Pustular Eruption Research Articles

Imsidolimab, an anti-interleukin-36 receptor monoclonal antibody, for the treatment of generalized pustular psoriasis: results from the phase II GALLOP trial.

Generalized pustular psoriasis (GPP) is a systemic inflammatory disease that can be severe, debilitating and life threatening. Uncontrolled activation of interleukin (IL)-36 proinflammatory activity may underlie the pathogenesis of GPP. Currently, GPP-specific treatment options are limited. To evaluate the efficacy and safety of the anti-IL-36 receptor antibody imsidolimab in patients with GPP. In an open-label, single-arm, multiple-dose study, patients with GPP were treated with imsidolimab to assess clinical efficacy, tolerability and safety. Patients received an intravenous dose of imsidolimab 750 mg on day 1, followed by three subcutaneous doses of imsidolimab 100 mg administered on days 29, 57 and 85. The primary efficacy endpoint was the proportion of patients who achieved a clinical response at weeks 4 and 16 following treatment with imsidolimab, as measured by the Clinical Global Impression scale. Eight patients were enrolled and six completed the study. Responses were observed as early as day 3, most rapidly for pustulation relative to other manifestations of GPP, with continued and consistent improvement across multiple efficacy assessments at day 8, day 29 and through day 113. Most treatment-emergent adverse events (TEAEs) were mild to moderate in severity. No patient discontinued the study owing to a nonserious TEAE. Two patients experienced serious adverse events (SAEs); no deaths were reported. Imsidolimab demonstrated a rapid and sustained resolution of symptoms and pustular eruptions in patients with GPP. It was generally well tolerated, with an acceptable safety profile, and is advancing to phase III trials. These data support the targeting of IL-36 signalling with a specific antibody - imsidolimab - as a therapeutic option for this severely debilitating condition.

Teaching Neuro Images : Longitudinally extensive transverse myelitis in neuro-Behçet disease

A 25-year-old man presented with subacute spastic paraparesis. He reported 2 previous episodes of spastic paraparesis with partial recovery. Recurrent oral and genital ulceration, pustular skin eruptions, and fever coexisted. Profound motor weakness, a sensory level at T10, oral ulceration, and a pustular eruption on the anterior abdominal wall were noted. Marked neutrophilia was noted in both blood and CSF. Neuromyelitis optica–immunoglobulin G autoantibody was negative. MRI (figure) demonstrated marked inflammatory changes. IV and oral steroids, followed by 6 months of pulsed IV cyclophosphamide, resulted in marked clinical improvement. Neuro-Behçet disease lies within the clinical differential for longitudinally extensive transverse myelitis.1,2

Hyper‐IgE syndrome presenting with early life craniosynostosis in monozygotic twin sisters

<scp>Hyper‐IgE</scp> syndrome presenting with early life craniosynostosis in monozygotic twin sisters

Trimethoprim - Sulfamethoxazole Induced Sweet Syndrome: A Case Report

Sweet syndrome (SS) also called as acute febrile neutrophilic dermatosis, is a rare inflammatory infectious skin reaction characterized clinically by tender, erythematous papules or plaque commonly appearing on the upper limbs, trunk, and head and neck. It is usually accompanied by fever and an elevation in the erythrocyte sedimentation rate (ESR), C- reactive protein. SS is classically triggered by infections, inflammatory bowel disease, pregnancy, malignancy or due to the administration of an associated drugs. Drug induce sweet syndrome (DISS) an uncommon subtype of SS. Drugs like antiepileptics like carbamazepine, diazepam, antibiotics like trimethoprim-sulfamethoxazole (TMP-SMX), minocycline, nitrofurantoin, norfloxacin, ofloxacin, contraceptives, non-steroidal anti-inflammatory drugs (NSAIDS) (celecoxib and diclofenac) and granulocyte – colony stimulating factor are the most common culprit. In this case, the patient was presented with oedematous and pustular eruption of nose with a rapid extension to the face, neck, upper trunk and the limbs which developed after taking TMP-SMX for 10 days accompanied with fever (42ºC) and cough. Laboratory test disclosed elevated WBC with notable increase in polymorphonuclear cells ,elevation of ESR (96mm/h), C-reactive protein (85ng/l, normal &lt;5) and α-2-globulin (10g/l, normal &lt;8). All these presentations clearly indicated that the patient is having DISS as per Walker and Cohen criteria for SS. TMP-SMX was discontinued upon which the lesions started to disappear. Clinical pharmacist must be more aware about the usage of TMP-SMX especially in immune compromised patients. Keywords: Drug induced sweet syndrome, Trimethoprim-sulfamethoxazole, Neutrophilic infiltration, Corticosteroids, oedematous papules,

Reactive Neutrophilic Dermatoses in Adult-Onset Immunodeficiency due to Interferon-Gamma Autoantibody and Their Associated Factors

Background: Adult-onset immunodeficiency (AOID) due to interferon-gamma autoantibody is a rare, acquired immunodeficiency disease. Reactive neutrophilic dermatoses (RND), predominantly Sweet syndrome (SS), and generalized pustular eruption have been reported repeatedly. Objectives: The aims of this study were to describe the cutaneous manifestations in AOID patients and determine the incidence of RND and associated factors using a larger population size than have been previously reported. Methods: A retrospective chart review of all confirmed AOID cases in Chiang Mai University Hospital from January 2006 to June 2020 was conducted. The demographics and characteristics of RND including type, onset, and laboratory information in every episode of cutaneous manifestations were collected. Generalized estimating equations of binary logistic regression were used to determine the indicators of RND. Results: A total of 146 patients with confirmed AOID were identified. Of these, 57 cases (39%) developed at least one episode of RND. Thirteen cases (23%) of the patients experienced RND twice during the follow-up period. All recurrence of RND displayed the same cutaneous phenotype, with the exception of 2 cases who had both SS and generalized pustular eruption. Finally, 49 episodes of SS and 22 episodes of generalized pustular eruption were included in the analysis. All patients with RND had concomitant active opportunistic infections, of which most were non-tuberculous mycobacterium (NTM) infection. NTM infection (prevalence odds ratio [POR] 2.87), lymphadenopathy (POR 3.30) as well as lower serum alkaline phosphatase (ALP) level (POR 0.71 for every 100-unit increment in ALP) were found to be significantly associated with RND occurrence. Conclusions: 39% of our AOID patients experienced RND once during the course of the disease. Notable factors associated with RND occurrence were concomitant NTM infection, lymphadenopathy, and lower level of ALP.

Unique Presentation and Unusual Cause of Acute Generalized Exanthematous Pustulosis: Case Report

Acute generalized exanthematous pustulosis (AGEP) is a febrile, pustular eruption that has been reported in all ages. Three weeks following oral terbinafine use, a 64-year-old male patient was admitted to the hospital for diffuse, nearly confluent erythematous plaques and desquamation, fevers, chills, and ulcer formation on his lower mucosal lip and tongue. Ten days prior to presentation, he was evaluated and discharged with a prednisone 60 mg taper for suspected erythema multiforme. Terbinafine was discontinued and the patient was monitored for systemic involvement. Recognition of subtle pustules on a background of EM-like lesions may facilitate the timely diagnosis and appropriate treatment of AGEP.

Dermoscopy of Acrodermatitis Continua of Hallopeau.

A 29-year-old female presented with recurrent episodes of painful pustules over the right thumb for the past 8 months. She was treated with oral antibiotics along with avulsion of the nail plate, but it did not improve her condition. She had no history of other skin disorders. On examination, the nail plate was absent. Erythematous plaque with scaling and few areas of crusting were present involving the nail bed and pulp of the right thumb [Figure 1a] along with a single pustule on the right thumb [Figure 1b]. Dermoscopy (Polarized contact light dermoscopy; Dermlite DL4; 10×) revealed yellowish-white scales and linear blood vessels over the nail bed along with few hemorrhagic spots [Figure 2a]. Additionally, yellow globules were seen on the lateral nail fold and dotted vessels arranged in a regular pattern were seen over the pulp area [Figure 2b]. A Gram stain was done from the pustule, which was sterile and showed only neutrophils. A diagnosis of acrodermatitis continua of Hallopeau (ACH) was made based on the clinical and dermoscopic correlation. She was treated with oral cyclosporine and topical calcipotriol with good improvement in 2 weeks [Figure 3a and b].Figure 1: (a) The nail plate is absent. Erythematous plaque with scaling and few areas of crusting is seen involving the nail bed and pulp of the right thumb. (b) A single pustule is seen on the right thumbFigure 2: (a) Dermoscopic image showing yellowish-white scales (black arrow) and linear blood vessels over the nail bed (blue arrow) along with few hemorrhagic spots (red arrow). (b) Dermoscopic image showing dotted vessels arranged in a regular pattern (yellow arrows) over the pulp area. (Polarized contact light dermoscopy; Dermlite DL4; 10×)Figure 3: Clinical (a) and dermoscopic (b) images showing near-complete resolution of the lesions after 2 weeks. (Polarized contact light dermoscopy; Dermlite DL4; 10X)ACH, a variant of pustular psoriasis, is a rare, sterile pustular eruption of the distal fingers and/or toes. It is a chronic and progressive condition and can lead to irreversible complications including onychodystrophy and osteitis, which may result in anonychia and distal phalanx osteolysis, respectively; hence, an early and prompt diagnosis is required.[1] Dermoscopy of pustular psoriasis reveals yellow globules and regularly distributed dotted vessels which on histology correspond to nonfollicular superficial pustules and dilated vessels in homogenously elongated dermal papillae, respectively.[2] E. Errichetti et al. described dermoscopic findings in two cases of ACH which showed whitish-yellowish hyperkeratosis/scaling along with single or multiple pustules, dotted and linear vessels, and hemorrhagic spots.[3] These findings aid in differentiating ACH from other localized conditions such as onychomycosis, nail lichen planus, acral hand eczema, and psoriatic onychopathy.[3] We report our case to highlight the dermoscopic features of ACH which can help in the preliminary evaluation, thus reducing the diagnostic delay. As biopsy over the distal digits is often distressing and difficult, dermoscopy can be considered a valuable tool in such cases. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Acrodermatitis Continua of Hallopeau: A Diagnostic Challenge.

Acrodermatitis Continua of Hallopeau: A Diagnostic Challenge.

Generalized Pustular Eruption in a Patient with Adult-Onset Immunodeficiency Due to Anti-Interferon-Gamma Autoantibodies.

Generalized Pustular Eruption in a Patient with Adult-Onset Immunodeficiency Due to Anti-Interferon-Gamma Autoantibodies.

Tozinameran (Pfizer-BioNTech COVID-19 vaccine)-induced AGEP-DRESS syndrome

Dear Editor, A 40-year-old woman with scalp psoriasis was maintained on topical mometasone furoate therapy from 2019 with no major psoriatic flares until she presented with one week of an acute generalised pustular eruption and fever in June 2021. Physical examination revealed numerous discrete and confluent pustules studded on her scalp, trunk and limbs, on

A Case of Pustular Drug Eruption due to Axitinib Triggered by Lower Leg Burn

患者：69 歳，女性。腎癌の既往があり，肺，肝臓，骨転移を合併している。自宅でストーブにあたっていて左下腿に熱傷を受傷した。浅在性 2 度熱傷と判断し，外来で保存的加療を行っていた。しかし，熱傷の上皮化が遷延し第 14 病日を過ぎて熱傷創やその周囲に膿疱や膿海がみられるようになってきた。強い疼痛を伴っていた。膿疱の生検病理組織像では角層内の好中球浸潤の他，表皮内，真皮内に多数の好酸球が認められた。第 19 病日に，投与中であったアキシチニブを被疑薬とした膿疱型薬疹と診断した。入院の上，同薬の休薬とプレドニゾロン（30 mg/day）の内服を行ったところ，膿海は速やかに上皮化した。 膿疱が消退したのでプレドニゾロンを漸減して退院した。文献を渉猟したところ，アキシチニブを含めたチロシンキナーゼ阻害薬は好中球の遊走能に影響を及ぼし，膿疱を形成しやすいことが示された。これらの薬剤の皮膚障害に対して皮膚科医は積極的に取り組むべきと結論した。

Pustular Psoriasis

A woman in her 60s with no past dermatologic history presented with a 4-month history of a pustular eruption. Before onset she had received steroid injections for cervical radiculopathy, but there were no other new medications or illnesses. Dermatologic exam showed more than 90% body surface area involvement with erythematous patches studded with pustules and diffuse skin sloughing (Figure 1) (Supplemental Figure 1, available online at http://www.mayoclinicproceedings.org). Skin biopsy specimens showed subcorneal neutrophilic pustules with hypogranulosis (Figure 2).

POSTSURGICAL ACUTE GENERALIZED EXANTHEMATOUS PUSTULOSIS: TIMING IS EVERYTHING

<h3>Introduction</h3> Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction commonly caused by antibiotics. <h3>Case Description</h3> A 62-year-old male was evaluated for a painful and burning pustular eruption three days after a hip arthroplasty. Medication timeline was notable for intraoperative (3 days prior) receipt of cefazolin, dexamethasone, hydromorphone, ropivacaine, and tranexamic acid. Two days prior, he received oxycodone, ondansetron, omeprazole, sennosides, and dexamethasone. One day prior to presentation he received meloxicam and aspirin. Physical examination revealed confluent erythema studded with pinhead-sized sterile pustules on the bilateral thighs, under arms (Figure 1A), and trunk. Complete blood count with differential showed mild neutrophilia (7.59; ref 1.56-6.45 × 10⁻⁹/L) and a complete metabolic panel was within normal limits. Histopathology of a punch biopsy from the right thigh revealed subcorneal pustules with epidermal spongiosis, mild papillary dermal edema and a mixed perivascular infiltrate (Figure 1B) consistent with AGEP. He was instructed to avoid all beta-lactams, non-steroidal antiinflammatory agents (NSAIDs), and steroids. The eruption completely resolved within 10 days of avoidance measures and antihistamines. There were plans to perform patch testing to cefazolin, prednisone, and dexamethasone. Unfortunately, the patient was lost to follow-up. <h3>Discussion</h3> AGEP is diagnosed clinically and confirmed by a skin biopsy. Patch testing may be utilized to evaluate for the culprit agent; but when this is not available, the timeline between medication exposure and index reaction may aid in causal agent identification. The median time from exposure to onset of AGEP is 1 day for antibiotics versus 11 days for all other medications.

Monkeypox Disease: An Emerging Public Health Concern in the Shadow of COVID-19 Pandemic: An Update.

The last few decades have witnessed an appalling rise in several emerging and re-emerging viral and zoonotic outbreaks. Such outbreaks are a lesson to learn from and seek insight into better disease monitoring and surveillance, thus preventing future outbreaks. Monkeypox, a viral zoonotic illness caused by the monkeypox virus, may no longer be endemic to the tropical rainforests of Central and West Africa. However, the current monkeypox outbreak in nonendemic countries is most likely due to failure to curb the disease dissemination in endemic African regions despite decades of constant outbreaks. The clinical manifestations are typified by a prodromal phase (fever, myalgia, malaise, and lymphadenopathy) followed by maculopapular or vesicular, or pustular cutaneous eruptions that eventually form encrustations and peel off. Children and the elderly, pregnant females, and individuals living with comorbidities (diabetes, HIV/AIDS, and lymphoproliferative ailments) are at a high risk of severe disease. Monkeypox is a self-limiting disorder, but its complications and pandemic potential signify its immense public health relevance. The recent ongoing monkeypox outbreak in nonendemic nations areas was identified with increased propensity in men who have sex with men (MSMs) with no travel history to endemic regions, emphasizing the changing trends in disease transmission. This review article provides an updated overview of the monkeypox disease taxonomy, pathogenesis, transmission, epidemiology, clinical and oral features, diagnostic aids, differential diagnosis, preventive aspects, and treatment protocol.

Homoeopathic management of Acne vulgaris: A case report

This is a case of 18yrs old female having pustular eruptions on face treated with individualised homoeopathic medicine. When patient came for consultation, she was presented with pustular eruptions which are painful and leaves scar marks on recovery since last 3 years. Systematic case taking followed by repertorzsation of the totality of symptoms was done by Synthesis repertory using RADAR software in order to choose the individualized remedy, and a few medicines including Nat mur, and others were shortlisted. The patient responded well to the individualized homoeopathic treatment, i.e., Nat mur 200 in 3 doses with gradual and steady recovery.Abbreviations: Aetius of Amida (AOA), Acne of life (AOL), Hypertension (HTN)

Damaging Alleles Affecting Multiple CARD14 Domains Are Associated with Palmoplantar Pustulosis

Palmoplantar pustulosis (PPP) is a severe pustular eruption that affects the palms and/or soles, with detrimental effects on quality of life. The disease is notoriously difficult to treat because its immune and genetic determinants remain poorly defined (Twelves et al., 2019). Although mutations of the IL36RN and myeloperoxidase MPO genes have been convincingly associated with generalized pustular psoriasis, they are rarely found in patients with PPP (Haskamp et al., 2020; Twelves et al., 2019; Vergnano et al., 2020).

Monkeypox: Cutaneous clues to clinical diagnosis

Monkeypox: Cutaneous clues to clinical diagnosis

34442 Pustular psoriasis of pregnancy: A unique presentation

A 31-year-old pregnant female at 35-weeks gestation presented with a 1-week history of a painful, generalized pustular eruption consisting of tender erythematous plaques embedded with numerous pinpoint pustules. The eruption initially involved the acral surfaces including the dorsal and palmar hands as well as dorsal feet, and it progressed proximally to the remainder of the extremities and lower abdomen. Associated symptoms included arthralgias, distal extremity swelling, and left axillary lymphadenopathy.

34617 Imsidolimab in the treatment of adult subjects with generalized pustular psoriasis: Design of a pivotal phase 3 clinical trial and a long-term extension study

Generalized pustular psoriasis (GPP) is a debilitating, and life-threatening systemic inflammatory disease with no approved therapies in the US or EU. Treatment of recurrent flares includes use of off-label systemic immunomodulators with potentially concerning safety risks. Imsidolimab (ANB019) is a high-affinity, humanized, anti-IL-36 receptor (IL-36R) monoclonal antibody that antagonizes IL-36 cytokine signaling that is being developed to treat GPP and other inflammatory dermatoses. A Phase 3, international, multicenter, randomized, double-blind, placebo-controlled study (ANB019-301) to evaluate the efficacy and safety of imsidolimab in adult subjects with active GPP was designed and initiated following completion of an open-label, single-arm, multiple-dose Phase 2 study (NCT03619902) that demonstrated rapid resolution of pustular eruptions and systemic symptoms in subjects with GPP flare.

Paracetamol Induced Acute Generalized Exanthematous Pustulosis- A Rare Case Report

Acute generalized exanthematous pustulosis (AGEP) is a rare cutaneous adverse drug reaction characterized by rapid occurrence of dozens to thousands pinhead-sized, non-follicular, sterile pustular eruptions. AGEP is infrequent with an incidence of one to five millions per year. There are only two previous reports of paracetamol induced AGEP in literature. The clinical course of AGEP is characterised by spontaneous resolution on drug withdrawal. Resolution is marked by a characteristic desquamation. Diagnosis of AGEP depends on morphology of skin lesions, presence of fever, laboratory and histopathological findings. Factors that favours the diagnosis of AGEP include onset of pustules within few hours or in few days after the causative agent is administered. The most frequent causative drugs are Aminopenicillins, ampicillin, amoxixillin, sulphonamides, pristinamycin, quinolones, hydroxychloroquine, terbinafin diltiazem. In some cases it is induced by bacterial, viral or parasitic infections. In our case, a 50 year male patient developed multiple pus filled lesions, burning sensations all over the body caused by administering paracetamol drug where, the lesions desiccated immediately after cessation of offending drug in two daysleaving exfoliations .Upon diagnosis the white blood cell count was increased indicating lymphocytosis. He was administered with antihistamines, emollients and corticosteroids during his course of stay in hospital. Pustular rashes were reduced and patient recovered with treatment. Paracetamol is one of the most widely used safer drug worldwide, herein draws special attention that no drug is completely safe hence proper medication history interview would be recommended to overcome the drugs causing adverse drug reactions, that can be possibly dangerous and life threatening.