Stevens Johnson syndrome/toxic epidermal necrolysis and erythema exsudativum multiforme

In the past, definition and name giving of erythema exsudativum multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis have been confusing. However, after a consensus classification, which separates erythema exsudativum multiforme from Stevens-Johnson syndrome and toxic epidermal necrolysis, it is now generally accepted that Stevens-Johnson syndrome and toxic epidermal necrolysis are variants within a continuous spectrum of severe, potentially fatal, mucocutaneous, mainly adverse drug reactions, whereas erythema exsudativum multiforme is a distinct, generally more mild entity with different clinical signs, mainly precipitated by infections e.g. herpes simplex virus.</p

Drug-induced pemphigoid and linear IgA disease

Drug-induced pemphigoid (DIBP) and drug-induced linear IgA bullous dermatosis (DILAD) can be difficult to differentiate from idiopathic bullous pemphigoid (BP) or mucous membrane pemphigoid (MMP), respectively linear IgA disease (LAD). Possible absence or only minor differences in clinical, histopathologic and immunopathologic features complicate the recognition. However, differentiation can be of major importance because of a different approach, prognosis, and treatment. Diagnosis is mainly based on a clear time-relation between start of the suspected drug(s) and onset of the lesions, but can be complicated by polypharmacy and comorbidity, especially in the elderly. After withdrawal of the culprit drug, both DIBP and DILAD tend to be self-limiting. With the introduction of immune checkpoint inhibitors in treatment of malignancies, pemphigoid variants may present as an immune-related adverse event, leading to a dilemma in treatment choices.</p

Drug-induced pemphigus

Drug-induced pemphigus can be induced or triggered by drugs, most often by penicillamine and other thiol-associated drugs. The time from first intake of the drug to onset of the reaction is often relatively long compared to other cutaneous adverse drug reactions. Clinically it presents most often as pemphigus vulgaris or pemphigus foliaceus. Opposing to idiopathic pemphigus, pruritus, a prodromal stage and absence of mucosal involvement are regularly observed, while immunopathological findings are similar. The pathogenesis is not completely known, but probably includes endogenous and exogenous factors, and is likely based on both a biochemical and immunological process. Withdrawal of the culprit drug is mandatory and can result in remission, although the disease not always subsides, especially in cases caused by non-thiol drugs. Additionally, drugs may also cause a flare or exacerbation of pre-existing pemphigus.</p

Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis

Mortality in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high. Apart from intensive supportive therapy, no generally accepted specific treatment regimen exists. The role of corticosteroids in SJS/TEN is controversial. It is possible that high-dose pulse therapy with corticosteroids might be an improvement on long-term lower dose therapy, by combining higher efficacy with a diminished risk both of infection and of delayed wound healing. The aim of this study was to evaluate the efficacy of dexamethasone pulse therapy with respect to mortality and healing time of patients with SJS/TEN. A small, uncontrolled series of consecutive inpatients with SJS/TEN was treated with dexamethasone pulse therapy. The efficacy of this treatment was assessed retrospectively using SCORTEN. Twelve patients were included over a period of 10 years. One patient died, while SCORTEN predicted a fatal outcome of 4 patients. Stabilization was reached after 2.3 days on average, total re-epithelialization after 13.9 days. The results of this study bear no statistical relevance due to the small number of patients. In conclusion, short-term dexamethasone pulse therapy, given at an early stage of the disease, may contribute to a reduced mortality rate in SJS/TEN without increasing healing time. A larger controlled trial is warranted to investigate further the use of dexamethasone pulse therapy in SJS/TEN

Acute generalized exanthematous pustulosis caused by morphine, confirmed by positive patch test and lymphocyte transformation test

Morphine, an opium alkaloid, frequently causes side effects such as hyperhidrosis and facial flushing, but serious cutaneous adverse drug reactions are seldom observed. Best known are Urticaria, erythema, and pruritus; sometimes pseudoallergic anaphylactoid reactions, and blisters are reported