Darier disease (DD) is a rare dominantly inherited genodermatosis characterized by loss of intercellular adhesion (acantholysis) and abnormal keratinization. DD is often difficult to manage. Numerous treatments have reportedly been used for the treatment of DD, with limited success. Systemic retinoids are considered the drug of choice for treating DD. However, their use is limited by potential deleterious side effects. Considering the recently reported efficacy of doxycycline for Hailey-Hailey disease, an inherited acantholytic skin disorder pathogenetically similar to DD, we report the case of a patient with extensive DD who showed a dramatic response to oral doxycycline monotherapy.

Idiopathic facial aseptic granuloma (IFAG) is a rare, benign pediatric dermatological lesion that occurs in children between 8 months and 13 years of age. The pathogenesis of IFAG is still unclear but it is likely to be associated with granulomatous rosacea in childhood. Here we describe a case of IFAG in a 13-year-old boy who showed a dramatic response to oral doxycycline and topical metronidazole, which supports the hypothesis that IFAG may belong to the spectrum of rosacea.

Background: Cutaneous melanomas have a high tumor mutational burden (TMB) because of mutations induced by ultraviolet radiations (UVR). TMB is a predictive biomarker of response to PD-1 inhibitors but it is unfortunately not yet routinely available. Considering photoaging as a result of repeated and cumulative exposure to UVR, we hypothesized that signs of photoaging around primary melanoma could predict response to PD-1 inhibitors.Methods: We conducted a retrospective bicentric study including 34 patients with stage IV melanoma treated with first-line immunotherapy. Five independent dermatologists assessed the grade of photoaging for each patient using two clinical scales, one descriptive and the second photo-analogic (McKenzie Photographic Scale), in a blinded, photographic assessment. The reliability of the clinical scales was statistically assessed by intraclass correlation coefficients (ICC). Outcomes were progression-free survival (PFS), overall survival (OS), and objective response rate (ORR).Results: The clinical scales, graded from 0 to 3, were reproducible, with an ICC of 0.68 for the descriptive scale and 0.72 for the photo-analogic scale. PFS was significantly higher in case of severe photoaging with both the descriptive scale and the photoanalogic scale (HR¼0.32, p<10-3, and HR 0.41, <10-3, respectively). Similarly, OS was higher when photoaging was assessed severe. Three-month ORR was better in cases of severe photoaging than in cases of mild/no photoaging, both with the descriptive and photo-analogic scales (77% vs. 24% and 61% vs. 25% respectively).Conclusions: Our study suggests that reliable determination of photoaging around a melanoma or its scar can be used as a predictive clinical biomarker of response to PD-1 inhibitors, higher grades being associated with better response rate and progression-free survival.

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