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Results for “Erythema Multiforme”

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PubMed2001

Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia.

Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia. Clinical manifestations are polymorphous, and include erythema, bullae, erythema multiforme-like lesions and severe mucous membrane involvement. PNP manifesting as lichenoid dermatitis has recently been observed. We describe two Italian men with fatal PNP featuring typical PNP autoantigens associated with chronic B-cell lymphocytic leukaemia. The first patient presented with an extensive blistering eruption, several erythema multiforme-like lesions and severe mucosal involvement. The second patient presented with a lichenoid dermatitis, then developed bullae, and died with an erythrodermic and exfoliative dermatosis resembling pemphigus foliaceus. Our patients represent two Italian cases of well-documented PNP. In patient 2, the sequence of clinical presentations was unique, and strongly supports the hypothesis of epitope spreading through chronic lichenoid inflammation of the dermo-epidermal junction exposing new self antigens, leading to the humoral response characteristic of PNP.

Fatal Outcome↗
PubMed2001

[Stevens-Johnson syndrome, unusual urologic involvement].

Stevens-Johnson Syndrome is an adverse drug reaction, considered a severe type of erythema exsudativum multiforme, fatal in about 5%. Characterized by erythema with vesiculobullous lesions in skin and mucous membranes. Implicated drugs are mainly anticonvulsivants and sulphonamides. Prompt recognition and withdrawal of the suspected drug is essential in treatment. Denuded skin areas must be treated with the same principles of burns. Systemic immunosuppression, mainly corticosteroids, remain controversial. We report a case of Stevens-Johnson Syndrome in a 43 year-old male who developed disuria and genital lesions with a detachment of foreskin and glans penis.

Adult↗
PubMed2003

Toxic epidermal necrolysis and Stevens-Johnson syndrome are induced by soluble Fas ligand.

The pathogeneses of toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS), both severe blistering diseases usually associated with drug intake, are not fully elucidated. Histologically, both TEN and SJS are characterized by extensive keratinocyte apoptosis. Previous studies have shown that keratinocyte apoptosis in TEN and SJS was induced by a suicidal interaction between Fas and Fas ligand (FasL), which are both expressed by keratinocytes. However, our preliminary examinations demonstrated that FasL is hardly detected on keratinocytes. We hypothesized that soluble FasL (sFasL) is secreted by peripheral blood mononuclear cells (PBMCs), and this interacts with the Fas expressed on keratinocytes in TEN and SJS. To justify this hypothesis, we investigated whether sFasL secreted by PBMCs could induce the keratinocyte apoptosis in TEN and SJS. Enzyme-linked immunosorbent assay analysis demonstrated that there was no significant sFasL increase in any samples of healthy controls (<40 pg/ml, n = 14) and patients with an ordinary erythema multiforme-type drug eruption (41.5 +/- 3.1 pg/ml, n = 14), whereas high concentrations are detected in all samples of TEN and SJS patients (TEN: 131.5 +/- 57.4 pg/ml, n = 8; SJS: 119.1 +/- 41.0 pg/ml, n = 14) (P < 0.0001). In vitro analysis using cultured keratinocytes revealed that the sera of TEN and SJS patients induced abundant keratinocyte apoptosis compared to erythema multiforme-type drug eruption sera. Furthermore, on stimulation with the causal drug, PBMCs obtained from TEN and SJS patients secreted high levels of sFasL. Taken together, these results indicate that sFasL secreted by PBMCs, not keratinocytes, plays a crucial role in the apoptosis and pathomechanism of TEN and SJS, and that the serum sFasL level may be a good indicator for the early diagnosis of TEN and SJS.

Adult↗
PubMed2003

[Cutaneous xanthomas associated with minocycline-induced cholestatic jaundice].

Minocycline is an effective treatment of acne vulgaris, especially for inflammatory forms. Prescription rates have increased in recent years accompanied by a number of reports concerning drug-induced side effects. An otherwise healthy woman developed an erythema multiform-like rash and and toxic hepatic damage causing cholestatic jaundice following long-term minocycline use. Unusual cutaneous lipid deposition also developed. Minocycline-induced side effects are reviewed.

Acne Vulgaris↗

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