Epidermolysis Bullosa Acquisita Responsive to Dapsone Therapy

2001 ◽  
Vol 5 (5) ◽  
pp. 397-399 ◽  
Author(s):  
Amy P. Hughes ◽  
Jeffrey P. Callen
Keyword(s):  
Epidermolysis Bullosa ◽  
Oral Prednisone ◽  
Epidermolysis Bullosa Acquisita ◽  
Effective Agent ◽  
Complete Control ◽  
Bullous Eruption ◽  
Immunofluorescence Studies ◽  
History Of ◽  
Blistering Disease ◽  
One Year

Background: Epidermolysis bullosa acquisita (EBA) is a chronic subepidermal blistering disease that is frequently resistant to therapy. Objective: A 58-year-old man who had a one-year history of a bullous eruption involving the hands, forearms, trunk, scalp, and oral mucosa. Histopathology revealed a subepidermal bulla, and direct and indirect immunofluorescence studies were consistent with EBA. The patient failed respond to niacinamide and tetracycline and oral prednisone 40 mg per day. Methods: Complete control of his blistering was achieved within two months of initiating oral dapsone, 150 mg per day. Conclusion: Dapsone may be an effective agent for some patients with EBA.

A rare occurrence of epidermolysis bullosa acquisita in a patient with retroviral disease

2017 ◽  
Vol 28 (12) ◽  
pp. 1255-1258
Author(s):  
Zambare Uddhao ◽  
Chhonkar Aditi ◽  
Nayak Chitra ◽  
Tambe Swagata
Keyword(s):  
Hiv Infection ◽  
Epidermolysis Bullosa ◽  
Epidermolysis Bullosa Acquisita ◽  
Rare Occurrence ◽  
Type Vii Collagen ◽  
Blistering Disease

Epidermolysis bullosa acquisita is a chronic subepidermal blistering disease associated with autoimmunity to type-VII collagen within anchoring fibrils located at the dermo-epidermal junction. This entity is rarely reported from India. It can have a variety of presentations. Its occurrence with retroviral disease has rarely been reported. Here, we present a case of epidermolysis bullosa acquisita in a patient with HIV infection.

scholarly journals Pemphigus Vulgaris Confined to the Gingiva: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Mitsuhiro Ohta ◽  
Seiko Osawa ◽  
Hiroyasu Endo ◽  
Kayo Kuyama ◽  
Hirotsugu Yamamoto ◽  
...  
Keyword(s):  
Case Report ◽  
Lichen Planus ◽  
Oral Mucosa ◽  
Pemphigus Vulgaris ◽  
Oral Lesions ◽  
Skin Involvement ◽  
Mucous Membranes ◽  
History Of ◽  
Blistering Disease ◽  
One Year

Pemphigus Vulgaris (PV) is an autoimmune intraepithelial blistering disease involving the skin and mucous membranes. Oral mucosa is frequently affected in patients with PV, and oral lesions may be the first sign of the disease in majority of patients. In some patients, oral lesions may also be followed by skin involvement. Therefore, timely recognition and therapy of oral lesions is critical as it may prevent skin involvement. Early oral lesions of PV are, however, often regarded as difficult to diagnose, since the initial oral lesions may be relatively nonspecific, manifesting as superficial erosions or ulcerations, and rarely presenting with the formation of intact bullae. Lesions may occur anywhere on the oral mucosa including gingiva; however; desquamtive gingivitis is less common with PV than other mucocutaneous conditions such as pemphigoid or lichen planus. This paper describes the case of a patient presenting with a one-year history of painful gingival, who is finally diagnosed as having PV.

Case Report of a 21-Year-Old Man With Epidermolysis Bullosa Acquisita

2018 ◽  
Vol 22 (3) ◽  
pp. 356-358 ◽  
Author(s):  
Janelle Cyr ◽  
Annie Liu ◽  
Danny Ghazarian ◽  
Sanjay Siddha
Keyword(s):  
Epidermolysis Bullosa ◽  
Clinical Findings ◽  
Epidermolysis Bullosa Acquisita ◽  
Direct Immunofluorescence ◽  
Early Age ◽  
Minor Trauma ◽  
Dorsal Hand ◽  
History Of ◽  
Polyclonal Gammopathy ◽  
High Index Of Suspicion

Epidermolysis bullosa acquisita (EBA) is a rare acquired type of mechanobullous disease affecting the dermal-epidermal junction (DEJ) of trauma prone acral surfaces. It manifests as tense vesicles, bullae, and milia and typically heals as atrophic hypo- or hyperpigmented scars. Classic noninflammatory mechanobullous EBA typically presents at a mean age of 48 years. A 21-year-old man presented with a 2-year history of nonpainful papular-vesicular lesions on his hands, knees, and toes after minor trauma to these areas. Physical exam revealed postinflammatory hypopigmented scarring and milia to the bilateral dorsal hands and bilateral extensor elbows and knees, with tense blisters on the dorsal hand and patella regions. Direct immunofluorescence revealed strong linear IgG and IgM with weak focal positivity for IgA and C3 at the DEJ. Blood work revealed an increased diffuse gamma region of 71 g/L (6-13 g/L) on serum protein electrophoresis. Pathology showed a fibrotic underlying dermis, with subepidermal bullae and separation and no significant inflammation. The patient was started on colchicine. This case showcases an unusual early age of presentation for mechanobullous EBA and illustrates the importance of interpreting pathology in the context of clinical findings and maintaining a high index of suspicion for EBA in younger patients who present with classic findings. This case is unique as it is the first report of an association between EBA and polyclonal gammopathy and could be suggestive of chronic inflammation, which would fit with our patient’s chronic history of EBA.

scholarly journals Pemphigus Vulgaris as Oral Mucosal Ulcer Progressive to Skin Lesion:

2014 ◽  
Vol 3 (2) ◽  
pp. 43-47
Author(s):  
SM Anwar Sadat ◽  
Akhter Imam
Keyword(s):  
Public Health ◽  
Pemphigus Vulgaris ◽  
Health It ◽  
Major Threat ◽  
Floor Of The Mouth ◽  
Autoimmune Blistering Disease ◽  
Life Threatening ◽  
History Of ◽  
Blistering Disease ◽  
One Year

Among all the life threatening diseases, autoimmune diseases are among most notorious and complicated diseases. As these diseases have no specific cause or responsible organism, it is therefore very difficult to distinguish and diagnose and thus stands as a major threat for public health. It is assumed that the reason for these diseases may be from genetic to environmental factors, from stress to idiopathic origin. Pemphigus Vulgaris(PV) is such an autoimmune blistering disease that frequently affects the mucous membrane and skin. Very often it starts from oral cavity and Dentists therefore the first to recognize. This paper describes the case of a patient presenting with a one-year history of painful ulcerated gingiva, tongue, floor of the mouth and even on GIT who is finally diagnosed as having PV spreading to generalized skin. DOI: http://dx.doi.org/10.3329/updcj.v3i2.17999 Update Dent. Coll. j: 2013; 3 (2): 43-47

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