may lead to blindness. Local and systemic corticoid hormones, dapsone, and periodontal therapy have all been used with variable success to treat the disease.     

The treatment of desquamative gingival lesions

The treatment of desquamative gingival lesions is dependent on the correct diagnosis of the underlying disease, which is frequently dermatologic. This is determined by clinical observations, as well as by histologic and immunologic examination of gingival biopsy specimens. The recommended treatment of desquamative lesions caused by lichen planus, cicatricial pemphigoid (benign mucous membrane pemphigoid), pemphigus, and psoriasis, the most common dermatoses causing gingival lesions, is summarized on the basis of 62 cases and reports in the literature.     

Gingival manifestations of childhood cicatricial pemphigoid

Cicatricial pemphigoid (CP) is found almost exclusively among middle-aged and elderly persons. This article describes a rare case of CP in a 14-year-old girl; it appeared on the mandibular anterior gingiva as desquamative gingivitis. Histologic examination of the lesions showed a subepithelial bulla. Immunofluorescence of gingival biopsy revealed immunoglobulin G, protein C3, and faint immunoglobulin A deposition along the basement membrane zone; results for normal skin were negative. Indirect immunofluorescence produced negative results. There are only six documented cases of childhood CP previously reported in the literature. The clinical and immunologic features of these cases are reviewed.     

Desquamative gingivitis associated with IgG/IgA pemphigoid presents a challenging diagnosis and treatment: a case report

BACKGROUND: Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune blistering disorders characterized by subepithelial separation and the deposition of immunoglobulins and complement along the basement membrane zone (BMZ). This disease is diagnosed with direct immunofluorescence testing showing a linear deposition of immunoglobulins and/or complement along the BMZ and indirect immunofluorescence testing showing circulating IgG (and sometimes IgA) autoantibodies along the BMZ. In this case report we describe desquamative gingivitis secondary to IgG/IgA pemphigoid and the management of this challenging variant of MMP. METHODS: Routine histology, direct immunofluorescence testing, and indirect immunofluorescence testing were utilized and correlated to the clinical findings to diagnose this unusual immunobullous disease. RESULTS: Direct and indirect immunofluorescence testing confirmed the clinical diagnosis of IgG/IgA pemphigoid as the cause of desquamative gingivitis and the other mucosal findings in this patient. A treatment program including dapsone and other drugs completely resolved the oral lesions after 14 months of therapy. CONCLUSIONS: Desquamative gingivitis associated with IgG/IgA pemphigoid can be challenging to diagnose and treat. After 14 months of treatment, a combination therapy consisting of dapsone with cimetidine and vitamin E to enhance drug efficacy and frequent intramuscular administrations of triamcinolone achieved control of both the oral and genital elements of IgG/IgA pemphigoid in this patient.     

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.     

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.     

Immunofluorescent studies in desquamative gingivitis

Twenty-seven patients (19 females and 8 males) with a clinically diagnosed desquamative gingivitis were used in this study. Twenty-seven additional patients with oral and skin diseases and with gingival involvement other than that of the de-squamative gingivitis type served as controls. Biopsy gingival specimens were sectioned and stained with H and E. The rest of the tissue specimen was cut with a cryostat and used for immunofluorescent assays of the tissue-bound antibodies (IgG, IgA, IgM, C3 and fibrin). Circulating antibodies were determined by using the indirect immunofluorescence method in serum samples. Normal human oral mucosa and several animal epithelial tissues were used as substrates. The immunopathologic findings suggest the diagnosis of lichen planus in the presence of linear or granular fibrin deposition at the basement membrane zone/mucosal submucosal interface with or without cytoid bodies and cicalricial pemphigoid, in the presence of circulating and/or tissue-bound immunoglobulins and C3 in a linear continuous pattern along the BMZ. It is suggested that an accurate diagnosis of the underlying disease of the desquamative gingivitis can be made on the basis of the clinical, histopalhologie, immunopathologic and follow-up findings.     

Expression of estrogen receptors in desquamative gingivitis

BACKGROUND: Most cases of chronic desquamative gingivitis (CDG) are shown by direct immunofluorescence (DIF) to be immune mediated diseases. Some patients present with similar clinical and microscopic findings as CDG but DIF staining is negative. It has been suggested that those cases of CDG may be hormone (estrogen) mediated and may be treated with estrogens with favorable results. METHODS: Gingival tissue from 24 cases of CDG and one case of ordinary gingivitis were studied for estrogen receptor (ER) expression using immunohistochemical techniques. Twenty-four of the 25 cases were female. Using standard DIF analysis, 11 of the CDG cases were diagnosed as benign mucous membrane pemphigoid, 10 as lichen planus or lichenoid mucositis (LP), and one as pemphigus. The remaining 3 cases were not diagnostic for a specific disorder (idiopathic). Five of the females had a history of estrogen substitute therapy. RESULTS: Twenty-two of 23 female CDG cases were positive for ER, although the degree of staining varied. A 32-year-old female with ordinary gingivitis, whose gingivitis varied with her menstrual cycle, did not stain for ER. A 50-year-old male and a 76-year-old female, both with gingival LP, also had negative staining for ER. CONCLUSIONS: There appears to be no correlation between diagnosed diseases (immunological versus idiopathic) and expression of ER in CDG gingiva. ER expression in the gingiva is probably not related to the presence or absence of estrogen supplementation. The results of this study do not support the use of estrogen in the treatment of idiopathic CDG.     

Update on mucous membrane pemphigoid: a heterogeneous immune-mediated subepithelial blistering entity.

Most oral involvement in the skin diseases (dermatoses) is related to mucous membrane pemphigoid or lichen planus. Mucous membrane pemphigoid was the subject of a European Symposium held in Turin, Italy, in June 1997. This review is based on that symposium. Mucous membrane pemphigoid is a subepithelial vesiculobullous disorder mainly of late middle age; it has a slight predilection for women. Whereas mucous membrane pemphigoid was formerly considered a single entity, it is now quite evident that a number of subepithelial vesiculobullous disorders may produce similar clinical pictures and also that a range of variants of mucous membrane pemphigoid exists, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated subepithelial blistering diseases has therefore been used. Diagnosis and management of immune-mediated subepithelial blistering diseases on clinical grounds alone are impossible; a full history, general and oral examinations, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. Most patients with mucous membrane pemphigoid affecting the mouth manifest desquamative gingivitis, a fairly common complaint typically seen in women who are middle-aged or older. Oral vesicles and erosions may also occur, and there can be a positive Nikolsky sign. Some patients have lesions of other stratified squamous epithelia, presenting as conjunctival, nasal, oesophageal, laryngeal, vulval, penile, or anal involvement. Apart from improving oral hygiene, immunomodulatory-in particular, immunosuppressive-therapy is typically required to control oral lesions in mucous membrane pemphigoid. No single treatment regimen reliably controls all these disorders.