Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection.

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at higher risk of developing a severe course of infections especially viral and malignancies than the general population. The aim of the study was to present complex diagnostic and therapeutic management of HV-LPD. The clinical diagnosis was confirmed at the histological and molecular level with next generation sequencing. HV-LPD was diagnosed in a patient with CVID and chronic active Epstein-Barr virus (CAEBV) infection. The patient was refractory to CHOP chemotherapy and immunosuppressive treatment in combination with antiviral drugs (prednisone, bortezomib, gancyclovir). The third-party donor EBV-specific cytotoxic T cells (EBV-CTL, tabelecleucel) were used, which stabilised the disease course. Finally, matched unrelated donor hematopoietic cell transplantation (MUD-HCT) was performed followed by another cycle of EBV-CTL.

Hydroa vacciniforme and solar urticaria.

Hydroa vacciniforme (HV) and solar urticaria (SU) are uncommon immunologically mediated photodermatoses. HV occurs almost exclusively in children, usually beginning in childhood and remitting spontaneously by adolescence. Association with chronic Epstein-Barr virus infection has been reported in HV, which raises the possibility of lymphoproliferative disorders in these patients. SU is characterized by skin erythema, swelling, and whealing immediately after sun exposure. Although several treatment options are available, the management of both conditions remains a challenge.

Efficacy of ω-3 polyunsaturated fatty acids for the treatment of refractory hydroa vacciniforme.

Hydroa vacciniforme (HV) is a rare photodermatosis. Several therapies, with sometimes severe side effects, have been used in isolated cases. We report a case of refractory HV successfully treated with dietary fish oil rich in ω-3 polyunsaturated fatty acids.

A survey examination of patients with hydroa vacciniforme.

Hydroa vacciniforme is a rare acquired photosensitivity disorder that manifests in childhood. We sought to identify the demographics, disease progression, symptoms, risk factors, and treatment efficacy associated with hydroa vacciniforme using a questionnaire. Nineteen surveys were included in the data analysis. Symptoms occurred throughout the year and the most common dermatologic manifestations included redness, blisters, bumps, scars, itching, and burning, predominantly on sun-exposed areas. The most common associated symptoms included oral ulcers, abdominal pain, and eye ulcers. Most participants limited their sun exposure to less than 10 hours weekly and stated sunscreen somewhat helped prevent breakouts. Most participants had no to limited improvement of their symptoms with treatments.

Erupción vesiculosa intermitente en un niño / Intermittent vesiculous eruption in a child

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Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma.

An 8-year-old Taiwanese girl had a 6-month history of a relapsing papulovesicular eruption on her face that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of large atypical lymphocytic cells expressing CD8. TCR-gamma gene rearrangement study revealed a monoclonal band present in the DNA extracted from the specimen. A diagnosis of CD8+ cutaneous T-cell lymphoma (CTCL) was made. The patient was treated with Chinese herbal drugs and her skin lesions waxed and waned. At this writing, 11 months after establishment of the diagnosis, the skin lesions have been limited to the facial area and no definite evidence of systemic involvement is noted. To our knowledge, this is the first case of CD8+ primary CTCL with clinical features resembling HV.

[Hydroa vacciniforme: dietary fish oil]. / Hydroa vacciniforme: traitement par huiles de poisson (Maxepa(R)).

BACKGROUND: Hydroa vaccinniforme is a highly uncommon photodermatosis acquired in childhood. The clinical course is dominated by the risk of varioliform scars. Numerous treatments have been proposed with variable efficacy. One recent open study suggested dietary fish oil could be useful. We report a case of hydroa vacciniforme treated successfully with dietary fish oil (Maxepa(R)). CASE REPORT: A 15-year-old girl consulted in May 1988 for vesiculobullous lesions typical of hydroa vacciniforme in photo-exposed areas. Anti-malaria drugs and photoprotection had been used for several years without success. Maxepa(R) was introduced in June 1998 and was followed by regression of the lesions within a few weeks despite the summer season. The treatment was interrupted at the patient's request due to fetid breath. Reintroduction of Maxepa(R) in April 1999 after an episode of recurrent lesions, again led to total resolution of the lesions within three weeks. DISCUSSION: Hydroa vacciniforme is an exceptional photodermatosis of uncertain etiology. It may possibly be related to an abnormal sensitivity to ultraviolet A. Fish oil rich in 3-omega polyunsaturated fatty acids would reduce the local inflammation triggered by sun exposure. Recent studies have demonstrated that dietary fish oil can increase the level of 3-omega polyunsaturated fatty acids in the epidermis and reduce the level of prostaglandins in the skin. Our case would appear to confirm the contribution of dietary fish oil to treatment despite the poor tolerance due to fetid breath.

Hydroa vacciniforme: A clinical and follow-up study of 17 cases.

BACKGROUND: Hydroa vacciniforme (HV) is a rare, sporadic, idiopathic photodermatosis characterized by vesicles and crust formation after sunlight exposure. The lesions typically heal with vacciniform scarring. OBJECTIVE: We identify and review the clinical features and follow-up data of Scottish patients with HV and report on the prevalence of this condition. This is the largest recent study of HV patients from a single center. METHODS: In this retrospective study, patients with HV were identified by means of the diagnostic database from the Photobiology Unit, Dundee. Patients were contacted and details of clinical features, duration of disease, results of investigations, and treatment were recorded. At review, disease progress was assessed. RESULTS: Between 1973 and 1997, 17 patients (9 males and 8 females) with a diagnosis of HV were investigated. Data from 15 patients showed a mean age at onset of 7.9 years (range, 1 to 16 years), with females (mean, 6.7 years; range, 2 to 12 years) having an earlier onset than males (mean, 8.7 years; range, 1 to 16 years). A bimodal age distribution was also identified with onsets between the ages of 1 and 7 years and 12 and 16 years. At review, spontaneous clearing had occurred in 9 patients (60%) with mean duration of disease being 9 years (range, 4 to 17 years). Males had longer disease duration (mean, 11 years; range, 5 to 17 years) than females (mean, 5 years; range, 4 to 7 years). Eight patients (53%) were sensitive in the UVA wave-band on monochromator phototesting, and 6 (40%) experienced papulovesicular lesions on repetitive broad-spectrum UVA irradiation. All patients received broad-spectrum sunscreens with variable results. Of the 5 patients treated with narrow-band UVB (TL-01) phototherapy, 3 reported beneficial results with an increase in tolerance to sunlight exposure and associated reduction in disease severity. CONCLUSION: The estimated prevalence of HV was at least 0.34 cases per 100,000 with an approximately equal sex ratio. Males had a later onset and longer duration of disease than females. Phototesting showed abnormal responses in the UVA wavebands in 53% of cases, whereas 60% of patients treated with prophylactic TL-01 phototherapy found it beneficial.

Dietary fish oil as a photoprotective agent in hydroa vacciniforme.

Hydroa vacciniforme is a troublesome and scarring photosensitivity disorder for which treatment is unsatisfactory. Dietary fish oil rich in omega-3 polyunsaturated fatty acids reportedly increases the resistance to ultraviolet-induced erythema and rash provocation in polymorphic light eruption. We report for the first time the response of hydroa vacciniforme to dietary fish oil. Three Caucasian boys with the condition were placed on MaxEPA, five capsules daily. Phototesting was performed at baseline and after 3 months supplementation. At baseline, low erythemal thresholds were seen to monochromated UVA at 350 and 370 nm in all three boys, while one also had a low threshold to 320 nm (UVA) and another showed a low threshold to 300 nm (UVB). Broad-band UVA provocation challenge produced typical skin lesions in all the subjects. Following fish oil, all the boys showed reduced erythemal sensitivity to UVA and one also showed reduced sensitivity to UVB. Provocation challenge revealed a reduced response in all three children. Clinically, these changes were accompanied by pronounced improvement in one child, mild improvement in the second child, but no improvement in the third. The third boy subsequently showed good clinical response to azathioprine.

A clinical study on severe hydroa vacciniforme.

OBJECTIVE: To describe a severe type of hydroa vacciniforme (HV) in order to provide basis for treatment and prevention of this disease. METHODS: Nine cases of HV were collected in our outpatient department, and clinical and laboratory examinations were performed. RESULTS: All the cases had erythemas, papules, bullae, erosions or ulcers on exposed sites, with recurrences, gradually resulting in deformation. Five patients suffered from hand deformation manifesting rigidity, flexor tetanus, or malposition of the first, second and third interphalangeal joints of hands. Two of the above mentioned patients manifested partial bone absorption of fingers. Five patients showed partial defect of auricle. Two patients showed saddle nose, and partial absorption of nasal cartilage. One patient displayed cicatricial contracture of lower lip, with incisor extrusion. Four patients showed opacity of cornea. Through laboratory examinations, porphyrinopathies were excluded. Phototests showed increased sensitivity to ultraviolet-A radiation (UVA). CONCLUSIONS: Based on the clinical features and laboratory examinations, these patients were diagnosed as having severe HV. It is believed that this condition belongs to a disease spectrum induced by ultraviolet light. Our analysis suggests that for children with severe type of HV, preventive measures and therapeutic agents should be taken as soon as possible in order to avoid deterioration of the disease and malformations.

Dietary fish oil reduces basal and ultraviolet B-generated PGE2 levels in skin and increases the threshold to provocation of polymorphic light eruption.

The sunburn response is markedly reduced by dietary fish oil rich in omega-3 polyunsaturated fatty acids. Because prostaglandins mediate the vasodilatation, we examined the effect of fish oil on ultraviolet (UV) B-induced prostaglandin metabolism. In addition we assessed the potential photoprotective effect of fish oil in light-sensitive patients. Thirteen patients with polymorphic light eruption received dietary supplements of fish oil rich in omega-3 polyunsaturated fatty acids for 3 months. At baseline and 3 months, the minimal erythema dose of UVB irradiation was determined, and a graded UVA challenge given to a forearm to assess the threshold dose for papule provocation. Suction blisters were raised on the other forearm, on control skin, and on skin irradiated with four times the minimal erythema dose of UVB 24 h previously, and blister fluid prostaglandin E2 was measured by radioimmunoassay. Following 3 months of fish oil, the mean minimal erythema dose of UVB irradiation increased from 19.8 +/- 2.6 to 33.8 +/- 3.7 mJ/cm2 (mean +/- SEM), p < 0.01. The UVA provocation test was positive in 10 patients at baseline, and after 3 months nine of these showed reduced sensitivity to papule provocation, p < 0.001. Before fish oil, PGE2 increased from 8.6 (SEM 2.1) ng/ml in control skin to 27.2 (11) ng/ml after UVB, p < 0.01. Following 3 months of fish oil, PGE2 decreased to 4.1 (1) and 9.6 (2.4) ng/ml in control and irradiated skin, respectively, p < 0.05. Reduction of UV-induced inflammation by fish oil may be due, at least partially, to lowered prostaglandin E2 levels. The photoprotection against UVA-provocation of a papular response suggests a clinical application for fish oil in polymorphic light eruption.

Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses.

Twenty patients with photodermatoses [actinic prurigo (n = 6), hydroa vacciniforme (n = 4), idiopathic solar urticaria (n = 1), amiodarone-induced photosensitivity (n = 1) and a range of cutaneous porphyrias (n = 8)] were treated with a 'hardening' course of narrow-band ultraviolet B (TL-01) phototherapy in springtime. The response to phototherapy was monitored subjectively, by interviewing patients after the summer, and objectively by monochromator phototesting, before and after phototherapy. Fifteen patients reported that treatment was worthwhile. Monochromator phototesting after phototherapy revealed a fourfold increase in the minimal erythema dose in those with abnormal photosensitivity to ultraviolet A wavebands. Adverse effects included erythema (seven patients), pruritus (five) and provocation of the eruption (four). We now routinely consider narrow-band UVB phototherapy for problem photodermatoses.

[Hydroa vacciniforme - action spectrum. UV - tolerance following photochemotherapy]. / Hydroa vacciniforme - Aktionsspektrum. UV-Toleranz nach Photochemotherapie.

In a 14-year-old boy with hydroa vacciniforme by UV-testing was found to be the provoking wave band region. Tolerance to artificial UV-and sunlight was achieved by inducing pigmentation with oral methoxsalen photochemotherapy (PUVA).