Background Current knowledge of angiolymphoid hyperplasia with eosinophilia (ALHE) derives from retrospective reports and case series, leading to a nonevidence-based treatment approach. Objective We sought to systematically review the literature relating to cutaneous ALHE to estimate its epidemiology and treatment outcomes. Methods A literature search of PubMed, EMBASE, Web of Science, and Google Scholar was conducted. Articles detailing cases of histologically confirmed cutaneous ALHE were included. Results In all, 416 studies were included in the review, representing 908 patients. There was no sex predominance among patients with ALHE. Mean age at presentation was 37.6 years. There was a significant association between presence of multiple lesions and pruritus, along with bleeding. Surgical excision was the most commonly reported treatment for ALHE. Treatment failure was lowest for excision and pulsed dye laser. Mean disease-free survival after excision was 4.2 years. There were higher rates of recurrence postexcision with earlier age of onset, longer duration of disease, multiple lesions, bilateral lesions, pruritus, pain, and bleeding. Limitations Potential for publication bias is a limitation. Conclusions Surgical excision appears to be the most effective treatment for ALHE, albeit suboptimal. Pulsed dye and other lasers may be effective treatment options. More studies are needed to improve the treatment of ALHE.

Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia (ALHE): A systematic review

Merkel cell carcinoma has been a focus of active scientific investigation in recent years and new information on the topic has emerged. Although uncommon, this primary cutaneous neuroendocrine carcinoma, usually involving the head/neck of elderly individuals, has a poor prognosis. Within the past two decades, an increase in the incidence of the tumor and the discovery of its link to the Merkel cell polyomavirus have focused medical attention on the lesion. The resulting studies have improved our understanding of the biology of the neoplasm and contributed to clinical care. Specifically, two pathogenic subsets of the tumor have come to light, the majority due to Merkel cell polyomavirus and the minority caused by ultraviolet radiation-induced genetic damage. This dichotomy carries prognostic implications favoring the former subset. In addition, having capitalized on the known susceptibility of the tumor to immune influences, investigators have recently discovered its responsiveness to immune checkpoint inhibition. This revelation has constituted a therapeutic milestone at the clinical level. Herein we provide an overview of the topic, outline updates in the field and place an emphasis on dermatopathologic aspects of Merkel cell carcinoma.

Merkel cell carcinoma: A review

Pellagra is a clinical syndrome caused by a deficiency of niacin (nicotinic acid) and/or its precursor tryptophan. The cardinal manifestations are 4 D's: dermatitis, diarrhoea, dementia and in worst case death. Increased use of isoniazid prophylaxis along with antiretroviral therapy in countries where latent tuberculosis is common has been associated with increased presentations with pellagra.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/phpp.12659

Pellagra a review exploring causes and mechanisms, including isoniazid-induced pellagra.

Importance Patients with extrapulmonary neuroendocrine carcinomas (EPNECs) receive essentially the same treatment as those with small cell lung cancer (SCLC) despite differences in origin, clinical course, and survival. This SCLC-based approach is attributable to the rarity of EPNECs, which impedes the use of randomized clinical trials. However, neuroendocrine carcinomas are becoming more common because of the increasing use of systemic cancer therapy for adenocarcinomas. This treatment can transdifferentiate certain adenocarcinomas into neuroendocrine carcinomas. In addition, the treatment landscape for SCLC is slowly changing, potentially impacting the treatment paradigms for EPNECs. Observations New information on tumorigenesis of EPNECs from different origins, either as a primary malignant tumor or after neuroendocrine differentiation from adenocarcinomas, demonstrates their biological similarity. Activated molecular pathways that appear to underlie the development of EPNECs are potentially targetable, and some of these targets, such as poly(adenosine diphosphate-ribose) polymerase, Wee1, and Aurora A kinase, are currently under investigation. Immune checkpoint inhibitors (ICIs) already constituted a new treatment modality for patients with SCLC and produced some promising results in patients with EPNECs. Conclusions and relevance Although only moderately effective, the introduction of ICIs signifies the first new option in systemic treatment of SCLC in decades. To prove the value of ICIs and other new drugs for patients with EPNECs, these patients should be included in clinical trials independent of the primary tumor site. Furthermore, to optimize clinical decision-making for patients with EPNECs, experts from the neuroendocrine tumor board should collaborate with members from tumor site-specific boards, which will require patient referral to a center with EPNEC expertise.

Current Treatment Strategies and Future Directions for Extrapulmonary Neuroendocrine Carcinomas: A Review

Hiperplasia angiolinfoide con eosinofilia. Estudio clínico patológico de 9 casos

INTRODUCTION Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasoproliferative disease of undetermined origin. It is characterized by the presence of nodular pseudo-tumors corresponding microscopically to a vascular proliferation within an inflammatory infiltrate made up of lymphocytes, macrophages, and eosinophils. The authors describe 7 cases of ALHE. METHODS The 7 cases were diagnosed over a period of 19 years (1990-2008). Clinical data and histological slides were brought from the departments of dermatology and pathology of the Rabta Hospital. RESULTS The 7 patients were 4 women and 3 men with an average age of 34.5 years. The cephalic localization was the most frequent. Lesions were solitary or multiple and formed papules or plaques of variable color. The diagnosis was based in all cases on histological findings. DISCUSSION The main disease in the differential diagnosis of ALHE is Kimura disease, but the 2 entities have several clinical and histological differences. The pathogenesis remains unclear and there is no consensus on the best treatment.

Angiolymphoid hyperplasia with eosinophilia: a study of 7 cases.

Acute generalized exanthematous pustulosis (AGEP) is an uncommon pustular eruption characterized by small nonfollicular pustules on an erythematous background, sometimes associated with fever and neutrophilia. Over 90% of cases are drug-induced; however, it can be caused in rare cases by other agents. We report two cases of AGEP secondary to ingestion of Pistacia lentiscus essential oil, the first two such cases to our knowledge. The cutaneous morphology, disease course and histological findings were consistent with a definite diagnosis of AGEP, based on the criteria of the EuroSCAR study group. These two cases highlight the need to consider herbal extracts as a potential rare cause of AGEP and to ensure the safety of herbal medicines.

Acute generalized exanthematous pustulosis induced by the essential oil of Pistacia lentiscus.

Resume Introduction L’osteosarcome est une tumeur maligne d’origine mesenchymateuse qui touche essentiellement les metaphyses des os longs. La forme extrasquelettique est rare et plus encore la localisation cutanee, qu’elle soit metastatique ou primitive. Nous rapportons un nouveau cas d’osteosarcome cutane primitif. Observation Une femme de 54 ans presentait sur le bras gauche une tumeur nodulaire sous-cutanee de consistance dure, evoluant depuis un an. Le diagnostic d’osteosarcome cutane etait retenu grâce a l’examen histologique de la lesion, montrant une proliferation sarcomateuse dermo-hypodermique secretant de l’osteoide. Le bilan d’extension clinique et radiologique permettait d’eliminer une eventuelle atteinte extracutanee, en particulier osseuse, confirmant ainsi le caractere cutane primitif de l’osteosarcome. Discussion L’osteosarcome cutane primitif est une tumeur rare dont le diagnostic est en general porte histologiquement.

Ostéosarcome cutané primitif

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis with unknown etiology. PG associated with infectious disease is very unsual. We report a singular case of PG with lymph nodes tuberculosis disease.

/pdf/pyoderma-gangrenosum-and-lymph-nodes-tuberculosis-disease-1i4ignodo0.pdf

Pyoderma Gangrenosum and lymph nodes tuberculosis disease: unusual association

• Introduction. Les mycetomes sont des affections chroniques de la peau et du tissu sous-cutane d'origine fongique ou bacterienne. Ils sont endemiques dans les regions tropicales et subtropicales, mais rares en Tunisie. Objectifs. Cette etude decrit les aspects epidemio-cliniques, paracliniques, therapeutiques et evolutifs des mycetomes en Tunisie. Malades et methodes. Il s'agit d'une etude retrospective colligeant tous les cas de mycetomes observes au service de dermatologie de l'hopital La Rabta de Tunis, entre 1982 et 2006. Resultats. Quinze cas de mycetomes ont ete observes (6 hommes et 9 femmes) avec un âge moyen de 53,2 ans. L'aspect clinique predominant etait celui d'un placard infiltre erythemato-pigmente multi-nodulaire parseme de fistules. Le siege podal etait retrouve dans 12 cas. L'issue de grains etait rapportee par 13 malades. Ils etaient d'origine actinomycosiques dans 9 cas, l'agent pathogene etant Actinomadura madurae, et fongiques dans 6 cas dus a Madurella mycetomatis dans 3 cas et Pseudallesheria boydii dans 2 cas. Le traitement etait base sur l'antibiotherapie generale en cas d'actinomycetomes, et les antifongiques oraux en cas d'eumycetomes. Conclusions. Les mycetomes demeurent une pathologie rare en Tunisie, avec une legere predominance feminine. Le siege podal est frequent. Le diagnostic est base sur l'examen direct, la culture et l'examen anatomopathologique. Le traitement n'est pas codifie et est souvent prolonge avec des rechutes frequentes.

Les mycétomes en Tunisie: 15 observations

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