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

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

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

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

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

Lichen nitidus (LN) is an uncommon chronic inflammatory skin disease composed of numerous, tiny, shiny, flesh-colored papules that are predominantly observed on the chest, abdomen, glans penis and upper extremities. The distribution of LN is most often localized, but in some cases it can become generalized. Because LN tends to be asymptomatic and presents spontaneous resolution within several years, it usually does not require treatment except in symptomatic, persistent and generalized cases. We describe a 28-yr-old man and a 7-yr-old boy with generalized LN where both cases improved with narrow-band ultraviolet B (NB-UVB) phototherapy plus topical steroid ointment. Both patients noted improvement within the first three treatments and showed almost complete resolution after 18 and 20 treatments, respectively. NB-UVB phototherapy may be an effective alternative therapy for the treatment of generalized LN, even for those patients in their childhood.
*Ultraviolet Therapy ; PUVA Therapy ; Male ; Lichen Nitidus/pathology/*radiotherapy ; Humans ; Child ; Adult

Cutaneous plasmacytosis is a rare disorder characterized by multiple red-brown plaques, mainly on the trunk, which histologically show a marked hyperplasia of mature plasma cells with no mitotic figures or cellular atypia. It is sometimes accompanied by extracutaneous symptoms like lymphadenopathy or interstitial pneumonia, but without plasma cell infiltration. Although many treatments such as corticosteroid lesional injection, cyclophosphamide, melphalan, systemic PUVA, and local tacrolimus application have been introduced for cutaneous plasmacytosis, there is no reported post-treatment data on cutaneous plasmacytosis in Korea. We report a case of a Korean male patient who was diagnosed with cutaneous plasmacytosis which was improved by PUVA therapy.
Cyclophosphamide ; Humans ; Hyperplasia ; Korea ; Lung Diseases, Interstitial ; Lymphatic Diseases ; Male ; Melphalan ; Plasma Cells ; PUVA Therapy* ; Tacrolimus