Is photodynamic therapy a relevant therapeutic option in refractory benign familial pemphigus (Hailey-Hailey disease)? A series of eight patients.

INTRODUCTION/BACKGROUND: Treatment of benign familial pemphigus or Hailey-Hailey disease (HHD), a rare inherited condition associated with a significant impairment of quality of life, is often challenging and disappointing with frequent relapses and infectious complications. Topical photodynamic therapy (PDT) may offer new perspectives in this difficult setting. MATERIAL AND METHODS: Eight patients with long-lasting HHD lesions refractory to multiple treatments were treated on at least one involved site with PDT using methyl-amino levulinate with a standardized protocol of three sessions of irradiation separated by 3-week intervals. RESULTS: A complete or partial clearing was achieved in all treated areas, and the result was satisfactorily maintained in all cases after a follow-up period ranging from 3 to 36 months. Results were of higher quality in non-inguinal areas. Tolerance was overall acceptable with local pain during and shortly after irradiation being the main limiting factor. DISCUSSION/CONCLUSION: Our series, although limited in size, emphasizes the interest of PDT in this difficult condition even though results may be incomplete. Treatment-related pain can be adequately managed by prior analgesics, cooling with sprayed water and local tumescent anesthesia. Overall, PDT appears as a relevant option in refractory HHD management with a favorable benefit/risk ratio.

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.