Cicatricial pemphigoid (Brunsting-Perry type). Case report and immunofluorescence findings.

We describe clinical and immunofluorescence findings of a patient with Brunsting-Perry-type cicatricial pemphigoid. Direct immunofluorescence showed tissue-fixed basement membrane zone antibodies similar to those characteristic of bullous pemphigoid. Circulating antibodies to the basement membrane zone were not found. Brunsting-Perry-type cicatricial pemphigoid probably represents a clinical variation midway in the cicatricial pemphigoid-bullous pemphigoid spectrum of disease. Management with intralesional corticosteroids was successful in controlling the skin lesions.     

Oesophageal webs preceding carcinoma and rupture of the oesophagus in cicatricial pemphigoid

A case in which oesophageal webs preceded the development of carcinoma and rupture of the oesophagus in a 77-year-old woman with cicatricial pemphigoid is reported. Oesophageal webs in cicatricial pemphigoid have been reported but are rare. Clinical, histological, radiological and post-mortem features are described. Western immunoblotting of serum demonstrated a 180-kDa antigen which comprises one of the antigens reported in cicatricial pemphigoid.     

Bullous pemphigoid evolving into cicatricial pemphigoid?

We describe three patients who initially presented with both clinical and immunological findings to support a diagnosis of bullous pemphigoid but whose subsequent course has been that of cicatricial pemphigoid. Mucosal scarring was accompanied by a fall in autoantibody titres in our three patients. These cases illustrate the difficulties clinicians may experience in assigning a specific diagnosis to patients. They also support the concept that bullous pemphigoid and cicatricial pemphigoid are part of a single disease spectrum. The most intriguing question is what specific factors determine the expression of a particular disease phenotype as bullous pemphigoid and cicatricial pemphigoid share target antigens and also the DQ7 allele.     

Cicatricial pemphigoid and rheumatoid arthritis.

A review of our experience with cicatricial pemphigoid revealed three patients with cicatrical pemphigoid and rheumatoid arthritis and one patient with ankylosing spondylitis who had a high titer of rheumatoid factor. A comparison of these four patients with patients who had bullous pemphigoid and rheumatoid arthritis shows similarities between cicatricial pemphigoid and bullous pemphigoid in relation to the development of rheumatoid arthritis.     

Topische Immunmodulatoren als Therapieoption für das orale vernarbende Pemphigoid

Oral cicatricial pemphigoid is a chronic autoimmune blistering disease which affects predominantly the gingiva and the buccal mucosa. The pathogenesis of this disease is still incompletely understood; however, there is compelling evidence that cicatricial pemphigoid might be mediated by T lymphocytes. Therefore, we performed immunomodulatory therapy with topical tacrolimus in patients with long-standing, therapy-resistant oral cicatricial pemphigoid. Following 3 months of treatment, complete healing and ongoing remission could be achieved.     

Immunofluorescent studies in ocular cicatricial pemphigoid

Twenty nine patients with cicatrizing conjunctivitis were studied; 17 with a clinical diagnosis of cicatricial pemphigoid, five with a clinical diagnosis of pseudopemphigoid caused by long-term application of topical medication and seven who had a cicatrizing conjunctivitis from other causes. Biopsies from clinically uninvolved bulbar conjunctiva were taken for direct immunofluorescence and blood was taken for indirect immunofluorescence using normal human conjunctiva, oral mucosa and skin as substrates. On direct immunofluorescence, in vivo bound immunoglobulins were found along the basement membrane in 10 of the 17 patients with cicatricial pemphigoid, one of the five with pseudopemphigoid and two of the seven with a cicatrizing conjunctivitis associated with other diseases. Circulating anti-basement membrane zone antibodies were found only when conjunctiva was used as a substrate. These were present in seven of the patients with cicatricial pemphigoid, three of those with pseudopemphigoid and two of those with a cicatrizing conjunctivitis caused by other diseases. These results indicate that direct immunofluorescence is a useful, but not absolute diagnostic marker for ocular cicatricial pemphigoid. The results in the pseudopemphigoid group argue that this is an immunologically mediated disorder indistinguishable from spontaneous cicatricial pemphigoid and probably triggered by the drugs. The presence of circulating antibodies should allow for precise identification of the antigen involved in cicatricial pemphigoid using SDS electrophoresis and Western blot analysis.     

DAPSONE AND SULFAPYRIDINE THERAPY OF PEMPHIGOID DISEASES

Since 1974, dapsone and sulfapyridine have been used to treat 30 patients with localized oral pemphigoid, 36 with cicatricial pemphigoid, and 11 with cicatricial pemphigoid limited to the eyes; 7 of these 77 patients were less than 50 years of age. Follow-up was J to 12 years (mean, 4.8 years). Of the 30 patients with localized oral pemphigoid, half were in remission and off treatment at a mean follow-up of 5 years. Of 25 patients with cicatricial pemphigoid who received dapsone therapy as initial treatment, 15 (60%) had a successful result. Sulfapyridine was successful in three patients and dapsone in six additional patients after other therapy. Thus, dapsone or sulfapyridine therapy was successful in 24 of 34 patients (71%). In 30 patients with pemphigoid limited to the ocular mueosa or cicatricial pemphigoid with ocular manifestations, 25 (83%) responded successfully to dapsone or sulfapyridine; there were no treatment failures among these 30 patients with ocular pemphigoid. Overall, of the 77 patients, of 55 who underwent an evaluable eotirse of dapsone or sulfapyridine therapy, 47 (85%) responded successfully.     

Transient Cutaneous Vesicles in a Teenager: The Final Piece to a Puzzle

Abstract: Cicatricial pemphigoid is a chronic blistering disease that predominantly affects the mucous membranes. It has a peak occurrence in the seventh decade. Pediatric cicatricial pemphigoid is a rare entity, with fewer than 20 cases reported. We report an 18-year-old man who was recently diagnosed with cicatricial pemphigoid after six years of diagnostic uncertainty.     

Occurrence of cicatricial pemphigoid and leiomyosarcoma in a psoriatic patient

A psoriatic patient who developed cicatricial pemphigoid and leiomyosarcoma of the abdomen is presented. The sequence of events indicates a relationship between the cicatricial pemphigoid and the malignant disease.     

Anti-epiligrin (laminin 5) cicatricial pemphigoid

Cicatricial pemphigoid is an autoimmune disease predominantly of elderly patients. Recently, a subtype of cicatricial pemphigoid with antibodies to epiligrin (laminin 5) was described. We report a patient in whom anti-epiligrin cicatricial pemphigoid was identified. He presented with cutaneous, oral and ocular involvement. He has shown initial improvement on corticosteroids and cyclophosphamide.     

Anti-epiligrin cicatricial pemphigoid with IgG autoantibodies to the beta and gamma subunits of laminin 5

Anti-epiligrin cicatricial pemphigoid is an autoimmune subepithelial blistering disorder of mucous membranes and skin. By immunoblot analyses, sera of most patients with antiepiligrin cicatricial pemphigoid have been shown to react specifically with the alpha3 chain of laminin 5. We describe the first patient with anti-epiligrin cicatricial pemphigoid in whom circulating IgG autoantibodies directed against the beta3 and gamma2-chains of laminin 5 were detected. Treatment with oral prednisolone was beneficial in controlling the disease.     

Antiepiligrin cicatricial pemphigoid: an underdiagnosed entity within the spectrum of scarring autoimmune subepidermal bullous diseases?

BACKGROUND: Antiepiligrin cicatricial pemphigoid (AECP) is a chronic autoimmune subepidermal blistering disease characterized by autoantibodies to laminin 5 and clinical features of cicatricial pemphigoid. Only a few patients with AECP have been described to date. The aim of the present study was to analyze the relative frequency of AECP among patients with the clinical phenotype of cicatricial pemphigoid. OBSERVATIONS: Serum from 16 consecutive patients with the clinical phenotype of cicatricial pemphigoid were included in this study. Nine patients had circulating IgG autoantibodies by indirect immunofluorescence on sodium chloride-split skin; patients' IgG bound to the epidermal side (n = 2), dermal side (n = 5), or both sides (n = 2) of this test substrate. Interestingly, all 5 cases with dermal binding immunoprecipitated laminin 5 from extracts and media of cultured keratinocytes, and 4 of these serum samples reacted with the alpha3 subunit of laminin 5 by immunoblotting. None of the patients with dermal binding of IgG demonstrated autoantibodies to type VII collagen. CONCLUSION: Our data suggest that, among patients with the clinical phenotype of cicatricial pemphigoid, AECP may be more frequent than previously assumed.     

Oesophageal involvement in cicatricial pemphigoid

Four cases of cicatricial pemphigoid complicated by oesophageal involvement are presented. All patients suffered dysphagia but repeated radiological studies were required for confirmation of oesophageal ulceration, webs and strictures. A combination of systemic drug therapy and oesophageal dilatation were necessary for the suppression of symptoms. The clinical and immunopathological features, management and complications of oesophageal involvement in cicatricial pemphigoid are discussed. Dermatologists should be aware of these features and make regular inquiries about swallowing difficulties in patients with cicatricial pemphigoid to guide appropriate investigations and treatment     

Immunopathology of cicatricial pemphigoid: studies of complement deposition.

Immunopathologic investigations were conducted on the sera and oral mucosal tissue specimens of 23 patients with cicatricial pemphigoid. A linear, continuous basement membrane zone pattern was noted in 83% of oral mucosal biopsy specimens studied. This pattern is indistinguishable from the pattern noted in immunofluorescence studies of bullous pemphigoid, herpes gestationis, and some cases of desquamative gingivitis. Complement studies provided data supportive of classical pathway activation in cicatricial pemphigoid tissue. Deposition of IgA with Factor B, properdin, and C3 raised the possibility of alternative pathway activation, a question requiring further study. Circulating antibasement membrane zone antibodies were noted in the sera of two patients with cicatricial pemphigoid.     

Mucous gland basement membrane immunofluorescence in cicatricial pemphigoid

Three patients are described with cicatricial pemphigoid and positive immunofluorescence findings in the basement membrane zone of mucous glands of the pharynx, mouth, and nose. These findings appear to be unique to cicatricial pemphigoid.     

(11) Oesophageal strictures in cicatricial pemphigoid

Cicatricial pemphigoid is a rare disease affecting the skin and mucous membranes. It is a disorder characterized by sub-epidermal blisters which heal with fibrosis and scarring. The diagnosis is confirmed by demonstration of immunoglobulins along the basement membrane of perilesional tissue using immunofluorescence. Ten patients with cicatricial pemphigoid who complained of dysphagia were investigated by barium swallow. An upper oesophageal stricture was demonstrated in eight of these. In the remaining two patients indirect laryngoscopy showed hypopharyngeal ulcers which could account for the dysphagia. A benign stricture of the upper third of the oesophagus is a rare finding and cicatricial pemphigoid should be recognized as a possible cause. The other clinical manifestations of cicatricial pemphigoid may be subtle. However, they can be seen on careful examination of the skin and mucous membranes which will enable the diagnosis to be made.     

Identification of cicatricial pemphigoid antigens.

The nature of skin antigens defined by antibodies in patients with cicatricial pemphigoid was studied with use of the 1 mol/L of sodium chloride split skin technique and Western immunoblot analysis. Antibodies in the serum samples of three of seven patients with cicatricial pemphigoid reacted to the epidermal side of 1 mol/L of sodium chloride split skin, and antibodies in the serum sample of one patient reacted to the dermal side. With Western immunoblot analysis, three patients had antibodies to antigens in the epidermal extracts of skin. The antibodies reacted in all patients to a 160-kd antigen and in one patient to an additional 230-kd antigen. These two antigens are similar in molecular weight to the 230-kd major bullous pemphigoid antigen and to the 160-kd minor bullous pemphigoid antigen. However, while the basement membrane zone antibodies present in cicatricial pemphigoid were most often directed to the 160-kd antigen, those present in 38 patients with bullous pemphigoid reacted most often (in 34 patients [89%]) to the 230-kd antigen. None of the serum samples reacted to antigens in dermal extracts that contained the epidermolysis bullosa acquisita antigen. These results indicate that the basement zone membrane antibodies present in cicatricial pemphigoid are directed in part to epidermal antigens that are similar in molecular weight to bullous pemphigoid antigens. However, the frequency of reactions to different basement membrane zone antigens differs in the two diseases, which may account for the clinical differences between the two conditions.     

Vulvar cicatricial pemphigoid of childhood

We report a 9-year-old girl with a vulvar autoimmune bullous dermatosis. A diagnosis of localized bullous pemphigoid or cicatricial pemphigoid was made on the basis of immunohistologic data. Since the lesions were unresponsive to topical corticosteroids but healed completely on dapsone at a dosage of 1.5 mg/kg/day, we favor the diagnosis of vulvar cicatricial pemphigoid. Only two such cases have been reported thus far. The diagnostic criteria and therapeutic modalities are discussed.     

Cicatricial pemphigoid rarely involves the scalp

We present a case of cicatricial pemphigoid with significant scarring alopecia as a major manifestation and review our series of cicatricial pemphigoid patients in Oxford (UK). Only lour of 54 patients had scarring alopecia of the scalp. While rare, this manifestation poses a difficult management problem; patients find this cosmetically distressing and there are no successful treatments available to date.     

Cicatricial pemphigoid with linear IgA deposit

A Japanese woman with typical clinical and histological manifestations of cicatricial pemphigoid was presented. Direct immunofluorescent (IF) investigation of perilesional skin revealed in vivo deposits of IgA but not of IgG, IgM, or C3. Indirect IF study revealed that this patient had circulating antibody against epidermal basement membrane zone of the IgA class. We would like to classify this case as cicatricial pemphigoid with IgA deposits rather than as a cicatricial variant of linear IgA bullous dermatosis.