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Laminar plantar hyperkeratosis on both feet with enhancement of areas under compressive stress (a) before treatment and (b) after three cycles of chemotherapy (punch marks on left sole)
Source publication
Acrokeratosis paraneoplastica is a rare paraneoplastic phenomenon associated with upper aerodigestive tract carcinomas, usually manifesting as psoriasiform keratosis over the acral sites. It is primarily seen in white males above the age of 40 years. Here we report a case of paraneoplastic acrokeratosis in a woman with serous ovarian cancer. To the...
Context in source publication
Context 1
... epidermal hyperpla- sia. Mycosis was excluded histologically. A regression of the cutaneous lesion together with the normalisation of the CA125 tumour marker (a drop to 9.3 U/mL) under first-line chemotherapy containing carboplatin and pacli- taxel was observed, which further supported the suspected diagnosis of a paraneoplastic phenomenon (Fig. ...
Citations
... Among the most frequently affected sites are the ears, nails, nose, fingers, palms, hands, soles, and feet. On a less common note, the knees and elbows can exhibit lesions in approximately 20% of patients, usually later in the disease's course [12][13][14][15][16]. ...
... Characteristic red to purple scaly or crusted plaques emerge on the cheeks, with scaling developing on the palms and soles, excluding the central areas. Fissuring, primarily on the feet, and nail pathology, manifesting as yellowing, thickening, onycholysis, and both horizontal and vertical ridging, may result in pain and disability [13,15,16]. ...
... In some instances, vesicles and bullae may emerge, mainly on the fingers, hands, and feet. Nail changes can vary significantly, ranging from typical thickening to atrophy and even complete loss [12,15]. ...
Acrokeratosis paraneoplastica, also known as Bazex syndrome, is a relatively understudied yet clinically important dermatological condition that is inextricably associated with squamous cell carcinoma, predominantly of the upper respiratory and gastrointestinal tracts. Manifesting as unique psoriasis-like cutaneous lesions, Bazex syndrome functions as an early warning signal for underlying malignancies, underscoring the urgent need for its timely diagnosis and intervention. Notwithstanding its clinical relevance, the molecular and cellular mechanisms underpinning its pathogenesis are not fully understood. To address these gaps, this comprehensive literature review undertook a meticulous search across reputable databases such as EMBASE, MEDLINE/PubMed, and Web of Science. Our analysis ventures into multiple putative pathogenic pathways, including shifts in Th2 immune responses, aberrant secretion of growth factors, and immunological reactions to tumor-specific antigens. We also detail the clinical phenotypes of Bazex syndrome and their chronological linkage with the corresponding malignancies. Finally, the review evaluates the therapeutic effectiveness of various approaches, including but not limited to targeted cancer treatments, PUVA therapy, and topical corticosteroids. This synthesis aims to arm healthcare providers with a nuanced understanding of Bazex syndrome, facilitating more accurate diagnosis and tailored treatment paradigms.
... Less common, the skin disorder can develop contemporaneously or after diagnosis of malignancy. In most cases, it affects Caucasian men over forty with an underlying neoplasm, which is frequently diagnosed as a squamous cell carcinoma of the upper aerodigestive tract or metastatic cervical lymphadenopathy [6]. Due to a similar clinical appearance, the cutaneous psoriasiform lesions can often be misdiagnosed as psoriasis or eczema but do not respond well to standard dermatological treatment, such as topical corticosteroids. ...
Acrokeratosis paraneoplastica Bazex is a rare paraneoplastic skin manifestation, typically causing acral psoriasiform lesions. Patients usually show erythematous hyperkeratosis with yellowish, adherent scales on the hands and feet or other acral locations such as ears or nose. We herein report a case of Bazex syndrome in a male patient, who was previously diagnosed with hepatocellular carcinoma. Our case report highlights this rare condition as early diagnosis may impact the patient’s course of tumor disease and prognosis.
... Acrokeratosis paraneoplastica (also known as Bazex syndrome) is a rare paraneoplastic skin disease in which cutaneous lesions appear in association with an underlying malignancy. These lesions generally consist of erythematous, violaceous, scaly plaques on acral locations (such as nose and ears), and on the hands and feet; and they precede cancer diagnosis in 65-70% of affected patients [1,2]. Thus, early clinical recognition is vital to improving patient outcomes. ...
... It includes symmetrical psoriasiform lesions on the acral regions, especially the nose, ears, hands and feet with an intensive violaceous erythema, and hyperkeratotic yellowish surfaces. Fingernails and toenails may be affected with dystrophic and subungual hyperkeratosis [1,2,5,6]. ...
... One theory involves an autoimmune response resulting from molecular mimicry of tumor antigens to epidermal cell growth factor receptors. Another possible explanation involves direct interaction between the skin and cytokines secreted by tumor cells, such as transforming growth factor and insulinlike growth factor [2,5,8]. ...
Background
Acrokeratosis paraneoplastica is an infrequent paraneoplastic disease in which cutaneous lesions appear and develop in a context of an underlying malignancy. Upper aerodigestive tract carcinomas are the most common neoplasias associated with this syndrome. The aim of this report is to raise awareness of this syndrome, since the recognition of the skin lesions could allow suspicion and diagnosis of the underlying neck malignancy at an early curable stage.
Methods
We report a case of a 62-year-old life-long smoking male who presented a several months evolution of multiple skin lesions, affecting both ear lobes, hands and feet. He was referred for appropriate diagnosis and treatment of the lesions.
Results
After further examinations and study of the patient, the diagnosis concluded in the detection of a laryngeal squamous cell carcinoma (SCC) causing the paraneoplastic skin-affecting syndrome (acrokeratosis paraneoplastica).
Conclusions
A correctly oriented clinical suspicion of the malignancy was possible due to the detection of suggestive skin lesions affecting ear lobes and other locations.
... Una teoría es la existencia de mimetismo molecular, una reacción cruzada entre antígenos tumorales y receptores del factor de crecimiento en las células epidérmicas. Otra explicación posible implica la interacción directa entre la piel y las citocinas secretadas por las células tumorales, como el factor de crecimiento transformante y el factor de crecimiento similar a la insulina [2,6]. Niveles bajos de vitamina A sérica también podrían influir en el desarrollo de las lesiones cutáneas características [3,4]. ...
Introduction and objective: Bazex syndrome is an infrequent paraneoplastic syndrome that is related to an upper aerodigestive epidermoid carcinoma. A case of characteristic Bazex syndrome is presented to contribute to an early diagnosis of the associated underlying tumor pathology. Case description: 57-year-old male, smoker and regular drinker, presented with a cervical mass associated with scaly and violaceous eczema on hands, feet, ears and knees. After examination and complementary tests, the diagnosis of T2N3M0 tonsillar squamous cell carcinoma and paraneoplastic syndrome was established. Conclusions: Oropharyngeal carcinoma is one of the most frequent tumors associated with Bazex syndrome. Therefore, if we observe these skin lesions, we should always rule out oncological pathology at that level.
... Acrokeratosis paraneoplastica (AP) or Bazex syndrome, is an obligate paraneoplastic skin disease characterized by peripheral psoriasiform lesions, palmoplantar keratosis and onychodystrophy. 1 It is linked with a variety of internal malignancies; the majority being squamous cell carcinoma of upper aerodigestive tract and metastatic cervical lymphadenopathy. 2 Usually, the skin lesions develop prior to the diagnosis of an internal malignant neoplasm. Less commonly, they can develop concurrently or after diagnosis of malignancy. ...
... Синдром Лезера-Трела (эруптивный себорейный кератоз) -факультативный паранеопластический дерматоз, характеризующийся внезапным появлением быстро увеличивающихся в размерах и количестве себорейных кератом [2,[6][7][8]. Признак впервые описан в 1890 г. немецким хирургом E. Leser и французским хирургом U. Trelat [9]. Встречается примерно с одинаковой частотой у мужчин и женщин. ...
... Acrokeratosis paraneoplastica, otherwise known as Bazex syndrome, is a paraneoplastic syndrome in which cutaneous lesions appear in association with an underlying malignancy. Early clinical recognition is vital to improving patient outcomes, considering that its manifestations precede cancer diagnosis in 65-70% of affected patients [1][2][3]. Hyperkeratotic plaques with associated erythema and scale present in a symmetric acral distribution and spread centripetally, accompanied by nail changes, including onycholysis and ungual dystrophy [3][4][5]. Ears and nails are most commonly affected, followed by the nose, hands, and feet [2]. ...
... Hyperkeratotic plaques with associated erythema and scale present in a symmetric acral distribution and spread centripetally, accompanied by nail changes, including onycholysis and ungual dystrophy [3][4][5]. Ears and nails are most commonly affected, followed by the nose, hands, and feet [2]. Skin changes typically improve with treatment of the underlying malignancy. ...
... Acrokeratosis paraneoplastica involves bilateral psoriasiform cutaneous lesions that initially present on acral surfaces, especially the ears, nose, hands, and feet, spreading centripetally [2,4,5]. Three clinical stages exist to describe the sequence of cutaneous lesions in relation to the underlying neoplasia: (1) in a locally asymptomatic malignancy, cutaneous lesions present on the nose, ear helices, fingers, toes, and nails; (2) in a locally symptomatic malignancy, palmoplantar surfaces are affected; (3) in a symptomatic malignancy left untreated, lesions advance to the patient's knees, elbows, and trunk [4,6]. ...
Background . Acrokeratosis paraneoplastica, or Bazex syndrome, is a paraneoplastic syndrome characterized by cutaneous psoriasiform lesions with associated acral erythema and scale, as well as nail changes, including onycholysis and ungual dystrophy. Its most advanced, severe form involves the trunk, elbows, and knees. It is typically associated with upper aerodigestive tract malignancies in males. Rare cases associated with gynecological cancers have been reported, including uterine adenocarcinoma, as well as ovarian and vulvar squamous cell carcinomas. Cutaneous manifestations often precede cancer diagnosis. In most reported cases, skin changes resolve when the underlying malignancy is adequately treated. Main Observations . We present the case of a 56-year-old female diagnosed with acrokeratosis paraneoplastica following the discovery of FIGO stage IIB cervical squamous cell carcinoma (SCC). Scaling, hyperpigmentation, xerosis, and fissuring were noted on the patient’s hands, feet, legs, arms, and lower back. Pitting was noted on her fingernails. Her cervical cancer was successfully treated with chemoradiotherapy, after which her cutaneous lesions persisted for two months before resolving. Conclusions . The presentation of acrokeratosis paraneoplastica in this context is atypical. Reports of associations with gynecological cancers, as in our patient’s case, are exceedingly rare.
Paraneoplastic syndromes are a group of rare disorders that can be triggered by an abnormal immune response to proteins from tumors of the lung, ovary, lymphatics, or breast. Paraneoplastic clinical syndromes affect < 1% of patients with cancer; however, the frequency of subclinical levels of paraneoplastic autoantibodies in asymptomatic patients with cancer is unknown. Numerous studies have reported that ovarian cancer patients show signs of paraneoplastic neurological syndromes (PNSs) before or after their cancers are diagnosed. PNSs arise from a tumor-elicited immune response against onconeural antigens that are shared by tissues of nervous system, muscle, and tumor cells. Studies on the serum IgGs obtained from ovarian cancer patients have indicated the presence of onconeural antibodies in the absence of any PNS symptoms. The occurrence of PNSs is low in ovarian cancer patients and it can be accompanied by onconeural antibodies. The diagnosis of PNSs is accompanied by a suspicion of a malignant tumor such that neurologists typically refer such patients for a tumor diagnostic workup. There will be tremendous utility if subclinical levels (without paraneoplastic neurological symptoms or myositis) of these autoantibodies to paraneoplastic antigens can be exploited to screen asymptomatic high-risk patients for ovarian cancer, and used as biomarkers in immunoassays for the early detection or recurrence of ovarian cancer. Ovarian cancer overall survival is likely to be improved with early detection. Therefore, a panel of onconeural antigens that can detect paraneoplastic autoantibodies in patient sera should provide diagnostic utility for an earlier therapeutic intervention. Here we review the usefulness of PNS and other paraneoplastic syndromes and their association with paraneoplastic antigens to exploit these autoantibody biomarkers to form diagnostic multi-analyte panels for early detection of ovarian cancer.
Acrokeratosis paraneoplastica Bazex (Bazex syndrome) is a rare paraneoplastic skin disease defined by erythematous, violaceous, scaly plaques on the hands and feet and on other acral locations such as nose and ears. Bazex syndrome is linked to a variety of underlying malignancies. Usually the skin lesions develop prior to the diagnosis of an internal malignant neoplasm with spontaneous remission after tumor removal. The objective of this study was to review the so far reported risk factors, diagnostic work up, prognosis and treatment options for Bazex syndrome in a systematic manner. This systematic review is based on a search in Medline, Embase and Cochrane Central Register for English and German articles from 1990 to 2015. Evidence on the diagnosis and treatment of Bazex syndrome is limited predominately to case reports or to small case series. There are no randomized controlled trials. A number of underlying tumor entities, predominately oropharyngeal neoplasms and tumors of the gastroenterological tract, but other malignancies were reported. Treatment modalities including topical and systemic corticosteroids, salicylic acid, topical vitamin D analogues, etretinate and PUVA therapy are often ineffective. Due to the small number of patients and the frequent misdiagnosis of this clinical entity, the aim of this systematic review is to call attention to this rare condition and to help clinicians to diagnose and treat Bazex syndrome effectively. Due to the good prognosis of the skin lesions and the tendency to resolve spontaneously if the underlying tumor is treated early, the differential diagnosis of Bazex syndrome should be taken into consideration when dealing with atypical psoriasiform cutaneous lesions. An early diagnosis may improve the patient's prognosis substantially. This article is protected by copyright. All rights reserved.
