Sporadic Porphyria in a patient with stage II melanoma treated with interferon α.

Porphyria cutanea tarda (PCT) is the most common form of human porphyria, due to reduced activity of uroporphyrinogen decarboxylase (UROD). There are many factors which can trigger PCT such as viral infections, excessive alcohol intake, iron overload, hepatotoxic drugs and hepatic tumours. Drug induced PCT is well documented but PCT induced by interferon α has rarely been described and only in cases of Hepatitis C Virus (HCV) infection or haematological malignancies. Here, we report the first case of de novo PCT induced by adjuvant interferon α (IFNα) therapy in a patient with stage II melanoma.

Solar urticaria: a time-extended retrospective series of 61 patients and review of literature.

BACKGROUND: Solar urticaria is a rare, disabling, chronic disease. Few large series are available. OBJECTIVE: To report the epidemiological, clinical, phototesting, treatment and patient outcome data of a large series. METHODS: Data from 61 patients' files were retrospectively retrieved in a tertiary referral centre. RESULTS: 43 women and 18 men were included (mean age at first symptoms: 34 y). 3 patients had a medical history of chronic urticaria and 29% of atopy. Urticaria occurred before the 15(th) minute of sun-exposure in 95% of the patients and resolved spontaneously after its interruption within 1h in 76.4%. Determination of the action spectra revealed UVA sensitization in 91.8% of the patients, alone (49.2%) or with UVB (24.6%) or visible light (14.75%). 61.7% of the patients received antihistamines, 75% noted a significant improvement. 36.2% benefited from UVA phototherapy and were satisfied. 3 patients reported complete remission after 4 to 11 years. CONCLUSIONS: Our study is the second largest in the literature. Main discrepancies with previous series concern the absence of associated photodermatoses and the predominance of UVA sensitization over visible light, which could be explained by suboptimal phototesting in the most ancient cases. Interpretation of our results is restricted because data were missing in 10 to 25% of the patients' files. The response to treatment was evaluated upon the dermatologist's observation, which highlights the need of validated tools to evaluate patients' disability and response to treatment.

Treatment of early stages of mycosis fungoides with narrowband ultraviolet B. A clinical, histological and molecular evaluation of results.

BACKGROUND: Narrowband ultraviolet B (UVB) phototherapy is increasingly used in mycosis fungoides (MF). OBJECTIVE: We report on the results obtained in a prospective series of early MF patients receiving this therapeutic regimen. METHODS: In total, 22 patients were treated. Therapeutic results were evaluated on clinical, histological and molecular levels. Patients were then submitted to a clinical follow-up. RESULTS: The cumulative number of treatments ranged from 22 to 48 (mean: 29). A complete clinical remission (CCR) was obtained in 18/22 patients, and a partial clinical remission in 4 cases. Complete or partial histological responses were achieved in 9/15 (all in CCR) and 4/15 patients, respectively. The molecular response was evaluated in 12 patients, and a disappearance of the dominant T cell clone in the skin was obtained in only 3 cases. After 4-48 months of follow-up (mean 20.1 months), 7/18 patients in CCR (39%) relapsed. CONCLUSION: Narrowband UVB phototherapy is a well-tolerated treatment of early-stage MF, and its efficiency is maximal in very early stages (Ia). Even though clinical results seem very similar to PUVA through indirect and tentative comparisons with historical series, relapses tend to occur earlier than with PUVA, especially when an incomplete histological or molecular response was achieved.

Ichthyosiform mycosis fungoides.

Malignancy-associated acquired ichthyosis is well known, but the ichthyosiform subset of mycosis fungoides (MF) is rarely reported. We report on two patients with a clinical presentation for whom diagnosis of mycosis fungoides was established on histological grounds. In both cases, long term remission was obtained with non aggressive therapies. This rare condition must be added to newly described forms of MF with epidermal hyperplasia such as keratosis lichenoides chronica like MF and pilotropic MF.