Neha Robinson

The new pemphigus variants

Pemphigus describes a group of autoimmune diseases characterized by blisters and erosions of the skin and mucous membranes, acantholysis by histology, and autoantibodies directed against epidermal cell surface components. Since the early 1970s, the following new clinical variants of pemphigus have been reported: pemphigus herpetiformis, IgA pemphigus, and paraneoplastic pemphigus. In recent years, significant data have been obtained from laboratory investigation on these rare and atypical variants, especially regarding their specific target antigens. We review these variants, their clinical presentations, histologic findings, immunopathology, target antigens, theories of pathogenesis, treatment modalities, and clinical courses.

Autoimmune blistering skin diseases

Emergency physicians, at the front line of patient care, are often confronted with a wide variety of dermatologic conditions. Prompt recognition is essential, especially for the autoimmune blistering skin diseases, many of which have considerable morbidity and mortality. Therefore, an accurate diagnosis is imperative for appropriate referral and initiation of therapy. This review article provides a concise yet thorough discussion of the clinical presentation, incidence, differential diagnosis and management of the commonly encountered autoimmune blistering skin diseases, some of which include pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita.

Characterization of BALB/c mice B lymphocyte autoimmune responses to skin basement membrane component type XVII collagen, the target antigen of autoimmune skin disease bullous pemphigoid

Bullous pemphigoid is an autoimmune blistering skin disease characterized by IgG autoantibodies targeting the skin basement membrane component type XVII collagen (BPAg2). To gain understanding of the diseases induction phase, we subcutaneously immunized adult BALB/c mice with peptides of human and/or the murine-equivalent BPAg2 pathogenic NC16A domain. Female mice were injected with peptides (human, murine, or combined human and murine), or PBS control emulsified in CFA, on a four-week interval. At the fourth and subsequent immunizations, all peptide-immunized mice were given murine peptides. Two weeks after the sixth immunization, ELISA detected IgG circulating autoantibodies against self peptides in 92% (47/51) of mice immunized with murine peptides; whereas none of the preimmune sera or the sera from PBS control-immunized mice reacted to the self peptides. In four mice their autoantibodies labeled mouse skin basement membrane. Breaking B-cell tolerance to BPAg2 sets the first step in dissecting the diseases induction phase.

Erosive Dermatitis and Progressive Neurological Symptoms—Quiz Case

A 32-year-old morbidly obese man (316 kg, body mass index, 102.7 [calculated as weight in kilograms divided by height in meters squared]) with no medical history presented with a 2-year history of 2 progressively enlarging tumors on his bilateral thighs. These tumors were asymptomatic, but they greatly interfered with ambulation. Physical examination revealed 2 pedunculated, oblong, soft, nontender subcutaneous tumors (Figure 1). The tumors grew from the distal right and proximal left thighs and measured 30 4 5 cm and 102 30 50 cm, respectively. The overlying skin was extremely edematous with several shallow pressure ulcerations. Focal lichenification and erythema were present on the larger tumor, reflecting chronic rubbing on the ground while walking. An intraoperative incisional biopsy specimen of the larger tumor was sent for histologic examination (Figure 2 and Figure 3). Ultrasonography demonstrated fatty tissue, edema, and minimal flow throughout the tumors. What is your diagnosis?