Noninfectious sternal wound inflammation after coronary artery bypass grafting in a patient with myelodysplastic syndrome: A no‐touch approach

Pyoderma gangrenosum (PG) is a rare, chronic inflammatory noninfectious dermatosis. It is associated with underlying systemic or hematological diseases such as myelodysplastic syndrome (MDS) and can be triggered after surgery. Recognition and diagnosis of PG can be difficult as it can mimic a wound infection. Misdiagnosis could lead to invasive procedures which worsen the disease and have possible disastrous aftermath. A 74‐year‐old male with a history of MDS presents with an atypical sternal wound inflammation. Diagnosis confirmed PG after skin biopsy. No surgical or invasive procedures were performed and the patient was treated on an outpatient basis with prednisolone, clobetasol cream, and cyclosporine. This case shows the importance of a rapid diagnosis of the disease. Awareness is required for the diagnosis of PG in a wound with pronounced livid borders, without improvement after antibiotic treatment or worsening after debridement. Rapid diagnosis and treatment reduce high healthcare costs, morbidity, and mortality

Paraneoplastic pemphigus

Paraneoplastic pemphigus is a rare but severe potentially fatal autoimmune disease characterized by severe stomatitis and a variety of cutaneous manifestations in association with an underlying neoplasia. Pulmonary involvement may also occur. The pathogenesis involves the production of autoantibodies against desmogleins, plakins and the protease inhibitor alpha-2 macroglobuline like-1, but T-cell mediated autoimmunity is also thought to play a role. Diagnosis usually relies on the demonstration of a specific subset of circulating autoantibodies in patient serum, although in a small subset of patients these autoantibodies might be absent. Due to it's rarity, there are no guidelines for the treatment of PNP. The general approach includes a variety of immunosuppressive agents and treatment of the underlying neoplasia. Despite treatment, paraneoplastic pemphigus has high mortality rates, often due to sepsis, respiratory failure or progression of the underlying malignancy.</p

The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases

Background: Pemphigus foliaceus is an autoimmune skin disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the skin, these antibodies deposit intraepidermally. On rare occasions, an additional "lupus band" of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations: We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of skin lesions. Direct immunofluorescence of skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions: High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectoclomain. In patients with pemphigus foliaceus, the circulating anti desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon. Arch Dermatol, 2012;148(10):1173-1178. Published online July 16, 2012. doi:10.1001/archdermatol.2012.189