Drug-Induced Pseudoporphyria: A Case Report.

Pseudoporphyria is an uncommon dermatosis resembling porphyria cutanea tarda (PCT). The exclusion of true porphyria, especially PCT, is critically essential for diagnosing pseudoporphyria. It has an unknown underlying pathophysiology with a normal or near-normal porphyrin profile. Pseudoporphyria has been associated with chronic renal failure and hemodialysis, medications, and tanning beds. In drug-induced pseudoporphyria cases, eliminating the suspected photosensitizing drug improves the disease typically within weeks to months (on average eight weeks). In genetically predisposed individuals, phototoxic metabolites may trigger the development of skin fragility, bullae, milia, and scarring on the dorsum of the hands and other sun-exposed areas. Wearing a broad-spectrum sunscreen and maintaining strict ultraviolet protection is essential in cases of pseudoporphyria. We report the case of a 20-year-old male who presented to us with complaints of photosensitivity and multiple erosions with irregular scars over photo-exposed areas involving the dorsum of the hands and face predominantly. The patient was evaluated further to determine the underlying cause. A wood's lamp examination of the urine was done, which did not show fluorescence. Based on clinical and laboratory findings, the diagnosis of pseudoporphyria was made, and the patient was started on the oral antimalarial agent hydroxychloroquine sulfate with strict sun protection.

Lupus Erythematosus Tumidus as a Distinct Uncommon Subtype of Cutaneous Lupus Erythematosus: A Case Report and Review.

Lupus erythematosus tumidus (LET) is an uncommon but distinct photosensitive subtype of cutaneous lupus erythematosus (CLE). It differs from discoid and subacute cutaneous lupus erythematosus (SCLE) clinically and pathologically. LET is marked by extreme photosensitivity and carries a much lower risk of progression to systemic disease. The differential diagnosis of LET includes polymorphic light eruption (PMLE) and Jessner's lymphocytic infiltration of the skin (JLIS) because of subtle alterations in the histopathology and the paucity of immunopathologic markers in LET. We report herein a case of LET with positive immunoglobulin (Ig) deposits on direct immunofluorescence (DIF) testing. LET resolved completely with strict sun avoidance and treatment with topical corticosteroids, without the sequelae of atrophy, scarring, or dyspigmentation.

Fitofotodermatosis: el peligro del sol y las plantas para la piel / Phytofotodermatosis: the danger of the sun and plants for the skin

Presentamos el caso de un paciente que padeció una reacción cutánea intensa tras el contacto con los frutos y las hojas de una higuera y posterior exposición solar. Se trata de una reacción no inmunológica llamada fitofotodermatosis. Es una erupción inflamatoria de la piel que se produce tras el contacto con sustancias fototóxicas (furocumarinas o psoralenos) y que puede llegar a ser de gran intensidad. Por ello requiere una anamnesis exhaustiva y su conocimiento por parte del equipo médico de Atención Primaria y pediatría, ya que su diagnóstico diferencial puede ser muy complejo.(AU)

We report the case of a patient who developed an intense skin reaction after contact with the fruits and leaves of a fig tree and subsequent sun exposure. This is a non-immunological reaction called phytophotodermatosis. It is an inflammatory skin rash that occurs after contact with phototoxic substances (furocoumarins or psoralens) and could be very intense. That is why it requires an in-depth history; its knowledge by primary care physicians and paediatricians, is important because the differential diagnosis could be very complex.(AU)

Solar urticaria involves rapid mast cell STAT3 activation and neutrophil recruitment, with FcεRI as an upstream regulator.

BACKGROUND: Solar urticaria is a rare photodermatosis characterized by rapid-onset sunlight-induced urticaria, but its pathophysiology is not well understood. OBJECTIVE: We sought to define cutaneous cellular and molecular events in the evolution of solar urticaria following its initiation by solar-simulated UV radiation (SSR) and compare with healthy controls (HC). METHODS: Cutaneous biopsy specimens were taken from unexposed skin and skin exposed to a single low (physiologic) dose of SSR at 30 minutes, 3 hours, and 24 hours after exposure in 6 patients with solar urticaria and 6 HC. Biopsy specimens were assessed by immunohistochemistry and bulk RNA-sequencing analysis. RESULTS: In solar urticaria specimens, there was enrichment of several innate immune pathways, with striking early involvement of neutrophils, which was not observed in HC. Multiple proinflammatory cytokine and chemokine genes were upregulated (including IL20, IL6, and CXCL8) or identified as upstream regulators (including TNF, IL-1ß, and IFN-γ). IgE and FcεRI were identified as upstream regulators, and phosphorylated signal transducer and activator of transcription 3 expression in mast cells was increased in solar urticaria at 30 minutes and 3 hours after SSR exposure, suggesting a mechanism of mast cell activation. Clinical resolution of solar urticaria by 24 hours mirrored resolution of inflammatory gene signature profiles. Comparison with available datasets of chronic spontaneous urticaria showed transcriptomic similarities relating to immune activation, but several transcripts were identified solely in solar urticaria, including CXCL8 and CSF2/3. CONCLUSIONS: Solar urticaria is characterized by rapid signal transducer and activator of transcription 3 activation in mast cells and involvement of multiple chemotactic and innate inflammatory pathways, with FcεRI engagement indicated as an early event.

Fig Tree-Induced Phytophotodermatitis: A Case Report on the Perils of a Hobby.

Phytophotodermatitis, a condition that results from sequential skin exposure to phototoxic chemicals contained within plants, followed by exposure to solar ultraviolet radiation, has been described with several plants and plant-based foods, namely members of the Moraceaefamily, which include Ficus carica L. This tree's branches, leaves, and fruit skin exude a milky sap or latex containing proteolytic enzymes and furocoumarins known to be photoirritants, easily absorbed upon skin contact. Oxygen-dependent and independent toxic reactions subsequent to sun exposure promote cell membrane damage and oedema, consequently leading to cell death. The diagnosis is confirmed with a detailed anamnesis, and photopatch testing is often useful to rule out a differential diagnosis. It is typically a self-limited condition, with few cases requiring treatment with topical or systemic corticosteroids. We report on a 55-year-old male patient who, following picking figs and pruning a fig tree while exposed to sunlight, developed erythematous and pruritic maculopapular lesions that progressed to blisters with residual hyperpigmentation. The diagnosis was further corroborated through photopatch testing, and the patient was recommended to avoid this recreational activity without symptoms' relapse. This case highlights the importance of considering phytophotodermatitis as a differential diagnosis when evaluating cases of dermatitis on exposed body surfaces and the importance of an exhaustive anamnesis. Identification of specific plant triggers and the performance of photopatch tests are essential to help confirm the diagnosis and guide avoidance recommendations.

Research progress and directions in photodermatology / 中华皮肤科杂志

As an important subdiscipline of dermatology, photodermatology has developed rapidly in recent years. It is not only theoretical science of photodamage-related dermatoses, but also practical science of treatment of photodamage-related dermatoses. This article introduces the basis of photobiology, the pathogenesis of photodamage-related dermatoses, and research hotspots in sunscreens, and elaborates new progress in the clinical application of targeted phototherapy and home phototherapy, with a view to providing a theoretical basis and practical guidance for the treatment of photodamage-related dermatoses.

Photohardening therapy for idiopathic actinic dermatoses / 中华皮肤科杂志

Photohardening therapy, also known as photodesensitization therapy, refers to the phototherapy and photochemotherapy of idiopathic actinic dermatoses, and its goal is to improve the patients′ tolerance to sunlight and prevent disease flares. Its mechanisms of action involve a variety of cellular and inflammatory factors. This therapy is suitable for all idiopathic actinic dermatoses, with definite efficacy and good safety. However, the treatment specificity usually leads to poor compliance. The development of UVA1 rush hardening and home phototherapy is expected to solve this problem.

Characterisation of serum lipidomic profiles in patients with chronic actinic dermatitis based on liquid chromatography-mass spectrometry / 中华皮肤科杂志

Objective:To investigate serum lipidomic profiles in patients with chronic actinic dermatitis (CAD), and to search for biomarkers of CAD.Methods:A retrospective analysis was conducted. Serum samples were collected from 46 patients with CAD and 16 age- and gender-matched healthy controls in the Guangzhou Institute of Dermatology from April 2011 to December 2021. Changes in serum lipid composition and expression were assessed by liquid chromatography-mass spectrometry. Principal component analysis, partial least squares discriminant analysis, and orthogonal partial least squares discriminant analysis were performed to screen differential biomarkers, and receiver operating characteristic (ROC) curve analysis was conducted to screen diagnostic markers. Comparisons of the age and gender distribution between groups were performed using t test and chi-square test, respectively. Results:The 46 CAD patients were aged from 30 to 84 (60.39 ± 10.52) years, including 41 males and 5 females; the 16 healthy controls were aged from 50 to 89 (59.81 ± 10.72) years, including 14 males and 2 females; there were no significant differences in the age or gender distribution between the two groups (age: t = 0.19, P = 0.853; gender: χ2 = 0.03, P = 0.859). Totally, 4 136 lipid molecules belonging to 40 subclasses were identified in the serum samples from CAD patients as well as healthy controls. Twenty-two differential lipid molecules were identified between the CAD patients and healthy controls, belonging to 9 subclasses (triglycerides, sphingomyelin, phosphatidylserine, phosphatidylethanolamine, monofatty acid glycerides, lysophosphatidylcholine, hexose ceramide, diglycerides, and cardiolipin). When the combinations of triglycerides (37.7e) and Na, those of monoglycerides (22.3) and NH 4, or those of phosphatidylserine (18.0_18.1) and H served as diagnostic markers separately, the areas under the ROC curve (AUCs) were all > 0.8, and the AUCs of 16 differential lipid molecules were all > 0.7. Conclusion:The serum lipid composition differed between healthy controls and CAD patients, and the combinations of triglycerides (37.7e) and Na, those of monoglycerides (22.3) and NH 4, and those of phosphatidylserine (18.0_18.1) and H may be promising biomarkers for the diagnosis of CAD.

Chronic actinic dermatitis: A 5-year clinical analysis of 488 patients in China.

BACKGROUND/PURPOSE: Chronic actinic dermatitis (CAD) is a spectrum of diseases with chronic photosensitivity occurring mostly among middle-aged and older men. We seek to explore the characteristics and pathogenesis of CAD among the Chinese population. METHODS: The medical records of 488 CAD cases diagnosed by phototesting at Huashan Hospital, Fudan University from January 2014 to December 2018 were analyzed retrospectively. RESULTS: Among the 488 patients, 344 were male and 144 were female. 84.8% of the cases were over 40 years old at the age of onset, while the remaining with an early age of onset had a prevalence of atopic history of 21.6%. Up to 45.0% of the patients reported excessive sun exposure and outdoor activities before the initiation of symptoms. The typical skin lesions were erythema, papules and plaques laid predominantly in sun-exposed areas. 42.8% of the cases showed sensitivity to UVB only, 20.7% were both sensitive to UVA and UVB, and 18.2% had UVA sensitivity only. The most predominant photoallergens were chlorpromazine (80.1%), thimerosal (17.2%), potassium dichromate (12.7%), etc. The most prevalent patch test allergens were potassium dichromate (24.4%), thimerosal (20.5%), formaldehyde (16.8%), etc. CONCLUSIONS: CAD was more commonly seen in males over 40 years old. The action spectrum of Chinese patients is primarily in the UVB range. Exposure to excessive sunlight or contact allergens and photoallergens are important risk factors. Photobiology tests are essential in detecting photosensitivity and recognizing potential photosensitizers. Early avoidance of confirmed photoallergens and sun exposure may prevent photosensitive reactions from progressing into persistent photosensitivity.

Review on photoprotection: a clinician's guide to the ingredients, characteristics, adverse effects, and disease-specific benefits of chemical and physical sunscreen compounds.

Photoprotection is a critical health prevention strategy to reduce the deleterious effects of ultraviolet radiation (UVR) and visible light (VL). Methods of photoprotection are reviewed in this paper, with an emphasis on sunscreen. The most appropriate sunscreen formulation for personal use depends on several factors. Active sunscreen ingredients vary in their protective effect over the UVR and VL spectrum. There are dermatologic diseases that cause photosensitivity or that are aggravated by a particular action spectrum. In these situations, sunscreen suggestions can address the specific concern. Sunscreen does not represent a single entity. Appropriate personalized sunscreen selection is critical to improve compliance and clinical outcomes. Health care providers can facilitate informed product selection with awareness of evolving sunscreen formulations and counseling patients on appropriate use. This review aims to summarize different forms of photoprotection, discuss absorption of sunscreen ingredients, possible adverse effects, and disease-specific preferences for chemical, physical or oral agents that may decrease UVR and VL harmful effects.

Pediatric Cutaneous Oncology: Genodermatoses and Cancer Syndromes.

Skin cancers represent the most common malignancy worldwide. In children, the diagnosis of skin cancer is rare and raises the possibility of an underlying genetic predisposition. Recent molecular advances have increased understanding of certain genetically determined regulatory pathways that constantly protect the skin from atypical cell growth and cancer. Knowledge about these underlying gene defects aids a dermatologist's ability to recommend confirmatory genetic testing and provides potential targets for future therapies. In this review, we outline genetic conditions important to dermatologists that are associated with skin cancer development and review the current approaches to the management of these patients.

In vitro Effect of Photodynamic Therapy with Curcumin and Methylene Blue Photosensitizers on Staphylococcus Aureus.

Statement of the Problem: Staphylococcus aureus (S.A) can colonize in the skin, nasal cavity, and oral cavity. In the oral cavity, it can cause dental caries and periodontal disease. Mouthwashes can be used as an adjunct to mechanical plaque control methods to decrease the load of oral microorganisms. Chlorhexidine (CHX) is a commonly used antimicrobial mouthwash with side effects such as changing the sense of taste, tooth discoloration, oral mucosal burning, allergy, and xerostomia. It also has adverse systemic effects, if swallowed. Purpose: This study aimed to assess the effect of photodynamic therapy (PDT) with curcumin and methylene blue (MB) photosensitizers and different laser parameters on S.A colony count. Materials and Method: In this in vitro experimental study, 99 samples of standard-strain S.A were subjected to PDT with curcumin and MB photosensitizers with/without irradiation of 660 and 445 nm laser with different exposure parameters, and CHX in 9 groups (n=11). The samples were cultured in microplates containing Mueller-Hinton agar, and the number of colony forming units (CFUs) was counted after 24 h of incubation at 37°C. Data were analyzed by the Kruskal-Wallis and Dunn tests. Results: The minimum colony count was noted in CHX group (CFUs=0) followed by MB and 660nm diode laser group irradiated for 100 s (CFUs=147.2727±169.35707). The difference in this respect was significant between MB+660nm diode laser for 100 s and other groups (p< 0.05) except for the MB + 660 nm diode laser for 60 s group. Conclusion: CHX is superior to laser for elimination of S.A. However, PDT with 660 nm diode laser + MB has considerable antimicrobial efficacy against S.A; increasing the duration of laser irradiation enhances the antimicrobial effect.

Pathogenesis of solar urticaria: Classic perspectives and emerging concepts.

Solar urticaria is a rare, immunologically mediated photodermatosis in which activation of cutaneous mast cells is triggered by specific wavelengths of solar electromagnetic radiation. This manifests clinically as the rapid development of cutaneous itch, erythema and wheal formation after several minutes of sun exposure. Disease mechanisms in solar urticaria remain incompletely elucidated and there have been few recent investigations of its pathobiology. Historic passive transfer experiments performed during the twentieth century provide support for a 'photoallergy' model of disease pathogenesis, wherein molecular alteration of a putative chromophore by solar electromagnetic radiation produces mast cell activation via an IgE-dependent mechanism. However, this model does not account for several observations made during passive transfer experiments nor does it explain a range of subsequent clinical and photobiological observations made in solar urticaria patients. Furthermore, increased understanding of the molecular dynamics underpinning cutaneous mast cell responses highlights the need to reformulate our understanding of solar urticaria pathogenesis in the context of this contemporary scientific landscape. In this review, we discuss the current understanding of solar urticaria pathogenesis and, by incorporating recent scientific and clinical observations, develop new hypotheses to drive future investigation into this intriguing disorder.

Actinic prurigo in a dermatological reference center in Colombia: 108 cases / Prurigo actínico en un centro dermatológico de referencia en Colombia: 108 casos.

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.

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.

Prurigo actínico en un centro dermatológico de referencia en Colombia: 108 casos / Actinic prurigo in a dermatological reference center in Colombia: 108 cases

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.

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.

A qualitative study of knowledge, behaviour and attitudes regarding vitamin D acquisition among patients with photosensitivity disorders.

BACKGROUND: Cutaneous exposure to sunlight is a major source of vitamin D. Individuals with photosensitivity disorders have symptoms provoked by sunlight and may not achieve the brief sunlight exposures that convey vitamin D acquisition. OBJECTIVE: To explore knowledge, behaviour and attitudes towards vitamin D and its acquisition in patients with photosensitivity. METHODS: Patients (n = 19) diagnosed with solar urticaria, erythropoietic protoporphyria or polymorphic light eruption at a specialist photoinvestigation centre participated in semi-structured focus groups to discuss vitamin D knowledge, acquisition behaviours and attitudes towards vitamin D acquisition through sunlight and diet. Discussions were analysed by thematic analysis using MAXQDA11. RESULTS: Knowledge of vitamin D was variable. There was good awareness that sunlight exposure is an important source but knowledge of dietary sources was poor. Patients had little concern for their own vitamin D status prior to attending the photoinvestigation centre. Most patients avoided sunlight exposure, were unable to achieve the guidance on sun exposure for healthy individuals and were aware this could affect their vitamin D status. Use of oral vitamin D supplements was common, and all were willing to consider supplements if required. Patients recommended improving education of clinicians to increase patient awareness of vitamin D, CONCLUSIONS: More targeted guidance is required on acquisition of vitamin D for patients with photosensitivity, supported by increased patient and clinician education.