RESUMO
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.
Assuntos
Síndromes Mielodisplásicas , Pioderma Gangrenoso , Idoso , Ponte de Artéria Coronária , Humanos , Inflamação , Masculino , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/terapia , Esterno/cirurgiaRESUMO
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 ectodomain. In patients with pemphigus foliaceus, the circulating antidesmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus bandlike appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon.