Immunoperoxidase examination of cutaneous infiltrates of mycosis fungoides and large-plaque atrophic parapsoriasis with OKT10

A monoclonal antibody (OKT10), which was developed recently, reacts with pro-thymocytes, T cell acute lymphoblastic leukemia (ALL) cells, cells in normal bone marrow (including plasma cells), and activated T cells. Tissues from patients with cutaneous T cell lymphoma were studied for the presence of OKT10-reactive cells with the use of an indirect immunoperoxidase technic. OKT10-reactive cells were identified in three of eight cases of mycosis fungoides, one of two cases of Sézary syndrome, with an equivocal reaction in one of ten cases of large-plaque parapsoriasis and in one of seven positive patch tests (allergic contact dermatitis). The biologic and possible clinical implications are discussed.     

Poikilodermatous mycosis fungoides and atrophic large-plaque parapsoriasis exhibit similar abnormalities of T-cell antigen expression

We studied the immunohistologic findings of skin biopsy specimens from 21 patients with poikiloderma (14 with mycosis fungoides [MF] and seven with atrophic large-plaque parapsoriasis [ALPP]). Both types of poikiloderma were similar with regard to T-cell antigen expression. In each case, most T cells expressed the CD4+ (helper/inducer) phenotype and lacked Leu-8 antigen. T cells were also deficient in Leu-9 antigen in most cases (MF, 11/14 [79%]; ALPP, 4/7 [57%]). These T-cell antigen deficiencies are similar to those described previously in various types of MF and indicate that such deficiencies are common in minimally infiltrated, patch-stage MF lesions. Because combined Leu-8/Leu-9 antigen deficiencies are uncommon in inflammatory skin diseases, our findings are consistent with the view that ALPP is an early form of MF, as had been suggested previously by results of clinicopathologic studies.     

Twenty-nail dystrophy treated with topical PUVA

A case of twenty-nail dystrophy treated with topical PUVA is described. The patient, a 19-year-old woman, had a 4-year history of a nail dystrophy involving all finger- and toe-nails. The finger-nail changes were treated with topical PUVA, dose 0.7-1.4 J/cm2 x 3/week. After 7 months excellent improvement was seen, while the untreated toe-nails were unaffected. A maintenance dose of 0.7 J/cm2 x 3/week was necessary to prevent recurrence. We suggest that topical PUVA is worth trying for the treatment of twenty-nail dystrophy.     

Treatment of Childhood Cutaneous T-Cell Lymphoma with Alpha-Interferon Plus PUVA

Abstract: All forms of cutaneous T-cell lymphoma are rare in childhood. We describe an 8-year-old boy with plaque-stage mycosis fun-goides stage HA whose cutaneous eruption had been present for 5 years. Histologic examination revealed the presence of a granulomatous infiltrate together with atypical lymphocytes within the dermis. The child had an excellent response to combination psoralen-UVA (PUVA) with interferon-α2a treatment and is currently in remission.     

Cutaneous carcinoma and PUVA lentigines in Thai patients treated with oral PUVA

A total of 113 Thai patients who were treated with oral PUVA from 1979 to 1992 were examined for long-term cutaneous side effects of PUVA. Two psoriatic patients developed PUVA keratosis on non-sun-exposed areas. Both were skin type IV and had had phototherapy with UVB and sunlight previously. The total doses of UVA were 909 J/cm² and 242 J/cm² respectively. One psoriatic patient developed Bowen's disease. He had had a cumulative dose of UVA 2207 J/cm². He also had a past history of arsenic intake and phototherapy with UVB and sunlight. PUVA lentigines were seen in 58 patients (51.4%). It was associated with older age at starting PUVA, higher cumulative UVA dose and greater number of PUVA treatment. This study suggests that previous exposure to other risk factors is important for inducing skin cancer in populations with skin phototype III, IV and V treated with oral PUVA.     

Generalised granuloma annulare successfully treated with PUVA

Disseminated granuloma annulare is an uncommon disorder in which both topical and systemic therapy may have limited success. Anecdotal reports have suggested that PUVA may result in complete clearance of disease; however, maintenance PUVA therapy has usually been required in order to maintain remission. We report the successful treatment of a patient with 5-methoxypsoralen over a 7-month period who remained in remission during a 20-month follow up period.     

Ocular findings in patients treated with PUVA

This 5-year prospective study of ophthalmologic findings in 1299 patients treated with oral 8-methoxypsoralen photochemotherapy (PUVA) for psoriasis failed to demonstrate a significant dose-dependent increase in the risk of developing symptomatic cataracts. These patients were instructed to wear UVA-blocking eyeglasses when exposed to sunlight and during treatment for a 12-h period beginning from the time of 8-methoxypsoralen ingestion. However, we did observe a small increase in the risk for development of nuclear sclerosis and posterior subcapsular opacities among patients who received at least 100 PUVA treatments, compared to patients with fewer than 100 treatments (relative risk = 2.3 and 3.0, respectively; p less than .05 both comparisons). We compared our results to those of a large, population-based study and found, after adjusting for differences in methods, that the prevalence of cataracts in our study patients, aged 52-75 years, was not significantly different. Since the latency period for development of symptomatic ocular abnormalities may be longer than 5 years, continued surveillance of our cohort and continued use of appropriate ocular protection by all patients treated with PUVA is indicated.     

Cutaneous carcinoma in psoriatic patients treated with PUVA

Eight of 216 psoriatic patients treated at The London Hospital with photochemotherapy (PUVA) have developed a total of twenty-five skin carcinomata. The type and site of tumour are discussed with special reference to the importance of PUVA compared with other risk factors. We suggest that a history of previous skin carcinoma, arsenic therapy or radiotherapy are relative contra-indications to PUVA therapy for psoriasis.     

Juvenile mycosis fungoides treated with bexarotene and PUVA

A 14-year-old Caucasian boy presented with a 4-month history of a slightly pruritic eruption that began on the hips and later extended to the trunk and upper and lower limbs. The patient did not present fever, weight loss, or asthenia. Physical examination revealed multiple, red, desquamative, oval patches with areas of healthy skin between them, which covered nearly 50% of the body surface area. The palms, soles, face, and mucosa were not affected. In addition, he presented two violet-colored infiltrated plaques on the left thigh and right buttock (Fig. 1). There were multiple, > 1 cm, freely mobile, axillary and inguinal nodes. In follow-up, the patient developed two red-colored, mobile, well-delimited cutaneous nodules of 2.5 cm in diameter in the right hemithorax and lumbar area. The lumbar nodule regressed spontaneously before treatment. The clinical diagnosis was mycosis fungoides. We obtained three skin biopsies, one from a patch lesion and the others from a nodule; the third was sent to a reference hospital to determine the rearrangement. Histologic examination was similar in the three biopsies and revealed an atypical lymphoid infiltrate in the superficial dermis with epidermotropism and a tumoral nodule of atypical, small-sized lymphocytes in the deep dermis and subcutaneous level (Fig. 2). The atypical infiltrate was CD3+, CD4+, CD8-, T-cell intracellular antigen (TIA)+/-, Epstein-Barr-encoded RNA (EBER)-, and CD56-. The biopsy of one left axillary adenopathy was compatible with mycosis fungoides (Fig. 3). Amongst the additional tests carried out was a blood analysis showing 5300 leukocytes (neutrophils, 35%; lymphocytes, 40.7%; monocytes, 16.8%; eosinophils, 6.40%) without Sézary cells, normal lactate dehydrogenase (LDH), immunoglobulin E (IgE) of 497 U/mL (normal, 3-100 U/mL), and beta2-microglobulin of 3.09 mg/L (normal, 1.64 +/- 0.58 mg/L). A bone marrow study and a thoraco-abdomino-pelvic scan were normal. The rearrangement in the skin was monoclonal, whereas in peripheral blood and lymph nodes it was polyclonal. With the diagnosis of mycosis fungoides stage IVA (according to the TNM classification), treatment was initiated with psoralen plus ultraviolet light A (PUVA), three times a week, plus oral bexarotene at a dose of 300 mg/m2/day. The parents were informed that this treatment was not approved for this age group and informed consent was obtained. The clinical tolerance to bexarotene was very good, although low doses of atorvastatin (10 mg/day) and 75-100 mg of thyroxine were needed to control the expected adverse reactions to oral retinoid. After 32 sessions of PUVA and 6 months of treatment with oral bexarotene, the skin patches regressed, except for the plaque on the left buttock and the nodule on the right hemithorax (Fig. 4). There was no evidence of lymphadenopathy clinically or via sonographic evaluation. Bexarotene was discontinued after patient clearance and resolution of adenopathies. Nevertheless, 5 months after discontinuation of oral treatment, the patient developed multiple, scaling, nonconfluent macules on the trunk and arms affecting almost 30% of the body surface area, which disappeared with the application of methylprednisolone aceponate. He did not present significant lymphadenopathies.     

Telangiectasia macularis eruptive perstans successfully treated with PUVA therapy

We present the case of a 58-year-old woman who was diagnosed as having telangiectasia macularis eruptiva perstans (TMEP) and was successfully treated with PUVA photochemotherapy. During the 6-month follow-up, no recurrence of pruritus or skin lesions was observed. TMEP represents a rare form of cutaneous mastocytosis, which is clinically characterized by reddish-brown telangiectatic macules symmetrically distributed over the trunk and extremities. Although in the majority of cases the disease is limited to the skin, systemic involvement may occur. The treatment of TMEP is challenging and several therapeutic modalities have been proposed in the past.     

Syringotropic cutaneous T cell lymphoma treated with PUVA therapy

Syringotropic cutaneous T cell Lymphoma (SCTCL) is a rare localized variant of CTCL. It is characterized by erythematous papules that, at histological examination, show dense dermal infiltrates of atypical T cells, that are preferentially located around hyperplastic eccrine sweat glands and ducts, with absent or minimal epidermotropism. Its relationship with mycosis fungoides and other CTCLs is not clarified and is still under discussion. Several treatment approaches have been suggested, but therapeutic results are often disappointing. We report the case of a patient with typical clinical and histopathological features of SCTCL and an excellent response to PUVA therapy.     

Phototoxic reactions to photoactive drugs in patients treated with PUVA

To determine the potentially adverse effects of photoactive medications taken in conjunction with oral methoxsalen photochemotherapy (PUVA) for psoriasis, we studied the incidence of phototoxic reactions in 1,125 patients treated with PUVA at 15 centers. During the initial (clearing) phase of PUVA therapy, patients using photoactive medications were no more likely to have diffuse delayed erythema than patients who did not use such medications. After clearing, patients 45 years of age or older who used photoactive drugs were 2.3 times as likely to discontinue PUVA therapy for at least one month owing to problems related to UV-induced burns than were those who did not use such drugs. Only 6% of patients older than 45 years who took photoactive medications had problems with phototoxicity sufficiently severe to contribute to permanent discontinuation of PUVA therapy.     

Lymphoma risk in psoriasis: results of the PUVA follow-up study

OBJECTIVE: To assess the risk of lymphoma in patients with psoriasis. DESIGN: Prospective cohort study that spans 30 years and a systematic review of the literature. SETTING: Sixteen university medical centers. PATIENTS: A total of 1380 patients with psoriasis who were initially treated with psoralen-UV-A (PUVA) from 1975 through 1976 and who underwent periodic interviews and physician examinations irrespective of their use of any treatment. MAIN OUTCOME MEASURE: Incidence of lymphoma relative to that expected in the general US population (original primary end point of the study). RESULTS: The incidence of lymphoma in patients who received PUVA and were not exposed to high levels of methotrexate was comparable to that expected in the general population (incidence rate ratio, 0.85; 95% confidence interval, 0.37-1.67) but was elevated among those exposed to high levels of methotrexate (> or =36 months) (incidence rate ratio, 4.39; 95% confidence interval, 1.59-12.06). CONCLUSION: Unless exposed to high levels of methotrexate, the risk of lymphoma among members of the PUVA Follow-up Study was comparable to that observed in the general population.     

Onycholysis in a case of atopic eczema treated with PUVA photochemotherapy

Onycholysis following the ingestion of psoralens and subsequent exposure to natural sunlight has been reported on several occasions and was first reported following photochemotherapy in 1978 by Ortonne and Baran from France and in 1979 by Mackie from Scotland. Mackie commented that she hoped to stimulate further reports of onycholysis induced by PUVA photochemotherapy in order to establish whether or not it was a definite complication of such treatment. Since then, there has been a dearth of similar reports. We describe a patient with severe atopic eczema and alopecia totalis who developed onycholysis of all finger nails and a toe nail during PUVA photochemotherapy.