Ocular cicatricial pemphigoid isa chronic, progressive disease characterized by cicatricial shrinkage of the conjunctiva, entropion, trichiasis, xerosis, and finally reduced vision from corneal opacification. It is a relatively rare, autoimmune disease. In the early stages, it can be easily overlooked as simple chronic conjunctivitis, so it can be untreated. We report a case of ocular cicatricial pemphigoid that resulted in significant morbidity. We were able to control the progression of cicatrization with aggressive immunosuppressive therapy.
Autoimmune Diseases ; Cicatrix ; Conjunctiva ; Conjunctivitis ; Entropion ; Pemphigoid, Benign Mucous Membrane* ; Pemphigoid, Bullous ; Trichiasis ; Vision, Low

The clinical spectruni of epidermolysis bullosa acquisita(EBA) is much broader than originally thought. Although the full extent of the clinical presenation is still being defined, it is now known that EBA include the followings: a non-inflammatory mechanobullous condition equating wit,h classical EBA; an inflammatory vesiculosullous eruption mimicking bullous pernphigoid; and a mucosal-centered disease with sarring similar to cicatricial pemphigoid. Among the nine cases of EBA, aged between 34 to 70 year-old, seen in recent years, three patients had mechanobullous lesions with skin fragilities and scarrings; three patients had inflammatory bullous eruptions, and three other patient had combined features of mechanobullous/inflammatory bullous lesions. Mucous membrane lesions were recognized in sex cases, and the rnos! frequent site of involvement was the oral mucosae. According to observations of these patients episodes of inflammatory bullous eruptions appeared to be present in seven cases and have been considered as early sympoms of the disease. It has been noted, however, that in two cases lesions develop d as an non-inflammatory mechanobullous from thonset. Based on the ability of EBA to mimick bullous pemphigoid or cicatricial pernphogoid and the fact that such cases have perhaps been missed, we feel EBA is more common than past literature has suggested.
Aged ; Blister ; Cicatrix ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Humans ; Mouth Mucosa ; Mucous Membrane ; Pemphigoid, Benign Mucous Membrane ; Pemphigoid, Bullous ; Skin

Aged ; Arthritis, Rheumatoid ; complications ; Female ; Humans ; Pemphigoid, Benign Mucous Membrane ; etiology

Cicatricial pemphigoid is a chronic autoimmune subepithelial blistering disease that predominantly involves mucous membrane with resultant scar formation. It may involve oral, ocular, nasal, pharyngeal, laryngeal, esophageal, and anogenital mucous membranes. Cicatricial pemphigoid is a heterogenous group of diseases with respect to the autoimmune target antigens including BP180(type XVII collagen), BP230, laminin 5(epiligrin), laminin 6, type VII collagen and other newly described antigen. We describe a patient with cicatricial pemphigoid in whom circulating IgA and IgG autoantobodies against BP180 and BP230 antigens were detected simunltaneously.
Autoantibodies* ; Blister ; Cicatrix ; Collagen Type VII ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Laminin ; Mucous Membrane ; Pemphigoid, Benign Mucous Membrane*

A 21 year-old lady had erythemas and bullae on the trunk for 20 days. Biopsy specimens showed subepidermal blister and deposits of IgG and IgA along the basement membrane zone (BMZ). Her serum antibodies of IgG and IgA were observed to recognize 290/145 kD antigens derived from A431-cell line. The other patient of 62 year-old woman had recurrent oral erosions and erosio-ulcerative plaques on her anterior chest for the past 2 years. Tissue specimens showed subepidermal blister and deposits of IgG and IgA at the BMZ. The patients serum had IgG and IgA antibodies which had binding specificities to the 230 kD pemphigoid antigen. The above two cases of epidermolysis billosa acquisita and cicatricial pemphigoid with IgG and IgA antibodies of comparable titers in each may be rarely encountered ones.
Antibodies* ; Basement Membrane ; Biopsy ; Blister* ; Epidermolysis Bullosa Acquisita ; Erythema ; Female ; Humans ; Immunoglobulin A* ; Immunoglobulin G* ; Middle Aged ; Pemphigoid, Benign Mucous Membrane* ; Pemphigoid, Bullous ; Thorax ; Young Adult

Desquamative gingivitis is characterized by a diffuse erythema of the free and attached gingiva associated with areas of vesiculation, erosion, and desquamation. Desquamative gingivitis is not a distinct disease entity but represents a reaction pattern of the gingiva to various stimuli. Pemphigus vulgaris, cicatricial pemphigoid, and lichen planus may presents as desquamative gingivitis. We observed 3 patients whose disease was limited to the gingiva, and studied them by light and direct immunofluorescence microscope. We classified them according to clinical, histologic, and immunopathologic observations. Identification of the underlying causes of desquamative gingivitis is of utmost importance and is dependent upon clinical, histologic, and immunologic criteria.
Diagnosis, Differential ; Erythema ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Direct ; Gingiva ; Gingivitis* ; Humans ; Lichen Planus ; Pemphigoid, Benign Mucous Membrane ; Pemphigus

Ocular cicatricial pemphigoid (OCP) is a chronic bilateral, blinding, cicatrizing form of conjunctivitis with relapsing and remitting periods. It has strong evidence for an immune type II hypersensitivity that leads to subconjunctival fibrosis and extensive systemic bullae formation. To the best knowledge of the authors, this is the first reported case of direct immunofluorescence (DIF) assay-proven OCP in an elderly Filipino man.

A 68-year-old male presented with bilateral corneal conjunctivalization, symblepharon, ectropion, conjunctival hyperemia testing positive with conjunctival biopsy for basement membrane antibodies with DIF for the left eye, while turning out negative for the right eye. He was managed as a case of OCP, both eyes, and was given topical steroids and antibiotics. Oral Dapsone was started by Dermatology and Rheumatology Services.

OCP is a rare autoimmune and blinding disease. Early diagnosis and prompt treatment are vital as ocular complications permanently affect the quality of life of patients as seen in our patient. DIF assay remains the gold-standard for diagnosis. Systemic immunosuppression is the mainstay of treatment. Adjunctive supportive topical medication may be given to alleviate ocular discomfort. A multidisciplinary approach is essential to provide holistic care to each patient.

Human ; Male ; Aged: 65-79 Yrs Old ; Ocular Cicatricial Pemphigoid ; Pemphigoid, Benign Mucous Membrane ; Direct Immunofluorescence Assay ; Fluorescent Antibody Technique, Direct