Severe and refractory solar urticaria treated with intravenous immunoglobulins: a phase II multicenter study.

BACKGROUND: Retrospective data have suggested the effectiveness of intravenous immunoglobulins (IVIG) for solar urticaria (SU). OBJECTIVE: We sought to prospectively assess the efficacy of IVIG for SU. METHODS: We conducted a multicentric phase II study to test the efficacy of a single course of IVIG (2 g/kg) in patients with severe and refractory SU. The primary outcome was remission of SU on phototesting at 12 weeks after IVIG treatment. Secondary objectives included clinical remission, improved quality of life, and 50% improvement in disease intensity as measured on a visual analog scale. RESULTS: Of the 9 patients who received IVIG injection, 2 showed remission of SU on phototesting, corresponding to a response rate of 22.2% (95% confidence interval 2.8%-60.0%). In all, 6 patients (67%) showed at least 1 response criterion after 4 weeks and 5 (56%) after 12 weeks. Response was maintained after 24 weeks for 2 patients and after 48 weeks for 1 patient. About half of the patients (56%) had moderate to severe headache. LIMITATIONS: Lack of control arm and small number of patients are limitations. CONCLUSION: A single course of IVIG appears insufficient to obtain prolonged significant control of SU; future evaluation of different schedules of IVIG administration is warranted.

Prognostic factors of paraneoplastic pemphigus.

OBJECTIVE: To identify the prognostic factors of overall survival in a series of patients with paraneoplastic pemphigus (PNP). DESIGN: Multicenter retrospective cohort study. SETTING: Twenty-seven dermatology departments in France. PATIENTS: A total of 53 patients (31 men and 22 women; median age, 59 years; age range, 30-88 years) were diagnosed as having PNP between 1992 and 2010. MAIN OUTCOME MEASURES: Overall Kaplan-Meier survival rates were estimated, and features associated with survival were assessed using univariate (log-rank test) and multivariate (Cox regression) analyses. RESULTS: The study included 53 patients with PNP. Thirty-six patients (68%) died during the study. The 1-, 3-, and 5-year overall survival rates were 49%, 41%, and 38%, respectively. The main causes of death were infections (n=21) and evolution of neoplasia (n=6). In univariate analysis, the main detrimental prognostic factors identified were erythema multiforme­like skin lesions (P=.05) and histologic keratinocyte necrosis (P=.03). None of the 5 patients with Castleman disease died during the study. After adjustment for age and sex in multivariate analysis, erythema multiforme­like skin lesions remained predictive of fatal outcome, with a 2-fold increase in death rate (hazard ratio [HR], 2.3; 95% CI, 1.05-5.03; P=.04). The prognosis of patients with PNP was even poorer when erythema multiforme­like skin lesions were associated with severe skin or mucosal involvement at presentation (HR of death, 3.0; 95% CI, 1.01-8.92; P=.049). CONCLUSION: Patients with PNP with erythema multiforme­like skin lesions and histologic keratinocyte necrosis, especially when associated with extensive lesions at presentation, are likely to have a more severe and rapid fatal outcome and should be managed very carefully.

Efficacy of ω-3 polyunsaturated fatty acids for the treatment of refractory hydroa vacciniforme.

Hydroa vacciniforme (HV) is a rare photodermatosis. Several therapies, with sometimes severe side effects, have been used in isolated cases. We report a case of refractory HV successfully treated with dietary fish oil rich in ω-3 polyunsaturated fatty acids.

Hidradenitis suppurativa: the role of deficient cutaneous innate immunity.

OBJECTIVE: To evaluate the expression of innate immunity markers at the site of nodules caused by hidradenitis suppurativa (HS). DESIGN: Prospective analysis of 12 patients with HS. SETTING: Unité de Cancéro-Dermatologie, Nantes Hospital, Nantes; Service de Dermatologie, Poitiers Hospital, Poitiers; and Service de Dermatologie, Clinique de Courlancy, Reims, France PATIENTS: Twelve patients (Hurley stage I or II) in whom the disease had progressed for at least 6 months and who had a minimum of 2 closed nodules in typical sites. MAIN OUTCOME MEASURES: Two biopsies were performed at baseline: one in a closed inflammatory nodule and one in healthy adjoining skin. Patients were treated for 3 months with zinc gluconate at a dosage of 90 mg/d. A new biopsy was then performed in the same nodule. Innate immunity markers (toll-like receptors 2, 3, 4, 7, and 9; intercellular adhesion molecule 1; interleukin [IL] 6 and 10; tumor necrosis factor; α melanocyte stimulating hormone; transforming growth factor ß; ß-defensin 2 and 4; and insulinlike growth factor 1) were studied by immunohistochemical analysis. RESULTS: We observed significantly decreased expression (P < .001) of all the innate immunity markers studied except IL-10 in nonlesional and lesional HS skin. The downregulation of innate markers was significantly stronger in lesional HS skin compared with normal skin except for tumor necrosis factor. Three months of zinc treatment induced a significant increase in the expression of all the markers involved in innate immunity. CONCLUSION: Our study demonstrates for the first time, to our knowledge, that a deficiency of the main innate immunity markers in typical HS sites may explain the development of chronic inflammatory nodules in this disease.

Papular and annular fixed urticarial eruption: a characteristic skin manifestation in patients with relapsing polychondritis.

BACKGROUND: Skin manifestations of relapsing polychondritis (RP) are usually nonspecific. OBJECTIVE: We report a series of patients with RP who presented with annular skin lesions. METHODS: The clinical and histologic features and follow-up data of patients with RP and an annular urticarial eruption were retrospectively reviewed. RESULTS: Ten patients (9 male, 1 female) (mean age 63.7 years) were included. All patients had tense, fixed, urticarial papules with an annular configuration predominantly located on the upper part of the trunk. Skin lesions occurred before the chondritis in 7 of 10 cases with a mean delay of 23 ± 13 months. Histologic examination consistently showed a lymphocytic vasculitis with no leukocytoclastic vasculitis, even when biopsies were repeated during the evolution (n = 7). Hematologic abnormalities were found in all cases. A myelodysplastic syndrome was found in 4 patients. Oral corticosteroids were effective in all cases, although skin lesions recurred during the decrease of corticosteroid doses in 4 cases. Five patients died during the evolution. LIMITATION: Retrospective case series design is a limitation. CONCLUSION: Annular and papular fixed urticarial eruption may represent a characteristic skin manifestation of RP. It is frequently associated with hematologic abnormalities and may auger a poor prognosis.

Long-term survivors in stage IV melanoma: a regional population-based study in France.

We aimed to better characterize exceptional cases of long survivors (LSs) among patients with stage IV melanoma. We performed a comprehensive regional investigation in the Champagne-Ardenne region, France. During the period 1994-2003, 316 patients died of melanoma whereas 10 patients diagnosed with distant metastases had a subsequent survival time > 4 years. These 10 LSs were characterized by a long delay (median: 58 months) prior to distant metastases and by frequent subsequent complete remissions (CRs). Eighteen episodes of CRs, lasting 3-139 months (mean: 26.4), were documented in 9 patients, for single or multiple tumors, for metastases of any site and with any treatment, even including spontaneous CRs. Four of 8 evaluable patients had clinical and/or biological features of auto-immunity. At 31 March 2007, 3 patients had died of melanoma and 1 of a chemo-induced leukaemia. The median survival time was 65 months (range: 52-139). These data suggest that long survival in stage IV melanoma might depend less on the site of metastases and specific therapies than on the patients themselves and their spontaneous antitumor control.

Comparison of histopathologic-clinical characteristics of Jessner's lymphocytic infiltration of the skin and lupus erythematosus tumidus: Multicenter study of 46 cases.

OBJECTIVE: We sought to identify criteria able to distinguish between Jessner's lymphocytic infiltration of the skin (JLIS) and lupus erythematosus tumidus (LET). METHODS: The following characteristics were recorded in a retrospective, multicenter analysis of patients with JLIS and LET: clinical features (number, size, type, and localization of lesions; photosensitivity; extracutaneous signs), histologic findings, phototesting, lupus serology, treatment, and outcome. Available histologic slides were reviewed blinded to the initial diagnosis using a pre-established grid. RESULTS: Univariate analysis of data from patients with JLIS (15 women, 17 men; mean age: 35 years) and LET (13 women, one man; mean age: 31 years) showed the following significant (P < .05) differences: more frequent back involvement and annular lesions in JLIS, as opposed to female predominance, more frequent face involvement, and plaques in LET. Phototesting, especially ultraviolet B, induced lesions in 18 of 26 patients with JLIS and all 4 with LET. The blinded histologic review (33 samples) only found slight epidermal atrophy and focal thickened dermoepidermal junction more frequent and perivascular lymphocyte infiltrations less dense in LET. The two groups of patients reclassified according histopathologic features (18 LET and 11 JLIS) showed only slight clinical differences (more frequent nasal bridge lesions in LET and annular lesions in JLIS). LIMITATION: The retrospective nature of the study is a limitation. CONCLUSION: JLIS and LET in this population showed more similarities than differences, supporting a continuous spectrum covering these two entities.