Porphyrias

Bickers, David R.

doi:10.1007/978-1-4899-7190-6_19

Cited by 10 publications

(29 citation statements)

References 60 publications

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“…Immediately before exposure to the PDT light source, the face was examined under Wood's light illumination (model No. 9312; Burton Medical Products, Chatsworth, Calif) to detect the characteristic coral-red fluorescence emitted by porphyrins 26 for comparison with the results obtained using the 14-to 18-hour ␦-ALA incubation. 25,28,29 Patients then received 16 minutes and 40 seconds (10 J/cm 2 ) of blue light.…”

Section: Experimental Designmentioning

confidence: 99%

“…24,25 The therapy uses a topically applied 20% solution of ␦-aminolevulinic acid (␦-ALA) that is absorbed and metabolized by epidermal cells to protoporphyrin IX (PpIX), 25 a highly photosensitizing compound in the heme biosynthesis pathway. 26 The treated area is then exposed to porphyrin-activating wavelengths of light in the Soret band (400-410 nm). 25,27 The FDA-approved protocol specifies a 14-to 18-hour delay between ␦-ALA application and light exposure, which was demonstrated in the pivotal studies to be sufficient for therapeutic photosensitization, 25 and restricts application to clinically apparent AKs.…”

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confidence: 99%

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A Trial of Short Incubation, Broad-Area Photodynamic Therapy for Facial Actinic Keratoses and Diffuse Photodamage

Touma¹,

Yaar²,

Whitehead³

et al. 2004

Arch Dermatol

229

131

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Section: Experimental Designmentioning

confidence: 99%

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confidence: 99%

A Trial of Short Incubation, Broad-Area Photodynamic Therapy for Facial Actinic Keratoses and Diffuse Photodamage

Touma¹,

Yaar²,

Whitehead³

et al. 2004

Arch Dermatol

229

131

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“…However, sclerotic skin changes with thickening of collagen bundles can occur in certain porphyrias, and an increase of collagen biosynthesis was observed following incubation of fibroblasts with uroporphyrins. 27 One theory holds that exposure to ionizing radiation induces a premature differentiation process in the fibroblast-fibrocyte cell system, resulting in an enhanced accumulation of postmitotic fibrocytes characterized by a severalfold increase in collagen synthesis. 28 Transforming growth factor ␤ is probably the major cytokine responsible for the fibrotic reaction in normal tissues following radiation therapy.…”

Section: Commentmentioning

confidence: 99%

Long-term Follow-up and Histological Changes of Superficial Nonmelanoma Skin Cancers Treated With Topical δ-Aminolevulinic Acid Photodynamic Therapy

Fink‐Puches¹,

Soyer²,

Hofer³

et al. 1998

Arch Dermatol

145

101

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To investigate the immediate and longterm effects of photodynamic therapy with ␦-aminolevulinic acid (ALA-PDT) on superficial basal cell carcinomas (BCC) and superficial squamous cell carcinomas (SCC).Design: Retrospective study with 60 months of maximal follow-up.Setting: University-based hospital in Graz, Austria.Patients: Forty-seven subjects with a total of 95 superficial BCC and 35 superficial SCC.Interventions: A compound of 20% ␦-aminolevulinic acid was topically applied under an occlusive and lightshielding dressing before exposure to either UV-A or different wave bands of polychromatic visible light (fullspectrum visible light, Ͼ515, Ͼ570, or Ͼ610 nm).Main Outcome Measures: Primary tumor responses and recurrence rates in the long-term follow-up, as well as histological changes associated with ALA-PDT, were studied.Results: The complete primary response rate for all wave bands of light was 86% (82/95) for superficial BCC and 54% (19/35) for superficial SCC. There was no statisti-cally significant difference among the response rates to the different wave bands of light. After a median follow-up of 19 months (range, 3-60 months) for BCC and 8 months (range, 3-47 months) for SCC, the overall recurrence rate was 44% (36/81) and 69% (11/16), respectively. At 36 months after therapy, the projected disease-free rate was 50% (95% confidence interval, 43%-57%) for BCC vs 8% (95% confidence interval, 7%-9%) for SCC (PϽ.001, log-rank test). Histopathologic studies revealed a significant increase of fibrosis in the dermis after ALA-PDT and appearance of a sharp border between fibrotic and nonfibrotic tissue. In 15 of 16 BCC examined, the border between fibrotic and nonfibrotic tissue was deeper in the dermis than the maximum tumor thickness before therapy (PϽ.001, Wilcoxon signed rank test). Similar histopathologic observations were made in SCC. Conclusions:Our study revealed poor long-term cure rates for superficial BCC and SCC treated with topical ALA-PDT and visible light. The histopathologic observations showing remarkable fibrosis in the dermis indicated that the effect of ALA-PDT reached deeper than the initial depth of invasiveness of the neoplastic tissue, suggesting in turn that the poor long-term results of ALA-PDT cannot be explained by insufficient penetration of the therapy effect.

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“…7 The pattern of excretion of urinary porphyrins is diagnostic for PCT. 6 Anuric patients or patients who did not present the excretion pattern of porphyrins (ratio URO/COPRO greater than 3:1) were excluded from the study. Disappearance of blisters and improvement of skin fragility were the criteria used for clinical remission, and reduction of total porphyrins in the 24-hour urine sample to normal parameters (females <159 μg and males <199 μg) was considered biochemical remission.…”

Section: Patientsmentioning

confidence: 99%

Estudo da imunofluorescência direta, imunomapeamento e microscopia ótica na porfiria cutânea tardia

Vieira¹,

Aoki

Oliveira

et al. 2010

An. Bras. Dermatol.

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L Study of direct immunofluorescence, immunofluorescence mapping and light microscopy in porphyria cutanea tardaEstudo da imunofluorescência direta, imunomapeamento e microscopia ótica na porfiria cutânea tardia Abstract: BACKGROUND: Even though porphyria cutanea tarda is the most frequent type of porphyria, there are few studies about its cutaneous physiopathology. OBJECTIVE: To evaluate skin changes in porphyria cutanea tarda using light microscopy and direct immunofluorescence before and after treatment with chloroquine. To perform antigen immunomapping of bullae to study their level of cleavage. METHODS: Light microscopy and direct immunofluorescence of 28 patients are reported in three different phases: 23 patients with active porphyria before treatment (Phase A), 7 patients with clinical remission during treatment (Phase B), and 8 patients with biochemical remission (Phase C). Immunomapping was performed on 7 patients. RESULTS: In active porphyria, direct immunofluorescence showed homogenous and intense fluorescence on the inside and on the walls of blood vessels as well as in the dermal-epidermal junction. In clinical remission (Phase B) and biochemical remission (Phase C), the deposit of immunoglobulins was maintained, but the deposit of complement was reduced in most cases. Immunomapping revealed no standard cleavage plane. CONCLUSION: No correlation was observed between clinical response and immunoglobulin deposits. The reduction of complement favors the hypothesis that activation of the complement cascade represents an additional pathway that leads to endothelial damage. Keywords: Fluorescent antibody technique; Microscopy; Porphyria cutanea tarda; Porphyria cutanea tarda/physiopathology Resumo: FUNDAMENTO: Apesar de a porfiria cutânea tardia ser a mais frequente das porfirias, há poucos estudos que abordam sua fisiopatologia cutânea. OBJETIVO: Avaliar as alterações cutâneas na porfiria cutânea tardia utilizando a microscopia ótica e a imunofluorescência direta, antes e depois do tratamento com cloroquina. Realizar o imunomapeamento antigênico da bolha para estudo do seu nível de clivagem. MÉTODOS: Relata-se a microscopia ótica e imunofluorescência direta de 28 pacientes em três fases diferentes: 23 pacientes com porfiria ativa antes do tratamento (Fase A), sete pacientes com remissão clínica durante o tratamento (Fase B) e oito pacientes com remissão bioquímica (Fase C). O imunomapeamento foi realizado em sete pacientes. RESULTADOS: Na porfiria ativa, a imunofluorescência direta demonstrou fluorescência homogênea e intensa no interior e na parede dos vasos e na junção dermoepidérmica. Na remissão clínica (Fase B) e na remissão bioquímica (Fase C), o depósito de imunoglobulinas se manteve, mas o depósito de complemento apresentou diminuição na maioria. O imunomapeamento não demonstrou plano de clivagem fixo. CONCLUSÃO: Não houve correlação entre a resposta clínica e os depósitos de imunoglobulinas. A diminuição do complemento favorece a hipótese de que a ativação da cascata do complemento representa u...

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Porphyrias

Cited by 10 publications

References 60 publications

A Trial of Short Incubation, Broad-Area Photodynamic Therapy for Facial Actinic Keratoses and Diffuse Photodamage

A Trial of Short Incubation, Broad-Area Photodynamic Therapy for Facial Actinic Keratoses and Diffuse Photodamage

Long-term Follow-up and Histological Changes of Superficial Nonmelanoma Skin Cancers Treated With Topical δ-Aminolevulinic Acid Photodynamic Therapy

Estudo da imunofluorescência direta, imunomapeamento e microscopia ótica na porfiria cutânea tardia

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