ABSTRACT
BACKGROUND: Many case reports have described the coexistence of autoimmune bullous diseases (AIBDs) and psoriasis. Among them, anti-laminin γ1 (p200) pemphigoid is the best known. OBJECTIVES: We sought to characterize patients with AIBDs and psoriasis and to investigate common AIBDs occurring in these patients. METHODS: This retrospective study included 145 patients with coexisting AIBD and psoriasis given a diagnosis from January 1, 1996, to July 31, 2013, at an academic dermatology department. Of these, 134 were consultation cases regarding AIBD diagnosis. RESULTS: Ratio of male to female patients was 5.7:1. Psoriasis onset preceded AIBD onset in most patients. Mean age at AIBD onset was 65.4 years, and mean duration between psoriasis and AIBD onset was 14.6 years. Most cases had single AIBD, whereas 16 cases had combined AIBDs. Bullous pemphigoid was the most prevalent (63.4%) followed by anti-laminin γ1 pemphigoid (37.2%). LIMITATIONS: Consultation cases may not have included mild AIBD cases. CONCLUSION: This study confirmed the association of psoriasis and anti-laminin γ1 pemphigoid. However, because bullous pemphigoid is a much more common disease, it is seen more frequently in patients with psoriasis than anti-laminin γ1 pemphigoid.
Subject(s)
Autoimmune Diseases/complications , Psoriasis/complications , Skin Diseases, Vesiculobullous/complications , Skin Diseases, Vesiculobullous/immunology , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Retrospective StudiesSubject(s)
ATP-Binding Cassette Transporters/genetics , Arachidonate 12-Lipoxygenase/genetics , Ichthyosis, Lamellar/genetics , Mutation , Asian People/genetics , DNA Mutational Analysis , Gene Frequency , Genetic Markers , Genetic Predisposition to Disease , Humans , Ichthyosis, Lamellar/diagnosis , Ichthyosis, Lamellar/ethnology , Japan/epidemiology , Malaysia/epidemiology , Phenotype , Risk FactorsSubject(s)
Autoantibodies/analysis , Ichthyosis, Lamellar/immunology , Laminin/immunology , Pemphigoid, Bullous/immunology , Skin/immunology , Aged , Biomarkers/analysis , Biopsy , Dermatologic Agents/therapeutic use , Drug Therapy, Combination , Humans , Ichthyosis, Lamellar/diagnosis , Ichthyosis, Lamellar/drug therapy , Male , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy , Skin/drug effects , Skin/pathology , Treatment OutcomeSubject(s)
Pemphigoid, Bullous/immunology , Pemphigoid, Bullous/pathology , Skin/immunology , Skin/pathology , Aged, 80 and over , Autoantigens/immunology , Autoimmunity , Carrier Proteins/immunology , Cytoskeletal Proteins/immunology , Dystonin , Female , Humans , Inflammation/immunology , Inflammation/pathology , Lymphocytes/pathology , Nerve Tissue Proteins/immunology , Neutrophils/pathology , Non-Fibrillar Collagens/immunology , Collagen Type XVIISubject(s)
Autoantibodies/immunology , Autoantigens/immunology , Immunoglobulin A/immunology , Immunoglobulin G/immunology , Non-Fibrillar Collagens/immunology , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/immunology , Fluorescent Antibody Technique, Indirect , Follow-Up Studies , Humans , Infant , Infusions, Intravenous , Lower Extremity , Male , Methylprednisolone/administration & dosage , Pemphigoid, Bullous/diagnosis , Pulse Therapy, Drug , Severity of Illness Index , Thorax , Treatment Outcome , Upper Extremity , Collagen Type XVIISubject(s)
Immunocompromised Host , Ki-1 Antigen/analysis , Molluscum Contagiosum/immunology , Mycosis Fungoides/immunology , Skin Neoplasms/immunology , T-Lymphocytes/immunology , Biopsy , Humans , Immunohistochemistry , Male , Middle Aged , Molluscum Contagiosum/therapy , Molluscum Contagiosum/virology , Mycosis Fungoides/diagnosis , Mycosis Fungoides/therapy , Neoplasm Staging , Skin Neoplasms/diagnosis , Skin Neoplasms/therapy , Steroids/administration & dosage , T-Lymphocytes/virology , Time Factors , Treatment Outcome , Ultraviolet TherapySubject(s)
Chemokine CCL17/blood , Desmocollins/immunology , Desmoglein 1/immunology , Immunoglobulin G/blood , Pemphigus/blood , Pemphigus/therapy , Aged, 80 and over , Anti-Inflammatory Agents/therapeutic use , Dapsone/therapeutic use , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Plasmapheresis , Prednisolone/therapeutic use , RetreatmentSubject(s)
Pemphigoid, Bullous/therapy , Plasmapheresis , Psoriasis/therapy , Aged , Anti-Inflammatory Agents/therapeutic use , Combined Modality Therapy , Cyclosporine/therapeutic use , Humans , Immunoglobulin G/blood , Immunosuppressive Agents/therapeutic use , Laminin/immunology , Male , Pemphigoid, Bullous/blood , Pemphigoid, Bullous/drug therapy , Prednisolone/therapeutic use , Psoriasis/drug therapyABSTRACT
BACKGROUND: Pemphigus shows geographically variable characteristics. OBJECTIVE: To study the clinical and immunologic characteristics of Indian pemphigus patients before and after treatment. METHODS: Twenty-six Indian pemphigus patients were analyzed with regard to age, gender, clinical features, treatments and response, the results of histopathology, direct and indirect immunofluorescence (IF), enzyme-linked immunosorbent assay (ELISA), and immunoblot analyses. RESULTS: There were 22 pemphigus vulgaris (PV) and 4 pemphigus foliaceus (PF) patients. Direct and indirect IF was positive in 95.8% and 56% of patients, respectively. Indices of ELISA were lower in our study. Immunoblot assays detected the 130 kDa desmoglein-3 in 10 PV patients and the 160 kDa desmoglein-1 in 1 PV patient; 190 kDa periplakin was unexpectedly detected in 8 patients. CONCLUSION: Indian pemphigus patients showed several unique characteristics, including younger population, predominance of PV, low ELISA indices, lower sensitivity of indirect IF and immunoblotting, and the presence of the 190 kDa periplakin in nearly one-third of patients.
Subject(s)
Pemphigus/immunology , Pemphigus/pathology , Adult , Age Factors , Anti-Inflammatory Agents/therapeutic use , Cohort Studies , Desmoglein 1/metabolism , Desmoglein 3/metabolism , Female , Humans , Immunologic Factors/therapeutic use , India , Male , Middle Aged , Pemphigus/therapy , Plakins/metabolism , Severity of Illness Index , Young AdultABSTRACT
Lichen planus pemphigoides (LPP) is a rare clinical variant of bullous pemphigoid (BP). A 35-year-old female patient presented to our hospital complaining of pruritic violaceous-colored plaques or papules on the extremities. Tense vesicles were also seen on the soles. Skin biopsies from the papules and vesicles demonstrated lichen planus and BP, respectively. Direct immunofluorescence demonstrated linear IgG and C3 deposition on the basement membrane zone. Indirect immunofluorescence on 1 M NaCl split skin detected IgG reactivity with the epidermal side. Enzyme-linked immunosorbent assay also detected anti-BP180 antibodies. After treatment with oral prednisolone alone had failed, low-dose cyclosporine A (CyA) was added. The clinical symptoms immediately improved and the titer of the anti-BP180 antibodies decreased. Although there is little information about the treatment of recalcitrant LPP, additional CyA appeared to be beneficial.
Subject(s)
Lymphoma, B-Cell/diagnosis , Paraneoplastic Syndromes/diagnosis , Pemphigus/diagnosis , Stevens-Johnson Syndrome/diagnosis , Asian People , Diagnosis, Differential , Enzyme-Linked Immunosorbent Assay , Fluorescent Antibody Technique, Direct , Humans , Immunoglobulin G/analysis , Leukocyte Count , Male , Middle AgedSubject(s)
Adenocarcinoma/immunology , Autoantibodies/blood , Cell Adhesion Molecules/immunology , Membrane Glycoproteins/immunology , Neoplasms, Unknown Primary/immunology , Pemphigoid, Benign Mucous Membrane/immunology , Plakins/immunology , Adenocarcinoma/secondary , Asian People , Carrier Proteins , Cytoskeletal Proteins , Dystonin , Enzyme-Linked Immunosorbent Assay , Fatal Outcome , Fluorescent Antibody Technique, Indirect , Glucocorticoids/therapeutic use , Humans , Lymphatic Metastasis , Male , Middle Aged , Mouth Mucosa/immunology , Mouth Mucosa/pathology , Nerve Tissue Proteins , Pemphigoid, Benign Mucous Membrane/drug therapy , Pemphigoid, Benign Mucous Membrane/pathology , Prednisolone/therapeutic use , KalininABSTRACT
Inherited keratinizing disorders are caused by mutations in the genes encoding cornified cell envelope proteins, enzymes and their inhibitors, adhesion molecules, cytoskeletal proteins and others in the epidermis. These molecules are known to regulate differentiation, proliferation and cell adhesions. Intriguingly, some keratinizing disorders show blistering skin lesions, while some inherited blistering disorders show abnormal keratinization. Therefore, hereditary keratinizing and blistering diseases are closely related and show overlapping genetic backgrounds. In this review, we overviewed keratinizing and blistering disorders in terms of overlapping of the two disease groups. Gene mutations in desmosomal components cause striate keratoderma, Naxos disease, epidermolytic palmoplantar keratoderma and plakophilin deficiency, which first show skin fragility and blisters and later hyperkeratosis. Gene mutations in hemidesmosomal components cause various forms of epidermolysis bullosa, some of which show hyperkeratosis on the nails, palms and soles, in addition to blister formation. Diseases with gene mutations in calcium pump proteins are Darier disease and Hailey-Hailey disease, which show clinicopathological overlaps and develop both keratinizing and blistering skin lesions. Finally, gene mutations in epidermal keratins cause epidermolysis bullosa simplex, epidermolytic ichthyosis, superficial epidermolytic ichthyosis, epidermolytic palmoplantar keratoderma and pachyonychia congenita/focal palmoplantar keratoderma, which show thickening of the palms and soles with underlying blister formation. In general, responsible proteins for diseases developing both keratinizing and blistering conditions are adhesion molecules, calcium pump proteins and keratins, but not connexins, cornified cell envelop proteins, enzymes or inhibitors. It is still unknown how particular keratinizing diseases develop blisters and vice versa.
Subject(s)
Blister/genetics , Epidermis/pathology , Hyperkeratosis, Epidermolytic/genetics , Keratins/genetics , Keratoderma, Palmoplantar/genetics , Skin Diseases/genetics , Arrhythmogenic Right Ventricular Dysplasia/genetics , Calcium/metabolism , Cell Differentiation , Epidermis/metabolism , Epidermolysis Bullosa/genetics , Hair Diseases/genetics , Humans , Keratins/physiology , Mutation , Pemphigus, Benign Familial/geneticsABSTRACT
Psoriasis greatly impacts the health-related quality of life of patients, including any dermatological conditions that are listed in the dermatology life quality index (DLQI). We investigated the relationships between DLQI and the degree of patient satisfaction using questionnaires among psoriasis patients treated only with topical corticosteroids. Patients who were dissatisfied with topical corticosteroids alone and agreed to receive cyclosporin were given low-dose oral cyclosporin. We assessed changes of the DLQI and the psoriasis area and severity index (PASI) scores in patients dissatisfied with treatment during the period of cyclosporin addition. Of 32 enrolled patients, 17 reported dissatisfaction with the current treatment of topical corticosteroids alone. There was a significantly positive correlation between the degree of patient satisfaction questionnaires and the DLQI of these 32 patients. Among the 17 dissatisfied patients, 12 patients agreed to receive additional cyclosporin therapy and five did not. The 12 patients who started on cyclosporin had a significantly lower PASI after 12 weeks than they did at baseline. The DLQI improved significantly after 12 weeks in the cyclosporin-treated patients. The 12 patients who agreed to receive cyclosporin showed a significantly lower DLQI at 12 weeks compared to the five patients who declined the addition of cyclosporin to their treatment. Assessing the degree of patient satisfaction with therapy using a questionnaire could be useful for improving clinical interventions in psoriasis patients. Low-dose oral cyclosporin could be effective in patients who are dissatisfied with topical corticosteroid treatment alone.