[Solar urticaria and polymorphous light eruption]. / Lichturtikaria und polymorphe Lichtdermatose.

Solar urticaria is a rare idiopathic photodermatosis. According to the current knowledge its pathogenesis is most likely based on an allergic type I reaction to an autoantigen activated by ultraviolet (UV) radiation or visible light. As many of the patients suffer from severe forms of the disease, it may therefore severely impair the quality of life of those affected. In contrast, polymorphous light eruption is a very common disease, which, according to the current data, can be interpreted as a type IV allergic reaction to a photoallergen induced by UV radiation. As the skin lesions heal despite continued sun exposure, the patients' quality of life is generally not significantly impaired. These two clinically and pathogenetically very different light dermatoses have shared diagnostics by means of light provocation and an important therapeutic option (light hardening). Herein, we present an overview of the clinical picture, pathogenesis, diagnosis and available treatment options for the above-mentioned diseases.

Botanical Briefs: Fig Phytophotodermatitis (Ficus carica).

Patients presenting with a linear, erythematous, blistering eruption may experience a sudden painful sunburn that seems to get worse rather than better with time. In warm climates, exposure to the common fig tree (Ficus carica) may be the culprit. Dermatologists should recognize fig phytophotodermatitis as a possible cause and help the patient connect their symptoms with the inciting agent as well as administer proper treatment.

Sun pain and solar dysesthesia: A new challenge in clinical practice.

BACKGROUND: A few patients report intense pain and other unpleasant sensations, such as burning, dysesthesia and hyperalgesia, after even brief exposure to the sun and in the absence of any skin lesion. Sometimes they also develop systemic symptoms, such as mild fever, fatigue, faintness and fainting. As a result, these patients carefully avoid even short-term sun exposure with a consequent severe negative impact on their lives. METHODS: We have reviewed the clinical findings and the results of photobiological investigations of 10 patients who presented this clinical picture. Six of these patients were previously described by our group with the diagnosis of sun pain. We have reviewed the similarities with other previously described disorders such as solar dysesthesia and PUVA pain and have evaluated possible pathogenetic mechanisms. RESULTS: During phototesting our patients experienced intense pain in the exposed area and in the surrounding skin, without any visible lesion, even with very low sub-erythemal doses. At follow-up, five patients were diagnosed with fibromyalgia, three with a major depressive disorder, one with bipolar syndrome and one with a conversion disorder. The pathogenesis remains unclear, but the use of a psychopharmacological treatment with antidepressants improved both the neuropsychiatric symptoms and sensitivity to the sun in most subjects. CONCLUSION: For patients with pain and other severe symptoms in the absence of skin lesions and clinical and laboratory manifestations of known photodermatoses, a neuropsychiatric evaluation should be suggested.

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.

Equipment developed for simplifying routine phototesting in dermatology.

Some people react abnormally when exposed to sunlight by getting easily burned or develop a rash. When testing a patient's level of photosensitivity in the clinic, the UVR dose to provoke erythema is determined by the minimal erythema dose (MED) test. Subsequently, a photoprovocation test is performed to detect abnormal skin reactions by daily exposing the skin to UVR for several consecutive days. Associated problems in MED testing include choice of an even skin area for testing, patients keeping still during the test, testing with different UVR doses simultaneously, and securing clear borders of erythema. To address these issues, a MED Test Patch was developed which adheres closely to the skin to ensure sharp erythema borders and provides six irradiation fields with decremental doses of 20%. For MED testing, we constructed a solar simulator and LED lamps with peak emissions at 309 and 370 nm, small enough to be mounted directly on to the MED Test Patch and accommodate patient movements. These lamps and a 415 nm LED can also be used for provocation testing which is best performed on the back where the skin is assumed to have identical UVR sensitivity, and the area is large enough for adjacent MED and provocation test fields. Reading of erythema is still performed by visual and tactile evaluation. The UVA and UVB MED test can be performed in 1 h. The advantage of these developments is an easy-to-use, standardized test method with improved accuracy of the results.

Facial clues to the photosensitive trichothiodystrophy phenotype in childhood.

Among genodermatoses, trichothiodystrophies (TTDs) are a rare genetically heterogeneous group of syndromic conditions, presenting with skin, hair, and nail abnormalities. An extra-cutaneous involvement (craniofacial district and neurodevelopment) can be also a part of the clinical picture. The presence of photosensitivity describes three forms of TTDs: MIM#601675 (TTD1), MIM#616390 (TTD2) and MIM#616395 (TTD3), that are caused by variants afflicting some components of the DNA Nucleotide Excision Repair (NER) complex and with more marked clinical consequences. In the present research, 24 frontal images of paediatric patients with photosensitive TTDs suitable for facial analysis through the next-generation phenotyping (NGP) technology were obtained from the medical literature. The pictures were compared to age and sex-matched to unaffected controls using 2 distinct deep-learning algorithms: DeepGestalt and GestaltMatcher (Face2Gene, FDNA Inc., USA). To give further support to the observed results, a careful clinical revision was undertaken for each facial feature in paediatric patients with TTD1 or TTD2 or TTD3. Interestingly, a distinctive facial phenotype emerged by the NGP analysis delineating a specific craniofacial dysmorphic spectrum. In addition, we tabulated every single detail within the observed cohort. The novelty of the present research includes the facial characterization in children with the photosensitive types of TTDs through the 2 different algorithms. This result can become additional criteria for early diagnosis, and for subsequent targeted molecular investigations as well as a possible tailored multidisciplinary personalized management.

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.

Clinical characteristics of patients with human immunodeficiency virus and immune-mediated photodermatoses: A retrospective study of 39 patients.

BACKGROUND: HIV/AIDS patients are susceptible to various infectious and inflammatory dermatoses. No systemic work has been done on HIV/AIDS patients with immune-mediated photodermatoses in China. Here, we aim to determine the clinical features of immune-mediated photodermatoses in HIV/AIDS patients. METHODS: A retrospective analysis of HIV/AIDS patients with immune-mediated photodermatoses was carried out with demographic data, clinical characteristics, laboratory data, and follow-up data at the First Affiliated Hospital of Kunming Medical University between 2012 and 2019. The data were subjected to statistical analysis. RESULTS: A total of 39 HIV/AIDS patients with immune-mediated photodermatoses were enrolled, including 22 cases of polymorphic light eruption (PLE), 16 cases of chronic actinic dermatitis (CAD), and one actinic reticuloid. The CD4 count at the visit of the HIV-positive CAD group was lower than the PLE group (p = .049). The HIV-positive CAD group was more sensitive toward UVB than the PLE group (p = .020) and had a lower MED-UVB value (p = .044). There was no significant difference in UV tests among different categories of skin types. CONCLUSION: Immune-mediated photodermatoses are a manifestation of the advanced symptom of HIV infection, and sometimes also the presenting feature of HIV infection. Compared with HIV-positive PLE patients, CAD patients showed higher sensitivity to UVB radiation and had a lower MED-UVB value. The primary treatment for immune-mediated photodermatoses in HIV/AIDS patients is HAART and sun avoidance.