Sunil Narayan Tolat; Anil Gosavi; Vasudha Belgaumkar; Ravindranath Chavan; Nitika Deshmukh; Sweta Tharewal; Nupur Warke
Abstract
Schnitzler’s syndrome is an autoinflammatory disorder presenting with wheals, monoclonal gammopathy, and signs of inflammation. A 55-year-old woman presented with reddish, moderately itchy wheals with intermittent fever and arthralgia for two years. Multiple erythematous, edematous plaques were ...
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Schnitzler’s syndrome is an autoinflammatory disorder presenting with wheals, monoclonal gammopathy, and signs of inflammation. A 55-year-old woman presented with reddish, moderately itchy wheals with intermittent fever and arthralgia for two years. Multiple erythematous, edematous plaques were noted all over the body. Dermographism was present. A diagnosis of chronic urticaria was considered and treated with antihistamines. The patient returned six weeks later with partial symptomatic relief. She was then investigated for the cause of chronic urticaria, and the following differentials were considered: systemic lupus erythematosus (SLE), dermatomyositis, urticarial vasculitis, and auto-inflammatory diseases. The patient was febrile, and her blood investigations revealed leukocytosis and a raised erythrocyte sedimentation rate along with IgM gammaglobulinemia and an M band on serum electrophoresis. Skin biopsy revealed a neutrophilic infiltrate in the dermis. Thus, based on the Strasbourg diagnostic criteria, a final diagnosis of Schnitzler syndrome was made. Urticarial rash is one of the most common complaints encountered by dermatologists. Other extremely uncommon diseases like autoinflammatory disorders (for example, Schnitzler syndrome) can mimic chronic urticaria. The appearance of the rash and associated symptoms should be carefully considered to identify these missed cases. Auto-inflammatory syndromes are severely debilitating, with little awareness among healthcare professionals. Thus, they are often recognized with a diagnostic delay of many years. Early diagnosis of such rare diseases is imperative for effective treatment and to prevent devastating long-term complications.
Anil Prakash Gosavi; Ravindranath Brahmadeo Chavan; Neelamkumari Bhatt; Nitika Deshmukh; Darshana Rajendra Kundale
Abstract
Bullous pemphigoid is the most common type of subepidermalautoimmune bullous disease. It usually affects older people;isolated cases among people younger than 65 have been reportedwith various presentations, usually more severe and active thanthose seen in the elderly. Toxic epidermal necrolysis (TEN) ...
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Bullous pemphigoid is the most common type of subepidermalautoimmune bullous disease. It usually affects older people;isolated cases among people younger than 65 have been reportedwith various presentations, usually more severe and active thanthose seen in the elderly. Toxic epidermal necrolysis (TEN) is apotentially life-threatening dermatologic disorder characterizedby widespread necrosis and bullous detachment of the epidermisand mucous membranes resulting in exfoliation and possiblesepsis. Various morphological variants of bullous pemphigoidhave been reported, with reports of the TEN-like variant ofbullous pemphigoid being scarce. In this study, we report a caseof bullous pemphigoid with TEN-like presentation in a middleagedfemale.
Anil Prakash Gosavi; Ravindranath Brahmadeo Chavan; Rahul Narendra Jaiswal; Vijay Deepak Joshi
Abstract
Methotrexate, an anti-psoriatic drug possessing immunoregulatory, antiproliferative, and anti-inflammatory properties,acts by blocking deoxyribonucleic acid and ribonucleic acid synthesis. It acts especially on rapidly dividing bone marrow cells and proliferating epithelia and mucosae. The toxicity of ...
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Methotrexate, an anti-psoriatic drug possessing immunoregulatory, antiproliferative, and anti-inflammatory properties,acts by blocking deoxyribonucleic acid and ribonucleic acid synthesis. It acts especially on rapidly dividing bone marrow cells and proliferating epithelia and mucosae. The toxicity of this double-edged sword occurs due to accidental excessive intake or daily dosing instead of weekly dosing. We report the case of a 31-year-old male with acute methotrexate toxicity presenting with ulcerations over pre-existing psoriatic plaques with foci of bleeding. Oral mucosal involvement in the form of buccal and palatal ulceration was present in the setting of sepsis, pancytopenia, and acute kidney injury. It is worth emphasizing the role of adequate prescription guidelines, patient education, stringent monitoring, early recognition of tell-tale signs of toxicity, timely leucovorin rescue, and the need for strict enforcement of regulations regarding the over-the-counter availability of such drugs, especially in developing countries.