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A 53-year-old woman presented with a 25-year history of acne excoriée and prurigo excoriée. Her symptoms began in 1988 coinciding with her husband’s death from a brain tumour when she was 27. The pruritus affected her quality of life and disturbed her sleep. She had scarring on her face and body resulting from persistent scratching. The pruritus proved refractory to treatment despite a multi-modal treatment approach including multiple topicals, phototherapy and systemic agents such as isotretinoin, antibiotics, anxiolytic agents and neuromodulators. She was extremely frustrated that various treatments had been ineffective at controlling the itch-scratch cycle. She was commenced on low dose naltrexone (LDN), 3 mg nocte, and she became itch free within a few weeks. She reports that the LDN has had a beneficial impact on her quality of life.

Background Prurigo nodularis (PN) is a debilitating, difficult-to-treat, intensely pruritic, chronic inflammatory skin disease characterized by hyperkeratotic skin nodules. The pathogenesis of PN is not well understood but is believed to involve cross talk between sensory nerve fibers, immune cells, and the epidermis. It is centered around the neuroimmune cytokine IL-31, driving an intractable itch–scratch cycle. Objective We sought to provide a comprehensive view of the transcriptomic changes in PN skin and characterize the mechanism of action of the anti–IL-31 receptor inhibitor nemolizumab. Method RNA sequencing of biopsy samples obtained from a cohort of patients treated with the anti–IL-31 receptor inhibitor nemolizumab and taken at baseline and week 12. Generation and integration of patient data with RNA-Seq data generated from reconstructed human epidermis stimulated with IL-31 and other proinflammatory cytokines. Results Our results demonstrate that nemolizumab effectively decreases IL-31 responses in PN skin, leading to effective suppression of downstream inflammatory responses including TH2/IL-13 and TH17/IL-17 responses. This is accompanied by decreased keratinocyte proliferation and normalization of epidermal differentiation and function. Furthermore, our results demonstrate how transcriptomic changes associated with nemolizumab treatment correlate with improvement in lesions, pruritus, stabilization of extracellular matrix remodeling, and processes associated with cutaneous nerve function. Conclusion These data demonstrate a broad response to IL-31 receptor inhibition with nemolizumab and confirm the critical upstream role of IL-31 in PN pathogenesis.

Black thyroid is characterized by a rare pigment change observed almost exclusively in patients taking minocycline. We present the case of a 72-year-old man diagnosed with T3N3bM0 stage IVB hypopharyngeal squamous cell carcinoma who had been taking minocycline for approximately 18 months as a treatment for prurigo chronica multiformis. Initial treatment consisted of total pharyngolaryngoesophagectomy, bilateral neck dissection, total thyroidectomy, pharyngeal reconstruction using a free jejunal autograft, and creation of a permanent tracheostoma. During surgery, black discoloration of the thyroid and trachea was observed. Postoperative histological findings confirmed the black discoloration, with deposits of dark-brown, melanin-like granules observed in the thyroid, trachea, thyroid cartilage, and cricoid cartilage. Therefore, the black discoloration of the thyroid associated with the use of minocycline can extend to the thyroid cartilage, cricoid cartilage, and trachea. This information is important for surgeons to recognize in order to prevent unnecessary resection due to misdiagnosis.

Prurigo nodularis (PN) is an understudied, pruritic inflammatory skin disease. Little is known about the effect of PN on quality of life and its associated economic burden.To quantify the impact of PN on quality of life and its economic implications.A cohort study of PN patients (n = 36) was conducted using the Health Utilities Index Mark 3 questionnaire. Control data from US adults (n = 4187) were obtained from the 2002-2003 Joint Canada/United States Survey of Health. Quality-adjusted life year loss and economic costs were estimated by comparing the Health Utilities Index Mark 3 scores of the PN patients with those of the controls.The PN patients had lower overall health performance compared to the controls, (mean ± SE, 0.52 ± 0.06 vs 0.86 ± 0.003, respectively, P .001). In multivariable regression, PN was found to be associated with worse health performance (coefficient -0.34, 95% CI [-0.46 to -0.23]), most prominent in the pain subdomain (coefficient -0.24, 95% CI [-0.35 to -0.13]). This correlated to an average of 6.5 lifetime quality-adjusted life years lost per patient, translating to an individual lifetime economic burden of $323,292 and a societal burden of $38.8 billion.These results demonstrate that PN is associated with significant quality-of-life impairment, similar to the level of other chronic systemic conditions. PN is also associated with a substantial individual economic burden, emphasizing the necessity of research on effective treatment options.

Background: Prurigo nodularis (PN) as an extremely pruritic and hyperplastic chronic dermatosis induces psychologically and physiologically stressful responses. PN-induced responses in the hypothalamic-pituitary-adrenal (HPA), hypothalamic-pituitary-gonadal (HPG) axes and endocannabinoid system (ECS) are abnormal. Extant studies on the PN’s pathogenesis mostly focused on the PN’s psychological responses. To date, the PN’s physiological responses remain not been fully uncovered yet. Objectives: To investigate the PN-induced physiological responses via the levels of five steroids and two endocannabinoids combined with their ratios in plasma and examine the association between the psychological and physiological responses. Materials and methods: Thirty-six patients with PN, 36 age- and gender-matched healthy controls were recruited. The PN’s psychological symptoms including pruritus severity, pain and life quality were measured with the visual analogue scale, the prurigo score index, numerical rating scale, verbal rating scale and dermatology life quality index. Their concentrations of steroids and endocannabinoids were determined with liquid chromatography-tandem mass spectrometry. Results: Compared to controls, the PN patients showed lower plasma levels in cortisol, cortisone, N-arachidonoyl-ethanolamine (AEA), and the ratio of DHEA to 1-arachydonoyl glycerol (1-AG), which negatively moderately and over correlated with PN’s symptoms, especially with the pruritus severity. Additionally, the PN patients exhibited higher levels in the ratios of testosterone and 1-AG to cortisol, which positively moderately and over correlated with pruritus severity. Thus, the seven biomarkers would be sensitive and reliable biomarkers for assessing the pruritus severity of PN because they met the screening criteria that the biomarkers show intergroup differences and showed moderate or over correlation with the pruritus severity of PN. Conclusions: To the best of our knowledge, this is the first study exploring PN-induced physiological responses. The findigs suggest that alterations in these three endocrine systems may lead to new insights to psychological mechanisms and responses to prurigo nodularis.

BACKGROUND Treatment of prurigo nodularis (PN) is challenging and new treatment options are needed. OBJECTIVE To evaluate the efficacy and safety of two oral doses of the kappa opioid agonist and mu opioid antagonist nalbuphine extended release (NAL-ER) tablets in a phase 2, multicentre, randomized, double-blind, placebo-controlled trial with an open-label, 50-week extension phase. METHODS Subjects with moderate-to-severe PN were randomized to NAL-ER 81 mg (NAL-ER81) or 162 mg (NAL-ER162) tablets twice-daily or placebo for 8 weeks of stable dosing following a 2-week titration period. Subjects completing Week 10 with a Worst Itch Numerical Rating Scale (WI-NRS) score ≥5 at the time of rollover (or during the observation period) were eligible for open-label treatment. RESULTS Of 63 randomized subjects, 62 were treated and comprised the modified intent-to-treat population (MITT), 50 completed 10 weeks of treatment. In the MITT analysis, 8 subjects (44.4%) treated with NAL-ER162 (P = 0.32) and 6 (27.3%) treated with NAL-ER81 (P = 0.78) achieved ≥30% reduction from baseline in 7-day WI-NRS at Week 10 (primary efficacy endpoint) vs. 8 (36.4%) in the placebo group. Itch reduction was significant among 8/12 (66.7%) subjects completing Week 10 treated with NAL-ER162 vs. placebo (8/20, 40.0%; P = 0.03). Additionally, 6 subjects (33.3%) treated with NAL-ER162 and 3 (13.6%) treated with NAL-ER81 achieved ≥50% reduction from baseline in 7-day WI-NRS at Week 10 (coprimary endpoint). Extended open-label treatment was associated with further improvements in itch reduction and favourable changes in PN lesion activity as assessed by Prurigo Activity Score. Adverse events occurred predominantly during dose titration and were of mild-to-moderate severity. The safety profile did not change with extended open-label treatment. CONCLUSION In adult subjects with PN, oral treatment with NAL-ER 162 mg twice daily provided measurable anti-pruritic efficacy in subjects completing ≥10 weeks of treatment and was well tolerated (ClinicalTrials.gov: NCT02174419).

Background In Germany, skin diseases are mainly treated in the 115 dermatological hospitals. Methods Health care and health economic analysis of dermatological inpatient care and prediction of future care needs based on primary and secondary data. Results Outpatient and inpatient care for dermatologic treatment indications is predominantly provided by dermatology specialists. Inpatient treatment was provided for 833,491 cases in 2018, corresponding to 4.21 % of all inpatient cases (19,808,687). Most common treatment cases were: epithelial skin cancer (total 87,386, of which dermatology clinics 52,608), followed by melanoma (23,917/17,774), psoriasis (19,291/13,352), erysipelas (73,337/11,260), other dermatitis (12,671/10,842), atopic dermatitis (AD) (11,421/9,734), and herpes zoster (26,249/9,652). With an average length of stay of 5.69 days, dermatology hospitals were in the bottom third. The proportion of inpatient indications cared for in dermatology hospitals was highest for prurigo (95.2 %), pemphigus (94.9 %), parapsoriasis (94.6 %), pemphigoid (90.3 %), eczema other than AD (85.6 %), and AD (85.2 %). While the total number of inpatient treatment cases in Germany has increased by an average of 17.5 % between 2000 and 2018, this is the case for 26.6 % of skin diseases and over 150 % for individual ones. The projection of current to future inpatient care suggests a continued high demand for inpatient care by dermatology hospitals. Conclusion Inpatient dermatological care will continue to be an indispensable component of qualified, socially necessary care in Germany.

Prurigo nodularis (PN) or chronic prurigo of nodular type (CNPG) is a subtype of chronic prurigo with severe pruritus and neuroimmune underlying pathophysiology occurring in a plethora of dermatological, systemic, neurologic, and psychiatric conditions.We review the increasing repertoire of biologics in the treatment of CNPG focusing on those targeting interleukins 4, 13, 31, oncostatin M and IgE. Presented information is based on a database research on current clinical trials (clinicaltrials.gov, European Clinical Trials Database (EudraCT), US clinical trial registry ICH-GCP) and a PubMed search for latest publications conducted with the combinations of the terms 'chronic prurigo,' 'prurigo nodularis,' 'pathophysiology,' 'treatment,' 'therapy', and 'biologics.'CNPG gets more and more attention as new therapeutic targets have been revealed in recent years, thus allowing the use of targeted approaches. The off-label advent of dupilumab offered advanced insight into the pathogenesis of CNPG and showed an impressive relief of pruritus in the vast majority of patients. New therapies including biologics (e.g. nemolizumab, tralokinumab, lebrikizumab), small molecules (e.g. neurokinin-1 receptor antagonists, janus kinase inhibitors) as well as mu-opioid receptor antagonists and nalbuphine, a μ-antagonist/κ-agonist, are in the pipeline and offer new hope for an improved future patient care.

BACKGROUND Prurigo nodularis (PN) is associated with a variety of systemic comorbidities, including infectious diseases such as HIV and viral hepatitis. There are limited data on other infectious disease comorbidities in patients with PN. AIM To characterize infectious disease hospitalizations among patients with PN and the associated cost burden. METHODS We searched the 2016-2017 National Inpatient Sample, a cross-sectional sample of 20% of all US hospitalizations, for infectious disease hospitalizations among patients with PN. Associations of PN with infections and related costs were determined using multivariable logistic and linear regression, adjusting for age, race, sex and insurance type. RESULTS PN was associated with any infection overall (OR = 2.98, 95% CI 2.49-3.56), and with HIV, cutaneous, hepatobiliary, central nervous system, bacterial, viral and fungal/parasitic infections and for sepsis. Patients with PN had a higher mean cost of care (US$11 667 vs. US$8893, P

Background: Prurigo nodularis (PN) is a highly pruritic disease that significantly impairs patient quality of life. Although the mechanism that causes pruritus is not clear, one hypothesis argues that neural hyperplasia, mast cell, and Merkel cell neurite complexes may be associated with PN pathogenesis. Objective: The objective of this study was to analyze whether special staining outcomes differed depending on the presence of atopic dermatitis (AD) and treatment response. Methods: A total of 209 patients diagnosed with PN was analyzed retrospectively. Patients were divided into two groups according to presence or past history of AD and by treatment response. Histopathologic features were obtained using the following stains: Giemsa, S-100, neuron-specific enolase, cytokeratin (CK)-20, CAM5.2, and CK8/CK18. Results: A total of 126 patients (60.29%) had AD, and 68 (32.54%) showed clinical improvement. There were no statistically significant differences in the staining results between the PN groups with AD (PN c AD) and without AD (PN s AD). Additionally, there were no statistically significant differences in staining results between the improved and non-improved groups. Conclusion: Implementing the special stains helped to identify PN pathogenesis. Because there were no statistically significant differences in the special stain results between the improved and non-improved groups, we conclude that mast cell proliferation, neural hyperplasia, and Merkel cell hyperplasia may not have a significant effect on treatment response. (Ann Dermatol 33(4) 333∼338, 2021)

We report the clinical and histopathological features of hydroa vacciniforme-like lymphoproliferative disorder in five indigenous and Mestizo children. All the children resided at higher altitudes, experiencing maximal solar exposure. All cases presented with prurigo along with Epstein-Barr virus infection. Histopathologic examination showed an atypical, CD30 + lymphocytic infiltrate with angiocentricity in all, while three cases demonstrated panniculitis-like infiltrate.

Prurigo nodularis (PN) is a debilitating chronic disease characterized by intense itching and excoriated hyperkeratotic nodules distributed on the trunk and extremities, especially the extensor surfaces. The pathophysiology includes complex and not yet well-understood mechanisms involving inflammation and dysregulation of the nervous system. Currently, there are no approved therapies by the Food and Drug Administration (FDA) and the few treatment approaches for this condition are often ineffective and related to severe side effects. An emerging therapeutic option is dupilumab, a monoclonal antibody for adults and adolescents with moderate-to-severe atopic dermatitis, that inhibits interleukin-4 receptor alpha subunit (IL4-Rα) and the signaling pathways activated by interleukin (IL)-4 and IL-13. These cytokines seem to be involved in the development and perpetuation of PN and other type-2 inflammation diseases. Data on this topic are limited, but the emergent positive effects of this drug, reported in the literature and summarized in this review, suggest that it can be a safe and efficient therapy in PN.

Although atopic dermatitis (AD) classically presents as eczematous, pruritic plaques in the flexural regions of children, AD can present with several morphologies and distributions that may vary wi...

Introduction: Prurigo nodularis (PN) is a chronic inflammatory skin disease characterized by intensely pruritic, hyperkeratotic nodules distributed on the trunk and extensor surfaces of the extremities. PN has a profoundly negative impact on sleep and quality of life in patients with PN. There are currently no U.S. Food and Drug Administration-approved agents and patients are often recalcitrant to current therapies, highlighting the importance of further research into this severely debilitating condition. Areas covered: A PubMed search was conducted to find available literature on the pathophysiology and clinical management of PN. In this review article, we discuss the current understanding of the pathophysiology, recommended diagnostic approach, and treatment options available for PN. Expert opinion/commentary: PN is an extremely difficult condition to treat, because there is a lack of effective therapies available due to our limited understanding of its pathophysiology. Currently, available treatment options are often multimodal due to the intersection of neuroimmune etiologic factors in the pathogenesis of PN. Fortunately, as our knowledge of PN expands, novel treatments targeting specific molecular biomarkers of PN are emerging, providing hope to this long-suffering patient population.

The impact of search strategies on systematic reviews (SR) of atopic dermatitis (AD) is unknown. The purpose of this review was to evaluate search strategies used in SR of AD and their impact on the frequency of manuscripts identified. MEDLINE and EMBASE were searched for SR related to AD. Simulations were performed by running combinations of search terms in MEDLINE and EMBASE. Overall, 250 SR met inclusion criteria, of which 225 specified search strategies. SR using 5–6 terms (20.0% to 12.1%) or ≥ 7 (40.0% to 18.8%) terms decreased, whereas SR using 3–4 terms numerically increased (18.8% to 30.2%) and 1–2 terms remained similar (37.5% to 38.9%) from 1999–2009 to 2015–2019. The most commonly searched terms were “atopic dermatitis” (n = 166), followed by “eczema” (n = 156), “dermatitis atopic’” (n = 81), “atopic eczema” (n = 74), “neurodermatitis” (n = 59), “Besniers prurigo” (n = 29), “infantile eczema” (n = 27), and “childhood eczema” (n = 19). Simulations revealed that “eczema” and “atopic dermatitis” yielded the most hits. The number of search terms that maximized hits in MEDLINE and EMBASE was 5 and 4, respectively. Search strategies for AD were heterogeneous, with high proportions of search strategies providing few search hits. Future studies should use standardized and optimized search terms.

BACKGROUND Chronic prurigo (CPG) is known as a high burdensome disease characterized by severe pruritus and multiple pruriginous lesions. Interestingly, the disease-specific burden is not well established and there are no data which compare the impact of CPG with chronic pruritus (CP) on non-lesional skin (CP-NL). OBJECTIVES To address this issue, we analysed datasets from 4484 patients with either CPG or CP-NL. METHODS Demographic medical data and additional information collected by validated patient reported outcome tools were analysed. The visual analogue scale and numerical rating scale (NRS) were used for assessing the pruritus intensity, the ItchyQoL for patients' quality of life, the Hospital Anxiety and Depression Scale and the Patient Needs Questionnaire' as a part of Patient Benefit Index for Pruritus for measuring the importance of 27 patient needs in terms of treatment goals. The Neuroderm questionnaire was used to assess the history of pruritus characteristics and the impact on sleep. RESULTS Patients with CPG suffered longer and with a higher intensity from pruritus [NRS worst the last 24 h, CPG 6.0 (4.0;8.0) vs. CP-NL 3.0 (5.0;7.0), P

Leprosy or Hansen’s disease is a chronic granulomatous infection that primarily affects the peripheral nerves and, consequently, the skin. Clinical manifestations vary from hypopigmentation to erythematous plaques, and it can present with leonine facies. We report a case of a patient with an uncommon clinical presentation of prurigo nodularis-like lesions without loss of sensation, for which two biopsy specimens demonstrated different histological subtypes. The first was the classic histology of lepromatous leprosy, whereas the other specimen revealed an atypical histoid leprosy variant pattern. This case report describes a patient with an atypical presentation of leprosy.

Resumen Introducción. El prurigo actínico es una fotodermatosis crónica. Afecta con mayor frecuencia a la población latinoamericana, predomina en mujeres y compromete la piel expuesta al sol, las conjuntivas y los labios. Objetivo. Actualizar la información sobre las características clínico-epidemiológicas y el tratamiento de pacientes con prurigo actínico en Colombia. Materiales y métodos. Se hizo un estudio de corte transversal que incluyó los registros clínicos de pacientes con prurigo actínico atendidos en el Servicio de Fotodermatología del Hospital Universitario Centro Dermatológico Federico Lleras Acosta entre el 2011 y el 2016, y se describieron sus características demográficas, clínicas e histopatológicas, así como su tratamiento. Resultados. Se incluyeron 108 pacientes, el 71,3 % de ellos mujeres y el 28,7% hombres, con predominio de los fototipos III-IV (70 %). La enfermedad se había iniciado durante la primera década de vida en el 66,4% de los casos y el 25 % de los pacientes tenía antecedentes familiares de la enfermedad. Las lesiones predominaban en el rostro (93,5 %), los antebrazos (79,6 %) y el dorso de las manos (70,4 %). También, se documentó compromiso ocular (87,9 %) y de los labios (88,8 %). Se hizo la prueba de fotoprovocación con radiación ultravioleta A en el 25 % de los casos y biopsia cutánea en el 19,4 %. Todos los pacientes se trataron con protección solar química y física. En los casos leves a moderados, se formularon corticoides tópicos (91,7 %) e inhibidores de la calcineurina (65,7 %), y en los graves, talidomida (33,3 %) y pentoxifilina (14,8 %). Conclusión. Las características de los pacientes colombianos con prurigo actínico son similares a las reportadas en otros países latinoamericanos: inicio temprano de la enfermedad, predominio en mujeres, compromiso frecuente de conjuntivas y labios, y adecuada respuesta al tratamiento tópico y sistémico. Abstract Introduction: Actinic prurigo is a chronic photodermatosis. It affects the Latin American population more frequently, predominantly women, and involves the sun-exposed areas of the skin, conjunctiva, and lips. Objective: To update the information on the clinical-epidemiological characteristics and treatment of patients with actinic prurigo in Colombia. Materials and methods: We conducted a cross-sectional study including the medical records of patients with actinic prurigo treated in the Photodermatology Service of Hospital Universitario Centro Dermatológico Federico Lleras Acosta between 2011 and 2016. We described the demographic, clinical, histopathological, and treatment characteristics of the patients. Results: We included 108 patients, 77 (71.3%) were women and 31 (28.7%) men, mainly with phototypes III-IV (70%). The disease had begun during the first decade of life in 66.4% of the cases and 25% of the patients had a family history with the condition. The lesions predominated on the face (93.5%), forearms (79.6%), and back of the hands (70.4%). Ocular (87.9%) and lip (88.8%) involvement was also documented. A photo-provocation test with UVA was performed in 25% of the cases and skin biopsies in 19.4%. Physical and chemical photoprotection was indicated in all patients. Mild to moderate cases were treated with topical corticosteroids (91.7%) and calcineurin inhibitors (65.7%) while severe cases received thalidomide (33.3%) and pentoxifylline (14.8%). Conclusion: The characteristics of actinic prurigo patients in Colombia are similar to those reported in other Latin American countries: early onset of the disease, predominance in women, frequent involvement of conjunctiva and lips, and adequate response to topical and systemic treatment.

Leprosy is a chronic infectious disease with a wide range of clinical manifestations. The early diagnosis of leprosy is a worldwide challenge. We present a case of leprosy with unusual severe pruritus and generalized excoriated papules and nodules.

Itching of the skin is a particularly unpleasant subjective sensation that causes the need to scratch. Physiological itching occurs in response to environmental irritants (friction, insect crawling, temperature changes, etc.) and disappears after elimination of the cause. Pathological itching is caused by changes in the skin or throughout the body and causes a strong need to get rid of itching by combing or any other method. Itching is a common symptom of local (dermatological) or general (systemic) disease. It can be limited (localized) or diffuse (generalized).One of the causes of itching is often noted endocrine diseases and metabolic disorders: hyper- and hypofunction of the thyroid gland, diabetes mellitus and hyperparathyroidism. In thyroid hyperfunction, generalized (inconstant and little-pronounced) itching of the skin is observed in the clinical picture only in 10 % of patients. It is accepted that itching of the skin in thyrotoxicosis syndrome is due to increased activity of kinin in combination with an increase in major metabolism and an increase in body and skin temperature (123).Itching caused by impaired bile secretion is characteristic of many diseases of the liver: primary biliary cirrhosis, sclerosing cholangitis, viral hepatitis caused by cholestasis medications and other causes of obstructive jaundice. It is in these cases that prurigo is usually generalized, but in some typical cases it is more pronounced on the feet and palms. With this pathology, itching is caused by obstruction of the biliary tract, however, and to this day, no close correlation has been found between serum bilirubin and the severity of itching.If it was previously thought that itching in pathology of the peripheral or central nervous system, such as multiple sclerosis, neuropathy, compression or irritation of nerves (e.g., paraesthetic dorsalgia, brachioradial itching) is also a significant symptom, then modern studies prove that in more cases, itching is not characteristic of most pathologies of the nervous system, so after a detailed anamnesis, examination and initial research, the patient should be referred to a related specialist, except in cases of about obvious postherpetic neuropathy, painless paresthesia, or brachioradial itching, which can often be guided in the provision of primary care.Instead, there are some psychological conditions and a number of psychiatric illnesses (obsessive-compulsive disorders, depression, and parasite illusions) that are most commonly diagnosed with the exclusion method. Neurotic squabbles are scattered on the body of the excorii, covered with scales that may occur in any area of the body that the patient may reach, but are often limited by limbs.

Prurigo nodularis (PN) and lichen simplex chronicus (LSC) are debilitating chronic pruritic diseases that can lead to and be exacerbated by psychosocial distress. However, little is known about the mental health (MH) comorbidities of PN/LSC. We sought to evaluate the likelihood and cost-burden of MH comorbidities and emergencies associated with PN/LSC. Data were examined from the 2002-2012 Nationwide Inpatient Sample, including a representative ~ 20% sample of US hospitalizations (n = 87,053,155 admissions). Inpatients with vs. without PN/LSC had higher odds of MH disorders overall (39.4% vs. 20.0%; adjusted odds ratio [95% confidence interval, CI] 2.26 [2.13-2.41]) and in all 15 individual MH disorders examined. Inpatients with vs. without PN/LSC were more likely to be admitted with a primary diagnosis of a MH disorder (4.5% vs. 2.2%; 2.16 [1.91-2.45]), particularly developmental, psychotic, and mood disorders, history of MH disorders or substance abuse, and cognitive disorders. PN/LSC were associated with prolonged inpatient length of stay, and an excess $0.69 million in inpatient costs of care indirectly related to hospitalization for MH disorders. In conclusion, inpatients with PN/LSC had increased likelihood of comorbid MH disorders and emergencies requiring hospitalization. Optimized approaches are needed for screening and managing MH comorbidities in PN/LSC.

Chronic nodular prurigo (CNPG) is a subtype of chronic prurigo, also called prurigo nodularis, and a chronic skin condition characterized by intensely pruritic nodular lesions. Although the exact cause of CNPG is unknown, it appears to result from a repetitive itch-scratch cycle induced by neuronal sensitization to chronic pruritus. CNPG is associated with an underlying dermatologic condition in about half of patients, and it can also be attributed to systemic, neurologic, psychogenic, or unknown causes. For most patients, multiple underlying causes are identified. Patients with CNPG often experience impaired quality of life, sleep disturbance, anxiety, and depression. To encourage consistent and accurate diagnosis and treatment of CNPG across regions, we are proposing a diagnostic and treatment algorithm that includes initial assessment of core symptoms, detailed dermatologic history and clinical examination, patient-reported outcomes, diagnostic workup, and recommended therapies. This information is supplemented with photographs to illustrate clinical appearance and disease severity. Because CNPG is often multifactorial and it can take months to years for lesions to heal, interdisciplinary cooperation and long-term management are important.

Dear Editor,Prurigo nodularis (PN) is an inflammatory skin disease characterized by intractably itchy nodules that often become excoriated and can ulcerate. PN has a significant burden of disease, ...

Patients with prurigo nodularis (PN) suffer from intractable itch and dramatic reduction in QOL. Although there is significant clinical heterogeneity in the presentation of PN, disease endotypes remain unknown. We assayed circulating plasma cytokine concentrations in patients with PN (n = 20) along with matched healthy controls and utilized an unsupervised machine learning algorithm to identify disease endotypes. We found two distinct clusters of patients with PN with noninflammatory (cluster 1) and inflammatory (cluster 2) plasma profiles. Cluster 2 had more African Americans (82%, n = 9 vs. 33%, n = 3; P = 0.028), higher Worst Itch Numeric Rating Scale scores (9.5 ± 0.9 vs. 8.3 ± 1.2; P = 0.036), and lower QOL as reflected by higher Dermatology Life Quality Index scores (21.9 ± 6.4 vs. 13.0 ± 4.1; P = 0.015). In addition, cluster 1 had a higher rate of myelopathy (67%, n = 6 vs. 18%, n = 2; P = 0.028). Compared with cluster 1, cluster 2 had higher levels of IL-1α, IL-4, IL-5, IL-6, IL-10, IL-17A, IL-22, IL-25, and IFN-α. With population-level analysis, African American patients with PN had higher erythrocyte sedimentation rate, C-reactive protein, ferritin, and eosinophils and lower transferrin than Caucasian patients with PN. These findings indicate discrete clusters of patients with PN with plasma biomarker profiles corresponding to distinct demographic and clinical characteristics, potentially allowing for precision medicine approaches to treat PN.

Prurigo pigmentosa (PP) is an uncommon inflammatory dermatosis first described in 1971. It is characterized by recurrent crops of pruritic erythematous papulovesicles that resolve with a macular reticulated hyperpigmentation. The exact etiology is yet to be determined, however with the expanded application of the ketogenic diet (KD) in recent years, conditions accompanied with ketosis are more commonly being described in association with PP. Antibiotics as well as resolution of ketosis can effectively treat the dermatitis. Given the publicity and growing popularity of the ketogenic diet and numerous references to the "Keto-Rash" on social media, we reviewed the KD-induced PP cases to raise awareness of this increasingly recognized entity and provide an update to clinicians, particularly dermatologists, regarding this possible side effect of KD.

Parents need information about their children’s health, and the Internet has become an essential repository for this information. However, there is almost no information about which topics are the most searched, consulted, or shared, or about the main characteristics of pediatric website users. The main aim of this research was to describe the profile of the users of a pediatric website, which topics they searched for, which topics were the most consulted, and which were the most shared. Users’ demographic data were analyzed regarding their use of the Internet to search for information about health. A pediatric website for parents was analyzed. A 26-item questionnaire collected demographical features of the users of the website. Descriptive and analytical analyses were performed. Most participants used general search engines for their health searches, and the most searched keywords were prurigo, barking cough, and laryngitis. The most visited topics were unexplained fever, snots, and laryngitis. The most shared were snots, lipotomy, and dizziness. The users were mainly women (67.8%), with an average age of 38.8 years, and one or two children (89%) with a mean age of 4.6 years. The users who mainly used the Internet for health purposes were women of younger age, and with a higher educational level.

Case report - Introduction Considering an auto-inflammatory disorder is important where recurrent fevers are a presenting feature. Performing a genetic panel is essential in these cases; however, it can be negative even in the presence of an underlying auto-inflammatory disorder. Our case highlights the importance of this with a reminder that a negative gene panel does not completely exclude an underlying auto-inflammatory disorder in the presence of suggestive history and clinical examination findings. Case report - Case description A 5-year-old girl presented with recurrent episodes of fever (>38 °C), abdominal pain and joint pains (back, wrist, hip, knee & ankle). Past medical history included episodes of self-resolving neutropenia. Initial clinical examination revealed mouth ulcers, a significant number of dental caries, a petechial rash and genital erythema. There was no active joint disease. A pustular like itchy rash developed on/off over time affecting the buttocks, genital area, knees and elbows. Episodes increased in frequency becoming once per month, lasting for 5 days on average, resulting in a significant amount of time off school. Initial differential diagnosis included periodic fever and cyclical neutropenia. White blood cells ranged from 4.3 to 20.1 (10^9/l), Neutrophils ranged from 1.1 to 11.4 (10^9/l), CRP 0.7 to 149.5 (mg/L), ESR 2 to 15 (mm/hr). Serum Amyloid A ranged from 8.0 to 370 (mg/L). Immunology and haematology consults were sought with cyclical neutropenia deemed unlikely. A primary immunodeficiency screen was negative. Initial genetic analysis for periodic fever was negative; however, this was later reanalysed and revealed a pathogenic mutation in the PSTPIP1 gene and a mutation in the TMEM173 gene. A mutation in the PSTPIP1 gene, on chromosome 15q24 is associated with Pyogenic Arthritis, Pyoderma gangrenosum and Acne (PAPA) Syndrome. A trial of colchicine was commenced, with eventual titration of the dose, achieving a good response. Occasional episodes now occur 1—2-times monthly with a reduced temperature rise, minimal arthralgia and are of a shorter duration. Dental extraction was also performed due to 9 periodontal abscesses. Skin care advice included use of emollient-barrier creams, antihistamines and oral antibiotics when indicated. Case report - Discussion PAPA – Pyogenic Arthritis, Pyoderma Gangrenosum and Acne are a triad of symptoms that together combine to form this rare auto-inflammatory disorder. It is an autosomal dominant condition. It is the result of a mutation of the (PSTPIP1/CD2BP1) gene located on chromosome 15. Studies of families affected have revealed a variable penetrance, including some asymptomatic carriers. Clinicians should be mindful when ascertaining what conditions and symptoms other family members have, as PAPA could be the underlying causative condition. The recurrent aseptic inflammation of the joints normally manifests within the first two decades of life. The skin symptoms manifest within the third decade. All three symptoms rarely appear instantaneously. The diagnosis is clinical with confirmation by genetic testing. Reported treatments includes the use of corticosteroids, arthrocentesis – for relieving pain, and biologics – namely anti IL1 and anti TNF therapy. We felt this case was interesting as the patient described demonstrated a good response to colchicine and highlighted the difficulties that can present in making the diagnosis. With a confirmed diagnosis the clinician can then appropriately follow up the patient, giving them and potential family members the best care available. Case report - Key learning points Our case highlights the importance of multi-specialty input particularly when faced with a rare or complex case in order to exclude other potential causes. Although genetic testing has significantly improved in helping with the diagnosis of auto-inflammatory disorders, it must be highlighted the importance of good clinical assessment and interpretation of more standard investigations. This is particularly relevant where genetic panelling is returned as negative and concerns persist of an underlying auto-inflammatory disorder. We hope to raise awareness of the varied presentation of PAPA syndrome particularly in the younger age groups where skin presentations may not be a predominant presenting feature. It is also important to emphasise that the main features of PAPA syndrome rarely present simultaneously. Current clear treatment pathways do not exist due to the rarity of this condition within the paediatric population – a number of agents are suggested in the literature with colchicine achieving good control in our patient described.

The worldwide spread of the novel coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has led to a global pandemic since its identification in Wuhan, China in December 2019.1 Few cases of COVID-19-associated dermatologic manifestations have been reported in the literature to date. This report describes the clinical features of a localized pruritic scarlatiniform rash of the ears and antecubital fossa on defervescence in a 29-year-old patient with COVID-19. Our case stands to further illuminate the dermatologic manifestations of this novel disease.

Prurigo simplex or "itchy red bump" disease is a controversial pruritic papular eruption. The objective of this study was to delineate the diagnostic criteria for this disease. A PubMed search was performed for: "prurigo simplex", "prurigo simplex subacuta", "subacute prurigo", "chronic papular dermatitis in adults" and "itchy red bump disease". Medical charts of patients with prurigo diagnoses in a university setting were analysed. The literature search revealed 32 relevant studies (431 patients) on different aspects of the disease. Out of 50 patients with prurigo diagnoses, 5 patients (4 women, mean age at onset 54 years) matched the description in literature. Small, severely itchy papules without secondary skin lesions continued to appear for years (mean 5.6 years). Skin biopsies revealed lymphocytic perivascular infiltrates, few eosinophils and occasionally spongiosis. Treatment with long-term metho-trexate or cyclosporine cleared the symptoms. Some major and minor diagnostic criteria for prurigo simplex are proposed and compared with chronic prurigo.

Background Prurigo Strophulus (PS) is a chronic and recurrent inflammatory disease caused by a hypersensitivity reaction to arthropod bites. The objective of this study was to determine the prevalence of PS and its clinical characteristic in our context. Methods We performed a cross‐sectional study during February to May 2017 in Dermatology's units of six hospitals in Yaoundé. Children with clinical signs of PS were included. A questionnaire was administered for data collection. Results A total of 112 children (62 boys and 50 girls) were included in the study, with a median age of 2 years; with range varied from 5 months to 16 years. The prevalence of PS was 5.4%. The most represented age group was 0–5 years (78.6%). PS lesions were most often located in exposed areas of body such as lower limbs (101/112; 90.2%), upper limbs (85/112; 75.9%) and face (19.6%). Papule‐vesicle (87.5%) was the predominant type of lesions. Conclusion PS is a common disease in Yaoundé (Cameroon). Papule‐vesicle lesions are the most frequent signs. It is usually found in exposure areas of body.

Actinic prurigo is a rare, idiopathic chronic photodermatosis of childhood characterized by excoriated papules, nodules, and plaques in sun-exposed areas. It is notoriously difficult to treat. The disorder involves a type IV hypersensitivity reaction driven by both Th1 and Th2 inflammatory pathways, the latter of which leads to secretion of IL-4, IL-5, IL-13, and production of B cells, IgE, and IgG4. Dupilumab, an IL-4 receptor antagonist, disrupts the Th2 pathway. We present a pediatric patient with severe, recalcitrant actinic prurigo who achieved rapid and sustained clearance with dupilumab.

Objectives Prurigo is a treatment‐resistant inflammatory disease of unknown etiology. Persistent and severe itch is a major and important clinical symptom, but pathological mechanisms and/or actual mediators for itch in prurigo remain to be defined. Methods We investigated blood levels of β‐endorphin, dynorphin A, and autotaxin in adult patients with prurigo (n = 18), including prurigo nodularis, prurigo chronica multiformis, and other forms. Patients with hemodialysis, hepatic dysfunction, and pregnancy were excluded. Immunofluorescence staining for β‐endorphin and autotaxin in lesional skin was also performed. In addition, β‐endorphin and autotaxin synthesis of cultured epidermal keratinocytes in response to several cytokine stimulation was assessed in vitro. Results Blood levels of β‐endorphin were higher in patients with prurigo than in control subjects (n = 20), while dynorphin A levels were comparable in the two groups. Blood autotaxin levels also were increased in patients with prurigo compared to control subjects. On the one hand, epidermal expression of β‐endorphin was increased in skin lesions of prurigo chronica multiformis, but not in prurigo nodularis. On the other hand, enhanced expression of autotaxin was observed in the epidermis in both prurigo nodularis and prurigo chronica multiformis. In vitro studies showed that β‐endorphin‐expressing epidermal keratinocytes increased following stimulation with TNF‐α, interleukin‐31, and/or IFN‐γ. Autotaxin was also detected in the cytoplasm of cultured epidermal keratinocytes. The fraction of autotaxin‐expressing cells increased when stimulated with TNF‐α and/or interleukin‐31. Conclusions Imbalance of opioid receptor signals and/or the autotaxin‐lysophosphatidic acid axis may contribute, at least in part, to the pathogenesis and intractable pruritus in some of the patients with prurigo.

Thalidomide has been used as an effective treatment for prurigo nodularis (PN) with a median dose of 200 mg, but the risk of peripheral neuropathy precludes long-term use. We analysed the efficacy of low-dose thalidomide (< 100 mg) in 17 patients with recalcitrant PN. Patients were initiated on thalidomide 50 mg on alternate days, and the dose was increased (doubled) in a stepwise manner, if needed, until a ≥ 50% reduction in score (partial response; PR) on a visual analogue scale (VAS) was achieved. Thalidomide then was continued at the same dose for 4 weeks to achieve ≥ 90% decrease in VAS score; if this was not achieved, the dose was increased to a maximum of 100 mg and continued until complete resolution of lesions (complete response; CR). Four patients discontinued thalidomide due to adverse effects. Four patients achieved PR, while 9 patients (n = 2 with 50 mg, n = 7 with 100 mg) achieved CR. No patient developed neuropathy. In addition, complete responders achieved an earlier ≥ 50% reduction in VAS score. Two patients relapsed after 12 months but responded to thalidomide 50 mg.

Chronic prurigo impairs quality of life and is immensely challenging to treat. Until recently, no clear definition or classification system was available for this disease. A European task force specialized in pruritus defined chronic prurigo as a distinct disease characterized by chronic pruritus, a lengthy scratching behaviour and the presence of pruriginous lesions. Papular, nodular, plaque, umbilicated and linear prurigo were identified as clinical subtypes according to the most prominent lesion type observed in the physical examination. Various clinical and pathophysiological aspects, which are common across the range of clinical manifestations of chronic prurigo, argue for chronic prurigo as a disease in its own right. Chronic prurigo should be clearly demarcated from other conditions such as so-called acute or subacute prurigo forms as well as from psychogenic self-inflicted skin lesions, since different diagnostic criteria apply for these diseases. This viewpoint essay provides a detailed definition and classification of chronic prurigo including its obligatory and associated diagnostic criteria and discusses chronic prurigo as a distinct disease as well as the demarcation to other relevant conditions.