Human ; Male ; Child: 6-12 Yrs Old ; Linear Iga Bullous Dermatosis ; Immunoglobulin A ; Skin Diseases

Introduction: Chronic bullous disease of childhood (CBDC) is a rare immune-mediated subepidermal vesiculobullous eruption, characterized by linear IgA deposition along the basement membrane zone of the skin. Although mostly idiopathic, CBDC may be triggered by factors such as infection, and drugs. Clinical and immunohistopathological features of drug-induced cases are heterogeneous and indistinguishable from the idiopathic form. Case report: A two-year-old Filipino male presented with pruritic vesicles and bullae on the back several days after finishing a course of cefuroxime, and cefaclor. Examination revealed multiple tense vesicles and bullae, some coalescing into a rosette pattern with central crusts on the perioral, scalp, neck, back, perineal, and perianal areas. Histopathology showed a subepidermal split with neutrophilic and eosinophilic infiltrates. Direct immunofluorescence revealed strong linear deposition of IgA, and granular deposits of C3 and IgM at the basement membrane zone, thus confirming the di- agnosis of CBDC. Dapsone at 2mg/kg/day was started, with oral prednisolone (1.3mg/kg/day), and cloxacillin syrup (40mg/kg/day). Topical care with betamethasone dipropionate and mupirocin ointment was included. After eight weeks, patient showed significant im- provement with few vesicles and resolved lesions healing with post-inflammatory hyperpigmentation. Conclusion We report a case of a two-year-old male presenting with vesiculobullous lesions after a course of cefuroxime, and cefaclor. As both were given and withdrawn in a period of close proximity, it is difficult to determine the probable culprit drug. Spontaneous resolution upon withdrawal of the suspected drug is variable. Systemic therapy such as dapsone may be necessary for treatment.
Linear IgA Bullous Dermatosis ; Cefaclor ; Cefuroxime

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

No abstract available.
Linear IgA Bullous Dermatosis ; Vancomycin

Linear immunoglobulin (Ig) A bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear IgA deposits at the basement membrane zone visualized with direct immunofluorescence (DIF). Most cases of LABD are idiopathic, but some are drug-induced with vancomycin being the most common causative agent. We herein report a patient presenting with blisters and erosive lesions, primarily in the intertriginous and flexor areas, consistent with a diagnosis of piperacillin-tazobactam-induced LABD based on the patient's clinical course and histopathology, DIF, and in vitro T-cell activation assay (TAA) findings. Only one case of piperacillin-tazobactam-induced LABD has been previously reported. In addition to its rarity, our case was also unique in that the skin lesions occurred in the intertriginous and flexor areas, uncommon locations for typical adult patients with LABD, and TAA strongly suggested an association with the causative drug.
Adult ; Basement Membrane ; Blister ; Diagnosis ; Fluorescent Antibody Technique, Direct ; Humans ; Immunoglobulin A ; Immunoglobulins ; In Vitro Techniques ; Linear IgA Bullous Dermatosis* ; Skin ; Skin Diseases ; T-Lymphocytes* ; Vancomycin

No abstract available.
Linear IgA Bullous Dermatosis* ; Lupus Erythematosus, Systemic*

No abstract available.
Linear IgA Bullous Dermatosis* ; Lupus Erythematosus, Systemic*

BACKGROUND: Linear IgA bullous dermatosis (LABD) is an autoimmune, chronic bullous disease characterized by sub-epithelial bullae with linear IgA deposits along the basement membrane. LABD primarily affects young children and adults. There has been no study on LABD in Korea to date. OBJECTIVE: The purpose of this study was to evaluate the clinical features, laboratory examinations, treatments, and outcomes of Korean LABD patients. Patient characteristics including age at disease onset, gender, medical associations, medications, immunofluorescence findings, disease duration, treatment, and outcome were analyzed. METHODS: A retrospective analysis was conducted on 16 LABD patients diagnosed at Gangnam Severance Hospital between 1999 and 2014. RESULTS: A total of 16 LABD patients were included in the study, 5 children and 11 adults. The mean ages at disease onset in children and adults were 3.2 and 41 years, respectively. Eighty percent of children with LABD showed complete remission. In adults, partial remission was achieved in 36.4%, and complete remission in 54.5% of patients. Two patients were diagnosed with drug-induced LABD, and 2 with ulcerative colitis-associated LABD. CONCLUSION: Our report differed from previous reports in that all 5 children with LABD were male, and the incidence in adults was higher in females than males. Most patients responded well to dapsone and oral prednisolone. Since LABD is rare and can be misdiagnosed as impetigo or bullous pemphigoid, diagnosis by immunofluorescence microscopy is necessary for proper treatment to attain disease remission.
Adult ; Basement Membrane ; Child ; Dapsone ; Diagnosis ; Female ; Fluorescent Antibody Technique ; Humans ; Immunoglobulin A ; Impetigo ; Incidence ; Korea ; Linear IgA Bullous Dermatosis* ; Male ; Microscopy, Fluorescence ; Pemphigoid, Bullous ; Prednisolone ; Retrospective Studies ; Ulcer

Diseases associated with immunoglobulin A (IgA) antibody include linear IgA dermatosis, IgA nephropathy, Celiac disease, Henoch-Schonlein purpura, etc. Although usually idiopathic, IgA antibody is occasionally induced by drugs (e.g., vancomycin, carbamazepine, ceftriaxone, and cyclosporine), malignancies, infections, and other causes. So far, only a few cases of IgA bullous dermatosis coexisting with IgA nephropathy have been reported. A 64-year-old female receiving intravenous ceftriaxone and metronidazole for liver abscess had purpuric macules and papules on her extremities. One week later, she had generalized edema and skin rash with bullae and was diagnosed with concurrent linear IgA dermatosis and IgA nephropathy. After steroid treatment, the skin lesion subsided within two weeks, and kidney function slowly returned to normal. As both diseases occurred after a common possible cause, we predict their pathogeneses are associated.
Carbamazepine ; Ceftriaxone ; Celiac Disease ; Drug Eruptions ; Edema ; Exanthema ; Extremities ; Female ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Kidney ; Linear IgA Bullous Dermatosis ; Liver Abscess ; Metronidazole ; Middle Aged ; Purpura, Schoenlein-Henoch ; Skin ; Skin Diseases* ; Vancomycin

Bullous pemphigoid (BP) is an acquired sub-epidermal immune-mediated blistering disease associated with a humoral and cellular response directed against self-antigens. Childhood BP is uncommon with only <80 reported cases in literature.Often,  lesions of childhood BP are localized rather than widespread; and, acral distribution is more common among infants. Clinically, it is often confused with chronic bullous disease of childhood (CBDC). BP can be differentiated from CBDC by the presence of immunoglobulin (Ig) G antibodies, rather than IgA at the basement membrane zone on direct immunofluorescence (DIF). We present a case of childhood BP in a one-year-old baby girl who presented with CBDC-like lesions. DIF showed IgG and C3 at the basement membrane zone. Treatment given included topical and oral sterioids due to the extent of lesions as well as antihistamine to reduce pruritus. Most of the lesions improved after two weeks and 80% resolved after two month of treatment with no note of scarring. However, continuous monitoring of the patient is advised due to the need for long-term treatment of these patients.

Human ; Female ; Infant ; Autoantigens ; Basement Membrane ; Blister ; Cicatrix ; Histamine H1 Antagonists ; Immunoglobulin A ; Immunoglobulin G ; Linear Iga Bullous Dermatosis ; Pemphigoid, Bullous ; Pruritus