Eosinophilic, polymorphic, and pruritic eruption associated with radiation treated with dupilumab in a patient with Hodgkin lymphoma

Pruritic urticarial papules and plaques of pregnancy (PUPPP), also known as polymorphic eruption of pregnancy, is a common dermatological condition of pregnancy that typically presents in the third trimester and resolves spontaneously after delivery. Lesions usually present around the striae gravidarum of the abdomen, with subsequent spread to the lower limbs. We report a case of PUPPP in a postpartum patient who presented with a pruritic rash affecting the buttocks and lower limbs, with abdominal sparing. The patient's clinical course, histological and lab findings, and response to treatment are discussed. This case contributes to the limited literature on the occurrence of PUPPP in postpartum patients and also PUPPP presenting without involvement of the abdomen. We aim to increase awareness of this atypical presentation and emphasise the need for a comprehensive evaluation in patients presenting with rashes in pregnancy and post partum.

Pregnancy induces significant hormonal and immunological changes that can alter the skin. Pregnancy-specificdermatoses include, polymorphic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis ofpregnancy, and atopic eruption of pregnancy, some of which are associated with adverse fetal outcomes, such aspreterm birth or low birth weight. In addition, common skin diseases, such as atopic dermatitis, psoriasis, acne,and urticaria, may follow a different clinical course during pregnancy. Management is primarily symptomatic,with topical corticosteroids, antihistamines, and narrowband ultraviolet B phototherapy, which are consideredto be relatively safe options, whereas systemic therapies require careful evaluation of fetal safety. This reviewsummarizes the classification, clinical features, and treatment strategies for pregnancy-related dermatoses, aimingto provide practical guidance for clinicians to optimize maternal and fetal care.

Polymorphic Mucocutaneous Eruption and Septic Shock in a 75‐Year‐Old Man

Polymorphic eruption of pregnancy with bullae on the soles: a case report.

Acne is a common inflammatory skin condition characterized by a polymorphous eruption, mainly affecting the face, neck, chest and back, especially in young adults. Therapy depends on severity. While mild cases are managed with topical products, moderate-to-severe cases may require systemic treatments such as antibiotics, isotretinoin, or hormonal contraceptives. Acne can cause scarring, and some types benefit from specialized dermatological treatment. Moderate acne unresponsive to treatment and severe cases should be referred to a dermatologist to prevent scarring and psychological sequelae.

Background: Pregnancy induces hormonal, immunologic, and vascular changes that profoundly affect dermatologic health. This systematic review aimed to assess the impact of pregnancy on dermatological disorders in terms of disease incidence, severity, maternal-fetal outcomes, and optimal management strategies. Methods: A systematic search was performed in PubMed, MEDLINE, and Web of Science databases, following PRISMA guidelines. Studies evaluating pregnant women with dermatological disorders, pregnancy-related dermatoses, and pre-existing morbidities, were included. The collaboratively extracted data included patient demographics, disease severity, treatment approaches, and pregnancy outcomes. Results: A total of 8490 pregnant cases with dermatologic changes and conditions caused by pregnancy were studied. The dermatological conditions were divided into physiological changes, pregnancy-related exacerbation of pre-existing skin conditions, and pregnancy-specific dermatoses. Intrahepatic cholestasis of pregnancy and pemphigoid gestationis were associated with increased rates of adverse fetal outcomes in patients with specific dermatoses, including increased preterm birth and fetal distress rates. The atopic eruption of pregnancy and polymorphic eruption of pregnancy were highly relevant, but their effect on fetal health was minimal. The efficacy and safety of treatment modalities, including corticosteroids, antihistamines, and ursodeoxycholic acid, were variable. Conclusions: Pregnancy drastically affects dermatological health, but the nature of the impact depends on the condition. Optimal maternal and fetal outcomes rely on early diagnosis and individualized management strategies. More randomized controlled trials are required to develop standardized diagnostic and treatment guidelines to enhance the quality of dermatologic care during pregnancy.

Introduction: Pruritic urticarial papules and plaques of pregnancy (PUPPP), also known as polymorphic eruption of pregnancy (PEP), is a common, self-limiting dermatosis of pregnancy. However, its specific characteristics and management outcomes in Indonesia, a diverse and populous nation, remain understudied. This study aimed to comprehensively assess the clinical features, risk factors, and management outcomes of PUPPP in an Indonesian population. Methods: A retrospective cohort study was conducted at Private Hospital in Jakarta, Indonesia, between January 2019 and December 2023. Medical records of pregnant women diagnosed with PUPPP were reviewed. Data collected included demographics, gestational age at onset, clinical presentation (lesion morphology, distribution, pruritus severity), associated symptoms, parity, pre-pregnancy BMI, weight gain during pregnancy, smoking history, presence of comorbidities, treatment modalities, and treatment outcomes (symptom resolution time, recurrence). Statistical analysis was performed using SPSS version 28. Results: A total of 285 pregnant women were included in the study. The mean age was 29.5 years (SD ± 4.8). The majority (72.3%) were primigravida. Onset was most common in the third trimester (88.4%). The most frequent presenting symptom was severe pruritus (94.7%), followed by erythematous papules (98.2%) and urticarial plaques (91.6%). Lesions predominantly affected the abdomen (96.5%), particularly the striae distensae (89.1%), with frequent involvement of the thighs (75.4%) and buttocks (62.1%). Higher pre-pregnancy BMI (p=0.012) and excessive gestational weight gain (p=0.003) were significantly associated with PUPPP development. Topical corticosteroids (85.6%) were the most commonly used treatment, followed by oral antihistamines (68.4%). Symptom resolution occurred within a mean of 10.2 days (SD ± 3.5) after treatment initiation. Recurrence was observed in 8.4% of cases. Conclusion: PUPPP in Indonesian women predominantly affects primigravida in the third trimester, presenting with severe pruritus and characteristic lesions on the abdomen, thighs, and buttocks. Higher pre-pregnancy BMI and excessive gestational weight gain appear to be significant risk factors. Topical corticosteroids and oral antihistamines are effective in achieving symptom resolution. These findings highlight the need for increased awareness and appropriate management of PUPPP in Indonesia.

Introduction and purpose: Pregnancy-specific dermatoses are a group of pruritic skin disorders that arise exclusively during pregnancy or the postpartum period. They encompass four primary conditions: atopic eruption of pregnancy (AEP), polymorphic eruption of pregnancy (PEP), intrahepatic cholestasis of pregnancy (ICP), and pemphigoid gestationis (PG). This review aims to provide a comprehensive analysis of their prevalence, clinical characteristics, underlying mechanisms, therapeutic strategies, and potential fetal risks. Results: These dermatoses often present with overlapping clinical features, making accurate differential diagnosis crucial. While some pose minimal risk to the fetus, others, such as ICP and PG, are associated with significant complications, including preterm birth and stillbirth. The underlying pathophysiology remains incompletely understood, with hormonal, immunological, genetic, and environmental factors playing key roles. Treatment strategies focus primarily on symptomatic relief, with topical corticosteroids and oral antihistamines being the mainstay of therapy, while ursodeoxycholic acid is the treatment of choice for ICP. Conclusions: A multidisciplinary approach is essential for optimal maternal and fetal outcomes. Further research is needed to better understand the mechanisms and develop more effective treatment options for these conditions.

Introduction and purpose: Pregnancy is a remarkable and delicate period in a woman's life, marked by significant physiological adaptations to accommodate the growing fetus. These changes affect every organ system, including the skin. The aim of this review paper is to raise awareness and assist clinicians in recognizing, diagnosing, and effectively managing these distinct skin conditions associated with pregnancy. Material and methods: An extensive examination of articles published in scientific journals was carried out through online research platforms PubMed and Google Scholar. We searched articles by entering keywords in appropriate configuration: “pregnancy dermatoses”, “pemphigoid gestationis”, “ polymorphic eruption of pregnancy”, “intrahepatic cholestasis of pregnancy”, “pustular psoriasis of pregnancy”, “atopic eruption of pregnancy”. Description of the state of knowledge: This diverse set of pregnancy-related skin conditions includes pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, atopic eruption of pregnancy, and pustular psoriasis of pregnancy. Summary: Early diagnosis and treatment are crucial to mitigate maternal and fetal complications, and in some conditions, prevent fatalities. Improving clinicians' in-depth understanding of these disease processes can enhance patient safety and quality of life during and after pregnancy.

A erupção polimórfica específica da gravidez é uma dermatose específica da gestação de desconhecida etiologia, mecanicamente induzida, mais comum em multíparas, primigestas e no terceiro trimestre da gestação. Apresenta ampla gama de diagnósticos diferenciais de dermatoses gestacionais, sendo necessário seu conhecimento clínico para correta investigação e diagnóstico oportuno. O presente trabalho é um relato de caso de gestante primigesta, de 34 semanas, com feto feminino, o que apresenta algumas diferenças na clássica epidemiologia da doença. Descritores: PUPP, erupção polimórfica específica da gestação, dermatose, dermatologia, gestaçã

Epstein-Barr virus-related polymorphic eruption in a pediatric patient.

Paraneoplastic pemphigus (PNP) is a rare and often fatal autoimmune blistering disorder associated with underlying malignancies, most commonly lymphoproliferative diseases. The condition is characterized by a complex interplay of immunological mechanisms that lead to a distinctive clinical presentation, including severe mucocutaneous lesions and a polymorphic skin eruption. The pathophysiology of PNP involves autoantibodies targeting desmosomal and hemidesmosomal proteins, as well as other epithelial adhesion molecules, resulting in widespread acantholysis and inflammation. Diagnosing PNP poses significant challenges due to its overlapping features with other pemphigus variants and autoimmune blistering diseases, necessitating a combination of clinical, histopathological, immunofluorescence, and serological evaluations. Therapeutic strategies for PNP are equally complex, requiring a multidisciplinary approach that addresses both the underlying malignancy and the severe autoimmune response. Despite advances in understanding the molecular underpinnings of PNP, the prognosis remains poor, with high mortality rates attributed to complications such as infections, respiratory failure, and the progression of the associated neoplasm. This review provides an in-depth analysis of the current knowledge on the pathophysiology, clinical manifestations, diagnostic challenges, and therapeutic approaches in paraneoplastic pemphigus, highlighting the need for early recognition and tailored treatment strategies to improve patient outcomes.

Sequential Progression from Pseudo-Vesicular Lesions to Polymorphic Eruption: A Diagnostic Dilemma.

Extensive polymorphic eruption and lymphocytosis in a 56‐year‐old male—A difficult diagnosis!

Abstract Polymorphic light eruption (PLE) is a common, debilitating disease. First-line management includes photoprotective measures, which are restrictive, and ineffective for severe cases. Many patients notice increased tolerance (hardening) to sunlight as summer progresses. This phenomenon can be induced by exposure to artificial sources of ultraviolet. Many studies have demonstrated the efficacy of both narrowband ultraviolet B radiation (NB-UVB) and psoralen–ultraviolet A therapy (PUVA) in reducing PLE flares (Ling TC, Clayton TH, Crawley J et al. British Association of Dermatologists and British Photodermatology Group guidelines for the safe and effective use of psoralen–ultraviolet A therapy 2015. Br J Dermatol 2016; 174: 24–55). NB-UVB is generally used first line due to simplicity of treatment and long-term safety. PUVA is second-line phototherapy and, as PUVA is associated with a dose-dependent risk of skin cancer, shorter treatment courses are desirable. A study by Palmer and Friedmann showed that six PUVA treatments provided as much protection as 12 in patients with PLE (Palmer RA, Friedmann PS. A comparison of six and 12 PUVA treatments in the prophylaxis of polymorphic light eruption. Clin Exp Dermatol 2004; 29: 141–3). We have retrospectively reviewed our patients with PLE who did not gain benefit with NB-UVB hardening and were treated with ‘accelerated’ PUVA. In the early spring, patients were treated on exposed sites with four UVA exposures after taking oral psoralen. The initial irradiation dose was 70% of the minimal phototoxic dose. Doses were then escalated by 1 J cm−2 each visit for a total of four exposures only. To prevent a significant flare of PLE during treatment, patients were prescribed oral prednisolone 20 mg once daily, commencing on the day of the first exposure and continued for the 2-week course of treatment. Fourteen patients (12 female, two male) with severe PLE and poor response to narrowband NB-UVB were treated. The age range was 25–67 years. Thirteen patients experienced a significant reduction in severity of their PLE flares. Only one patient (male, 39 years) found the treatment unsuccessful. Half of the patients went on to have further courses of accelerated PUVA. No patient experienced significant adverse effects. Our experience has demonstrated that only four exposures of oral PUVA can induce significant skin hardening, preventing PLE flares. This has the significant advantage of a short course of treatment and reduction in cumulative exposure for patients who frequently require repeated courses of treatment. Oral steroid cover is necessary to suppress reactions during treatment but was well tolerated in all cases.

IntroductionDeficiency of adenine deaminase 2 (DADA2) is caused by biallelic pathogenic variants in the gene on chromosome 22q11. It is autosomal recessive with incomplete penetrance, and a variable presentation. It typically manifests as a systemic vasculopathy with early onset strokes, cutaneous manifestations, immunodeficiency and cytopenia. Traditional immunosuppressants have limited efficacy, but vasculitic manifestations have been reported to respond to biologic TNF-inhibitors. We report a patient with confirmed ADA2 deficiency whose cutaneous manifestations were previously classified as Sneddon syndrome. After a period of stability on adalimumab she developed marked cutaneous ulceration that healed following a therapy switch to intravenous infliximab.Case descriptionWe report a 39-year-old female with a long clinical history that started in childhood when she developed Diamond-Blackfan anaemia that went into remission following corticosteroid treatment and transfusions. In 2006, she suddenly had two thalamic infarcts and was thoroughly investigated but was found to have unremarkable vascular imaging, a negative antiphospholipid syndrome and autoantibody testing, a modestly elevated CRP (5-15 mg/L) and a notably persistent low serum IgM. She continued to experience frequent migraines associated with transient sensorimotor symptoms. Subsequently, she developed recurrent botchy, polymorphic macular eruptions on the lower limbs. A deep cutaneous biopsy demonstrated lymphocytic predominant perivascular inflammation of deep dermal vessels associated with thrombus formation and endothelial proliferation. A putative diagnosis of Sneddon’s syndrome was made. However, after the subsequent identification of ADA2-deficiency vasculitis, the patient underwent genetic screening which identified a compound heterozygosity for two novel ADA2 mutations (c6634_636, delTTC (p.Phe212del) and c.1225C>T (p.Pro409Ser)). Her mother was as a carrier.She was started on adalimumab injections to which she had a good clinical response with improved cutaneous disease and no further neurological symptoms. However, after two years of disease stability, she then developed a significant ‘punched out’ ulcer on the mid-calf which was painful and increasing in size to a maximum of 2 x 3 cm. Dermatology colleagues were reluctant to re-biopsy due to a high likelihood of non-specific appearances and increased risk of poor healing leading to worsening ulceration. Drawing on experience from rheumatoid arthritis that a switch in anti-TNF biological will often restore efficacy following secondary non-response to adalimumab; we empirically switched treatment to intravenous infliximab, with a reducing course of prednisolone (40mg per day tapering to 5mg after 12 weeks). This was associated with a notable improvement within one month and a complete healing of the ulcer within 4 months.DiscussionDADA2 remains a rare condition with a carrier frequency estimated at 1 in 236 persons worldwide and a predicted disease prevalence of approximately 1 in 222,000 persons. Patients with DADA2 who have haematological manifestations tend to present during early infancy, whereas delayed presentation is common in patients with vasculitis and systemic inflammation. It should be suspected in patients with early onset strokes, livedo and other haematological and immunological features. Additionally, many previously diagnosed cases of Sneddon syndrome may in fact have DADA2, with a low IgM level favouring the later. Diagnosis can be confirmed by genetic studies or by a biochemical assay that demonstrates near-absent levels of ADA2 activity in the plasma or serum. Interestingly, on screening of adult patients with polyarteritis nodosa (PAN) 3.4% possessed biallelic pathogenic ADA2 variants and similarly, in genomic survey of patients with Diamond-Blackfan anaemia 2% possessed biallelic pathogenic ADA2 variants in the absence of the ribosomal protein gene mutations associated with it. Treatment is phenotype based with a preference for lifelong TNF inhibitor and biosimilar use in those with vasculitis and/or systemic inflammation, though not currently commissioned for this indication. Glucocorticoids, disease-modifying antirheumatic drugs, and other biologic agents may partially ameliorate systemic inflammation. Unfortunately, they are all ineffective for severe hematologic manifestations and hematopoietic cell transplantation (HCT) is currently the only treatment option. Treating asymptomatic patients and mildly symptomatic patients picked up on screening remains senior clinician led on a risk benefit basis. Disease activity is clinically monitored with an overall mortality rate estimated at 8%.Key learning pointsThe main learning points for me included an increased awareness of this syndrome, its variable presentation, association with other autoimmune illnesses, diagnosis, and treatment initiation. In addition, I learnt about screening of family members, weighing the benefits versus risks when considering treating mildly symptomatic and asymptomatic patients, clinical monitoring for response, and when to switch to other anti-TNFs and biosimilars.From this conference, I would like to explore the experience of our rheumatology colleagues with similar cases.

Hormonal, vascular, immunological and metabolic changes in pregnancy can cause many changes in body systems. This condition has an impact on skin changes associated specifically with pregnancy (dermatoses in pregnancy). These changes can be physiological or pathological. This condition is usually considered normal and ignored by health workers but can affect the quality of life in pregnant women. This study was conducted to determine the prevalence of both physiological and pathological skin changes in pregnancy. In this systematic review, literature was reviewed through three databases in accordance with the 2020 PRISMA guidelines. Inclusion criteria were primary literature or journals, full text, in English or Indonesian, published in 2013-2023, examined the prevalence of skin disorders in pregnancy, cross-sectional study design sectionals, cohorts. The study that cannot be accessed in full text and in the form of a review is excluded from the literature review. Most skin disorder changes in pregnant women are in the form of physiological disorders with the type of hyperpigmentation. While the most pathological changes are Atopic Eruption of Pregnancy (AEP) and Polymorphic Eruption of Pregnancy (PEP).

Polymorphous eruption of pregnancy

Introduction: Pregnancy is a physiological state of women which is associated with intricate endocrinological, immunological, metabolic & vascular changes. So pregnancy makes a woman susceptible to various changes in skin & appendages. Alteration to the immune state of the pregnant women is necessary to allow mother to tolerate genetically different tissue during pregnancy. Decrease in cell mediated immunity in normal pregnancy is mainly responsible for the higher frequency and severity of certain infectious diseases. Some of which can also be transmitted to the baby during child birth. Moreover there are marked changes in the levels of sex hormones particularly oestrogen & progesterone and this can lead to profound changes in skin. Objective: To find the prevalence of dermatological manifestations in pregnant patients presenting in a tertiary care hospital Study Design: Descriptive/Cross-sectional Study Setting: The study was done in the Dermatology unit DHQ Hospital Faisalabad. Duration of Study: The study was carried out from 1st October 2016 to 10th March 2017 Subjects and methods: 185 pregnant women presenting at any gestational age in dermatology department and patients referred from Gynae department with dermatological complaints were included in the study. Patients with pre-existing medical conditions such as hypertension and diabetes were excluded. Complete history and clinical features of the patients were accessed and relevant investigations were done to support the diagnosis. Final diagnosis was made on basis of history, clinical examination and laboratory confirmation of disease as per operational definition. LFT’s for intrahepatic cholestasis of pregnancy was done from DHQ laboratory and sample for skin biopsy for pemphigoid gestationis were sent to pathology laboratory FMU. All the data was recorded in a well-structured questionnaire. Results: Age range in this study was from 18 to 40 years with mean age of 30.129±3.26 years and mean duration of disease was 5.864±1.79 week. Majority of the women were with 2nd trimester of pregnancy (67%). 60% of women had no occupation. Striae Gravidarum was seen in 48.1%, Linea Nigra 45.9%, Melasma 25.4%, Hirsutism 16.2%, Palmer Erythema 8.1%, Atopic eruption of Pregnancy 11.9%, Polymorphic eruption of Pregnancy 3.2%, Intrahepatic Cholestasis of Pregnancy 1.6%, Pemphigoid gestationis 1.1%, Acne Vulgaris 9.7%, Vulvovaginal candidosis 8.1%, Urticaria 6.5% and Scabies was 5.4%. Practical Implication: We set out to investigate the prevalence of dermatological manifestations during pregnancy, as well as other clinical characteristics affecting pregnant women in Pakistan. Conclusion: If infections during pregnancy are diagnosed at the earliest stage it may prevent morbidity during and after antenatal period. Keywords: Pregnancy; Dermatological manifestations; Frequency, Clinical Patterns, Pemphigoid Gestationis, Acne Vulgaris, Vulvovaginal Candidosis,