Cicatricial Pemphigoid (cicatricial + pemphigoid)

Distribution by Scientific Domains

Kinds of Cicatricial Pemphigoid

  • ocular cicatricial pemphigoid


  • Selected Abstracts


    Early Onset Childhood Cicatricial Pemphigoid: A Case Report and Review of the Literature

    PEDIATRIC DERMATOLOGY, Issue 2 2010
    Monia Kharfi M.D.
    We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation. [source]


    Vulvar Cicatricial Pemphigoid of Childhood

    PEDIATRIC DERMATOLOGY, Issue 1 2004
    A. Schoeffler M.D.
    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. [source]


    Early Onset Childhood Cicatricial Pemphigoid: A Case Report and Review of the Literature

    PEDIATRIC DERMATOLOGY, Issue 2 2010
    Monia Kharfi M.D.
    We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation. [source]


    Tissue-engineered tear secretory system: Functional lacrimal gland acinar cells cultured on matrix protein-coated substrata

    JOURNAL OF BIOMEDICAL MATERIALS RESEARCH, Issue 1 2007
    Shivaram Selvam
    Abstract Dry eye is a general term that refers to a myriad of ophthalmic disorders resulting in the inadequate wetting of the corneal surface by the tear film. Dry eyes are typically treated by the application of artificial tears. However, patients with lacrimal insufficiencies such as Stevens-Johnson syndrome, chemical and thermal injuries, or ocular cicatricial pemphigoid have very limited options because of the short duration and action of lubricating agents. As a therapeutic strategy, we are working to develop a bioengineered tear secretory system for such patients. This article describes the growth and physiological properties of purified rabbit lacrimal gland acinar cells (pLGACs) on several matrix protein-coated polymers such as silicone, collagen I, copolymers of poly- D,L -lactide- co -glycolide (PLGA; 85:15 and 50:50), poly- L -lactic acid (PLLA), and Thermanox® plastic cell culture coverslips. Monolayers of acinar cells were established on all of the polymeric substrata. An assay of ,-hexosaminidase activity in the supernatant medium showed significant increases in protein secretion, following stimulation with 100 ,M carbachol on matrix protein-coated and uncoated polymers such as silicone, PLGA 85:15, and PLLA. Our study demonstrates that PLLA supported the morphological and physiological properties of purified rabbit lacrimal gland epithelial cells more successfully than the others. © 2006 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater, 2007 [source]


    Vulvar Cicatricial Pemphigoid of Childhood

    PEDIATRIC DERMATOLOGY, Issue 1 2004
    A. Schoeffler M.D.
    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. [source]


    Atypical herpes simplex infection masquerading as recalcitrant pemphigus vulgaris

    AUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 4 2007
    Andrew H Kalajian
    SUMMARY A 57-year-old woman presented with refractory genital erosive disease. One year earlier she experienced gingival fragility; direct immunofluorescence resulted in the diagnosis of cicatricial pemphigoid, and prednisone therapy led to initial improvement. Initial skin biopsy of her genital erosions demonstrated full-thickness ulceration with viral cytopathic change and a re-epithelializing subepidermal separation. Indirect immunofluorescence revealed intercellular IgG staining on monkey oesophagus at a titre of 1:320 consistent with pemphigus, leading to the diagnoses of pemphigus vulgaris with herpetic superinfection. Immunosuppressive treatment initially led to improvement; however, disease subsequently recurred as extensive genital erosions. We diagnosed atypical herpes simplex virus infection and oral candidiasis, discontinued all immunosuppressive medications, and initiated antiviral and antifungal therapy. Dramatic resolution was observed and the patient has remained free of disease for 13 months while taking only prophylactic famciclovir. [source]


    Antiepiligrin (laminin 5) cicatricial pemphigoid complicated and exacerbated by herpes simplex virus type 2 infection

    AUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 4 2001
    Tanya K Gilmour
    SUMMARY A 50-year-old man with antiepiligrin (laminin 5) cicatricial pemphigoid (AeCP) involving the eyes, mouth and skin required a combination of systemic drug therapies to suppress the ocular disease. Herpes simplex virus type 2 infection of the mouth and pharynx precipitated an acute deterioration, with laryngeal involvement and an increase in oral ulceration. This is an unusual complication of long-term immunosuppression and illustrates some of the difficulties in the management of patients with AeCP. Clinical improvement was obtained with oral antiviral therapy and adjustment of his immunosuppressive regimen. [source]


    U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases

    BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2004
    R.M. Vodegel
    Summary Background, Epidermolysis bullosa acquisita (EBA) can be differentiated from other subepidermal bullous diseases by sophisticated techniques such as immunoelectron microscopy, salt-split skin antigen mapping, fluorescence overlay antigen mapping, immunoblot and enzyme-linked immunosorbent assay. Objectives, To determine whether the diagnosis can also be made by routine direct immunofluorescence microscopy. Methods, We studied frozen skin biopsies from 157 patients with various subepidermal immunobullous diseases. Results, We found three distinct ,linear' fluorescence patterns at the basement membrane zone: true linear, n-serrated and u-serrated. The true linear pattern, often seen in conjunction with either the n- or the u-serrated pattern, was found in any subepidermal immunobullous disease with nongranular depositions. In bullous pemphigoid, mucous membrane pemphigoid, antiepiligrin cicatricial pemphigoid, p200 pemphigoid and linear IgA disease the n-serrated pattern was found, corresponding with depositions located in hemidesmosomes, lamina lucida or lamina densa. However, in EBA and bullous systemic lupus erythematosus the u-serrated staining pattern was seen, corresponding with the ultralocalization of type VII collagen in the sublamina densa zone. The diagnosis of EBA with IgG or IgA autoantibodies directed against type VII collagen was confirmed by immunoelectron microscopy, salt-split skin antigen mapping, fluorescence overlay antigen mapping or immunoblotting. Conclusions, Using this pattern recognition by direct immunofluorescence microscopy we discovered several cases of EBA which would otherwise have been erroneously diagnosed as a form of pemphigoid or linear IgA disease. [source]


    Tumour necrosis factor-, in conjunctivae affected by ocular cicatricial pemphigoid

    ACTA OPHTHALMOLOGICA, Issue 7 2007
    Miguel Cordero Coma
    Abstract. Purpose:, The presence of tumour necrosis factor-, (TNF-,) in conjunctivae affected by ocular cicatricial pemphigoid (OCP) was investigated. Methods:, Biopsy specimens from the conjunctivae of eight patients with OCP, three patients with atopic keratoconjunctivitis (AKC) and two normal subjects were studied for the expression of TNF-, by immunohistochemistry. Two independent, masked investigators evaluated the specimens. All samples were similarly processed by a third investigator. Results:, No TNF-, was discerned in the normal conjunctival sections; small amounts of TNF-, were observed in the atopic keratoconjunctivitis specimens. TNF-, was present in substantial amounts in conjunctival sections of patients with OCP. The expression of TNF-, was detected in both epithelial and stromal cells of conjunctivae from OCP patients. Conclusions:, The presence of TNF-, in conjunctivae affected by OCP may indicate that this cytokine plays an important role in the production and maintenance of conjuctival inflammation response and subsequent conjunctival scarring in patients with OCP. Further studies clarifying this potential role are warranted. [source]


    A review of high-dose intravenous immunoglobulin (hdIVIg) in the treatment of the autoimmune blistering disorders

    CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2001
    S. Jolles
    High-dose intravenous immunoglobulin (hdIVIg) is being used increasingly for dermatological indications. Its mode of action is via a number of proposed mechanisms and it is not associated with the many side-effects of steroids and other immunosuppressive agents. The evidence for using hdIVIg in the treatment of autoimmune bullous disorders is based on uncontrolled trials and case reports. However, there are now 62 reported patients and this review aims to make a critical assessment of the current data. This has been obtained from a Medline search of the English literature from 1966 to 2000 for pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, pemphigoid gestationis, cicatricial pemphigoid, epidermolysis bullosa acquisita and linear IgA disease. Taken together hdIVIg was effective in 81% of the patients with blistering disease. Patients appear to be more likely to respond when hdIVIg is used as adjunctive therapy (91% response rate) than as monotherapy (56% response rate). hdIVIg may offer a safe potential therapeutic avenue for resistant cases of the autoimmune bullous disorders but should be further assessed using double-blind placebo-controlled trials. [source]


    Cicatricial entropion repair with hard palate mucous membrane graft: surgical technique and outcomes

    CLINICAL & EXPERIMENTAL OPHTHALMOLOGY, Issue 4 2008
    Brighu N Swamy
    Abstract Background:, The use of a hard palate mucous membrane graft (HPMMG) has been previously described for upper and lower eyelid cicatricial entropion repair. The objective of this paper is to review the surgical technique and postoperative complications in a large series of patient who underwent hard palate grafting for the management of cicatricial entropion. Methods:, The medical records of 107 patients representing 147 eyelids undergoing surgical management of cicatricial entropion with HPMMG were reviewed. The surgical technique is described. Results:, 147 eyelid operations (74 upper, 73 lower) were performed on 107 patients (46 male, 61 female), with a mean age of 63 years (range 12,87). The aetiology of the cicatricial entropion included idiopathic (41%), trauma (5.6%), chronic blepharitis (16.8%), chemical injury (3.7%), ocular cicatricial pemphigoid (8.4%), trachoma (7.5%) and other (16.8%). Patients were followed postoperatively for an average of 21 months (range 6,120). Ninety-four per cent of patients noted symptomatic improvement. The postoperative complications included excess keratin (29%), recurrence of cicatricial entropion (4.1%), punctuate epithelial erosion (2.7%), graft shrinkage (0.7%) and donor site bleeding (2.0%). Conclusions:, Cicatricial Entropion with hard palate mucous membrane grafting for both upper and lower eyelid surgery offers high symptomatic and anatomical cure rates. The requirement for further surgical intervention is low. [source]