Finger nodules with a papulovesicular hands and feet eruption: a complicated human Orf virus infection.

BACKGROUND: Orf virus (ORFV) is the pathogen responsible for Orf, a zoonotic viral infection that can be spread to humans from sheep and goats. Here, we present a case of human Orf complicated by an immune-related reaction, to raise awareness of this under-recognized disease avoiding unnecessary investigations and overtreatment. CASE REPORT: A 51-year-old woman with no previous medical history presented with a one-week history of three asymptomatic swelling nodules with a grey necrotic center and red outer halo on her index finger. At physical examination there was also a pruritic papulovesicular eruption on her hands and feet. She reported a recent contact with a goat which had a similar nodular lesion in its mouth. A biopsy of the lesions was performed and a diagnosis of Orf complicated by widespread erythema multiforme was made based on the clinical and histopathological features. The lesions spontaneously resolved within the next 2 weeks. CONCLUSIONS: Orf is not very prevalent in our region, so we performed a biopsy of the lesion to guide us toward a diagnosis. However, we should remember that the diagnosis of ecthyma relies on clinical evaluation and epidemiological criteria.

Reality of drug-induced erythema multiforme: A French pharmacovigilance study.

BACKGROUND: Since the 2002 SCAR study, erythema multiforme (EM), a post-infectious disease, has been distinguished from Stevens-Johnson syndrome (SJS), drug-induced. Nevertheless, EM cases are still reported in the French pharmacovigilance database (FPDB). OBJECTIVES: To describe EM reported in the FPDB and to compare the quality and the characteristics of the reports. METHODS: This retrospective observational study selected all EM cases reported in the FPDB over two periods: period 1 (P1, 2008-2009) and period 2 (P2, 2018-2019). Inclusion criteria were 1) a diagnosis of clinically typical EM and/or validated by a dermatologist; 2) a reported date of onset of the reaction; and 3) a precise chronology of drug exposure. Cases were classified confirmed EM (typical acral target lesions and/or validation by a dermatologist) and possible EM (not-otherwise-specified target lesions, isolated mucosal involvement, doubtful with SJS). We concluded possible drug-induced EM when EM was confirmed, with onset ranging from 5 to 28 days without an alternative cause. RESULTS: Among 182 selected reports, 140 (77%) were analyzed. Of these, 67 (48%) presented a more likely alternative diagnosis than EM. Of the 73 reports of EM cases finally included (P1, n=41; P2, n=32), 36 (49%) had a probable non-drug cause and 28 (38%) were associated with only drugs with an onset time ≤4 days and/or ≥29 days. Possible drug-induced EM was retained in 9 cases (6% of evaluable reports). Etiological work-up was more often performed in period 2 than 1 (53.1% vs 29.3%, P=0.04), and the time to onset from 5 to 28 days was more frequent in period 2 (59.2% vs 40%, P=0.04). CONCLUSIONS: This study suggests that possible drug-induced EM is rare. Many reports describe "polymorphic" rashes inappropriately concluded as EM or post-infectious EM with unsuitable drug accountability subject to protopathic bias.

Lyme Neuroborreliosis with Intracranial Hypertension and Erythema Multiforme: A Rare Presentation.

A 9-year-old previously healthy boy presented with high-grade intermittent fever, severe headache associated with neck stiffness for 5 days, rash over trunk and extremities for 4 days, vomiting for 3 days and diplopia for 2 days. There was no history of seizures, abnormal body movements, altered sensorium or focal deficits. On examination, he had maculopapular erythematous rashes over the trunk and extremities and erythema multiforme. He had bilateral abducens nerve palsy and the rest of the cranial nerve, sensory and motor examination was normal. He had neck stiffness and positive Kernig's sign. Fundus examination showed grade 4 papilledema. Cerebrospinal fluid workup revealed elevated opening pressure, lymphocytic pleocytosis, normal protein and glucose levels. Neuroimaging showed features suggestive of intracranial hypertension. Borrelia IgM and IgG antibodies came positive. The uniqueness of our case lies with two rare presenting manifestations of Lyme neuroborreliosis in the same child.

Cutaneous Toxicities Associated with Immune Checkpoint Inhibitors: An Observational, Pharmacovigilance Study.

Cutaneous immune-related adverse events (cirAEs) are the most prevalent complication to arise from immunotherapy and cause significant morbidity. We aimed to determine the spectrum, timing, clinical features, and outcomes of cirAEs by conducting an observational pharmacovigilance study using VigiBase, the World Health Organization's global database of individual case safety reports from over 130 member countries (ClinicalTrials.gov, number NCT04898751). We compared adverse event reporting in patients who received immune checkpoint inhibitors (91,323 adverse events) with those of the full reporting database (18,919,358 adverse events). There were 10,933 cases of cirAEs within 51 distinct dermatologic types, with 27 specific eruptions with disproportionate signal represented (information component [IC]025 > 0). Of these 27 eruptions, there were eight cirAEs with n > 100 reports, including vitiligo (IC025 = 4.87), bullous pemphigoid (IC025 = 4.08), lichenoid dermatitis (IC025 = 3.69), erythema multiforme (IC025 = 1.03), toxic epidermal necrolysis (IC025 = 0.95), Stevens‒Johnson syndrome (IC025 = 0.41), drug eruption (IC025 = 0.11), and eczematous dermatitis (IC025 = 0.11). There were differences in time to onset after immune checkpoint inhibitor initiation, with a median of approximately 1 month (erythema multiforme, Stevens‒Johnson syndrome, and toxic epidermal necrolysis), 2 months (drug eruption and eczematous dermatitis), 4 months (lichenoid dermatitis), and 5‒6 months (bullous pemphigoid and vitiligo). CirAEs are diverse, dependent on cancer type, and have distinct and different onset times that are linked to the cirAE subtype.

Mycoplasma pneumoniae-induced rash and mucositis: A new entity.

Mycoplasma pneumoniae is a well-known cause of community-acquired pneumonia, mostly associated with dermatological manifestations especially with mucosal involvement and targetoid cutaneous lesions. For many years, it was considered among the spectrum of erythema multiforme. Recently, some authors have recommended the creation of a new syndrome called "mycoplasma-induced rash and mucositis." This new syndrome has distinct epidemiological, clinical and histological features making it different from drug-induced Stevens-Johnson syndrome, toxic epidermal necrosis and erythema multiforme. Herein, we report two patients with acute Mycoplasma pneumoniae respiratory tract infection presenting severe mucocutaneous lesions in accordance with this new syndrome.

Patch testing versus interferon-gamma release assay in evaluation of drug eruptions.

Delayed-onset T-cell-mediated cutaneous adverse drug reactions are an uncommon but potentially serious result of medication exposures. Identification of culprit medications is crucial, but clinical diagnosis is often difficult. Patch tests and interferon-gamma release assays (IFNγ-RA) were previously reported as potentially useful ancillary tests, while rechallenges remain the reference standard test. We compared the number of positive test results with patch testing and IFNγ-RA for drugs implicated as possible causes of cutaneous reactions. Fifty-one patients with a suspected cutaneous drug eruption underwent patch testing and IFNγ-RA for suspected drugs. Participants were followed up at least 9 months after the onset of the rash with results compared with the clinical diagnosis. Forty-two patients presented with morbilliform/eczematous eruptions; five were diagnosed with fixed drug eruption (FDE) and four with erythema multiforme. None had positive patch testing to the drugs tested. A total of 8/51 (15.6%) patients had positive reaction by the IFNγ-RA, and an additional 11 (21.6%) patients had borderline results. Positive or borderline results were more likely in patients with FDE (80%) than morbilliform/eczematous eruptions (30.9%) or erythema multiforme (25%). Our study emphasizes the necessity of additional effective ancillary tests in the evaluation of drug eruptions and supports the use of IFNγ-RA for drug testing as a tool for identifying medications associated with cutaneous drug eruptions.

Neuropsychiatric manifestations in an adolescent male with Rowell syndrome.

A 15-year-old boy presented with fever, skin, and oral lesions for 4 weeks. The cutaneous lesions were suggestive of subacute cutaneous lupus erythematosus and erythema multiforme. His clinical, histopathological, and immunological features were indicative of Rowell syndrome and he satisfied the diagnostic criteria of Rowell syndrome proposed by Zeitouni et al. He subsequently developed neurological manifestations and was diagnosed to have neuropsychiatric systemic lupus erythematosus. We report this case for the unusual occurrence of a rare entity like Rowell syndrome in an adolescent male with co-existence of neuropsychiatric systemic lupus erythematosus.

Acute Oral Lesions.

A variety of acute oral lesions may be encountered in the scope of dermatology. Oral lesions may be single or multiple; may arise secondary to infectious, immune, congenital, medication use, or idiopathic causes; and may take a variety of forms. A thorough evaluation of the oral cavity is required to assess patients with oral lesions. Affected patients may be monitored, treated, or referred to an appropriate specialist for further management as needed. Many acute oral lesions are self-limiting in nature and patients may require only assessment and reassurance. Several common acute oral lesions are discussed in this article.

A case of Good's syndrome complicated by erythema multiforme.

Good's syndrome (GS) is a rare, adult-onset combined B cell and T cell immunodeficiency with an associated thymoma. These patients have an increased risk of bacterial, fungal, viral and opportunistic infections. This report describes a 75-year-old female patient who presented with a full body rash and an anterior mediastinal mass. She underwent a biopsy of her rash and mass, which revealed erythema multiforme and WHO Type A thymoma, respectively. During her hospitalisation, she was also found to have oropharyngeal candidiasis, methicillin-susceptible Staphylococcus aureus bacteraemia and herpes simplex virus type 2 (HSV-2) skin lesions. Based on the number of infections and severity of her rash, an immunocompromised state was suspected. Immunological testing revealed a B cell and T cell deficiency as well as low serum immunoglobulins. This combination of hypogammaglobulinaemia and thymoma led to a diagnosis of GS. While there have been many case reports of GS, this is the first report of the immunodeficiency presenting with erythema multiforme.

The rash that presents as target lesions.

We have explored the rash that appears as target lesions, with the central and dominant diseases belonging to the Stevens-Johnson syndrome/toxic epidermal necrolysis group. After presenting the clinical patterns of an individual target lesion and classifying them into different types of lesions, the contribution has been organized with groups characterized by such specific findings according to the type of lesion: flat or raised, typical or atypical, presence or absence of fever, presence or absence of mucosal ulcerations, presence or absence of arthralgias, and/or internal organ involvement. Other specific features, such as histologic appearance, immunofluorescence findings, and laboratory changes, are considered. We provide clinicians with an algorithmic, systematic, and logical approach to diagnose the condition of the patients who present with targetoid lesions, and enable them to differentiate between those with serious systemic and life-threatening diseases from others with ordinary skin ailments.

Painful Oral Lesions.

Painful oral vesiculoerosive diseases (OVD) include lichen planus, pemphigus vulgaris, mucous membrane pemphigoid, erythema multiforme, and recurrent aphthous stomatitis. OVD lesions have an immunopathic cause. Treatment is aimed at reducing the immunologic and the following inflammatory response. The mainstay of OVD management is topical or systemic corticosteroids to include topical triamcinolone, fluocinonide, and clobetasol, whereas systemic medications used in practice can include dexamethasone, prednisone, and prednisolone. Oral herpetic lesions can be primary or recurrent. If management is desired, they can be treated by topical or systemic antiviral drugs. Topical antiviral creams include prescription acyclovir, penciclovir and over-the-counter docosanol.