Spectrum of comorbid autoimmune diseases in patients with chondrodermatitis nodularis helicis: a 17-year retrospective study of 215 patients in Asturias, northern Spain

Background: The cause of chondrodermatitis nodularis helicis (CN) (Winkler's disease) is unknown, but potential associations with autoimmune diseases have been suggested in case reports, however, studies with large case series are lacking. Objectives: To clarify the frequency of chronic inflammatory and autoimmune diseases (CADs), and associated gender and age distribution, in a large cohort of patients with CN. Materials & Methods: The frequency of CADs (systemic and cutaneous) was assessed in 215 patients (65.1% males and 34.9% females; median age: 69.6 years) with a histopathological diagnosis of CN (2000-2017). Endocrine diseases were not included. Statistical analysis included Fisher's exact test and multivariate logistic regression analysis. Results: Twenty different CADs were diagnosed in 15.34% patients with CN. The most frequent were polymyalgia rheumatica (six patients), psoriasis (four patients, one with psoriatic arthritis), rheumatoid arthritis (three patients), CREST syndrome (two patients), vitiligo (two patients), and chronic dermatitis (two patients). Several CADs were strongly associated with tobacco smoking. Systemic CADs were more frequent in females (OR: 3.814; CI 1.513-9.613; p = 0.005; multivariate logistic regression analysis). Differences according to age at onset were not significant. Conclusion: We characterize, for the first time, the spectrum of CADs as well as age and gender distribution in patients with CN based on the largest cohort of patients to date. The possible accumulation of different disorders that are strongly associated with tobacco smoking (Buerger's disease, pulmonary Langerhans cell histiocytosis, rheumatoid arthritis, Lupus erythematosus, and others) merits further investigation, but the rarity of some of them makes this challenging.

Retrospective study of apremilast drug survival in psoriasis patients in a daily practice setting: A long-term experience.

There is limited evidence about the real-world survival of apremilast in patients with psoriasis, especially over the long term. To evaluate the long-term survival of apremilast and its predictive factors when used to treat psoriasis. A retrospective hospital-based study, including data collected from 104 patients. Survival curves were estimated using the Kaplan-Meier estimator. Proportional hazard Cox regression models were used for multivariate analysis. The average duration of the treatment before discontinuation was 28.82 months (95% CI, 22.08-35.57 months) and the median was 12 months (95% CI, 2.68-21.31 months). The retention rates were 51% (1 year), and 33% (5 years). The survival study revealed statistically significant differences between patients with PASI<10 and those in the PASI≥10 group (log-rank test, p < 0.001). The 5-year prevalences were 64% for patients with a PASI of <10 and 5% for those with an index ≥10. In the PASI < 10-patient group, the retention rates were 77% (1 year) and 64% (5 years). Furthermore, 66% of patients who continued apremilast treatment for more than 2 years were receiving off-label doses (30 mg/day). Apremilast may be a suitable and efficient alternative for the treatment of psoriasis patients in the PASI<10 group.

Dermatitis anular liquenoide de la infancia / Annular lichenoid dermatitis of youth

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Ustekinumab Drug Survival in Patients with Psoriasis: A retrospective Study of Real Clinical Practice.

Background and objectives: The efficacy and safety of ustekinumab have been proved in clinical trials. In daily clinical practice, knowing the factors that determine survival differences of biological drugs allows psoriasis treatment to be optimized as a function of patient characteristics. The main objectives of this work are to understand ustekinumab drug survival in patients diagnosed with plaque psoriasis in the Hospital Universitario Central de Asturias (HUCA Dermatology Department, and to identify the predictors of drug discontinuation. Materials and Methods: A retrospective hospital-based study, including data from 148 patients who were receiving ustekinumab (Stelara®) between 1 February 2009 and 30 November 2019, were collected. Survival curves were approximated through the Kaplan-Meier estimator and compared using the log-rank test. Proportional hazard Cox regression models were used for multivariate analyses while both unadjusted and adjusted hazard ratios (HR) were used for summarizing the studied differences. Results: The average duration of the treatment before discontinuation was 47.57 months (SD 32.63 months; median 41 months). The retention rates were 82% (2 years), 66% (5 years), and 58% (8 years). Median survival was 80 months (95% confidence interval. CI 36.9 to 123.01 months). The survival study revealed statistically significant differences between patients with arthritis (log-rank test, p < 0.001) and those who had previously received biological treatment (log-rank test, p = 0.026). The five-year prevalence in patients still under treatment was 80% (those without arthritis) and 54% (arthritis patients). In the multivariate analysis, only the patients with arthritis had a lower rate of drug survival. No statistically significant differences were observed for any of the other comorbidities studied. The first and second most frequent causes of discontinuation were secondary failure and arthritis inefficacy, respectively. Conclusion: Ustekinumab is a biological drug conferring high survival in plaque psoriasis patients. Ustekinumab survival is lower in patients with arthritis.

Unusual presentation of herpes simplex virus infection in a boxer: 'Boxing glove herpes'.

Herein, we describe a patient with lesions of cutaneous herpes simplex virus 1 (HSV-1) infection over the knuckles of both hands in the context of an outbreak among boxers. Interestingly, the infection had an unusually long duration (4 weeks), and was not acquired directly through skin-to-skin contact, as it usually does among athletes (herpes gladiatorum). In our case, transmission was acquired through the use of shared boxing gloves contaminated by HSV-1. To the best of our knowledge, herpes gladiatorum, or wrestler's herpes, has not been described previously in boxers and infection over the knuckles is not commonly reported.

Psoriasis and type 2 diabetes risk among psoriatic patients in a Spanish population.

There is accumulating evidence showing a relationship between psoriasis and an increased risk of developing cardiovascular risk factors, including diabetes mellitus type 2 and ischemic heart disease. Our aim was to investigate if there is any difference in the diabetes risk profile among psoriatic patients based on clinical findings. To test this, we carried out a prospective and descriptive hospital-based study. Our results suggest that the highest risk of suffering from diabetes mellitus type 2 among psoriatic patients is in patients suffering from non-familial and late-onset disease and in patients suffering from psoriatic arthritis.

Psoriasis induced by cetuximab: a paradoxical adverse effect.

Cetuximab, an epidermal growth factor receptor inhibitor, is a chemotherapeutical agent featuring well-known adverse dermatological effects related to tumour-response prognosis. In psoriasis, the epidermal growth factor receptor is overexpressed; hence, the expectation would be that an epidermal growth factor receptor inhibitor could improve psoriatic lesions. We report a case of psoriasis induced by cetuximab, which was a surprising adverse effect.

Síndrome auriculotemporal de Frey. Un caso de presentación bilateral en un lactante / Frey auriculotemporal syndrome. A bilateral case in a child

El síndrome auriculotemporal o síndrome de Frey se caracteriza por la aparición de enrojecimiento, hiperhidrosis, o ambos, en la zona inervada por elnervio auriculotemporal como respuesta a un estímulo gustativo desencadenado por la ingesta de distintos alimentos. Por este motivo puede confundirseen ocasiones con alergias alimentarias. Se debe generalmente a una lesión del nervio auriculotemporal, si bien en ocasiones no existe ningún antecedentetraumático. Su aparición en la infancia y su presentación bilateral, como el caso que presentamos a continuación, son muy poco frecuentes

Frey syndrome or auriculotemporal nerve syndrome manifests as flushing, sweating, or both, localized to the distribution of the auriculotemporal nerve,in response to gustatory stimuli. Ocasionally it may be misinterpreted as foog allergy. It usually occurs as a result of injury to auriculotemporal nerve, butthere are cases with no known previous trauma. It is rarely described in children as well as the bilateral involvement, as the case we presented

Apocrine carcinoma, adenopathies, and raised TAG-72 serum tumor marker.

BACKGROUND: The detection of tumor-associated glycoprotein-72 in the serum of patients with carcinomas, basically of the colon, has proved to be of great use in the follow-up of these gastrointestinal adenocarcinomas. RESULTS: We report the case of a male patient presenting adenopathies in the right axilla. The histologic study of an adjacent skin tumor enabled the diagnosis of a cutaneous apocrine carcinoma. Among the studies made, the increase in the serum antibody CA72.4 can be highlighted. The tumor marker was negative after the extirpation of the skin tumor and the axillary adenopathies. CONCLUSION: To our best knowledge, this is the first case in which a tumor serum marker is associated with a cutaneous apocrine carcinoma, a fact that should be confirmed with further patients. Its use in the monitoring of this infrequent skin neoplasia is also noteworthy.

Dermatosis eosinofílicas (I) / Eosinophilic dermatoses (I)

El eosinófilo es una célula efectora de la respuesta inmune con importante tropismo hacia los tejidos. Las enfermedades cutáneas en las que existe una infiltración tisular por eosinófilos constituyen un grupo aparentemente heterogéneo. Se realiza una revisión de las características principales de las enfermedades conocidas como dermatosis eosinofílicas. En esta primera parte del trabajo se incluye la foliculitis pustulosa eosinofílica, la celulitis eosinofílica, la hiperplasia angiolinfoide con eosinofilia, el granuloma facial, la úlcera eosinofílica de la mucosa oral, la papuloeritrodermia y la dermatitis eosinofílica paquidérmica. En analogía a la visión integradora aceptada para las dermatosis neutrofílicas se discutirá la posible unidad patogénica de este grupo de enfermedades (AU)

Eritema elevatum et diutinum con afectación palmar y asociación con p-ANCA / Erythema elevatum et diutinum with palmar involvement associated to p-ANCA

El eritema elevatum et diutinum es una forma localizada, crónica y benigna de vasculitis leucocitoclástica cutánea que evoluciona hacia una peculiar fibrosis concéntrica y depósito secundario de material lipídico. Su causa es desconocida, pero se supone en relación con el depósito vascular de inmunocomplejos. Entre los posibles problemas médicos asociados se incluyen enfermedades hematológicas, especialmente la gammapatía monoclonal IgA. Una mujer de 24 años presentaba múltiples pápulas y placas localizadas simétricamente sobre la superficie de extensión de las extremidades, especialmente en torno a zonas articulares. La exploración reveló además múltiples placas elevadas, firmes, que afectaban por completo la superficie palmar de las manos. La biopsia de un elemento reciente descubrió un infiltrado neutrofílico dérmico y polvillo nuclear alrededor de vasos con vasculitis leucocitoclástica. En los estudios complementarios se encontró positividad para anticuerpos anticitoplasma de neutrófilo (p-ANCA) (anti-mieloperoxidasa [MPO]). El tratamiento con sulfona 100 mg/día condujo a una respuesta clínica espectacular. Describimos un ejemplo de eritema elevatum et diutinum con una distribución atípica afectando las palmas, circunstancia escasamente referida. Además discutimos el posible significado de la asociación con una prueba positiva para p-ANCA. (AU)

Xantogranulomatosis subcutánea elastolítica (piel laxa xantogranulomatosa) / Elastolytic subcutaneous xanthogranulomatosis (xanthogranulomatous slack skin)

La xantogranulomatosis subcutánea es una peculiar xantomatosis que probablemente corresponde a una variante muy xantomatizada y con elementos múltiples del xantogranuloma juvenil subcutáneo. Los individuos afectados presentan múltiples nódulos subcutáneos de consistencia firme y localización preferente en el tronco. Los nódulos corresponden a masas xantomatosas subcutáneas que no se acompañan de xantomas cutáneos. Desde el punto de vista histológico se trata de cúmulos celulares bien circunscritos, localizados en dermis profunda e hipodermis y compuestos por una mezcla de células xantomatosas y células gigantes de tipo cuerpo extraño y de tipo Touton. Los estudios analíticos de los lípidos séricos resultan normales. Un varón de 52 años consultó porque a lo largo de los últimos años toda la piel de la espalda y zona superior del tórax había ido adquiriendo un aspecto irregular, laxo y plegado. En estas zonas presentaba múltiples lesiones nodulares subcutáneas, de consistencia firme. Microscópicamente los nódulos estaban constituidos por una población predominante de células xantomatosas y algunas células gigantes multinucleadas. En el interior del citoplasma de muchas de las células gigantes se observó la presencia de cuerpos asteroides. Además presentaba xantomas en las conjuntivas bulbares y un intenso linfedema en ambas piernas. Consideramos que este caso es un ejemplo más de la denominada xantogranulomatosis subcutánea con dos peculiaridades clínicas: el hallazgo de piel laxa en las zonas afectadas y la asociación con linfedema y elefantiasis cutánea en miembros inferiores. Por otra parte hemos reseñado el peculiar hallazgo histológico de cuerpos asteroides en el interior de las células gigantes del infiltrado xantomatoso (AU)