Acral Hemorrhagic Darier Disease. / Enfermedad de Darier acral hemorrágica.

Darier disease is an autosomal-dominant inherited condition caused by mutation of a gene, which produces a protein involved in calcium channel regulation. The disease has a variety of manifestations and lacks consistent genotype-phenotype correlations. Acral hemorrhagic Darier disease causes macules, papules, vesicles and/or hemorrhagic blisters on the extremities. Other classic signs of the disease may be present in the same patient or relatives. Histopathology reveals dyskeratosis and suprabasal acantholysis with hemorrhagic lacunae. We report 3 new cases of this type of Darier disease triggered by injuries. Response to retinoid therapy was good.

Interobserver reliability of store-and-forward teledermatology in a clinical practice setting.

INTRODUCTION AND OBJECTIVES: Although many studies have evaluated the diagnostic reliability of store-and-forward (SF) teledermatology, the reliability of the technique for the diagnosis of general skin conditions in a clinical practice setting has never been demonstrated. We evaluated the reliability of SF teledermatology in clinical practice by analyzing the diagnostic agreement achieved in a subgroup of patients from the DERMATEL-2 study. MATERIAL AND METHODS: Patients referred from primary care settings were randomized to 3 groups: SF, a combination of videoconferencing and SF technology (VC-SF), and a control group. This article focuses on the SF group. Clinical data were recorded and photographs taken by primary care physicians, who forwarded the data electronically. Each SF consultation package was assessed by 3 dermatologists (D1,D2,D3). Subsequently all the patients were assessed by a single dermatologist (D1) in a face-to-face consultation. Finally, 2 other dermatologists (D4,D5) assessed the agreement between the diagnoses obtained by SF and FF. RESULTS: In total, 457 patients (200 males and 257 females) aged between 2 months and 86 years were randomized (192 to SF, 176 to VC-SF, and 89 to the control group). The diagnostic categories were as follows: tumors (49.4%), inflammatory (25.7%), adnexal (11%), infectious (9.4%) and other processes (4.4%) Since 170 patients had consultations deemed valid for analysis, the study included a total of 510 SF assessments. Most of the images and clinical records were of high quality (71.2% and 91.2% respectively), and diagnostic confidence was high in 81.4% of the cases studied. In 58.4% of cases the condition was managed exclusively by teledermatology. Levels of complete and aggregate interobserver agreement between SF and face-to-face evaluators were 0,72 and 0.90, respectively, for diagnosis and 0.61 and 0.80 for treatment. Diagnostic agreement correlated with the image quality (P < .001), diagnostic confidence (P<.001), felt need for conventional consultation (P<.001), and the quality of the clinical record (P=.013). CONCLUSION: The interobserver reliability of SF diagnosis in clinical practice is good. Dermatologists are able to predict errors in diagnosis by analyzing their own diagnostic confidence and evaluating the quality of the images.

Reconstructing Ear Defects Using the Revolving Door Island Flap: A Case Series. / Reconstrucción de defectos auriculares mediante el «colgajo en isla en puerta giratoria¼. Serie de casos.

BACKGROUND AND OBJECTIVES: Defects of the anterior region of the pinna pose a reconstruction challenge owing to the complex topography and difficult access. We report our experience using the revolving door island flap (RDIF) to reconstruct pinna defects and present the surgical results. MATERIAL AND METHODS: Retrospective descriptive study of patients undergoing ear reconstruction using RDIF at different sites of the anterior region after resection of malignant tumors. RESULTS: Seventeen patients underwent surgery between 2011 and 2019 for squamous cell carcinoma (n=7) and basal cell carcinoma (n=10). Conventional histology showed disease-free surgical margins in all the resected tissue specimens. The mean follow-up period was 40 months. One local recurrence of squamous cell carcinoma was observed, which responded well to radiation therapy; all patients were free of disease at the end of follow-up. Only 3 patients presented minor complications (depression of the flap surface in 1 case and retraction of the ear toward the mastoid process in 2 cases). Subjective rating scales were used to evaluate cosmetic and functional outcomes as assessed by both the dermatologist and the patient. The normal contour of the pinna was conserved in all patients and no patients required additional corrective surgery. CONCLUSIONS: Reconstruction of the pinna using RDIF allowed for satisfactory repair following complete resection of the tumors, while conserving structure and functionality. RDIF has been reported to be a very good option for reconstruction of the pinna. It is a simple technique that makes it possible to repair large defects with minimal risk of necrosis. In our series, the cosmetic results were excellent and recurrence rates were very low. RDIF provided optimal results in repairing lesions in the concha and in other regions, such as the antihelix, triangular fossa, and scapha.

Functional Surgery for Malignant Subungual Tumors: A Case Series and Literature Review. / Cirugía funcional en tumores malignos subungueales. Serie de casos y revisión de la literatura.

BACKGROUND AND OBJECTIVES: Amputation is the conventional treatment for malignant subungual tumors (MSUTs), namely, subungual squamous cell carcinoma (SUSCC) and subungual melanoma (SUM). Functional surgery consisting of wide local excision (WLE) of the nail unit can preserve function without modifying prognosis in such cases. We present a series of MSUTs treated with WLE of the nail unit, describe the technique, and review its indications. MATERIAL AND METHODS: Retrospective observational study of MSUTs treated with WLE of the nail unit between 2008 and 2017. The technique consisted of en bloc supraperiosteal excision of the nail unit with a margin of 5mm followed by repair with a full-thickness graft. RESULTS: Eleven MSUTs were treated in the study period: 7 SUMs (4 in situ; mean thickness, 1.17mm; range, 0-4mm) and 4 SUSCCs (mean thickness, 3.4mm; range, 1.6-6mm). WLE of the nail unit was performed in 9 patients and amputation in 2 patients with invasive SUM. Mean follow-up was 39 months (range, 12-96 months) and no local or regional recurrences were detected. One of the 2 patients who underwent amputation developed metastasis to the brain and died. In our review of the literature, we identified 5 series of patients with SUSCC treated with WLE of the nail unit (105 patients) and 14 series of patients with SUM (243 patients). Based on an analysis of these cases and ours, it would appear that WLE of the nail unit is associated with a very low rate of local recurrence (<7%) and offers better functional and cosmetic outcomes than amputation. CONCLUSIONS: WLE of the nail unit is the treatment of choice for SUSCC without bone involvement and for thin noninvasive SUM (Breslow depth <1mm). It is also feasible in intermediate-thickness SUMs when detailed histologic examination of the margins confirms complete resection. Amputation, by contrast, is the treatment of choice for SUSCCs with bone involvement, very thick SUMs (>4mm), and recurrent tumors.