Eczema Herpeticum: Clinical Insights and Pathogenesis Hypotheses on Basolateral Adhesion Proteins.

Eczema herpeticum (EH) is a severe viral complication caused by the herpes simplex virus (HSV) that occurs in individuals with compromised skin barriers, such as those with atopic dermatitis (AD). EH is characterized by the rapid spread of HSV across skin lesions, potentially leading to systemic involvement. Although commonly observed in the context of AD, EH can also arise in various dermatological conditions, necessitating prompt recognition and management by healthcare providers. This case report details the diagnosis and treatment of EH in a five-year-old girl with a history of AD who presents with fever and painful skin lesions. Despite the absence of confirmatory tests initially, a positive IgM anti-HSV-1 serology, combined with clinical presentation, supported the diagnosis of EH. The patient received intravenous aciclovir, resulting in significant improvement within 48 hours. This case highlights the importance of early diagnosis and treatment, particularly when confirmatory tests are not available. The report discusses the clinical presentation of EH, which includes vesicular lesions, fever, and rapid progression. The differential diagnosis includes chickenpox, impetigo, eczema vaccinatum, and contact dermatitis. Understanding the epidemiology and pathogenesis of EH, especially in relation to AD, is crucial for effective management. The case also introduces a novel hypothesis linking structural protein alterations to immune dysfunction in EH, suggesting a need for further research. Acyclovir remains the gold standard for treating EH, and timely intervention is essential. This case underscores the necessity of a diagnostic algorithm in the absence of guidelines and highlights the role of IgM serology and clinical judgment in managing suspected EH cases.

Efgartigimod-associated Kaposi's varicelliform eruption and herpetic conjunctivitis in a patient with seropositive ocular myasthenia gravis: a case report and review.

Background: Efgartigimod (Efgartigimod alpha fcab, Vyvgart™) is a pioneering neonatal Fc receptor (FcRn) antagonist for the treatment of severe autoimmune diseases mediated by pathogenic immunoglobulin G (IgG) autoantibodies, including myasthenia gravis (MG). It is a well-tolerated drug with minor side effects, such as headache and upper respiratory (lung) and urinary tract infections. Here, we present a case of Kaposi's varicelliform eruption (KVE) and herpetic conjunctivitis related to efgartigimod in a 60-year-old patient with ocular MG (OMG). Case description: A 60-year-old Chinese male suffered from acetylcholine receptor antibody positive (AChR Ab+) OMG for 8 years. During this period, he underwent first-line treatment with systemic corticosteroids, cyclosporine, cyclophosphamide, and so on, but had poor symptom improvement. On the recommendation of his attending neurologist, he received one cycle of intravenous efgartigimod (10mg/kg, once weekly for 4 weeks). The patient experienced fever, widespread painful blisters, and edema on the face on the third day after his last intravenous infusion. The patient also complained of increased secretions and a foreign body sensation in both eyes. Laboratory tests confirmed infection with herpes simplex virus (HSV). A diagnosis of efgartigimod-associated KVE and herpetic conjunctivitis was made. After intravenous administration (5mg/kg, 3 times a day, every 8 hours) for 10 days, the patient was cured without residual complications. Conclusions: This case is the first report of a patient with KVE and herpetic conjunctivitis related to efgartigimod in PubMed. This is rare and unusual. Clinicians should be alert to the rare symptoms related to efgartigimod.

Severe pityriasis rubra pilaris complicated with Kaposi's varicelliform eruption and cutaneous MRSA infection case report.

A 62-year-old woman presented to our hospital with erythroderma affecting 100 % of body surface area, skin scaling and a body temperature of 37.3o C. The lesions initially appeared on her scalp 6 months prior, then psoriasis was diagnosed. Topical corticosteroids were prescribed, which were ineffective. After 2 months the rash spread to the rest of the body, accompanied by nail changes and hair loss. The patient was subsequently admitted to the local hospital, where following clinical evaluation, oral methotrexate 10 mg once weekly was initiated for 6 weeks. Despite the administered treatment the patient's health and skin condition deteriorated, manifesting with an appearance of new lesions. By the time of admission to our hospital erythroderma affecting 100 % of body surface area covered with wide skin scales and punched-out erosions on the torso, lower eyelid ectropion, loss of scalp hair and thickened yellow nail plates were observed. Skin biopsy revealed histological changes consistent with pityriasis rubra pilaris diagnosis. Polymerase chain reaction test from erosions confirmed the presence of herpes simplex virus 1/2 and culture results identified methicillin-resistant Staphylococcus aureus. Given the considerations of pityriasis rubra pilaris, hematologic disorders and paraneoplastic syndrome, a comprehensive work-up for haematological and oncological disorders was conducted, which yielded no significant findings. The patient was treated with intravenous corticosteroids, antibiotics, and antiviral drugs. Isotretinoin was initiated following the histological confirmation of pityriasis rubra pilaris. By the time of discharge, the patient's condition improved. During a follow-up visit 43 weeks after the initiation of isotretinoin, the skin was almost clear. The described case highlights the rare possibility of developing Kaposi's varicelliform eruption in patients with pityriasis rubra pilaris and demonstrates that isotretinoin is a safe and effective treatment option for this condition.

Kaposi's Varicelliform Eruption: A Potentially Life-Threatening Complication of Atopic Dermatitis.

Introduction: Kaposi's varicelliform eruption (KVE), also known as eczema herpeticum or eczema vaccinatum, is an acute dermatosis that affects patients with chronic dermatopathies. The diagnosis is primarily clinical and is characterised by the presence of a vesicular exanthema on physical examination. The exanthema subsequently evolves into crusted lesions with typical circular ulcerations in 'punched-out' areas on the skin affected by the underlying dermatopathy. Case description: We present the case of a 6-year-old patient who presented to the Paediatric Emergency department with skin lesions consistent with eczema herpeticum. The patient's management was initially outpatient; however, due to the slow progression of the condition, hospitalisation and intravenous antiviral treatment were initiated. Discussion: KVE affects patients with chronic dermatoses, especially atopic dermatitis. It is important to know the clinical presentation for an early suspicion. KVE is a medical emergency that requires prompt diagnosis and treatment. It can progress to secondary viraemia, which can be fatal in up to 10% of immunocompetent individuals and up to 50% of immunocompromised individuals. It is important to be aware of this condition and to start early treatment with antivirals, especially given the high prevalence of atopic dermatitis in our population. This condition is one of the most serious complications that can occur in these patients. LEARNING POINTS: To facilitate early suspicion and diagnosis, disseminate information about eczema vaccinatum.Emphasise the importance of initiating antiviral treatment early to prevent potential complications of eczema herpeticum.If left untreated, Kaposi's varicelliform eruption can result in up to a 10% mortality rate in immunocompetent individuals and a 50% mortality rate in those who are immunocompromised.

Kaposi's Varicelliform Eruption After Treatment With Ixekizumab in a Patient With Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris (PRP) is a rare condition characterized by red-orange plaques with islands of sparing with follicular and palmoplantar hyperkeratosis. The disease can be difficult to treat and often requires patients to trial multiple therapeutic options. In recent years, targeted biologic therapies have increasingly been trialed due to their relative efficacy and favorable safety profile. Ixekizumab, an interleukin-17 inhibitor, is one such therapy that has demonstrated efficacy in PRP with few reported adverse events. We present a PRP patient who developed Kaposi's varicelliform eruption followed by a pseudomonal superinfection three months after initiation of ixekizumab.

A rare case of eczema herpeticum associated with HIV: A case report.

Eczema herpeticum (EH), also known as Kaposi's varicelliform eruption, is a disseminated herpes simplex virus infection seen in patients with underlying skin conditions, most commonly atopic dermatitis. Monomorphic vesicles and "punched-out" erosions with hemorrhagic crusts over eczematous regions are the hallmarks of EH's presentation. Here, we discuss a first reported case of eczema herpeticum in a patient living with well controlled HIV with prior steroid use. A 30-year-old male patient living with HIV presented to the hospital with a generalized rash involving the face, neck, arms, hands, low back region, and both feet. Herpes simplex 1 and 2 by PCR DNA were detected from external auditory ear canal drainage. The patient was treated with intravenous acyclovir and responded well. He had long term history of eczema and required acyclovir prophylaxis later.

Erupción variceliforme de Kaposi en un paciente adulto mayor con desnutrición severa

El eccema herpético o erupción variceliforme de Kaposi, a pesar de ser una infección viral poco frecuente, se considera una urgencia dermatológica, debido a su alto potencial de diseminación, gravedad y mortalidad en adultos. Los pacientes inmunosuprimidos suponen un alto riesgo de colonización cutánea y visceral, además de una mayor probabilidad de presentar otras infecciones causadas por el virus del herpes simple. Se caracteriza por presentar lesiones en piel de tipo vesículas umbilicadas agrupadas o diseminadas que evolucionan a erosiones hemorrágicas perforadas, que se localizan principalmente en cabeza, cuello y tórax; son dolorosas y tardan entre 2-6 semanas en sanar y por lo general se asocian a compromiso sistémico. El diagnóstico es clínico; en caso de duda, se puede utilizar Test de Tzanck, que es una herramienta rápida para confirmar infección por herpes virus; tiene una sensibilidad de 40-80 % y especificidad hasta del 100 %. El manejo consiste en terapia antiviral sistémica; el tratamiento antibiótico está indicado si existe riesgo sobreinfección bacteriana. El tratamiento oportuno de la infección es clave para la evolución hacia la recuperación del paciente.

Eczema herpeticum or Kaposi's varicelliform eruption is a rare viral infection; however, it's considered a dermatologic urgency due to the high potential for dissemination, severity and mortality. Immunosuppressed patients have a high risk of skin and visceral colonization in addition to a higher probability of other infections caused by herpes simplex virus. Kaposi's varicelliform eruption is characterized by vesicopustules, some umbilicated, others eroded and extended in clusters. They may also present hemorrhagic crusts with an erythematous base. The most commonly affected sites are head, neck, and trunk. These lesions are painful, they take from 2 to 6 weeks to heal and are usually associated with systemic signs of infection. Diagnosis is mainly clinical. In case of doubt, the Tzanck allows a rapid diagnostic approach with a sensitivity of 40-80 % and specificity up to 100 % in herpes virus. Antiviral treatment proved to be effective; additional antibiotic treatment is required if there is risk of bacterial infection. The timely treatment of the infection is the key in the evolution towards the recovery of the patient.

Kaposi Varicelliform Eruption With Contact Dermatitis in a Person Living with AIDS.

Kaposi varicelliform eruption (KVE) is a cutaneous dissemination of a viral infection, which is mostly caused by herpes simplex virus (HSV) in the setting of certain underlying skin diseases. KVE occurs mainly in infants and children, but very rarely in adults. Here, we report a case of KVE with contact dermatitis in a 36-year-old man with acquired immunodeficiency syndrome (AIDS), who was referred to our deparment with pruritic well-defined facial erythema and multiple vesicular lesions. A punch biopsy and immunohistochemical examination established the diagnosis of KVE with contact dermatitis. After treatment with valacyclovir and antihistamines, facial lesions achieved complete remission. With this case report, KVE has specific manifestation in clinic, histopathology and immunohistochemistry, which could guide the early diagnosis and improve prognosis.

Six Cases of Erythrodermic Pemphigus Foliaceus: A Case Report.

Pemphigus foliaceus (PF) is one of the causes of erythroderma; however, to date, there have been relatively few reported cases. We herein describe 6 cases of erythrodermic PF. In all 6 cases, PF was a direct cause of erythroderma because the patients had not undergone any medical treatments and neither had any other skin diseases nor were taking any drugs that typically cause erythroderma. Serum levels of IgE and thymus and activation-regulated chemokine were elevated in 5 of the 6 cases, whereas soluble interleukin-2 receptor and squamous cell carcinoma-related antigen were markedly increased in all cases, suggesting that those markers are strong indicators of skin surface damage. All patients were treated with predonisolon (PSL), of which PSL pulse was added in 4 patients and intravenous immunoglobulin was added in 4 patients. Furthermore, all patients except for one were older adults, among whom 2 cases developed Kaposi's varicelliform eruption, and died, and another 2 patients, respectively, died of gastrointestinal bleeding and sepsis. Kaposi's varicelliform eruption is a complication of erythrodermic PF associated with poor prognosis, and thus caution is necessary when considering the diagnosis. Furthermore, elderly people are more likely to have complications due to PSL, which may result in death. Inappropriate treatment and delay in treatment may cause erythroderma, so early diagnosis and treatment are necessary.

Crusted Scabies Complicated with Herpes Simplex and Sepsis.

Crusted scabies is a rare and extremely contagious infestation by Sarcoptes scabiei. Kaposi's varicelliform eruption (KVE) refers to herpes simplex virus infection superimposed on pre-existing dermatosis such as atopic dermatitis, Darier's disease, and pemphigus. We report a case of KVE superimposed on crusted scabies in a middle-aged woman. Her condition was complicated with sepsis. She was treated with IV meropenem, vancomycin, and acyclovir and was released 2 weeks later in good condition. To our knowledge, only rare cases of crusted scabies complicated by KVE have been reported.

Eczema herpeticum emerging during atopic dermatitis in infancy.

BACKGROUND: Eczema herpeticum (EH) is an acute disseminated viral infection that develops in the presence of an existing skin lesion, often on the ground of atopic dermatitis (AD). Morbidity and mortality of EH can be minimized by starting antiviral therapy at the earliest time in diagnosis. CASE: Herein we report five infants diagnosed with EH in the course of AD treatment. All patients had early onset, moderate to severe AD and needed intermittent topical corticosteroid (TCS) therapy. In physical examination, newly formed, TCS-resistant vesiculo-papular skin lesions were recognised on the present dermatitis. The presence of AD with food allergy and moderate to severe eosinophilia were other prominent findings. CONCLUSION: All patients were misdiagnosed as AD exacerbation. Therefore, EH should be considered in the differential diagnosis of AD exacerbation especially in the infants with moderate to severe AD.

Kaposi's varicelliform eruption in a patient with metastatic melanoma and primary cutaneous anaplastic large cell lymphoma treated with talimogene laherparepvec and nivolumab.

BACKGROUND: Immune-directed therapies have become front-line therapy for melanoma and are transforming the management of advanced disease. In refractory cases, multi-modal immunoncology (IO) approaches are being utilized, including combining immune checkpoint blockade (ICB) with oncolytic herpes viruses. Talimogene laherparepvec (T-VEC) is the first genetically modified oncolytic viral therapy (OVT) approved for the treatment of recurrent and unresectable melanoma. The use of IO in patients with concomitant malignancies and/or compromised immune systems is limited due to systematic exclusion from clinical trials. For example, a single case report of a solid organ transplant patient successfully treated with T-VEC for metastatic melanoma has been reported. Furthermore, the use of ICB in T-cell malignancies is limited and paradoxical worsening has been described. To our knowledge, this is the first report of dual ICB/T-VEC being administered to a patient with concurrent primary cutaneous anaplastic large cell lymphoma (pcALCL) and melanoma. CASE PRESENTATION: Here we present the case of a patient with concomitant primary cutaneous ALCL and metastatic melanoma, progressing on anti-programmed death (PD)-1 therapy, who developed Kaposi's varicelliform eruption after receiving the first dose of Talimogene laherparepvec. CONCLUSION: This case highlights the complexities of care of patients with coexistent cancers, demonstrates rapid progression of primary cutaneous ALCL on nivolumab and introduces a novel adverse effect of Talimogene laherparepvec.

Herpeticum-like rash in a child with atopic dermatitis: early clinical suspicion is valuable.

Dermatological conditions may be associated with serious underlying medical conditions which require urgent treatment. We describe the case of a 6-year-old boy with erythematous vesicles with erosion and crusting on face, cheeks, and forehead. Due to the medical history of atopic dermatitis, eczema herpeticum was suspected and appropriate treatment was immediately initiated. This resulted in significant improvement of skin lesions.

[Dermatoses and herpes superinfection: A retrospective study of 34 cases]. / Dermatoses et surinfection herpétique : étude rétrospective de 34 cas.

BACKGROUND: Although varicelliform Kaposi eruption is a well-known complication of dermatoses, it has not been widely investigated. AIM: To investigate features of dermatoses and herpes superinfection in patients hospitalized in a dermatology department. PATIENTS AND METHODS: We performed a single-centre, retrospective study between 2008 and 2014 that included cases of Kaposi varicelliform eruptions defined by positive PCR of an unconventional site of herpetic recurrence in a setting of active dermatitis. A record was compiled of each case giving details of the history, clinical and laboratory findings, therapeutic data and outcome. RESULTS: Thirty-four cases of Kaposi varicelliform eruptions in 30 subjects were studied. Mean age at diagnosis was 63.3±24.2 years. The underlying dermatoses were as follows: 7 pemphigus, 6 bullous pemphigoid, 3 cicatricial pemphigoid, 3 atopic dermatitis, 1 Darier disease, and 14 other dermatoses. Patients presented with skin (94.1 %) or mucous membrane lesions (62 %), mostly erosive (79 %), vesicular (27 %) or bullous (41 %), often painful (56 %) or pruritic (29 %). At diagnosis, 41.2 % were undergoing systemic immunotherapy and 24 % were on topical corticosteroids. PCR was positive for HSV1 in 20 cases and for HSV2 in 4 cases, and indeterminate in 10 cases. Lymphocytopenia was seen in 59 % of cases. The majority of patients received treatment. Nine patients experienced at least one relapse. CONCLUSION: Our study confirms the over-representation not only of the expected dermatoses (pemphigus and atopic dermatitis), but also of others such as pemphigoid and acute dermatoses; these results should be investigated in a more systematic prospective study.

A rare complication of follicular hair unit extraction: Kaposi's varicelliform eruption.

Follicular hair unit extraction (FUE) is becoming a popular type of hair transplantation recently. Kaposi's varicelliform eruption (KVE) is an uncommon skin emergency due to cutaneous dissemination of several types of viruses, most notably herpes virus, over the lesions of preexisting skin disorders. A 34-year-old man visited our dermatology outpatient clinic with a blistering, itchy and tender eruption on his head and body. He had undergone follicular FUE for androgenic alopecia 12 days previously, and 5 days after the procedure, umbilicated and/or hemorrhagic vesiculopustules appeared firstly on the occipital scalp skin where the hair units were taken. The lesions had rapidly spread over the upper chest and back. After the operation, he had taken oral methylprednisolone, amoxicillin clavulanate and had used fusidic acid ointment without any benefit. Bacterial culture of the pustules yielded no microorganism, while Tzanck smear from the vesicles revealed multinuclear giant cell groups. Based on a diagnosis of KVE, we treated the patient with oral valacyclovir hydrochloride 1000 mg 3 times a day for 14 days. Symptoms cleared rapidly, pustules and vesicles dried in a few days, and re-epithelialization of the eroded areas started at the end of the first week. The reported complications of FUE include necrosis of the donor site, postoperative hyperesthesia, recipient area folliculitis, keloids, bleeding, infection and pyogenic granuloma. Up to this date there are only three reports of KVE developing just after dermatological surgery, including dermabrasion, laser resurfacing, and skin grafting. According to our knowledge, this is the first case of KVE occurring after the FUE procedure. We think that the traumatic effects and skin barrier disruption due to operation and immune alteration due to postsurgical steroid treatment might have precipitated the activation and dissemination of latent herpesvirus infection.

Increased large unstained cells value in varicella patients: A valuable parameter to aid rapid diagnosis of varicella infection.

Varicella is a highly contagious infection caused by varicella zoster virus. Sometimes it is difficult to differentiate between other viral infections such as Kaposi's varicelliform eruption (KVE) and disseminated herpes zoster (HZ). The large unstained cells (LUC) value is a differential count parameter reported by routing hematology analysis. LUC have been studied previously, but never been reported in the context of varicella or in dermatological published work. The aim of this study was to compare the LUC values in varicella patients with that in KVE and disseminated HZ patients. Sixty-nine varicella patients, 30 KVE patients and 11 disseminated HZ patients were included in this retrospective study. All data were analyzed using SPSS version 17.0 or GraphPad Prism version 5.0. The mean percentage of LUC (%LUC) in varicella patients was higher than the upper limit of normal reference range and it was increased compared to %LUC of both KVE (P < 0.0001) and disseminated HZ (P = 0.0051) patients. %LUC of varicella patients significantly decreased with clinical improvements (P = 0.0017). %LUC was significantly increased in varicella patients and corresponded with clinical improvements. Patients with %LUC of 3.55 or more favor the diagnosis of varicella over both KVE and disseminated HZ with 71.01% sensitivity and 84.44% specificity. We suggest that %LUC can assist in making a precise diagnosis of varicella in confusing cases.