A case of acne fulminans presenting with systemic symptoms resembling acute appendicitis with maculopapular purpura-like eruption exacerbated by systemic steroids.

Acne fulminans, the most severe form of acne, comprises ulcerative nodulocystic lesions with general, abdominal, and skeletal symptoms acute in onset. In our case, the patient initially had been managed as acute appendicitis. Antibiotic therapy had been administered in full doses with systemic corticosteroids, usually recommended as primary treatment. Few days later, we observed the complete palette of clinical manifestations (vasculitis-like lesions and joint pain) and the classic signs (nodules and pustules on face and chest) allowing the correct diagnosis. Our treatment was peculiar: a systemic antibiotic therapy, based on cephalosporines, proved to be effective.

Ocular involvement in pemphigus.

PURPOSE: To report the occurrence of ocular involvement in the setting of pemphigus and discuss its relationship with disease activity and prognostic significance. DESIGN: Retrospective case reports. METHODS: Five patients, aged 38 to 65 years, diagnosed with pemphigus according to clinical, histopathologic, and immunopathologic criteria (n = 4 pemphigus vulgaris; n = 1 superficial pemphigus) developed ocular symptoms and signs consistent with the disease, ranging from mild conjunctivitis to blisters and prominent erosions of the bulbar/palpebral conjunctiva or at the eyelid margin. RESULTS: Ocular involvement in our series mostly followed skin disease or represented the stigmata of quiescent localized pemphigus. One of five patients had fatal outcome from myocardial infarction, whereas in the remaining cases significant improvement was achieved with oral prednisolone. CONCLUSIONS: Ocular pemphigus is probably underdiagnosed and its frequency appears underestimated. It does not seem to correlate with disease severity, but may persist chronically after healing of cutaneous lesion.

Blue rubber-bleb nevus syndrome: report of a case associated with osteoid osteomas.

Blue rubber-bleb nevus syndrome (BRBNS), or Bean's syndrome, is a rare angiomatosis characterized by multiple cavernous hemangiomas of the skin, mucosae and frequently of other sites. A 49-year-old male patient had been affected since birth by multiple angiomas localized in the skin, lips, oral cavity, cranial theca, and central nervous system; intrauterine rupture of angiomas in the right parietal lobe had caused partial hypotrophic paralysis of the left hemisoma. In addition to BRBNS, the patient was affected by three osteoid osteomas: this never-described clinical association is here discussed.