Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Finally, she reached complete remission of mycosis fungoides. However, new yellowish lesions started to appear 1 year after discontinuing the phototherapy. A physical examination revealed multiple yellowish plaques on both extremities. The plaques were well circumscribed and slightly elevated. All laboratory tests were normal. A biopsy specimen showed multiple nodular deposits of eosinophilic amorphous material in papillary dermis and upper reticular dermis. The deposits represented apple green birefringence on Congo red stain viewed under polarized light. Acellular small nodules in the upper dermis consisted of randomly oriented, non-branching, 6.67~12.7 nm thick amyloid fibrils on electron microscopy. We report an interesting and rare case of secondary cutaneous amyloidosis after narrow-band UVB therapy and PUVA therapy in a patient with mycosis fungoides.
Acitretin ; Aged ; Amyloid ; Amyloidosis* ; Biopsy ; Birefringence ; Bowen's Disease ; Carcinoma, Basal Cell ; Congo Red ; Dermis ; Eosinophils ; Extremities ; Female ; Ficusin ; Humans ; Leg ; Microscopy, Electron ; Mycosis Fungoides* ; Phototherapy ; Physical Examination ; Plaque, Amyloid ; Porokeratosis ; PUVA Therapy ; Skin ; Ultraviolet Therapy

Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Finally, she reached complete remission of mycosis fungoides. However, new yellowish lesions started to appear 1 year after discontinuing the phototherapy. A physical examination revealed multiple yellowish plaques on both extremities. The plaques were well circumscribed and slightly elevated. All laboratory tests were normal. A biopsy specimen showed multiple nodular deposits of eosinophilic amorphous material in papillary dermis and upper reticular dermis. The deposits represented apple green birefringence on Congo red stain viewed under polarized light. Acellular small nodules in the upper dermis consisted of randomly oriented, non-branching, 6.67~12.7 nm thick amyloid fibrils on electron microscopy. We report an interesting and rare case of secondary cutaneous amyloidosis after narrow-band UVB therapy and PUVA therapy in a patient with mycosis fungoides.
Acitretin ; Aged ; Amyloid ; Amyloidosis* ; Biopsy ; Birefringence ; Bowen's Disease ; Carcinoma, Basal Cell ; Congo Red ; Dermis ; Eosinophils ; Extremities ; Female ; Ficusin ; Humans ; Leg ; Microscopy, Electron ; Mycosis Fungoides* ; Phototherapy ; Physical Examination ; Plaque, Amyloid ; Porokeratosis ; PUVA Therapy ; Skin ; Ultraviolet Therapy

Chronic graft-versus-host disease (cGVHD) is a common complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT), which is also one of the major causes of patients' death following transplantation. Recently, extracorporeal photochemotherapy (ECP) has shown a considerable efficacy in cGVHD treatment, which is based on the infusion of autologous peripheral blood mononuclear cells collected by aphesis, incubated with the photoactivable drug 8-methoxypsoralen (8-MOP) and UV-A irradiation. The therapeutic effect of ECP is mainly achieved by the induction of cell apoptosis, influencing the function of dendritic cells and the induction of immune tolerance. ECP has many advantages in the treatment of cGVHD, such as no increasing the risk of infection in patients, unaffecting the graft-versus-leukemia effect, nearly no side effect and so on. Many medical centers have done a lot of research on the treatment of cGVHD in both children and adults by using ECP and achieved good results. CD19(+) CD21(-) B lymphocytes, serum BAFF and serum TNFα can be used to measure and early evaluate the efficacy of ECP treatment. The effect of ECP is associated with many factors, and certain complications may occur during the treatment. At present, the application of ECP treatment is limited by the unclear mechanisms, varying treatment cycles in different studies, and small number of patients in clinical research. In the near future, with deeper basic research, increasing the case number and standard clinical treatment, ECP will have a more extensive application prospects. This review focuses mainly on the clinical advances of ECP in the treatment of cGVHD.
Apoptosis ; Graft vs Host Disease ; Humans ; Leukocytes, Mononuclear ; Photochemotherapy ; Photopheresis ; Ultraviolet Rays

OBJECTIVETo evaluate the efficacy and safety of topical PUVA treatment of refractory lesions of mycosis fungoides.

METHODSFrom January 2008 to 2014, a total of 10 patients (4 males and 6 females) with mycosis fungoides were treated with topical PUVA in Peking Union Medical College Hospital, including 7 cases in plaque stage and 3 cases in tumor stage. The average number of lesions were 1.9±0.9. The median age of these patients was (46.0±9.4) years. The average course of disease was (12.4±7.7) years. Psoralen was applied topically on treatment area 30 min before total body UVA irradiation treatment, 3 times a week. And the efficiency and safety of the therapy were evaluated.

RESULTSAll the patients were treated with topical PUVA with a median total dose of (161.60±135.96) J/cm2 in an average of (18.10±14.61) fractions. Total dose of UVA was (1 953.25±829.73) J/cm2, and total number of treatment was (261.90±116.79) fractions. The total treatment time was (45.80±26.64) months. Complete clinical response (CR) rate was 60.0%, partial response (PR) rate was 30.0%, and the overall response rate (CR+PR) was 90.0%. One patient showed no response. No severe acute or chronic side effects were observed.

CONCLUSIONTopical PUVA therapy is effective in the treatment of refractory lesions of mycosis fungoides with little severe side effects.

Adult ; Female ; Ficusin ; therapeutic use ; Humans ; Male ; Middle Aged ; Mycosis Fungoides ; drug therapy ; pathology ; PUVA Therapy ; Photosensitizing Agents ; therapeutic use ; Treatment Outcome

Pigmented purpuric dermatosis (PPD) represents a group of cutaneous lesions exhibiting petechiae, pigmentation, and occasionally telangiectasia in the absence of an associated venous insufficiency or hematological disorder. PPD may resolve spontaneously but tends to persist for months to years. Various treatment modalities such as oral griseofulvin, pentoxifylline, cyclosporine, ascorbic acid, topical corticosteroids, and PUVA therapy have been used with unsatisfactory results. Recently, some studies reported that PPD showed a dramatic response to narrowband ultraviolet B (UVB) phototherapy. In these studies, narrowband UVB phototherapy was an effective treatment method with few side effects. Here, we present the case of a 7-year-old boy with generalized PPD that improved rapidly following narrowband UVB phototherapy.
Adrenal Cortex Hormones ; Ascorbic Acid ; Child* ; Cyclosporine ; Griseofulvin ; Humans ; Male ; Pentoxifylline ; Phototherapy* ; Pigmentation ; Purpura ; PUVA Therapy ; Skin Diseases* ; Telangiectasis ; Venous Insufficiency

OBJECTIVEOnly a few clinical reports in the treatment of early mycosis fungoides (MF)(IA, IB, IIA stage) are available in the literature. The purpose of this study was to compare the efficacy and safety of narrow-band UVB and psoralen plus ultraviolet A (PUVA) photochemoterapy in 24 patients with early-stage MF, and explore a new approach for the treatment of early mycosis fungoides.

METHODSA total of 24 identified early mycosis fungoides patients received PUVA, NB-UVB and a combined therapy of PUVA followed by NB-UVB (n = 9/6/9) irradiation. A retrospective study was carried out to analyze the sex, age of onset, TNM stage, treatment, and duration of treatment, and times of treatment, duration of maintenance treatment, effective and recurrence in these patients. The data were analyzed using SPSS 17.0 and a two-sided test at the α = 0.05 level of significance was conducted.

RESULTSOf the 24 patients studied, the average treatment was 104.5 (95% CI, 75.71-133.29) times. The average duration of treatment was 12.88 (95% CI, 9.90-15.85) months. The average maintenance treatment time was 11.08 (95% CI, 2.13-20.04) months. The effective rate (CR+PR) of PUVA treatment was 88.9%, recurrence rate was 11.1% (n = 9). In the NB-UVB treatment group, the effective rate was 100.0%, and the recurrence rate was 33.3% (n = 6). In the PUVA followed by NB-UVB (combination therapy) treatment group, the effective rate was 77.8% and the recurrence rate was 55.6% (n = 9). There were no significant differences among the three groups in terms of number of treatments, treatment duration, maintenance treatment duration, effective rate and recurrence rate (P > 0.05).

CONCLUSIONSPUVA and NB-UVB are effective and safe in the targeted therapy of early stage mycosis fungoides. The combined therapy of PUVA followed by NB-UVB can reduce the total PUVA dose and risk of developing skin cancer.

Combined Modality Therapy ; methods ; Ficusin ; Humans ; Mycosis Fungoides ; therapy ; Neoplasm Recurrence, Local ; PUVA Therapy ; Photochemotherapy ; Physical Examination ; Retrospective Studies ; Treatment Outcome ; Ultraviolet Therapy

OBJECTIVETo determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides (MF).

METHODSFrom January 2008 to January 2013, a total of 11 patients with tumor-stage MF were treated with local radiation therapy in Peking Union Medical College Hospital. The median age of these patients was 53.36 ± 14.45 years. Female-male ratio was 1:1.2. The average course of disease was 10.82 ± 3.37 years. All the patients were treated with local electronic beam irradiation with a total median dosage of 48.55 ± 9.51 (40-74) Gy in an average of 24.55 ± 5.57 (20-40) fractions, 5 fractions per week.

RESULTSThe median follow-up time was 55.27 ± 29.3 (13-103) months. No severe acute or chronic side effects of irradiation were observed. Complete clinical response (CR) rate of the radiated sites was 54.5% (6/11), partial response (PR) rate was 36.4% (4/11), and the overall response rate (CR+PR) was 90.9%. One patient showed no response.

CONCLUSIONLocal radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.

Adult ; Aged ; Antineoplastic Agents ; administration & dosage ; therapeutic use ; Chemoradiotherapy, Adjuvant ; methods ; Disease-Free Survival ; Female ; Humans ; Interferons ; administration & dosage ; therapeutic use ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; pathology ; radiotherapy ; Male ; Middle Aged ; Mycosis Fungoides ; drug therapy ; pathology ; radiotherapy ; Neoplasm Staging ; PUVA Therapy ; methods ; Palliative Care ; methods ; Radiotherapy Dosage ; Skin Neoplasms ; drug therapy ; pathology ; radiotherapy ; Treatment Outcome

BACKGROUND: The safety of extracorporeal photopheresis (ECP) in steroid-refractory chronic graft-versus-host disease (SR-cGVHD) has been explored in multiple studies but reported response rates (RR) vary significantly across studies. METHODS: We conducted a meta-analysis to assess the efficacy of ECP for SR-cGVHD. A search of electronic databases for studies published between 1984 and 2012 was conducted. End points included RR: complete response (CR), overall response rates (ORR), and organ-specific RR. The initial search generated 312 studies, of which 18 met the selection criteria (N=595). A random effects model was used for pooled rates. RESULTS: Pooled CR rates and ORR were 29% (confidence interval [CI], 19-42%) and 64% (CI, 65-82%), respectively. One-year overall survival was available for 4 studies only and was 49% (CI, 29-70%). The pooled RR for skin, liver, ocular, oral, lung, gastrointestinal and musculoskeletal SR-cGVHD was 74%, 68%, 60%, 72%, 48%, 53%, and 64%, respectively. There was a significant heterogeneity among studies due to differences in ECP schedules and duration. No significant differences in responses to ECP for pediatric and adult populations were found. Sensitivity analysis could not be undertaken due to a limited number of prospective studies. CONCLUSION: ECP is an effective therapy for oral, skin, and liver SR-cGVHD, with modest activity in lung and gastrointestinal SR-cGVHD.
Adult ; Appointments and Schedules ; Graft vs Host Disease* ; Humans ; Liver ; Lung ; Patient Selection ; Photopheresis* ; Population Characteristics ; Skin

BACKGROUND: Alopecia areata (AA) is believed to be an organ-specific autoimmune disease in which a mononuclear cell infiltrate develops in and around anagen hair follicles. There is no definitive therapy for AA. OBJECTIVE: We sought to determine whether the combination therapy of cyclosporine and psoralen plus ultraviolet A (PUVA) could be an effective treatment for severe AA. METHODS: A total of 41 patients with severe AA were treated with oral cyclosporine and topical PUVA. Cyclosporine was given at an initial daily dose of 200 mg for adult and 100 mg for children for periods of up to 16 weeks. Eight-methoxypsoralen (Methoxsalen) was applied topically 20 minutes prior to ultraviolet A (UVA) exposure, and the patients were irradiated with UVA twice a week for 16 weeks. RESULTS: Of the total 41 patients, 2 (7.3%) patients were lost to follow-up, and 1 (2.4%) patient discontinued the treatment due to abdominal discomfort. Six (14.6%) patients were treated for less than 12 weeks. Of remaining 32 patients, 3 (9.4%) showed excellent response, 3 (9.4%) showed good response, 12 (37.5%) showed fair response, and 14 (43.7%) showed poor response. CONCLUSION: Although limited by its uncontrolled character, this study shows that the combination therapy with cyclosporine and PUVA may be an additional choice for severe and recalcitrant AA.
Adult ; Alopecia ; Alopecia Areata ; Autoimmune Diseases ; Child ; Cyclosporine ; Ficusin ; Hair Follicle ; Humans ; Lost to Follow-Up ; PUVA Therapy ; Retrospective Studies

Mycosis fungoides palmaris et plantaris (MFPP) is a rare form of mycosis fungoides that is confined to the palms and soles. The clinical manifestation of MFPP is often confused with inflammatory palmoplantar dermatoses. Mycosis fungoides is usually considered as a disease of middle age, but it is rarely developed at any age. A 10-year-old girl was referred to us with a 2-year history of recalcitrant palmoplantar dermatoses. Other clinics had treated her for more than 2 years, but all medical treatments turned out to have had no effect, despite her young age. She had not had any atopic dermatitis or allergic contact dermatitis. Histopathologic findings showed inflammatory cell infiltration and lymphocytic epidermotrophism. Monoclonal TCR-rearrangement was positive, so we diagnosed her as having MFPP. We tried to treat her with topical PUVA therapy and she improved within 3 months. Herein, we report on a case of mycosis fungoides Palmaris et plantaris in a 10-year-old girl that was treated successfully with topical PUVA.
Child ; Dermatitis, Allergic Contact ; Dermatitis, Atopic ; Humans ; Middle Aged ; Mycosis Fungoides ; Organophosphorus Compounds ; PUVA Therapy ; Skin Diseases