Algorithmic approach toward diagnosis of patients with congenital photosensitivity disorders and review of literature.

The congenital photosensitivity disorders present as cutaneous signs and symptoms secondary to photosensitivity, extracutaneous manifestations, and a predisposition to malignancy. Diagnosis of these conditions mainly depend on clinical findings as the molecular analysis is not always feasible. A review of all the related articles collected after a thorough literature search using keywords, "congenital AND photosensitivity NOT acquired" and the individual diseases was done. A total of 264 articles were included in the review. An algorithm for diagnosis of the different congenital photosensitivity disorders based on the various clinical presentations has been proposed. An early suspicion and diagnosis of the different congenital photosensitivity disorders is the cornerstone behind prompt institution of prevention and treatment, and decreasing the associated morbidity.

How I treat erythropoietic protoporphyria and X-linked protoporphyria.

Erythropoietic protoporphyria (EPP) is an inherited cutaneous porphyria caused by reduced expression of ferrochelatase, the enzyme that catalyzes the final step in heme biosynthesis. The resultant accumulation of protoporphyrin IX leads to severe, painful cutaneous photosensitivity, as well as potentially life-threatening liver disease in a small percentage of patients. X-linked protoporphyria (XLP) is clinically similar to EPP but results from increased activity of δ-aminolevulinic acid synthase 2, the first step in heme biosynthesis in the bone marrow, and also causes protoporphyrin accumulation. Although historically the management of EPP and XLP (collectively termed protoporphyria) centered around avoidance of sunlight, novel therapies have recently been approved or are in development, which will alter the therapeutic landscape for these conditions. We present 3 patient cases, highlighting key treatment considerations in patients with protoporphyria, including (1) approach to photosensitivity, (2) managing iron deficiency in protoporphyria, and (3) understanding hepatic failure in protoporphyria.

Photodermatoses: what's new.

PURPOSE OF REVIEW: The purpose of this review is to summarize and highlight the recent literature in photodermatoses. In the past year, there have been many developments in this heterogeneous group of conditions. RECENT FINDINGS: This review is divided by photodermatoses type, which include idiopathic photodermatoses, photodermatoses secondary to exogenous agents, photodermatoses secondary to endogenous agents (the porphyrias), and genodermatoses. The idiopathic photodermatoses section focuses on case series and reports highlighting new disease presentations or further disease characterization and new treatment strategies for these disorders. The second section discusses a unique case and has a brief update on photoallergens. Clinical, diagnostic, and treatment updates for porphyrias are discussed in Section 3. For genodermatoses, we discuss complications and neoplastic risk of xeroderma pigmentosum and a few highlights from other rare disorders. Finally, we conclude with a brief overview of photoprotection updates, from assessing sun-damaged skin to the most effective photoprotective agents. SUMMARY: Up-to-date information will help providers identify and manage this rare group of disorders.

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.

Diltiazem-associated Photodistributed Hyperpigmentation.

Diltiazem is a calcium-channel blocker commonly used for the treatment of hypertension. Common adverse effects include dizziness, headache, and edema. Fewer than 20 cases of diltiazem-associated photodistributed hyperpigmentation have been reported in the literature. Here, we present the case of a 71-year-old woman with new-onset facial hyperpigmentation 6 months after initiating treatment with diltiazem.

Dermatitis actínica crónica / Chronic actinic dermatitis

RESUMEN La dermatitis actínica crónica (DAC) - dermatitis por fotosensibilidad, reacción solar persistente o su variante extrema el reticuloide actínico- es una fotodermatosis crónica, propia del adulto más frecuente en el sexo masculino provocada por rayos ultravioletas (UVB), (UVA) y luz visible. El diagnóstico es clínico, caracterizado por placas eccematosas y liquenificadas pruriginosas en aéreas expuestas al sol. Se cree que el DAC se debe a la fotosensibilización secundaria de un antígeno endógeno de la piel.

ABSTRACT Chronic actinic dermatitis (ACD) - photosensitivity dermatitis, persistent solar reaction or its extreme variant actinic reticuloid - is a chronic photodermatosis, typical of the adult male caused by ultraviolet (UVB), (UVA) and visible light. The diagnosis suggested by clinical researchers, characterized by eczematous and lichenified pruritic plaques in areas exposed to the sun. It is believed that DAC is due to the secondary photosensitization of an endogenous skin antigen.

Photosensitivity and cancer immune-targeted therapies.

The novel group of immunological agents used for solid tumors has importantly improved the quality of life and the survival rate of oncologic patients. Compared to conventional chemotherapy agents, they are more effective and less toxic. However, adverse cutaneous effects are commonly observed, and in some cases, they may induce treatment discontinuation, with heavy impact on patient prognosis. Among these, photosensitive reactions, either phototoxic or photoallergic, are increasing. Much remains to be clarified on the understanding of their prevention, diagnosis, and management. We have reviewed the literature about photosensitive reactions occurring during oncologic immunotherapies. Early dermatological diagnosis and adequate management, with oncologist's cooperation, is fundamental.

Phototherapy in the Evaluation and Management of Photodermatoses.

Ultraviolet light (UV) and visible light are important components in the diagnosis of photodermatoses, and UV has the unique ability to also be used to manage photodermatoses. Phototesting, provocative light testing, and photopatch testing can provide important information in diagnosing patients with photodermatoses; phototesting can be used to determine the starting dose for phototherapy in these patients. Once photosensitivity is established, narrowband UVB and UVA1 therapy have helped to improve the quality of life of photosensitive patients, such as those with polymorphous light eruption, chronic actinic dermatitis, and solar urticaria.

Photosensitivity and photoprotection in patients with lupus erythematosus.

Lupus erythematosus (LE) represents a spectrum of inflammatory autoimmune disease comprising varying clinical entities ranging from primary cutaneous to systemic disease. There is a clear relationship between ultraviolet irradiation (UVR) and the clinical manifestations of LE in both adult and pediatric populations. Although it has been established that UVR exacerbates pre-existing LE, it remains unclear whether UVR induces the development of the disease. This review serves to discuss effective photoprotective measures in LE and describe the pathogenic relationship of UVR and LE.

Mild cutaneous reactions to drugs.

Adverse reactions to drugs are not frequent in childhood. Cutaneous reactions are the most frequent in this age group. Mild cutaneous reactions are immediate or delayed adverse reactions that do not seriously compromise the clinical condition of children. The patients usually early improve and recover the state of health. Although it is difficult to define the prevalence accurately, we could affirm that the rate adverse reaction to drugs are often over estimated by both the families and the physicians. Therefore, children may be prone to loss of school days and inappropriate or sub-optimal treatments. However, the identification of a true adverse reaction to drugs allows adequate treatment and alert to further exposure to harmful drugs.

[The cutaneous porphyrias]. / Porphyries cutanées.

The porphyrias are a group of metabolic disorders resulting from an innate abnormality in haem biosynthesis, and the clinical settings of which vary according to the genetic enzyme abnormality in question. These are genetic disorders with autosomal dominant or recessive inheritance of varying penetrance, and whose clinical expression differs according to the preferential location of haem precursors. Different classifications have been proposed according to genetic inheritance, the enzyme anomaly at issue, and clinical expression. The clinical classification distinguishes between acute porphyria (acute intermittent porphyria, porphyria variegata, hereditary coproporphyria), bullous cutaneous porphyrias (porphyria cutanea tarda, porphyria variegata and hereditary coproporphyria), painful photosensitive acute cutaneous porphyrias (erythropoietic protoporphyria and X-linked dominant protoporphyria), and rare recessive porphyrias (congenital erythropoietic porphyria, Doss porphyria, hepatoerythropoietic porphyria and harderoporphyria). Treatment depends on the clinical expression of the disorder.

Clinical advances in photosensitive epilepsy.

Photosensitive epilepsy is the most common subset of reflex epilepsy in humans. It is characterized by an abnormal electroencephalogram trait known as photoparoxysmal response (PPR) corresponding to ictal symptomology in response to intermittent photic stimulation. Increased exposure to photic stimulation through technological and societal advance has had a significant impact on the incidence and interest in photosensitive epilepsy. Paralleled scientific developments have deepened our understanding of the neurophysiology underlying photosensitivity, especially in the context of deep genomic sequencing and neuroimaging. Furthermore, given the relatively predictable nature of photosensitive epilepsy, there is substantial interest in translating advancements in this field to epilepsy as a whole. Herein, we comprehensively review the epidemiology, clinical features and novel insights into the mechanism behind photosensitive epilepsy with a focus on the clinical implications of new advancements.