The chameleon rash: a review of the polyphenotypic dermatoses of dermatomyositis.

Dermatomyositis (DM) is an autoimmune connective tissue disease that is included in the idiopathic inflammatory myopathies. Cutaneous manifestations are a prominent part of the condition: some skin signs in DM are common to most patients, while other signs are encountered infrequently. A number of features are pathognomic for DM. The demonstration of myositis-specific antibodies (MSAs) in DM has extended the ability to define phenotypic subgroups. It appears that the presence of certain MSAs confers susceptibility to specific clinical features, an association which reveals a serotype-phenotype relationship. In this review article we have provided a detailed summary of common and under-recognized cutaneous manifestations of DM.

Contrast media and cutaneous reactions. Part 1. Immediate hypersensitivity reactions to contrast media and gadolinium deposition.

Contrast media (CM) are commonly used worldwide to enhance the quality of imaging, which is invaluable for diagnostic accuracy. Adverse reactions to CM are uncommon, but frequently involve cutaneous symptoms. This two-part article reviews adverse events secondary to CM that are relevant to the practising dermatologist. Part 1 will classify CM, address immediate hypersensitivity reactions and review the newly described condition, gadolinium deposition disease. Part 2 will cover the delayed hypersensitivity reactions of iodinated contrast medium including severe cutaneous adverse reactions and iododerma.

Contrast media and cutaneous reactions. Part 2: Delayed hypersensitivity reactions to iodinated contrast media.

Contrast media (CM) are an indispensable part of modern medical imaging. Adverse reactions to CM are uncommon, but frequently involve cutaneous symptoms. This two-part article reviews adverse events secondary to CM that are relevant to the practising dermatologist. Part 1 covers the classification of CM, immediate hypersensitivity reactions to CM and the newly described condition, gadolinium deposition disease. Given that there has only been two case reports to our knowledge of a delayed adverse reaction to gadolinium-based CM, this second part will focus on cutaneous delayed reactions caused by iodinated CM (ICM). Delayed hypersensitivity reactions to ICM commonly present as maculopapular exanthems, but more rarely, they can manifest as fixed drug eruptions, acute generalized exanthematous pustulosis, drug-related eosinophilia and systemic symptoms, Stevens-Johnson syndrome/toxic epidermal necrolysis, symmetrical drug-related intertriginous and flexural exanthema, graft-versus-host disease, vasculitis and iododerma. Delayed reactions to ICM may be underdiagnosed, as cutaneous symptoms may be attributed to oral medications, particularly if patients are on multiple drugs.

Malnutrition and skin disease in Far East prisoners-of-war in World War II.

During the Second World War, thousands of captured British and Commonwealth troops were interned in prisoner-of-war (POW) camps in the Far East. Imprisonment was extremely harsh, and prisoners developed multiple pathologies induced by physical hardship, tropical infections and starvation. Immediately after the war, several POW doctors published their clinical experiences, including reports of skin disease caused by malnutrition. The most notable deficiency dermatoses seen in Far East POWs were ariboflavinosis (vitamin B2 or riboflavin deficiency) and pellagra (vitamin B3 or niacin deficiency). A lack of vitamin B2 produces a striking inflammatory disorder of scrotal skin. Reports of pellagra in POWs documented a novel widespread eruption, developing into exfoliative dermatitis, in addition to the usual photosensitive dermatosis. A review of the literature from 70 years ago provides a reminder of the skin's response to malnutrition.

Long-term complications of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN): the spectrum of chronic problems in patients who survive an episode of SJS/TEN necessitates multidisciplinary follow-up.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe adverse reactions to drugs that cause a life-threatening eruption of mucocutaneous blistering and epithelial sloughing. While the acute complications of SJS/TEN are well described, it is increasingly recognized that survivors may develop delayed sequelae, some of which can be associated with significant morbidity. Studies of long-term SJS/TEN outcomes mostly focus on mucocutaneous and ocular complications. However, other internal organs, such as the respiratory tract and gastrointestinal tract, can be affected. Psychological sequelae are also frequent following the trauma of widespread epidermal necrolysis. An appreciation of the 'chronic' phase of SJS/TEN is needed by clinicians caring for individuals who have survived the acute illness. This review aims to provide an update on the breadth and range of sequelae that can affect patients in the months and years following an acute episode of SJS/TEN.

Generalized pustular psoriasis associated with Epstein-Barr virus.

Generalized pustular psoriasis (GPP) is a rare and severe variant of psoriasis. We report a case of a 79-year-old woman who presented with generalized pustular psoriasis and significant Epstein-Barr virus (EBV) viraemia. Serial measurements of EBV DNA showed a correlation with the deterioration in her clinical condition. We speculate that EBV reactivation triggered the development of GPP, and propose that further investigation is required into the association between EBV and GPP.

Disseminated coxsackievirus A6 affecting children with atopic dermatitis.

Coxsackievirus A6 (CV-A6) is an emerging pathogen that has in recent years been associated with atypical hand, foot and mouth disease. This manifests as a generalized papular or vesicular eruption, which may be associated with fever and systemic disturbance. We report a series of six children presenting to a single centre in the UK with disseminated CV-A6 infection on a background of atopic dermatitis (AD). Our patients exhibited a widespread papular or vesicular eruption in association with exacerbation of AD. Several of our cases mimicked eczema herpeticum, but the extent was more generalized, and individual lesions were discrete rather than clustered and were less circumscribed in character. This series highlights that CV-A6 infection may be encountered in the UK, and should be considered in the differential diagnosis of an acute exacerbation of AD, particularly in children.

Outcomes of jaundice in advanced hepatocellular carcinoma - a sub-Saharan perspective.

BACKGROUND: Jaundice is a marker of advanced disease and poor outcomes in hepatocellular carcinoma (HCC). The aim of this study was to describe and analyse the management and outcomes of jaundiced HCC patients at a large academic referral centre in sub-Saharan Africa (SSA). METHODS: Treatment-naïve adult HCC patients who presented with jaundice between 1990 and 2023 were analysed. RESULTS: During the inclusion period, 676 HCC patients were treated at Groote Schuur Hospital. The mean age of the 126 (18.6%) who were jaundiced was 48.8 (± 13.2) years. Eighty-nine (70.6%) were male. Ninety-four (74.6%) patients with jaundice secondary to diffuse tumour infiltration had best supportive care (BSC) only. Thirty-two had obstructive jaundice (OJ); four were excluded because of missing hospital records. In 28 of these patients, 16 underwent biliary drainage (BD) and 12 received BSC only. The mean overall survival (OS) of the 126 patients was 100.5 (± 242.3) days. The patients with diffuse tumour infiltration had an OS of 105.9 (± 273.3) days. The patients with OJ survived 86.5 (± 135.0) days. There was no significant difference in OS between the three patient groups (p = 0.941). In the OJ group, patients who underwent BD survived longer than the BSC group (117.9 ± 166.4 vs. 29.2 ± 34.7 days, p = 0.015).

Drug reaction with eosinophilia and systemic symptoms: is cutaneous phenotype a prognostic marker for outcome? A review of clinicopathological features of 27 cases.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) describes a heterogeneous group of severe adverse reactions to medications. The cutaneous phenotype has a number of guises, accompanied by a variety of systemic features including fever, haematological abnormalities and visceral involvement, most commonly the liver. Clinical markers of prognosis have not been identified. OBJECTIVES: To assess the cutaneous signs and dermatopathological features of DRESS in order to identify potential prognostic markers. METHODS: We reviewed the clinical features, dermatopathology and outcomes of 27 consecutive cases of DRESS presenting to a single unit. RESULTS: Four distinct patterns of cutaneous involvement were identified: an urticated papular exanthem (13/27 patients), a morbilliform erythema (three of 27), an exfoliative erythroderma (three of 27) and an erythema multiforme-like (EM-like) reaction consisting of atypical targets (eight of 27). All patients mounted a fever, most developed lymphadenopathy (24/27) and peripheral eosinophilia (25/27) and the most common organ involved was the liver (27/27). Review of the dermatopathic features of patients with DRESS demonstrated a superficial spongiotic dermatitis in the majority of cases (16/27). A smaller number of cases showed basal cell vacuolar degeneration and necrotic keratinocytes (nine of 27). The patients with these biopsy findings more commonly had an EM-like cutaneous phenotype, and more severe hepatic involvement. Three patients died, two following failed liver transplants. CONCLUSIONS: Our series is the first in which a detailed dermatological assessment has been made of consecutive patients presenting with DRESS, and the largest U.K. series to date. Our results suggest a possible prognostic role of the cutaneous and dermatopathic findings in DRESS in predicting the severity of visceral involvement in this syndrome. What's already known about this topic? • Drug reaction with eosinophilia and systemic symptoms (DRESS) has a heterogeneous clinical presentation, with a skin eruption of variable morphology. • DRESS carries considerable morbidity and mortality, usually hepatic in origin, although renal, pulmonary and pericardial involvement can be seen. What does this study add? • The cutaneous phenotype in DRESS can be categorized as an urticated papular exanthem, a morbilliform erythema, exfoliative erythroderma or erythema multiforme-like (EM-like). • An EM-like eruption DRESS may be prognostic of more severe hepatic involvement.

Drug reaction with eosinophilia and systemic symptoms (DRESS): an original multisystem adverse drug reaction. Results from the prospective RegiSCAR study.

BACKGROUND: Cases of severe drug hypersensitivity, demonstrating a variable spectrum of cutaneous and systemic involvement, are reported under various names, especially drug reaction with eosinophilia and systemic symptoms (DRESS). Case definition and overlap with other severe cutaneous adverse reactions (SCAR) are debated. OBJECTIVES: To analyse the spectrum of signs and symptoms of DRESS and distribution of causative drugs in a large multicentre series. PATIENTS AND METHODS: RegiSCAR, a multinational registry of SCAR, prospectively enrolled 201 potential cases from 2003 to mid-2009. Using a standardized scoring system, 117 cases were validated as showing probable or definite DRESS. RESULTS: The male/female ratio was 0.80; females were borderline significantly younger than males. Next to the ubiquitous exanthema, the main features were eosinophilia (95%), visceral involvement (91%), high fever (90%), atypical lymphocytes (67%), mild mucosal involvement (56%) and lymphadenopathy (54%). The reaction was protracted in all but two patients; two patients died during the acute phase. Drug causality was plausible in 88% of cases. Antiepileptic drugs were involved in 35%, allopurinol in 18%, antimicrobial sulfonamides and dapsone in 12% and other antibiotics in 11%. The median time interval after drug intake was 22 days (interquartile range 17-31) for all drugs with (very) probable causality, with differences between drugs. CONCLUSION: This prospective observational study supports the hypothesis that DRESS is an original phenotype among SCAR in terms of clinical and biological characteristics, causative drugs, and time relation. The diversity of causative drugs was rather limited, and mortality was lower than that suggested by prior publications.