Clinical presentation, sonographic features and treatment options of segmental stiff skin syndrome.

Segmental stiff skin syndrome is a rare genetic connective tissue disease, which is often misdiagnosed. High-frequency ultrasonography can represent a useful clinical adjunct in the differential diagnosis of this condition, in conjunction with the clinical and histopathological findings. Treatment options are limited and evidence is scarce. We present the clinical, sonographic and histological features of five paediatric patients diagnosed at our institution and discuss their response to treatment.

Classification of the cutaneous manifestations of COVID-19: a rapid prospective nationwide consensus study in Spain with 375 cases.

BACKGROUND: The cutaneous manifestations of COVID-19 disease are poorly characterized. OBJECTIVES: To describe the cutaneous manifestations of COVID-19 disease and to relate them to other clinical findings. METHODS: We carried out a nationwide case collection survey of images and clinical data. Using a consensus we described five clinical patterns. We later described the association of these patterns with patient demographics, the timing in relation to symptoms of the disease, the severity and the prognosis. RESULTS: The lesions may be classified as acral areas of erythema with vesicles or pustules (pseudo-chilblain) (19%), other vesicular eruptions (9%), urticarial lesions (19%), maculopapular eruptions (47%) and livedo or necrosis (6%). Vesicular eruptions appear early in the course of the disease (15% before other symptoms). The pseudo-chilblain pattern frequently appears late in the evolution of the COVID-19 disease (59% after other symptoms), while the rest tend to appear with other symptoms of COVID-19. The severity of COVID-19 shows a gradient from less severe disease in acral lesions to more severe in the latter groups. The results are similar for confirmed and suspected cases, in terms of both clinical and epidemiological findings. Alternative diagnoses are discussed but seem unlikely for the most specific patterns (pseudo-chilblain and vesicular). CONCLUSIONS: We provide a description of the cutaneous manifestations associated with COVID-19 infection. These may help clinicians approach patients with the disease and recognize cases presenting with few symptoms. What is already known about this topic? Previous descriptions of cutaneous manifestations of COVID-19 were case reports and mostly lacked illustrations. What does this study add? We describe a large, representative sample of patients with unexplained skin manifestations and a diagnosis of COVID-19, using a consensus method to define morphological patterns associated with COVID-19. We describe five clinical patterns associated with different patient demographics, timing and prognosis, and provide illustrations of these patterns to allow for easy recognition.

Efficacy and safety of wet wrap therapy for patients with atopic dermatitis: a systematic review and meta-analysis.

Wet wrap therapy (WWT) consists of topical steroids administered under a layer of wet cotton bandages or garments. Several trials with WWT have reported promising results in atopic dermatitis (AD). However, no systematic review and meta-analysis on its efficacy and safety has been published. Our objective was to conduct a systematic review of the literature on WWT in AD to assess its efficacy and safety. We included randomized controlled trials among patients of all ages with a diagnosis of AD based on predefined criteria or made by a dermatologist. Electronic searches were performed from 1970 to 30 March 2016 in the Cochrane Central Register of Controlled Trials, MEDLINE, Embase and the World Health Organization International Clinical Trials Registry. Selection of studies and data extraction were performed independently by two researchers, and discrepancies were resolved by consensus. Six trials comparing WWT with topical steroids in children or adults with AD were included. Sample sizes ranged from 19 to 51 patients. Results on clinical severity and quality of life were reported incompletely and proved heterogeneous across studies. A nonsignificant tendency to increased risk of mild skin infections was observed in those treated with WWT (pooled relative risk 6·35, 95% confidence interval 0·83-48·55). The overall grade of quality of evidence for the efficacy and safety outcomes was low. In conclusion, the evidence that WWT is more effective than conventional treatment with topical steroids in AD is of low quality. Further clinical trials should establish the efficacy of WWT in AD.

Cutaneous adverse events associated with heparin.

Heparin use is associated with various cutaneous reactions, with the most common being immune-mediated skin lesions and bleeding complications. In this review, we compile the dermatological side-effects of heparin reported in the literature, and provide a clear approach to their adequate management.

Nodular lesions arising in a large congenital melanocytic naevus in a newborn with eruptive disseminated Spitz naevi.

Congenital malignant melanoma within a pre-existing large congenital melanocytic naevus (CMN) is exceedingly rare. Its incidence is difficult to determine due to the small number of reported cases and because of problems associated with diagnosis. Some benign nodular proliferations (called proliferative nodules) arising in CMN, while rare, are significantly more common and can mimic malignant melanoma clinically or histologically. There are no reported cases of congenital melanoma or benign proliferative nodules in CMN in patients who also had eruptive disseminated Spitz naevi. We describe a girl who was noted to have a dark-brown plaque with several large erythematous nodules affecting the scalp at delivery, in addition to multiple erythematous dome-shaped papules that developed in a disseminated manner over several months, beginning at 10 days of age. It was difficult, not only clinically but also histologically, to determine the benign or malignant nature of all of these lesions. As primary cutaneous melanoma, atypical proliferative nodules in CMN, bland CMN or CMN with foci of increased cellularity and Spitz naevi show clear differences in the genetic aberration patterns, comparative genomic hybridization (CGH) could be a diagnostic help in ambiguous cases such as this. CGH performed on this patient showed multiple DNA copy number changes in the most atypical nodule, but such alterations could not be found in the remainder of the lesions. CGH showed differences between the nodular lesions that occurred in the CMN and helped us in supporting the diagnosis of this unique case of benign proliferative nodules and a possible congenital melanoma arising in a large CMN, associated with multiple widespread eruptive Spitz naevi.

[Septic emboli to the skin following angioplasty]. / Embolismos sépticos cutáneos tras angioplastia.

Invasive vascular procedures have good efficacy and safety profiles and are now widely used for the diagnosis and treatment of many cardiovascular disorders. However, they do have potential complications that can occasionally be life-threatening. We present a new case of infectious pseudoaneurysm following percutaneous transluminal coronary angioplasty and complicated by septic emboli to the skin. It is a rare condition characterized by persistent bacteremia, sepsis of unknown origin, and regional septic emboli. Histopathology of the skin lesions typically reveals gram-positive coccobacilli and septic vasculitis. The condition carries a significant morbidity and mortality, making early diagnosis essential. Both cholesterol and septic emboli should be considered in the differential diagnosis of skin lesions after invasive vascular procedures.

Are Oral Mucosal Changes a Sign of COVID-19? A Cross-Sectional Study at a Field Hospital. / ¿Son las alteraciones en la mucosa oral un signo de COVID-19? Estudio transversal en un Hospital de Campaña.

BACKGROUND: Coronavirus disease 19 (COVID-19) has many manifestations, including respiratory, thrombotic, neurologic, digestive, and cutaneous ones. Cutaneous manifestations have been classified into 5 clinical patterns: acro-ischemic (pseudo-chilblain), vesicular, urticarial, maculopapular, and livedoid. Oral manifestations have also been reported, but much less frequently. PATIENTS AND METHODS: We performed a cross-sectional study in which we examined the oral mucosa of 666 patients with COVID-19 at the IFEMA field hospital in Madrid in April 2020. RESULTS: Seventy-eight patients (11.7%) had changes involving the oral mucosa. The most common were transient anterior U-shaped lingual papillitis (11.5%) accompanied or not by tongue swelling (6.6%), aphthous stomatitis (6.9%), a burning sensation in the mouth (5.3%), mucositis (3.9%), glossitis with patchy depapillation (3.9%), white tongue (1.6%), and enanthema (0.5%). Most of the patients also reported taste disturbances. CONCLUSION: COVID-19 also manifests in the oral cavity. The most common manifestations are transient U-shaped lingual papillitis, glossitis with patchy depapillation, and burning mouth syndrome. Mucositis with or without aphthous ulcers or enanthema may also be observed. Any these findings may be key clues to a diagnosis of COVID-19.

Baseline description of the Spanish Academy of Dermatology infantile haemangioma nationwide prospective cohort. Comparison of patients treated with propranolol in routine clinical practice with previous pivotal clinical trial data. / Descripción basal de la cohorte prospectiva de hemangioma infantil a nivel nacional de la Asociación Española de Dermatología. Comparación de los pacientes tratados con propranolol en la práctica clínica rutinaria y los datos de un ensayo clínico pivotal previo.

BACKGROUND: There are several therapeutic options for infantile haemangiomas (IH). Propranolol is used according to a pivotal trial. We aimed to describe the characteristics of IH in clinical practice, including the therapies used, and to compare the characteristics of patients treated with propranolol with those of the trial to assess its external validity. METHODS: Consecutive patients attending 12 Spanish hospitals from June 2016 to October 2019 were included (n=601). RESULTS: The mean age was 3.9 (SD:1.9) months, with a 2:1 female-to-male ratio. Most IHs were localized (82%, 495), superficial (64%, 383) and located in the face (25%, 157) and trunk (31%, 188). Median size was 17 (IR: 10-30) x 12 (IR: 7-20) mm. Complications were found in 16 (3%) patients. Treatment was initiated for 52% (311). Most patients received timolol (76%, 237); propranolol was reserved for complications or high-risk IHs. Aesthetic impairment was the main reason for starting therapy (64%, 199). Several characteristics of the patients and IHs treated with propranolol are similar to those of the pivotal clinical trial, but 1/3 of IHs did not reach the minimum diameter to meet the inclusion criteria, and important prognostic information was not reported. CONCLUSIONS: As most patients receive treatment for aesthetic impairment, there is a need to better understand the aesthetic results of therapies and to increase evidence on the use of timolol, which is currently the most common therapy. Propranolol is being used in a population generally similar to that of the trial; however, this statement cannot be definitely confirmed.

Idiopathic Facial Aseptic Granuloma: Clinical and Ultrasound Findings in 3 Cases. / Granuloma aséptico facial idiopático: hallazgos clínicos y ecográficos de 3 casos.

Idiopathic facial aseptic granuloma is a typical childhood disease characterized by the presence of one or more asymptomatic nodules on the cheek. Although pathogenesis remains unclear, the disease is thought to be a type of childhood rosacea. It resolves spontaneously, yet it could be confused with other lesions that require treatment. We present clinical and ultrasound findings and outcome from 3 new cases. In 2 cases, the lesion presented as childhood rosacea. Ultrasound revealed a characteristic pattern, with variations depending on the stage of development. High-frequency ultrasound can facilitate the differential diagnosis and thus obviate unnecessary biopsy or excision.

[Acrocyanosis as a form of presentation of progressive systemic sclerosis]. / Acrocianosis como forma de debut de una esclerosis sistémica progresiva.

Acrocyanosis is an infrequent entity characterized by persistent and symmetrical erythrocyanotic discoloration of the hands, feet and face, which is not preceded by episodes of previous paleness. Acrocyanosis can be secondary to a variety of underlying causes, but is uncommon as a form of presentation of systemic sclerosis. We present a new clinical case of this disease and discuss its etiopathogenic, clinical, diagnostic and therapeutic characteristics; in addition to, we seek to distinguish this term from others that might be confused in the literature.