Idiopathic linear IgA bullous dermatosis treated with prednisone

2021 ◽  
Vol 14 (5) ◽  
pp. e242237
Author(s):  
Christopher Cantoria Garces ◽  
M Fahad Salam ◽  
Brian Nohomovich ◽  
Merryl Treasa Varghese
Keyword(s):  
Case Reports ◽  
Acute Onset ◽  
Direct Immunofluorescence ◽  
Dermoepidermal Junction ◽  
Mucosal Surfaces ◽  
Bullous Dermatosis ◽  
History Of ◽  
Linear Iga Bullous Dermatosis ◽  
Linear Band ◽  
Subepidermal Blister

We present a case of a 43-year-old man with a medical history of paroxysmal atrial fibrillation that presented with acute onset generalised vesiculobullous rash of 1-week duration. The rash was initially noticed on his groin and then spread to his hands, feet and mucosal surfaces. Laboratory tests were unremarkable, including an extensive infection aetiology work-up. Punch biopsies were obtained of a fresh lesion and were stained with H&E and sent for direct immunofluorescence. Light microscopy and immunofluorescence study demonstrated a subepidermal blister with predominant neutrophilic infiltrates and a linear band of IgA at the dermoepidermal junction, respectively. The patient was diagnosed with linear IgA bullous dermatosis and was subsequently treated with 0.5 mg/kg of prednisone daily following previous case reports. At 1-week follow-up as an outpatient, the bullae became crusted, and the rash was nearly completely regressed.

scholarly journals Lichen Planus Pemphigoides Induced by Enalapril: A Case Report and a Review of Literature

2017 ◽  
Vol 9 (3) ◽  
pp. 217-224 ◽  
Author(s):  
Woranit Onprasert ◽  
Kumutnart Chanprapaph
Keyword(s):  
Lichen Planus ◽  
Early Recognition ◽  
Case Reports ◽  
Direct Immunofluorescence ◽  
Drug Induced ◽  
First Case ◽  
Bullous Dermatosis ◽  
Culprit Drug ◽  
History Of ◽  
Hands And Feet

Lichen planus pemphigoides (LPP) is a rare autoimmune bullous dermatosis. The clinical presentation of LPP may mimic bullous pemphigoid making the diagnosis difficult. A thorough clinical, histopathological, and immunological evaluation is essential for the diagnosis of LPP. The etiology is largely idiopathic; however, there are several case reports of drug-induced LPP. We report an 81-year-old Thai woman with underlying hypertension and type 2 diabetes mellitus who presented with a 4-week history of multiple tense bullae initially on the hands and feet that subsequently expanded to the trunk and face. Enalapril was commenced to control hypertension. The histopathology and direct immunofluorescence were compatible with LPP. Circulating anti-basement antibodies BP180 was also positive. The patient was treated with topical corticosteroid with a modest effect. Enalapril was discontinued and complete resolution of LPP occurred within 12 weeks. There was no recurrence after a 1-year follow-up period. To the best of our knowledge, we present the first case of enalapril-induced LPP. Early recognition and prompt discontinuation of the culprit drug allow resolution of the disease. Medication given for LPP alone, without cessation of the offending drug, may not change the course of this condition.

scholarly journals A Rare Case of Vancomycin-Induced Linear Immunoglobulin A Bullous Dermatosis

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Pinky Jha ◽  
Kurtis Swanson ◽  
Jeremiah Stromich ◽  
Basia M. Michalski ◽  
Edit Olasz
Keyword(s):  
Joint Infection ◽  
Immunoglobulin A ◽  
Colchicine Treatment ◽  
Left Knee ◽  
Dermoepidermal Junction ◽  
Common Drug ◽  
Prosthetic Joint ◽  
Bullous Dermatosis ◽  
History Of ◽  
Linear Iga Bullous Dermatosis

Linear IgA bullous dermatosis (LABD) is an autoimmune vesiculobullous disease, which is typically idiopathic but can also rarely be caused by medications or infections. Vancomycin is the most common drug associated with LABD. Lesions typically appear 24 hours to 15 days after the first dose of vancomycin. It is best characterized pathologically by subepidermal bulla (blister) formation with linear IgA deposition at the dermoepidermal junction. Here we report an 86-year-old male with a history of left knee osteoarthritis who underwent a left knee arthroplasty and subsequently developed a prosthetic joint infection. This infection was treated with intravenous vancomycin as well as placement of a vancomycin impregnated joint spacer. Five days following initiation of antibiotic therapy, he presented with a vesiculobullous eruption on an erythematous base over his trunk, extremities, and oral mucosa. The eruption resolved completely when intravenous vancomycin was discontinued and colchicine treatment was begun. Curiously, complete resolution occurred despite the presence of the vancomycin containing joint spacer. The diagnosis of vancomycin-induced linear IgA bullous dermatosis was made based on characteristic clinical and histopathologic presentations.

scholarly journals A Case of Linear IgA Bullous Dermatosis Induced by Aspirin Therapy

2020 ◽  
Author(s):  
Mohammad Nabavi ◽  
Afshin Rezaeifar ◽  
Ali Sadeghinia ◽  
Saba Arshi ◽  
Sima Bahrami ◽  
...  
Keyword(s):  
Direct Immunofluorescence ◽  
Causative Role ◽  
Drug Induced ◽  
Autoimmune Blistering Disease ◽  
Bullous Dermatosis ◽  
Causative Agents ◽  
History Of ◽  
Blistering Disease ◽  
Linear Iga Bullous Dermatosis ◽  
String Of Pearls

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease that may be triggered by some diseases and medications. For the latter one, non-steroidal anti-inflammatory drugs (NSAIDs) have been identified as one of the potential causative agents to develop LABD. Here, a rare case of drug-induced LABD is introduced. A 13-month-old Iranian boy presented with a history of generalized blisters, displaying the classic “string of pearls” sign who was eventually diagnosed as a case of LABD. In his admission, he was diagnosed whit Mucocutaneous lymph node syndrome and treated with aspirin.  Some features like appearing the characteristic lesions one week following the administration of aspirin, rapid clearance of lesions after the withdrawal of the drug, and reappearance of new lesions after readministration of aspirin were highly suggestive of aspirin-induced LABD. To establish the diagnosis, we used the “Naranjo probability score” which determined the probable causative role of aspirin. The diagnosis was confirmed by showing the positive IgA deposition in the basement membrane zone in a direct immunofluorescence study of the skin biopsy. The child was treated with dapsone with dramatical response to the drug.

scholarly journals Linear IGA bullous dermatosis potentially triggered by vaccination

2022 ◽  
Vol 36 ◽  
pp. 205873842110212
Author(s):  
Alberto Corrà ◽  
Veronica Bonciolini ◽  
Lavinia Quintarelli ◽  
Alice Verdelli ◽  
Marzia Caproni
Keyword(s):  
Basement Membrane Zone ◽  
Dermoepidermal Junction ◽  
Pathogenetic Role ◽  
Autoimmune Blistering Disease ◽  
Bullous Dermatosis ◽  
Iga Antibodies ◽  
Blistering Disease ◽  
Adults And Children ◽  
Linear Iga Bullous Dermatosis

Linear IgA bullous dermatosis (LABD) is a mucocutaneous autoimmune blistering disease affecting both adults and children. It is caused by IgA antibodies targeting multiple antigens along the basement membrane zone, leading to disruption of dermoepidermal junction and development of bullous lesions which often presents in characteristic arrangement. Although most LABD cases have been reported to be idiopathic, different triggers have been described, including several drugs and infection. However, the occurrence of vaccine-induced cases of LABD is not widely known and accepted due to the few reports available. We present two cases of LABD occurred following different triggers, rising the suspicion for a possible pathogenetic role of vaccines.

A bullous rash

2020 ◽  
pp. edpract-2020-319179
Author(s):  
Vanessa Migliarino ◽  
Alberto Di Mascio ◽  
Irene Berti ◽  
Andrea Taddio ◽  
Egidio Barbi
Keyword(s):  
Bacterial Culture ◽  
Skin Lesions ◽  
Lower Limbs ◽  
Antibody Detection ◽  
Topical Steroids ◽  
List Type ◽  
Bullous Dermatosis ◽  
History Of ◽  
Linear Iga Bullous Dermatosis ◽  
Severity Of The Disease

A 3-year-old boy presented with a 5-day history of bullous skin lesions localised mainly in the upper and lower limbs and in the genital region (figure 1). Lesions were not pruritic nor painful and showed a central crust. There was no family history of skin disorders or autoimmune diseases. The child never had fever and his physical examination was otherwise unremarkable.Figure 1Bullous skin lesions forming around a central crust, localised in the upper and lower limbs.QuestionsWhat is the most likely diagnosis based on this clinical presentation?Bullous impetigo.Bullous pemphigoid.Linear IgA bullous dermatosis.Dermatitis herpetiformis.What would be the next step in the investigation to confirm your diagnosis?Skin biopsy.Swab test for bacterial culture with an antibiogram.Anti-transglutaminase antibody detection.What is the mainstay of management?Dapsone.Systemic steroids.Topical steroids.All of the above answers are correct, according to the severity of the disease.Answers can be found on page 02.

Other Vesiculobullous Diseases

2020 ◽  
Author(s):  
Aakaash Varma ◽  
Annette Czernik ◽  
Jacob Levitt
Keyword(s):  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Tissue Transglutaminase ◽  
Direct Immunofluorescence ◽  
Cicatricial Pemphigoid ◽  
Positive Serology ◽  
Fixed Drug Eruption ◽  
Varicella Zoster ◽  
Bullous Dermatosis ◽  
Linear Iga Bullous Dermatosis

Less common immunobullous diseases include cicatricial pemphigoid, epidermolysis bullosa acquisita, and linear IgA bullous dermatosis. Diagnosis of these entities are made through direct immunofluorescence, sometimes requires salt-split skin, as well as, in the case of cicatricial pemphigoid, mucosal scarring. As in pemphigus vulgaris and bullous pemphigoid, common therapies include rituximab, prednisone, and IVIg. Dapsone can be particularly effective in linear IgA bullous dermatosis and bullous lupus. Dermatitis herpetiformis is a rare cutaneous manifestation of gluten sensitivity, characterized by pruritic vesicles on extensor surfaces, that responds to dapsone and gluten avoidance. This diagnosis is confirmed with biopsy and positive serology for anti-tissue transglutaminase IgA. Blistering hypersensitivity reactions include TEN, SJS, erythema multiforme, and fixed drug eruption. All are characterized by varying degrees of keratinocyte necrosis. Common to the management of all include cessation of the offending agent. TEN can be managed by cyclosporine, TNF-inhibition, or—more controversially—IVIg. SJS can be effectively managed with systemic steroids. EM responds variably to a number of agents, including antiviral nucleoside analogues, prednisone, thalidomide, apremilast, and tofacitinib. Infectious causes of blisters include Staphylococcus aureus, HSV, and varicella zoster virus. Epidermolysis bullosa comprises a variety of genetically defective structural proteins of the skin. Recessive variants and those affecting deeper proteins carry more severe phenotypes. Management is best achieved at specialty centers and involves careful wound care as well as prevention of friction. Gene therapy is on the horizon for these disorders. Other blistering entities, mechanical or inflammatory in nature, are also discussed at the end of this chapter. This review contains 13 figures, 1 table, and 86 references. Keywords: Blisters, bullae, bullous, pemphigoid, necrolysis

scholarly journals Chronic bullous disease of childhood – A case report / Hronična bulozna bolest Kod Dece – Prikaz Slučaja

2010 ◽  
Vol 2 (4) ◽  
pp. 137-142
Author(s):  
Đorđi Gocev ◽  
Katerina Damevska ◽  
Suzana Nikolovska ◽  
Ljubica Pavlova ◽  
Nada Petrova
Keyword(s):  
First Episode ◽  
Direct Immunofluorescence ◽  
Rapid Progression ◽  
Upper Respiratory Infection ◽  
Bullous Disease ◽  
Systemic Corticosteroids ◽  
Bullous Dermatosis ◽  
The Face ◽  
Linear Iga Bullous Dermatosis

Abstract Linear IgA bullous dermatosis is a chronic, acquired, autoimmune subepidermal vesiculobullous disease. Both children and adults are affected. It is characterized by direct immunofluorescence findings of linear immunoglobulins class A (IgA) deposits along the dermal-epidermal junction (basement membrane zone). In children, the disease is commonly referred to as chronic bullous disease of childhood and it mostly affects children between 2 and 5 years. The onset of the disease is acute; the first episode is the most severe, while recurrences tend to wax and wane in severity and last till puberty or even longer. Diaminodiphenylsulfone is the treatment of choice, although systemic corticosteroids are reported to be very effective as well. We report a 3-year-old boy with a vesiculobullous eruption which developed one week following administration of cephalexin for upper respiratory infection. He was referred to our Clinic from other health institutions as treatment failure for suspected strophulus or impetigo bullosus. On admission, the patient had fever and numerous vesiculobullous and erosive lesions distributed on the face and trunk. After immunohistological verification, the treatment with prednisone 25 mg/d was introduced, due to rapid progression of the disease and the fact that diaminodiphenylsulfone was not available. Improvement occurred after 2 weeks, so the dose was carefully tapered, taking into account the possibility of adrenal suppression. The medication was completely excluded within the next three months. No serious side effects were observed, except transitory hirsutism. The patient has had no relapses over the last 20 months of clinical follow-up.

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