Kaposi varicelliform eruption: an unusual presentation caused by varicella zoster virus in a healthy adult patient - a case report.

BACKGROUND: Kaposi Varicelliform Eruptions (KVE), also known as eczema herpeticum, is a rare and potentially life-threatening dermatological condition primarily attributed to herpes simplex virus (HSV) infection, with less frequent involvement of Coxsackie A16, vaccinia, Varicella Zoster, and smallpox viruses. Typically associated with pre-existing skin diseases, especially atopic dermatitis, KVE predominantly affects children but can manifest in healthy adults. Characterized by painful clusters of vesicles and sores on the skin and mucous membranes, it often masquerades as other dermatological disorders. Non-steroidal anti-inflammatory drugs (NSAIDs) are commonly used for pain relief and inflammation, though their potential role as KVE triggers remains uncertain. CASE REPORT: Here, we present a case of an 18-year-old female with KVE attributed to Varicella Zoster virus (VZV) and successfully treated with oral acyclovir within a week, underscoring the significance of early recognition and intervention. KVE can manifest with systemic symptoms like fever, fatigue, and lymphadenopathy and may involve multiple organ systems, necessitating possible antibiotic use for complications. CONCLUSION: This case underscores the importance of prompt KVE identification and consideration of antiviral therapy to enhance patient outcomes. Further research is warranted to elucidate predisposing factors for this rare condition.

[Eccema herpético en una paciente con dermatitis atópica].

Background: Eczema herpeticum is an infection caused by herpes simplex virus in patients with atopic dermatitis, among its complications we can find meningitis, encephalitis, acute liver failure, and Staphylococcus aureus infection. Case report: We report the case of a female patient of 5 years of age, with a history of atopic dermatitis complicated by eczema herpeticum, who was treated initially without relief. Her hospital stay was complicated with cross infections, which prolonged her course. Dermatology diagnosed eczema herpeticum. Immediately after the start of treatment, the patient showed improvement. Conclusions: Eczema herpeticum is a rare complication of atopic dermatitis, it must be suspected based on patient history and physical examination. Therefore, early recognition and diagnosis are of clinical importance. Without an appropriate approach, these patients can present shock, sepsis, and death.

Antecedentes: El eccema herpético es una infección causada por el virus del herpes simple, que afecta a pacientes con dermatitis atópica. Las principales complicaciones son meningitis, encefalitis, insuficiencia hepática aguda e infección por Staphylococcus aureus. Reporte de caso: Paciente pediátrica de 5 años, con antecedente de dermatitis atópica complicada con eccema herpético, que recibió tratamiento sin reacción satisfactoria. Durante la hospitalización tuvo infecciones nosocomiales que prolongaron su estancia. Luego de la evaluación por personal del servicio de Dermatología se estableció el diagnóstico de eccema herpético, con adecuado tratamiento, seguimiento y egreso sin complicaciones. Conclusiones: El eccema herpético es una complicación rara de la dermatitis atópica, que debe diagnosticarse con base en los antecedentes personales patológicos y la exploración física adecuada. La atención oportuna es de relevancia clínica, pues los pacientes pueden tener complicación serias (choque, sepsis, incluso la muerte). Palabras clave: Eccema herpético; dermatitis atópica; infección nosocomial; Staphylococcus aureus.

Systemic herpes simplex virus infection in a child with eczema herpeticum.

Eczema herpeticum (EH) is a disseminated cutaneous infection with herpes simplex virus (HSV) that develops in patients with atopic dermatitis. The kinetics and clinical significance of HSV viremia in EH are poorly understood. Herein, we report HSV DNAemia in a child with EH 12 months after the completion of chemotherapy for Hodgkin lymphoma.

Conditions That Simulate the Environment of Atopic Dermatitis Enhance Susceptibility of Human Keratinocytes to Vaccinia Virus.

Individuals with underlying chronic skin conditions, notably atopic dermatitis (AD), are disproportionately affected by infections from members of the herpesviridae, papovaviridae, and poxviridae families. Many patients with AD experience recurrent, widespread cutaneous viral infections that can lead to viremia, serious organ complications, and even death. Little is known about how the type 2 inflammatory environment observed in the skin of AD patients impacts the susceptibility of epidermal cells (keratinocytes) to viral pathogens. Herein, we studied the susceptibility of keratinocytes to the prototypical poxvirus, vaccinia virus (VV)-the causative agent of eczema vaccinatum-under conditions that simulate the epidermal environment observed in AD. Treatment of keratinocytes with type 2 cytokines (IL-4 and -13) to simulate the inflammatory environment or a tight junction disrupting peptide to mirror the barrier disruption observed in AD patients, resulted in a differentiation-dependent increase in susceptibility to VV. Furthermore, pan JAK inhibition was able to diminish the VV susceptibility occurring in keratinocytes exposed to type 2 cytokines. We propose that in AD, the increased viral susceptibility of keratinocytes leads to enhanced virus production in the skin, which contributes to the rampant dissemination and pathology seen within patients.

Eczema herpeticum subsequent to septic shock in early pregnancy: a first case report.

BACKGROUND: Eczema herpeticum (EH) is a severe skin complication caused by human simplex virus (HSV) infection concomitant with immune dysfunction and dermatological conditions, mainly atopic dermatitis. We present the first case of EH subsequent to sepsis-related immunological suppression in pregnancy. CASE PRESENTATION: Septic shock developed in a 30-year-old primiparous woman at 14 weeks of pregnancy during admission for hyperemesis gravidarum. Although her life-threatening status due to sepsis improved by prompt treatment, on day 3 of treatment in the intensive care unit, blisters suddenly erupted on her face and neck and spread over her body. EH was diagnosed according to HSV type-1 antigen positivity and a past medical history of EH and atopic dermatitis. Antiviral agents were administered immediately, with positive results. Her general condition improved quickly, without central nervous system defects. This is the first report of EH following septic shock in early pregnancy. At present, we speculate that EH develops as a complication due to immunological changes in the late phase of sepsis because sepsis is mainly characterized by both an inflammatory state in the acute phase and an immunosuppressive state in the late phase. Pregnancy can also contribute to its pathogenesis, as it causes an immunosuppressive state. Mortality due to EH is relatively high; in this case, a history of EH and atopic dermatitis contributed to the initiation of prompt medical interventions for the former, with improvement in the patient's severe condition. The combination of immunological changes in sepsis and pregnancy can cause HSV reactivation, resulting in EH recurrence. CONCLUSIONS: In conclusion, if dermatological symptoms develop in a pregnant woman with a history of EH and/or atopic dermatitis treated for sepsis, EH should be suspected based not only on clinical features but also on immunological changes along with sepsis, and prompt medical interventions should be initiated.

Darier Disease Presenting with Recurrent Kaposi Varicelliform Eruption in a 10-year-old Boy with Seborrheic Dermatitis.

We present a case of a 10-year-old boy with a longstanding history of seborrheic dermatitis (SD) referred to the Allergy and Immunology Department for recurrent Kaposi varicelliform eruption (KVE) secondary to herpes simplex 1 (HSV-1) infection and possible primary immunodeficiency. The patient was the second child of non-consanguineous parents, with an older, healthy brother. Family history was negative for primary immunodeficiency and skin disorders. The patient's skin problems began in infancy when he was diagnosed and treated by a dermatologist for SD. From preschool age, he was under the care of a pediatric neurologist and a defectologist for a sensory processing disorder. For the last two years, the patient had been receiving chlorpromazine therapy for aggressive behavior. The first episode of KVE was diagnosed at the age of six, following potent topical corticosteroid therapy for SD and sun exposure, another known risk factor for HSV infection. After the third KVE episode, prophylaxis with oral acyclovir was initiated. The skin changes were treated with topical steroids and oral antibiotics during disease flares, with poor clinical response. On presentation, the patient was in good general health, adipose, and of unremarkable somatic status, except for numerous symmetrical yellowish-brown keratotic papules and plaques on the forehead, cheeks, and the lateral side of the neck (Figure 1). The nail plate had multiple red and white longitudinal streaks and V-shaped notches on the distal free end of the nail plate (Figure 2). The allergy tests revealed increased total immunoglobulin E (IgE) and sensitization to ragweed. Immunological workup showed normal immunoglobulins and good specific immunity (good vaccine response and normal humoral response to HSV-1) but a decreased number of T- cells (CD3+ 1020/µL (1320-3300), CD3+CD8+ 281/µL (390-1100) with normal T-cell response after antigen stimulation. The diagnosis of Darier disease (DD) was confirmed based on medical history, clinical findings and histological finding of focal suprabasal acantholysis and dyskeratosis (Figure 3). Low-dose oral retinoid therapy was initiated with modest clinical response after 6 months of therapy. In the light of recent publication (1), we initiated intravenous immunoglobulin (IVIG) substitution (400 mg/kg every month) with excellent clinical response. After 4 months, the patient's skin improved in terms of reduced inflammation, scab healing, and reduced itching. Acyclovir prophylaxis was continued. The patient had no new episodes of KVE during follow-up. Kaposi's varicelliform eruption (KVE) or eczema herpeticum occurs in a chronic inflammatory skin disease such as atopic dermatitis (AD), SD, Hailey-Hailey disease, allergic contact dermatitis, psoriasis, and DD (2). It is considered a dermatologic emergency due to its high mortality rate if misdiagnosed or left untreated (3). DD is a rare autosomal dominant genodermatosis of variable expressivity caused by mutations in the ATP2A2 gene, which encodes a sarco/endoplasmic reticulum calcium ATPase (SERCA2) highly expressed in keratinocytes (4). The onset of the disease usually occurs between the ages of 6 and 20 years. There are several clinical variants of DD: hypertrophic, verrucous, vesicular-bullous (dyshidrotic), erosive, and predominantly intertriginous forms (4). The fact that skin lesions occurred in infancy and a negative family history for skin diseases could be the reason our patient was initially misdiagnosed with seborrheic dermatitis. Due to the variable expressivity of the disease, it is impossible to exclude the diagnosis in other family members, and genetic testing of the patient and family members is therefore planned. A co-occurrence of neuropsychiatric abnormalities such as epilepsy, mental impairment, and mood disorders have been reported in patients with Darier disease, and these disorders were also present in our patient (5), indicating a correct diagnosis. Patients with DD have a high propensity for severe viral, bacterial, and fungal skin infection, probably due to local disruption of the skin barrier function or as the result of an underlying defect in general host defence (6). The occurrence of KVE in patients with DD is rare (7) and possibly caused by a disturbances in cell-mediated immunity (8). Despite abnormal findings in cellular immunity in some patients with DD, no consistent or specific abnormalities of the immune system have yet been demonstrated (6). Our patient had a decreased number of cytotoxic T-cells with normal T-cell response after antigen stimulation (in contrast with the findings of Jegasothy et al. (6)) and normal humoral response to HSV-1 infection. Recurrent KVE in our patient could be related to immune system dysfunction as an additional risk factor, along with impaired skin barrier. The excellent clinical response to IVIG speaks in favor of the role of antibody immune response in preserving the skin barrier. Occurrence of KVE in patients with mild DD (as in the case of our patient) and in some patients immediately preceding clinical skin manifestations of disease, argues very strongly against the second supposition. The severity of DD is variable and has a chronic course with frequent exacerbations and remissions. Known exacerbating triggers are: heat, sweat, sun exposure, friction, medication, and infection (9,10). The disease is chronic, and management is focused on the improvement of the skin appearance, relief of symptoms (e.g., irritation, pruritus, and malodor), and prevention or treatment of secondary infections. Topical (emollients, corticosteroids, retinoids, 5-fluorouracil, tacrolimus, pimecrolimus), physical (excision, electrodessication, dermabrasion, ablative laser, photodynamic therapy), and systemic (oral antibiotics, antiviral drugs, antimicrobial prophylaxis, vitamin A, retinoids) therapies are among the treatment options, all of which are of limited effect (2,11,12). IVIG substitution could be beneficial in some patients with Darier disease (1). In conclusion, this case highlights the association of DD with impaired cellular immunity and indicates the importance of proper diagnosis due to adequate management and avoidance of possible fatal outcomes. However, whether a subtle abnormality of T-cells in DD predisposes the patient to KVE remains unclear. Possible underlying mechanisms should be investigated further.

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.

Recurrent eczema herpeticum - a retrospective European multicenter study evaluating the clinical characteristics of eczema herpeticum cases in atopic dermatitis patients.

BACKGROUND: Eczema herpeticum (EH) is a disseminated viral infection of eczematous skin disease with the herpes simplex virus. Knowledge on clinical characteristics, risk factors and recurrent disease is limited. Our aim was to better define clinical characteristics and risk factors for EH and especially for recurrent EH. METHODS: A retrospective analysis of EH cases assessed the history, clinical signs, prior treatment and laboratory results using a predefined questionnaire. RESULTS: A total of 224 EH cases from eight European centres were included. Extrinsic AD was identified as risk factor for EH, and only one patient suffered from intrinsic AD. Early onset of AD was identified as risk factor for recurrent EH. Pretreatment with topical steroids, systemic steroids, topical calcineurin inhibitors or plain emollients reflected standard therapy. Many patients showed AD lesions without EH, but skin without AD lesions was never affected by herpetic lesions. CONCLUSION: Patients with clinically active, extrinsic AD are at risk of EH. Recurrent EH is associated with confounders of severe atopic distortion and requires active AD lesions for clinical manifestation. Recurrent eczema herpeticum mainly affects patients with early onset of AD.

Seborrhea herpeticum: cutaneous herpes simplex virus infection within infantile seborrheic dermatitis.

Eczema herpeticum has been well described in the setting of atopic dermatitis (AD) and other dermatoses. We present the case of a 2-month-old infant boy with cutaneous herpes simplex virus (HSV) infection within existing diffuse infantile seborrheic dermatitis. Providers should be aware that cutaneous HSV can be confined to a seborrheic distribution and may represent underlying epidermal dysfunction secondary to seborrheic dermatitis.

[Infantile atypical hand-foot-mouth disease with features of eczema herpeticum and acrodermatitis]. / Enfermedad boca-mano-pie atípica infantil con rasgos de eczema herpetico y de acrodermatitis.

Hand-foot-mouth disease can present atypically, including forms with more numerous lesions and/or morphologically different from the classic presentation. It may even mimic other viral diseases. We present the case of a 2-year-old child previously diagnosed with atopic dermatitis, who presented with papules and umbilicated vesicles affecting the perioral area and limbs, predominantly in pressure areas, as well as in areas with previous atopic lesions. Although he was clinically diagnosed with herpetic eczema, tests results were negative for herpes virus. However, positive entorovirus polymerase chain reaction results were obtained from the content of a vesicle, a pharyngeal exudate and a stool sample.

La enfermedad boca-mano-pie puede presentarse con formas atípicas, con lesiones más generalizadas y morfología diferente a la forma clásica. A veces, incluso simula otras enfermedades víricas. En las formas atípicas, existe la tendencia a afectar a las zonas de traumatismo o inflamación. Se presenta el caso de un niño de 2 años con antecedente de atopia, que consultó por presentar lesiones papulosas y vesiculosas umbilicadas que afectaban a la zona perioral, los miembros superiores e inferiores, con predominio en las zonas de presión y de dermatitis atópica previa. Aunque clínicamente se diagnosticó eczema herpético, las pruebas complementarias fueron negativas para herpes virus. Sin embargo, la reacción en cadena de la polimerasa del contenido de una vesícula, del exudado faríngeo y de heces fue positiva para enterovirus.

Diffuse facial rash in a former collegiate wrestler.

The patient described a history of "recurrent impetigo," which led to an uncommon diagnosis.

Disseminated herpes simplex virus: a case of eczema herpeticum causing viral encephalitis.

Eczema herpeticum is a dermatological emergency causing a mortality of up to 10% if untreated. It frequently presents in a localised form and rarely disseminates via haematogenous spread with pulmonary, hepatic, ocular and neurological manifestations. Although it commonly appears on a background of atopic dermatitis, many other dermatological conditions have been described preceding this disease. Eczema herpeticum can be easily mistaken for folliculitis and is often treated accordingly with antibacterial drugs; therefore patients will often deteriorate before a diagnosis of eczema herpeticum has been considered.

Persisting Ulcerating Lesions After Cyclosporine Therapy for Erosions Found in Flexural Sites.

In February 2012, a patient with a 15-year history of painful, pruritic eruptions presented with a severe exacerbation that had lasted for the previous week. The patient also reported severe headaches, a fever of 38.9°C (102.1°F), a cough, and diarrhea, which had now resolved. Physical examination showed erythematous macerated patches with erosions in the left submammary region, bilateral axillae, and vaginal area (Figure 1), and greasy scale covering the scalp. Despite lessening of the lesions in the submammary region and abdomen with the administration of minocycline 100 mg twice per day and cyclosporine 100 mg three times per day, the patient continued to complain of persistent painful ulcerations in the labia majora and perianal area (Figure 2). The patient was started on acyclovir 400 mg three times per day for 10 days for positive herpes simplex viral culture and ampicillin for Proteus infection of the area.