Pustular Rash Research Articles

CLINICAL CASE OF CAVERNITIS-COMPLICATED MONKEYPOX INFECTION

Abstract The article presents a clinical case of monkeypox (MPOX) infection, a rare acute viral zoonosis until 2022, previously endemic in Africa, but currently recorded worldwide, including Europe and Bulgaria. The clinical case describes a 35-year-old man, healthy before the current disease, hospitalized to urology clinic with a diagnosis of cavernitis-complicated acute balanoposthitis. In the department, surgical treatment of liberatio cordi periglandularis penis was performed. The combination of intoxication syndrome with characteristic elements of pustular rash, initially treated as staphyloderma, served as the basis for infectious disease specialist to assume that the patient had MPOX, which was subsequently confirmed by using polymerase chain reaction (PCR) method, after which the patient was transferred to the infectious disease clinic for isolation and treatment, which included detoxication, antibiotic and analgesic therapy. Upon discharge, the patient observed the quarantine regime until all elements of the rash had completely healed, and one-month follow-up observation after discharge showed no deviations from the norm. Due to the presence of a urogenital complication, cavernitis, a rare for this infection, a multidisciplinary approach was required, including urologists, dermatologists and infectious disease specialists. The surgical, pathogenetic and symptomatic treatment led to the patient complete recovery. Although monkeypox-related urogenital complications are rare, they can occur, which requires increased attention to such atypical disease manifestations. MPOX is a rare viral disease that has attracted the attention of health professionals due to its increasing incidence in countries where it is not typically endemic. The key finding of the current study is the necessity for increased vigilance by professionals of various specialties to rare and emerging infectious diseases such as MPOX to control their spread.

Clinical characteristics of suspected cases of human mpox (monkeypox) in Katako-Kombe, Democratic Republic of the Congo 2023: challenges and key responses.

This study aimed to describe the clinical and epidemiologic characteristics of suspected cases of human mpox in one of the most affected health zones, Katako-Kombe, Sankuru Province, in the Democratic Republic of the Congo. Also, to identify key challenges to prevent and improve the health of the affected community. Between January 26, 2023 and November 30, 2023, the DRC reported its highest incidence of mpox cases,with a total of 12,569 suspected cases in 156 health zones from 22 of the 26 country's provinces. The Katako-Kombe Health Zone registered 369 suspected cases and 17 deaths, making it one of the hotspots of the outbreak. Ten villages and centres were affected, with an attack rate (AT) of 2.15‰ and a case fatality rate (CFR) of 4.6%. Epi-curves showed an increase in clinical cases from January to May 2023, followed by a decrease until August 2023 and a resurgence of cases in September 2023, with a reproduction rate of 1.29. Furthermore, the occurrence of complications appeared to increase the CFR with 7 (14%), 11 (18.03%), and 9 (23.68%) for encephalitis, anemia, and pulmonary complications, respectively. AT, CFR, and transmission rate appeared to be lower in the current epidemic than in previous outbreaks and in Clade I. Critically, delayed medical care increases the incidence of complications and CFR. Therefore, mpox requires early diagnosis and prompt public health response. Including the possibility of mpox in the differential diagnosis of patients presenting with a vesicular or pustular rash is suggested.

Mpox (Monkeypox) On Board a Naval Warship.

We report a case of mpox in an active duty male on board a naval warship, who presented with a chancre-like penile lesion and ulcerating lymphadenopathy in the setting of a nonreactive treponemal test. Despite empirical therapy for sexually transmitted infections, he developed a fever and a generalized pustular rash. The patient was placed in isolation, and contact tracing was performed. No further spread of mpox was identified despite close contact with multiple shipmates and a pet rabbit. This case highlights the obligation of medical providers who care for our military personnel and veterans to offer JYNNEOS vaccine to high-risk individuals. Additionally, with the rise in clade I mpox cases prompting a recent global health emergency declaration by the World Health Organization, it is essential to implement proper testing methods and treatments when considering mpox in the differential diagnosis because of its association with other sexually transmitted infections.

Atypical neonatal cutaneous myiasis: A diagnostic challenge mimicking pustular rash in a resource-limited setting.

Neonatal cutaneous myiasis is a rare disease in newborns. Although it is unlikely, the level of suspicion for this condition should be high, especially in tropical regions. Treatment may deviate from the standard approach when necessary, and chemical debridement can be considered, particularly for smaller lesions. Ultimately, clinical judgment plays a key role in decision-making. Myiasis is the infestation of the skin of a mammal by larvae or maggots. The skin is the most affected organ. However, it can also affect other organs of the body. Gasterophilus and Hypoderma are two flies that produce creeping myiasis, a type of cutaneous myiasis our patient had. The infestation is common in Sub-Saharan Africa and most especially among rural dwellers. We report on a case of a 7-day-old term neonate who reported to St. Mary's Hospital Lacor, a Private-not-for-Profit hospital situated in the Northern region of Uganda, who was admitted as a case of neonatal sepsis with a focus on the skin initially, however, while on the ward was eventually diagnosed with Cutaneous Myiasis. The management plan included chemical debridement with Hydrogen peroxide, IV antibiotics, and other supportive therapies, and the neonate was ultimately discharged home after 9 days in the NICU. This case report aims to inform healthcare workers of the importance of heightening the index of suspicion of myiasis for neonates who present with sudden-onset rash that can resemble pustules and be mistaken for a pustular rash. Additionally, chemical debridement, in this case, underscores the importance of an innovative approach to managing cutaneous myiasis in resource-limited settings. Mass education and awareness programs focusing on proper hygiene practices, safe handling of newborns, and early recognition of symptoms can help mitigate the risk of myiasis.

Acute generalized exanthematous pustulosis: European expert consensus for diagnosis and management.

Acute generalized exanthematous pustulosis (AGEP) is a rare, usually drug-induced, acute pustular rash. Despite the lack of strong data supporting the effectiveness of topical or systemic corticosteroids in this drug reaction, they are widely used. More generally, there is no consensus on the diagnostic modalities and the management of patients with AGEP. We aimed to provide European expert recommendations for the diagnosis and management or patients with AGEP. Members of the ToxiTEN group of the European Reference Network (ERN)-skin, all dermatologists and/or allergologists with expertise in drug reactions, elaborated these recommendations based on their own experience and on a review of the literature. Recommendations were separated into the following categories: professionals involved, assessment of the diagnosis of AGEP, management of the patient and allergological work-up after the acute phase. Consensus was obtained among experts for the list of professionals involved for the diagnosis and management of AGEP, including the minimum diagnostic work-up, the setting of management, the treatments, the modalities and the timing of allergological work-up and follow-up. European experts in drug allergies propose herein consensus on the diagnosis and management of patients with AGEP. A multidisciplinary approach is warranted, including dermatologists, allergologists and pharmacovigilance services.

A rare blood transfusion complication: Bacillus thermoamylovorans bacteremia and diffuse pustular rash

Bacillus thermoamylovorans is a species of bacillus that causes structural defects in milk by contaminating milk with its heat-resistant spores. We report a case of diffuse pustular rash on the extremities and bacillus thermoamylovorans bacteremia in blood culture after erythrocyte suspension replacement. On admission, his acute phase reactants were elevated and he had a generalized rash. After appropriate antibiotherapy, his lesions regressed almost completely. There is no case report of blood transfusion-associated bacillus thermoamylovorans bacteremia in the literature. This case is presented to contribute to the literature to show that Bacillus thermoamylovorans bacteremia may play a role in the etiology of pustular rash and infectious reactions associated with blood transfusion.

Recognizing rare rashes: A case of acute generalized exanthematous pustulosis.

Acute generalized exanthematous pustulosis (AGEP) is a rare, pustular rash that occurs most commonly after exposure to a medication (typically antibiotics or diltiazem). This case describes a patient who developed a widespread pustular eruption shortly after beginning empiric antibiotics for community-acquired pneumonia. Diagnosis of AGEP was difficult in this scenario due to the patient's pulmonary infection and atypical skin biopsy results. However, after AGEP was correctly identified, the offending agents were discontinued and the patient had subsequent resolution of her symptoms.

'Monkeypox 2.0': Case series on a reconditioned virus causing sexually transmissible disease in urban population.

MPOX (Monkeypox) viral infection, a zoonotic disease previously confined to the African sub-continent, has caught attention worldwide recently due to its resurgence in a new 'avatar' among urban communities. Dermatologists in the U. A. E. started to see patients with fever and a self-limiting pustular necrotic rash that was negative for all other infectious investigations. We performed a prospective observational multicenter clinical study of the demographics, skin manifestations, and outcomes of patients presenting with necrotic pustular lesions and/or fever. 35 cases of PCR confirmed MPOX cases, mostly in the expatriate population, were followed up and found to have high-risk heterosexual contact on an average of 1week prior to disease onset. We found that they have characteristic annular pustular lesions with necrotic center or "Smoke ring pustules' in all cases. Lesion tenderness and predilection for the lower abdomen, pubic area, and genitalia were observed. Most cases were systemically stable, with fever lasting for an average of 4days and elevated CRP levels. Genital lesions were prone to secondary bacterial infections. The disease was severe, with larger annular plaques in one of our patients found to be living with HIV. The overall prognosis in healthy individuals is good, with lesions healing within an average of 2weeks without scarring. 'New world MPOX' should be unclassified from zoonosis to a sexually transmitted infection (STI) capable of transmission in an urban population. Our findings can help in early clinical suspicion and differentiation from other STI's for primary and secondary health care physicians.

Newborn with a pustular rash.

Newborn with a pustular rash.

Fahr's syndrome with dermatological manifestation

Fahr's syndrome is a rare condition characterized by the presence of calcifications in the basal ganglia, typically secondary to a disorder of phosphocalcic metabolism, primarily hypoparathyroidism. It is generally challenging to clinically suspect as it can remain asymptomatic or manifest with diverse symptoms not corresponding to any specific clinical pattern. The dermatological expression of this syndrome is exceptional and is linked to hypoparathyroidism and hypocalcemia. In a hypocalcemic environment, keratinocytes exhibit significant proliferative activity, which may explain the pustular rash in our patients. Diagnosis relies on the presence of bilateral and symmetrical calcifications in the basal ganglia. Treatment involves calcium and vitamin D supplementation. Therefore, it is important to consider this syndrome in the presence of any aseptic pustulosis associated with disorders of phosphocalcic metabolism, particularly in cases with associated neuropsychiatric pathologies. We retrospectively report observations from six patients with Fahr's syndrome revealed through dermatological manifestations over a five-year period.

Real-World Efficacy of Spesolimab: Reports from Four Patients with Generalized Pustular Psoriasis

Introduction: Generalized pustular psoriasis (GPP) is a rare, chronic, autoinflammatory skin disease characterized by widespread pustular eruptions that may be accompanied by systemic symptoms. GPP is painful, distressing, and adversely affects patient quality of life (QoL). Spesolimab is a first-in-class anti-IL-36 receptor monoclonal antibody approved for the treatment of GPP flares. Methods: We describe 4 cases from our respective clinical practices, where individuals with GPP were treated successfully with spesolimab. Results: Four patient cases of GPP were included; an 18-year-old male, a 58-year-old female; a 36-year-old male, and a 40-year-old female. One patient had a history of GPP, and had experienced 3 prior flares in the preceding year. Two patients had a history of plaque psoriasis. Events preceding the current GPP flare included hydroxychloroquine treatment (n=2), and corticosteroid treatment (n=3). All patients presented with sudden onset of worsening pustular rash (affecting lower extremities [n=1]; extremities and trunk [n=1]; whole body [n=2]). Two patients had systemic symptoms at presentation (1 case: fever, chills, malaise, fatigue; 1 case: severe weakness), and required hospitalization. Two patients received an initial mis-diagnosis of acute generalized exanthematous pustulosis (AGEP). Significant impacts on QoL were reported; all 4 patients were unable to perform normal daily activities; the 18-year-old patient was targeted at school because the skin lesions were perceived to be contagious. The 4 patients received multiple therapies prior to, and concomitantly with, spesolimab treatment, including topical agents, antibiotics, corticosteroids, retinoids, immunosuppressants, and biologics. All 4 patients received two 900 mg intravenous infusions of spesolimab with treatment intervals between 7 and 20 days. Treatment was given at an outpatient infusion center (n=3*), or using a home infusion service (n=1); *1 patient received their first infusion as an inpatient. Significant clinician-assessed improvement in pustules occurred within 1 week of the first spesolimab infusion in 3 patients; the remaining patient reported improvement 1-2 weeks after the second infusion. One of the hospitalized patients experienced 75% improvement in itching, pain, and pustules within 24 hours of the first spesolimab infusion, and was discharged the next day. Spesolimab treatment also improved QoL, with patients experiencing rapid improvements in pain, and their ability to resume their normal daily activities. Conclusions: These cases demonstrate the efficacy of spesolimab in the real-world, highlighting the ability of spesolimab treatment to rapidly improve GPP symptoms and patient QoL. Administration of spesolimab in an inpatient setting allowed for rapid patient improvement and discharge the next day. These cases also highlight the association of certain drugs as triggers for GPP flares, as well as the importance of AGEP in the differential diagnosis of GPP

Validation of a new extraction-free real-time PCR test to detect MPOX virus

The monkeypox (Mpox) virus has raised significant concerns given its recent spread with an increasing number of confirmed cases worldwide. In this study, we evaluated the performance of a laboratory developed test (LDT) using BioGX Xfree hMPXV/OPXV reagents for the qualitative detection of non-variola Orthopoxviruses and Mpox virus DNA, in swabs from human pustular or vesicular rash specimens. Analytical and clinical testing analysis were carried out on two different platforms: the BD MAX™ System (BD Diagnostics) and the new pixl.16 Real-Time PCR Platform (BioGX), using a synthetic Mpox virus DNA (ATCC VR-3270SD) and residual clinical samples previously identified with an EUA approved Mpox real-time PCR assay. In the end, the Xfree hMPXV/OPXV LDT proved to be a sensitive, specific, and reproducible test for the detection of Mpox on both platforms evaluated with the pixl.16 having an advantage of a small footprint and providing faster TAT facilitated by an extraction-free workflow.

Monkeypox Virus Immune Evasion and Eye Manifestation: Beyond Eyelid Implications.

Monkeypox virus (MPXV), belonging to the Poxviridae family and Orthopoxvirus genus, is closely related to the smallpox virus. Initial prodromal symptoms typically include headache, fever, and lymphadenopathy. This review aims to detail various ocular manifestations and immune evasion associated with the monkeypox viral infection and its complications, making it appropriate as a narrative review. Common external ocular manifestations of MPXV typically involve a generalized pustular rash, keratitis, discharges, and dried secretions related to conjunctival pustules, photophobia, and lacrimation. Orthopoxviruses can evade host immune responses by secreting proteins that antagonize the functions of host IFNγ, CC and CXC chemokines, IL-1β, and the complement system. One of the most important transcription factors downstream of pattern recognition receptors binding is IRF3, which controls the expression of the crucial antiviral molecules IFNα and IFNβ. We strongly recommend that ophthalmologists include MPXV as part of their differential diagnosis when they encounter similar cases presenting with ophthalmic manifestations such as conjunctivitis, blepharitis, or corneal lesions. Furthermore, because non-vaccinated individuals are more likely to exhibit these symptoms, it is recommended that healthcare administrators prioritize smallpox vaccination for at-risk groups, including very young children, pregnant women, older adults, and immunocompromised individuals, especially those in close contact with MPXV cases.

Results of Combined Pathogenetic Therapy in Netherton Syndrome: Clinical Case

Background. Netherton syndrome is a severe autosomal recessive disease based not only on genetically determined keratinization disorders, but also on immune system dysregulation.Clinical case description. Boy K., 9 years old, diagnosed with severe atopic dermatitis and revealed Netherton syndrome during examinations. The dupilumab was administered due to the presence of severe polysensitization and clinical signs of allergic process. Wave-like course of the disease was observed during this therapy. Patient had acute exacerbation — generalized pustular rash — at the 7th month of treatment. The therapy with secukinumab was initiated according to the foreign literature on pathogenetic treatment of Netherton syndrome with interleukin (IL) 17 inhibitor. It has shown positive dynamics. Currently patient is administered with the combined therapy of IL-17 and IL-4/13 inhibitors with significant positive effect.Conclusion. Genetically engineered biologic drugs targeting both the Th17 and Th2 have shown their efficacy in Netherton syndrome management.

OA06 Behçet's associated myositis: an unusual cause of exercise-induced leg pain

Abstract Introduction This case describes a 29-year-old gentleman of Cypriot heritage who presented with exercise-induced bilateral lower leg pain and swelling which made it difficult for him to cycle. Symptoms progressed over months to calf pain at rest accompanied by fatigue. He subsequently presented with central visual loss in the right eye, followed by the left. The second episode of visual loss coincided with development of oro-genital ulceration and, following rheumatology review, a diagnosis of Behçet’s disease was made. The case is significant in terms of the chronicity and nature of presenting symptoms which is not typically described in this disease. Case description A 29-year-old gentleman presented with exercise-induced bilateral lower leg pain and swelling for which he was reviewed in emergency care on four occasions. He received treatment for presumed cellulitis and underwent US-doppler studies, excluding thromboembolic disease. MRI of the lower legs was requested given lack of diagnosis with ongoing symptoms. This demonstrated bilateral muscle oedema in the antero-lateral compartments and lateral gastrocnemius heads, prompting rheumatology referral. Prior to attending rheumatology, the patient developed bilateral sequential visual loss. This was attributed to right acute macular neuroretinopathy, followed by significant left posterior pole choroidal ischaemia 2 months later. Each presentation was associated with transient low-grade anterior uveitis, vitritis, and the presence of a pre-papillary vitreous infiltrate. Initial treatment included a six-week oral prednisolone taper from 60mg daily. He reported a progressive leg pain which initially occurred during cycling but later affecting his walking. He was of Cypriot heritage, with no family history of autoimmune disease. A focused history revealed oral ulceration and an acne-like pustular rash over the back. His gait was antalgic with minimal toe-off phase. Investigations demonstrated raised ESR and CRP, negative autoimmune immunology, negative myositis ENA, normal CK, and positive HLA-B51. Given these features, a provisional diagnosis of Behçet’s was made. Nerve conduction studies were requested to consider whether changes could be secondary to denervation. They displayed non-specific S1 neurogenic changes and small tibial motor amplitudes. Subsequent MRI lumbar spine excluded radiculopathy. Repeat CK remained within normal limits. Muscle biopsy was sought and performed with a clinical suspicion of atypical myositis which has previously been reported in Behçet’s. At the second episode of visual loss, he was admitted under ophthalmology and treated with intravenous methylprednisolone and azathioprine. The case was discussed with our regional centre of excellence for Behçet’s and infliximab at 5mg/kg was commenced. Discussion This case was notable for the complexity and atypical presenting features with calf pain induced by exercise likely due to atypical myositis (biopsy report still awaited at time of submission). More classical Behçet’s disease symptoms including oro-genital ulceration were significantly later. The case highlights the importance of multidisciplinary working and involving specialist centres especially when rapid escalation to biologic treatment is required. Leg symptoms are persistent but improved despite ongoing treatment with prednisolone and azathioprine with the recent addition of infliximab. Visual symptoms have stabilised and skin involvement has settled. Management Rationale At the time of first review in rheumatology clinic, high-dose steroid treatment had already been initiated; therefore, expediting the investigative aspect of his management felt most appropriate, particularly given that myositis seemed to be the predominant feature at that time. In joint consultation with neurophysiology and neurology, putting the EMG and MRI findings in context, MRI of the lumbar spine was felt necessary to exclude discogenic disease causing denervation oedema. Unfortunately, visual symptoms progressed rapidly after this, requiring admission, which precluded an earlier opportunity for biopsy but leading to quick authorisation and organisation of treatment with infliximab. Key learning points This case highlights an atypical presentation of Behçet’s disease including associated likely atypical myositis. Causes of exercise-induced leg pain in a young, fit patient need to be considered: The need for multidisciplinary discussion both across specialties and with regional centres is key in order to progress with diagnostics and treatment In sharing this case for discussion, we wish to highlight possible causes of exercise-induced calf pain and a pathway for investigation of this. Our patient had atypical myositis as the cause for his symptoms in the context of multi-system disease. Both his myositis and ophthalmology presentations were somewhat atypical but multidisciplinary working, along with the development of some further symptoms, were helpful in making the diagnosis.

Resurgence of Human Monkey Pox: A Review

After being prevailing in Central and West Africa throughout the 1970s, sporadic cases of monkeypox sickness have surfaced in recent years. On the other hand, the monkey pox outbreak in the United States made headlines and garnered international attention in 2003. In 2022, the virus has caused a catastrophic pandemic, with over 50 nations being affected. There were 183 new cases of monkeypox recorded to WHO in April 2023, representing a 0.2% rise in overall cases, and 14 new deaths from the disease. Monkey pox shares the poxviridae family with the smallpox virus. One explanation for the rise of monkeypox in people is the discontinuation of smallpox immunizations. The zoonotic infectious illness is distinguished by a pustular rash resembling smallpox and systemic infection with severity ranging from mild to severe. Though the condition is self-limiting, the consequences it causes can be deadly. The illness has no particular therapy, however, it can be treated using antivirals that are effective against smallpox. Vaccines for smallpox have also been shown to be effective. The current article addresses the signs and symptoms of monkeypox sickness and the transmission pathways and treatment choices.

Adjuvant Topical Interferon Alpha 2b for the Treatment of Monkeypox Ocular Manifestations.

The aim of this study was to report a case of ocular Mpox that responded favorably to treatment with topical interferon and oral doxycycline. This is a case report of a previously healthy 24-year-old woman who developed a pustular rash, headache, fever, arthralgia, sore throat, and asthenia 3 weeks before attending to our clinic. Her main complaint at the moment of the visit was pain, photophobia, foreign body sensation, blurred vision, red eye, and discharge on the left eye. The slit-lamp examination of the left eye showed severe conjunctival hyperemia associated with tarsal follicles, 360 degrees ciliary injection, diffuse corneal epithelial edema with white linear epithelial infiltrates, pigmented and nonpigmented keratic precipitates, and two 1-mm peripheral corneal ulcers with white infiltrates, associated with positive fluorescein staining. Anterior chamber cellularity and flare were mildly present. Mpox with ocular manifestations diagnosis was confirmed by real-time quantitative reverse transcription polymerase chain reaction assay (qRT-PCR) testing; samples were taken from corneal, conjunctival, and nasopharynx swab as well as a skin scab. Topical interferon alpha 2b 1 MIU/mL every 6 hours for 1 month and oral doxycycline 100 mg BID were administered along with other medications with consequent decrease of inflammation and malaise symptoms 1 week later, associated with uncorrected visual acuity improvement. Alternative and efficacious treatment options for Mpox ocular manifestations are needed to prevent further disease progression and sequelae in countries with no access to the gold-standard therapy. Topical interferon alpha 2b and oral doxycycline have shown adequate response as shown with this patient.

PA12 A case of pustular psoriasis in epidermolytic ichthyosis: coincidence or common pathway?

Abstract An 8-year-old boy with epidermolytic ichthyosis (EI) due to a heterozygous KRT1 mutation presented with a striking bullous skin eruption. Microbiological swabs were sent, and oral antibiotics were prescribed for suspected bullous impetigo. He deteriorated clinically, developing a fever and more extensive pustular rash reminiscent of generalized pustular psoriasis (GPP). Skin biopsies from two distinct cutaneous sites revealed histology strongly supportive of pustular psoriasis. Oral ciclosporin was commenced in addition to intensive topical emollient therapy. Following widespread desquamation, the pustular eruption fully resolved. Recurrence upon weaning of ciclosporin necessitated the introduction of ustekinumab. The rash remains controlled on this therapy. The EI is caused by heterozygous mutations in KRT1 or KRT10, and presents at birth with erythroderma and widespread skin denudation. The erythema and skin fragility abate in early childhood, giving way to prominent hyperkeratosis. Severe palmoplantar keratoderma is seen in those with KRT1 mutations. The GPP is a rare but life-threatening condition characterized by sterile pustules with or without systemic involvement. Interleukin (IL)-36-mediated inflammatory signalling plays a pathogenic role, as evidenced by loss of function mutations in IL36RN, which encodes an IL-36 receptor antagonist (Marrakchi S, Guigue P, Renshaw BR et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365:620–8), and the efficacy of IL-36 receptor inhibitor spesolimab in a recent phase II trial. Both EI and GPP are rare, and the confluence of these entities in this case is striking. Four other cases in the literature describe a GPP-like eruption in patients with congenital ichthyoses. The coexistence of these conditions might be explained by their immunological profiles. Studies of the skin of patients with congenital ichthyoses have shown immune dysregulation with T helper 17/IL-23 skewing, and upregulation of associated inflammatory cytokines, including IL-36γ. The latter encodes for an IL-36 receptor agonist (Paller AS, Renert-Yuval Y, Suprun M et al. An IL-17-dominant immune profile is shared across the major orphan forms of ichthyosis. J Allergy Clin Immunol 2017; 139:152–65). Thus, increased IL-36 receptor agonist activity in congenital ichthyosis could theoretically lead to GPP in genetically predisposed individuals with already upregulated IL-36 receptor-mediated signalling.

T-cell chronic active EBV diagnosed in a 65-year-old male presenting as idiopathic hypereosinophilic syndrome

A 65-year-old male from Nigeria, admitted with dehydration and painful oral ulcers, is seen by allergy/immunology for hypereosinophilia.The patient had idiopathic eosinophilia (mean 5,000 cells/uL) ongoing for nine years. There were no evident drug or infectious causes. The eosinophilia peaked at 28,000 cells/µL, accompanied by parotid swelling, pustular skin rash, and elevated serum-IgG4 level (400 mg/dL). Rheumatology evaluated for IgG4-related disease and hypereosinophilic syndrome. This work-up demonstrated elevated IgE (39,000 IU/mL) and tryptase (40 ng/mL) levels. Bone marrow biopsy showed a hypercellular marrow with erythroid hyperplasia and eosinophilia. Submandibular gland, skin, and excisional lymph node biopsies demonstrated IgGsecreting plasma cells, lymphocytic/eosinophilic infiltrates, and lymphoid hyperplasia, respectively. All biopsies stained negative for IgG4. Treatment for idiopathic hypereosinophilia was initiated with corticosteroids, rituximab, and mycophenolate mofetil (MMF). Symptomatic improvement was attained on MMF (1000 mg twice daily) with the addition of mepolizumab (300 mg every 4 weeks).He unfortunately progressed to high fevers with elevated ferritin (7,286 ug/L) and soluble IL-2Ra (31,276.7 pg/mL) levels. He did not meet full criteria for hemophagocytic lymphohistiocytosis and improved after corticosteroids. EBV PCR from blood was >220,000 IU/mL.Dedicated immune work-up showed a non-specific population of lymphocyte-like cells without clear lineage markers (CD2dim/- surface CD3-CD4-CD8-CD7-CD56-CD49d-CD294dim) on peripheral blood flow cytometry, with otherwise normal T, B, and NK cell enumeration. There was reduced cytotoxic killing function of CD8+ T cells and NK cells by chromium-51 release assay. Sorted peripheral blood mononuclear cells demonstrated positive EBV viral load in B cells (1,018,239 IU/mL), T cells (857,418 IU/mL),NK cells (237,459), and myeloid cells (532,641 IU/mL). EBV-encoded RNA was positive within lymph node T cells, most consistent with a diagnosis of T-cell CA-EBV. The Invitae 407 gene immunodeficiency panel was sent; however, a pathogenic variant was not identified. The patient was seen by hematology/oncology with plans for hematopoietic stem cell transplant. Unfortunately, he succumbed to complications from CA-EBV and multiorgan failure.We describe a male with lymphocytic-variant hypereosinophilic syndrome and a longstanding constellation of inflammatory and lymphoproliferative symptoms due to CA-EBV. His course initially eluded his care team and only after careful multidisciplinary discussion was a diagnosis reached.

Newborn with a pustular rash.

Journal of Paediatrics and Child HealthEarly View Case Image Newborn with a pustular rash Dr Adambarage Chandima De Alwis, Dr Adambarage Chandima De Alwis orcid.org/0000-0002-7871-7517 Department of Paediatrics, Bathurst Base Hospital, Bathurst, New South Wales, AustraliaSearch for more papers by this authorDr Jessica Lin, Dr Jessica Lin Department of Paediatrics, Bathurst Base Hospital, Bathurst, New South Wales, AustraliaSearch for more papers by this author Dr Adambarage Chandima De Alwis, Dr Adambarage Chandima De Alwis orcid.org/0000-0002-7871-7517 Department of Paediatrics, Bathurst Base Hospital, Bathurst, New South Wales, AustraliaSearch for more papers by this authorDr Jessica Lin, Dr Jessica Lin Department of Paediatrics, Bathurst Base Hospital, Bathurst, New South Wales, AustraliaSearch for more papers by this author First published: 13 April 2023 https://doi.org/10.1111/jpc.16413Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation