INTRODUCTION: Linear IgA bullous disease (LABD) is a rare autoimmune blistering disease characterized by subepithelial bullae and linear IgA deposition along the basement membrane zone of the epidermis. Lesions present as pruritic papulovesicles and tense bullae which may coalesce forming annular or polycyclic urticarial plaques with blistering on the edge of the lesions forming the classic “string of pearls” sign. Lesions may affect the face, trunk, and extensor extremities. Incidence rates range from 0.5 to 2.3 cases per million individuals per year. Due to its rare occurrence, there are only a fewdocumented reports on cases of LABD, particularly in the Filipino population. CASE REPORT: A 33 year-old Filipino female consulted because of a 3-week history of severely pruritic vesicles and crusts on the face, trunk, and arms. Patient noted no gastrointestinal symptoms on consultation. Skin punch biopsy revealed subepidermal blisters with collection of neutrophils at the dermal papillae. Direct immunofluorescence showed strong (+2) deposits of linear IgA at the dermo- epidermal junction in perilesional skin thus validating the diagnosis. The patient’s serum was negative for IgA anti-tissue transglutaminase and IgA antiendomysial antibodies. Patient was treated with topical corticosteroids and Dapsone 50 mgs OD with remarkable improvement. CONCLUSION: Linear IgA bullous disease has very few reported cases especially in the Philippine setting. Dapsone is considered the first-line treatment. The disease may persist for a decade or longer, and relapses may occur. Careful history-taking accompanied by the histological, immunofluorescence, and serological findings must be done to ensure proper treatment and good prognosis.
Dermatitis Herpetiformis ; Linear IgA Bullous Dermatosis ; Immunoglobulin A

PURPOSE: Autoimmunity is an alternative etiology of gastric inflammation, the initiating event in the gastric carcinogenic cascade. This mechanism may be an increasingly important cause of gastric cancer with the waning prevalence of its primary etiologic factor, chronic Helicobacter pylori infection. MATERIALS AND METHODS: PubMed and EMBASE were searched up to September 2018. Autoimmunity and 96 specific manifestations were considered for associations with gastric cancer risk. Random effects analysis was used to calculate pooled relative risk estimates (RR) and 95% confidence intervals (CI). RESULTS: We found a total of 52 observational studies representing 30 different autoimmune diseases. Overall, the presence of an autoimmune condition was associated with a gastric cancer pooled RR of 1.37 (95% CI, 1.24 to 1.52). Among the 24 autoimmune conditions with two or more independent reports, nine were significantly associated with increased gastric cancer risk: dermatomyositis (RR, 3.69; 95% CI, 1.74 to 7.79), pernicious anemia (RR, 2.84; 95% CI, 2.30 to 3.50), Addison disease (RR, 2.11; 95% CI, 1.26 to 3.53), dermatitis herpetiformis (RR, 1.74; 95% CI, 1.02 to 2.97; n=3), IgG4-related disease (RR, 1.69; 95% CI, 1.00 to 2.87), primary biliary cirrhosis (RR, 1.64; 95% CI, 1.13 to 2.37), diabetes mellitus type 1 (RR, 1.41; 95% CI, 1.20 to 1.67), systemic lupus erythematosus (RR, 1.37; 95% CI, 1.01 to 1.84), and Graves disease (RR, 1.27; 95% CI, 1.06 to 1.52). CONCLUSION: Our analysis documents the wide range of autoimmune diseases associated with gastric cancer. These associations may reflect unreported links between these conditions and autoimmune gastritis. Further studies are warranted to investigate potential causal mechanisms.
Addison Disease ; Anemia, Pernicious ; Autoimmune Diseases ; Autoimmunity ; Dermatitis Herpetiformis ; Dermatomyositis ; Diabetes Mellitus ; Epidemiology ; Gastritis ; Graves Disease ; Helicobacter pylori ; Inflammation ; Liver Cirrhosis, Biliary ; Lupus Erythematosus, Systemic ; Prevalence ; Stomach Neoplasms

OBJECTIVE: Dapsone (diaminodiphenyl sulfone, DDS) is currently used to treat leprosy, malaria, dermatitis herpetiformis, and other diseases. It is also used to treat pneumocystis pneumonia and Toxoplasma gondii infection in HIV-positive patients. The most common adverse effect of DDS is methemoglobinemia from oxidative stress. Ascorbic acid is an antioxidant and reducing agent that scavenges the free radicals produced by oxidative stress. The present study aimed to investigate the effect of ascorbic acid in the treatment of DDS induced methemoglobinemia. METHODS: Male Sprague-Dawley rats were divided into three groups: an ascorbic acid group, a methylene blue (MB) group, and a control group. After DDS (40 mg/kg) treatment via oral gavage, ascorbic acid (15 mg/kg), MB (1 mg/kg), or normal saline were administered via tail vein injection. Depending on the duration of the DDS treatment, blood methemoglobin levels, as well as the nitric oxide levels and catalase activity, were measured at 60, 120, or 180 minutes after DDS administration. RESULTS: Methemoglobin concentrations in the ascorbic acid and MB groups were significantly lower compared to those in the control group across multiple time points. The plasma nitric oxide levels and catalase activity were not different among the groups or time points. CONCLUSION: Intravenous ascorbic acid administration is effective in treating DDS-induced methemoglobinemia in a murine model.
Animals ; Ascorbic Acid* ; Catalase ; Dapsone ; Dermatitis Herpetiformis ; Free Radicals ; Humans ; Leprosy ; Malaria ; Male ; Methemoglobin ; Methemoglobinemia* ; Methylene Blue ; Nitric Oxide ; Oxidative Stress ; Plasma ; Pneumonia, Pneumocystis ; Rats* ; Rats, Sprague-Dawley ; Tail ; Toxoplasmosis ; Veins

BACKGROUND: Dermatitis herpetiformis (DH) is a rare autoimmune blistering disease characterized by pruritic papulovesicular eruptions. It can be differentiated from other vesiculobullous skin diseases by skin biopsy with direct immunofluorescence. DH is most often associated with gluten enteropathy (celiac disease) also an uncommon disease.

CASE PRESENTATION: We report a case of a 20-year-old male with a five-week history of multiple vesicles and bullae associated with fever and body malaise. Lesions were symmetrically found on the entire face, axillae, trunk, buttocks and lower extremities. Hematology revealed leukocytosis with neutrophilic predominance. Skin punch biopsy revealed subepidermal blister with neutrophilic predominance. A direct immunofluorescence (DIP) of the perilesional skin showed granular IgA staining at the tips of the dermal papillae, consistent of DH.

TREATMENT AND OUTCOME: The patient was started with cloxacillin and mupirocin ointment for the infected skin lesions. Dapsone 100 mg daily was started with significant improvement of lesions after seven days. Endoscopy was suggested to investigate for gluten enteropathy, however patient did not consent. The patient was advised on gluten-free diet (GED). Complete resolution of lesions with postinflamamtory hypo- and hyperpigmented scarring were noted after nine weeks of dapsone therapy.

CONCLUSION: Bullous skin lesions present as a great challenge to non-dermatologists, hence a thorough investigation was clearly of utmost importance in arriving at a diagnosis of DH in this case. Internists must be aware that skin lesions could provide clues of other internal diseases, such as in DH and gluten enteropathy. Treatment of DH can be as simple as diet modification and avoidance of gluten¬containing food.

Human ; Male ; Adult ; Diet, Gluten-free ; Dermatitis Herpetiformis ; Glutens ; Celiac Disease ; Blister ; Mupirocin ; Dapsone ; Cicatrix ; Fluorescent Antibody Technique, Direct ; Leukocytosis ; Buttocks ; Cloxacillin

OBJEVTIVETo investigate the expression of transient receptor potential lvanilloidreceptor 4 (TRPV4) protein in pemphigus vulgaris (PV), bullous pemphigoid (BP), dermatitis herpetiformis (DH), and epidermolysis bullosa acquisita (EBA), and explore the role of TRPV4 in the pathogenesis of these diseases.

METHODSTRPV4 protein in normal skin tissues and lesions of PV, BP, DH, and EBA were detected with immunohistochemistry.

RESULTSThe positivity rate of TRPV4 protein expression was 61.90% in PV, 81.81% in BP, 72.22% in DH, and 68.42% in EBA. TRPV4-positive rates in these lesions were significantly lower than the rate in normal skin tissues (93.33%) and also differed significantly among these lesions (PV

CONCLUSINLow TRPV4 expressions may affect the formation and reconstitution of skin connection. TRPV4 may play an role in the occurrence and development of autoimmune bullous skin disorders.

Dermatitis Herpetiformis ; metabolism ; Diagnosis, Differential ; Epidermolysis Bullosa Acquisita ; metabolism ; Humans ; Pemphigoid, Bullous ; metabolism ; Pemphigus ; metabolism ; Skin ; pathology ; TRPV Cation Channels ; metabolism

Aged ; Anti-Bacterial Agents ; therapeutic use ; Dermatitis Herpetiformis ; drug therapy ; Female ; Humans ; Minocycline ; therapeutic use ; Niacinamide ; therapeutic use ; Pemphigus ; drug therapy

Anti-p200 pemphigoid is an autoimmune subepidermal bullous disease characterized by circulating and tissue-bound autoantibodies directed against a 200 kD protein of the human dermis. We report a 78-year-old male who was presented with bullous eruptions on the trunk, extremities, which clinically resemble bullous pemphigoid, epidermolysis bullosa aquisita, linear IgA dermatosis or dermatitis herpetiformis. Oral muscosa of the lower lip was also affected. Histopathological examination of a skin biopsy specimen from the trunk revealed subepidermal blister and infiltration predominantly by neutrophils. Direct immunofluorescence revealed linear deposits of IgG, C3 and IgA at the basement membrane zone. Indirect immunofluorescence using salt-split skin showed that IgG antibodies bound on the dermal side. Immunoblotting with dermal extracts showed that the patient's IgG autoantibodies reacted with a 200 kD protein. The patient showed good response to dapsone.
Aged ; Antibodies ; Autoantibodies ; Basement Membrane ; Biopsy ; Blister ; Dapsone ; Dermatitis Herpetiformis ; Dermis ; Epidermolysis Bullosa ; Extremities ; Fluorescent Antibody Technique, Direct ; Fluorescent Antibody Technique, Indirect ; Humans ; Immunoblotting ; Immunoglobulin A ; Immunoglobulin G ; Linear IgA Bullous Dermatosis ; Lip ; Male ; Neutrophils ; Pemphigoid, Bullous ; Skin

Anti-p200 pemphigoid is an autoimmune subepidermal bullous disease characterized by circulating and tissue-bound autoantibodies directed against a 200 kD protein of the human dermis. We report a 78-year-old male who was presented with bullous eruptions on the trunk, extremities, which clinically resemble bullous pemphigoid, epidermolysis bullosa aquisita, linear IgA dermatosis or dermatitis herpetiformis. Oral muscosa of the lower lip was also affected. Histopathological examination of a skin biopsy specimen from the trunk revealed subepidermal blister and infiltration predominantly by neutrophils. Direct immunofluorescence revealed linear deposits of IgG, C3 and IgA at the basement membrane zone. Indirect immunofluorescence using salt-split skin showed that IgG antibodies bound on the dermal side. Immunoblotting with dermal extracts showed that the patient's IgG autoantibodies reacted with a 200 kD protein. The patient showed good response to dapsone.
Aged ; Antibodies ; Autoantibodies ; Basement Membrane ; Biopsy ; Blister ; Dapsone ; Dermatitis Herpetiformis ; Dermis ; Epidermolysis Bullosa ; Extremities ; Fluorescent Antibody Technique, Direct ; Fluorescent Antibody Technique, Indirect ; Humans ; Immunoblotting ; Immunoglobulin A ; Immunoglobulin G ; Linear IgA Bullous Dermatosis ; Lip ; Male ; Neutrophils ; Pemphigoid, Bullous ; Skin

Anti-p200 pemphigoid is a recently defined subepidermal immunobullous disease. Most anti-p200 pemphigoid patients show the clinical features of bullous pemphigoid, dermatitis herpetiformis and linear IgA disease. It is characterized by the binding of circulating IgG autoantibodies to the dermal side of 1 M NaCl split skin, and by reactivity of these autoantibodies to a unique 200-kD antigen on immunoblot of dermal extract. On immunoelectron microscopic examination, these autoantibodies deposit at the lamina lucida-lamina densa interface. Herein, we report two cases of anti-p200 pemphigoid, as well as their favorable response to treatment with systemic corticosteroid, colchicine and dapsone.
Autoantibodies ; Colchicine ; Dapsone ; Dermatitis Herpetiformis ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Pemphigoid, Bullous ; Skin

Adolescent ; Dermatitis Herpetiformis ; complications ; Esophageal Diseases ; etiology ; Humans ; Male ; Mucous Membrane ; pathology