Acrokeratosis Paraneoplastica Research Articles

Bazex Syndrome (Acrokeratosis Paraneoplastica): A Narrative Review of Pathogenesis, Clinical Manifestations, and Therapeutic Approaches.

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, includingbut not limited totargeted 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.

DP12 Painful oral keratosis and skin lesions: a paraneoplastic diagnostic conundrum

Abstract A 70-year-old man was referred to oral medicine with extensive, painful oral hyperkeratosis affecting the tongue, buccal mucosa and palate. Medical history included a previous diagnosis of prostate cancer. Histology from multiple oral biopsy specimens showed only hyperparakeratosis and acanthosis, with no fungi or dysplasia. Owing to the absence of oral inflammation, the pain was treated as neuropathic. The patient then developed skin lesions and dermatology review was requested. Skin examination revealed marked hyperkeratotic, verrucous plaques affecting the extensor surfaces of the limbs and dorsal hands, as well as the plantar feet bilaterally. The nails were unaffected. There was violaceous discolouration of the pinnae. Given the history of prostate cancer, a paraneoplastic process was suspected. Two incisional biopsies were taken, which showed epidermal acanthosis with necrotic and dyskeratotic keratinocytes throughout the epidermis. There was a dense eosinophilic inflammatory infiltrate. Direct and indirect immunofluorescence was negative. The appearances were suggestive of a paraneoplastic phenomenon, including acrokeratosis paraneoplastica. Haematological investigations identified an IgG monoclonal paraproteinaemia with raised kappa:lambda ratio. Following further investigation, including bone marrow biopsy and positron emission tomography computed tomography, a diagnosis of monoclonal gammopathy of undetermined significance was made by haematology. A repeat bone marrow biopsy 7 months later confirmed evolution to myeloma. As CRAB criteria (hypercalcaemia, renal insufficiency, anaemia and bone lesions) were absent, a period of observation followed. A trial of oral steroids improved the cutaneous eruption, but he developed significant side-effects. Oral mycophenolate, methotrexate and acitretin were used as steroid-sparing agents, with a poor clinical response. The skin lesions progressed to a widespread psoriasiform eruption on the trunk and lower limbs with the formation of bullae. Further biopsies showed an eosinophil-rich infiltrate with negative immunofluorescence, raising the possibility of an eosinophilic dermatosis of haematological malignancy. After 2 years, given the worsening skin symptoms, it was decided to commence bortezomib, cyclophosphamide and dexamethasone (VCD) chemotherapy. Despite four cycles of VCD treatment, the skin lesions worsened and as the paraprotein levels did not reduce, treatment was stopped. Subsequently, further elevations in serum free light chains prompted treatment with lenalidomide. This resulted in a marked improvement in the skin and reduced pain from the oral lesions, as well as a reduction in serum free light chains. Acrokeratosis paraneoplastica (Bazex syndrome) is characterized by digital hyperkeratosis and paronychia, which may progress to involve the limbs and trunk. Eosinophilic dermatosis of haematological malignancy is characterized by a pruritic, papular skin eruption with a vesiculobullous component. This case presents a diagnostic conundrum, with evolving features of two different paraneoplastic conditions.

Bazex Syndrome Associated with Angioimmunoblastic T-cell Lymphoma

Bazex syndrome, also known as Acrokeratosis paraneoplastica, is a paraneoplastic disorder characterized by erythematous psoriasiform plaques involving the nose, ears, and acral sites. Although classically associated with squamous cell carcinomas of the upper aerodigestive tract, it has also been reported in association with adenocarcinoma, genitourinary tumors, multiple myeloma, and rarely, peripheral T-cell lymphoma and follicular lymphoma in-situ. Herein, we present a patient with Bazex syndrome associated with angioimmunoblastic T-cell lymphoma (AITL), a rare association not previously reported in the literature.

An asymmetric morbilliform eruption in an adult male

An asymmetric morbilliform eruption in an adult male

Paraneoplastic acrokeratosis (Bazex syndrome)

Paraneoplastic acrokeratosis (Bazex syndrome)

Dermatological paraneoplastic syndromes in gastroenterology: prediction of the inevitable or guidance to action? Part II

The second part of the article provides considerations for the proliferative and inflammatory dermatoses accompanying various malignant tumors of digestive organs. The most widespread paraneoplastic syndromes (PNS) have been described, including the acquired hypertrichosis lanuginosa, malignant black acanthosis, acquired padermatoglyphia, which, as a rule, accompany and sometimes lead to the appearance of malignant neoplasms of the gastrointestinal tract and lungs. The high probability has been emphasized for the diagnosis of benign black acanthosis in patients with obesity, insulin resistance, diabetes mellitus, patients taking medications for a long time. The authors presented typical manifestations of rarer PNS: paraneoplastic acrokeratosis, circular migrating erythema, paraneoplastic dermatomyositis. The detailed information provided regarding Leser — Trélat syndrome, which described the relationship between the appearance of numerous seborrheic keratomas and the development of malignant internal organ neoplasms. The possible relationship between Leser — Trélat syndrome and malignant black acanthosis was highlighted.The authors presented a clinical case with typical manifestations of dermatological PNS (Leser — Trélat syndrome) in a patient with a serous pancreatic cystadenoma. Despite the extremely low probability of malignant transformation of this neoplasm, the rapid growth of a pancreatic cyst (atypical for a benign formation) with simultaneous increase in the number and size of seborrheal adenoma was observed during medical observation. The results of 2‑year observation of the patient after the prophylactic pancreas resection have been outlined.

Clinical, Imaging, and Pathologic Features of Conditions with Combined Esophageal and Cutaneous Manifestations.

A variety of clinically significant conditions can affect both the esophagus and the skin. Esophageal and cutaneous manifestations may directly reflect the underlying disease process, as in infections such as herpes simplex virus, bullous diseases such as epidermolysis bullosa and mucous membrane pemphigoid, connective tissue diseases such as systemic sclerosis, and inflammatory diseases such as lichen planus. Alternatively, esophageal and cutaneous findings may result from conditions that are closely associated with and potentially pathognomonic for but distinct from the underlying disease process, as in genetic diseases such as Cowden syndrome or paraneoplastic syndromes such as acrokeratosis paraneoplastica. Other diseases such as Crohn disease may have cutaneous manifestations that directly reflect the same underlying inflammatory process that affects the gastrointestinal tract or cutaneous manifestations that represent reactive or associated conditions distinct from the underlying inflammatory process. The cutaneous manifestations of disease may precede, coincide with, or follow the esophageal manifestations of disease. The authors present the characteristic clinical features and imaging findings associated with common and uncommon conditions that have esophageal and cutaneous manifestations. Each condition is presented with a brief overview, discussion of salient clinical and cutaneous manifestations, and description of the typical esophageal imaging findings, with particular attention to implications for diagnosis, prognosis, and treatment. Recognition of potential associations between cutaneous lesions and esophageal imaging findings is important for establishing a specific diagnosis or generating a meaningful differential diagnosis.

Bazex syndrome in a patient with head and neck malignancy

Bazex syndrome (acrokeratosis paraneoplastica) is an uncommon dermatologic condition associated with an underlying malignancy, most commonly squamous cell carcinomas of the head and neck. This article describes Bazex syndrome in a 56-year-old woman who presented with bilateral cervical lymphadenopathy and a mass at the base of her tongue.

Acrokeratosis paraneoplastica (Bazex syndrome) - a systematic review on risk factors, diagnosis, prognosis and management.

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 tumour 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 tumour entities, predominately oropharyngeal neoplasms and tumours 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 was to call attention to this rare condition and to help clinicians to diagnose and treat Bazex syndrome effectively. Because of the good prognosis of the skin lesions and the tendency to resolve spontaneously if the underlying tumour 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.

Paraneoplastic Acrokeratosis (Bazex Syndrome) associated with lung adenocarcinoma

Paraneoplastic Acrokeratosis (Bazex Syndrome) associated with lung adenocarcinoma

Acral manifestations of paraneoplastic and collagen vascular diseases

The skin often signals a number of systemic disease, making skin findings of paramount significance. Paraneoplastic diseases and collagen vascular diseases are vitally important illnesses. Paraneoplastic diseases and collagen vascular diseases may also occur with many different acral skin findings. Paraneoplastic skin diseases, associated with some cancers, are by definition nonmalignant skin disorders. These diseases can occur before, simultaneously, or after the diagnosis of cancer. Acral paraneoplastic diseases include acanthosis nigricans maligna, acquired pachydermatoglyphia, acrokeratosis paraneoplastica, palmoplantar keratoderma, and paraneoplastic nail disorders. Collagen vascular diseases include the acral skin findings of lupus erythematosus, scleroderma, dermatomyositis, and rheumatoid arthritis. Any acral skin finding may be encountered as the first finding of an undiagnosed malignancy or collagen vascular diseases. The role of the dermatologist is significant for often being the first physician to suspect a malignancy or collagen vascular disease.

Acrokeratosis paraneoplastica in the setting of early pancreatic adenocarcinoma

Acrokeratosis paraneoplastica in the setting of early pancreatic adenocarcinoma

Paraneoplastic syndromes in daily clinical practice

Paraneoplastic syndromes consist of disorders that accompany benign and malignant tumours, but are not directly related to mass effects or invasion by the primary tumour or its metastases. The pathophysiology of mostly paraneoplastic syndromes is not well known. The usual mechanism of their development is the aberrant production of substances (protein hormones, hormone precursors or hormone-like substances) by tumour tissue or autoimmune disorder. The neurological paraneoplastic syndromes are autoimmune disorders that can affect almost any part of the nervous system. The endocrine paraneoplastic syndromes are often certain clinical syndromes like paraneoplastic hypercalcaemia or the aberrant production of hormones by cancers. Many haematologic conditions, including anaemia, leucocytosis, thrombocytopenia or thrombocytosis, coagulapathy have been reported in association with cancer. Also paraneoplastic rheumatic syndromes have been reported in association of cancer. Skin can be involved in cancer in different ways: by metastases, as a part of a genetic disorder with a cutaneous component in which there is an inherited predisposition to the later development of malignancy, as a part of an acquired syndrome due to the toxicity of a carcinogen that induces malignant change and accompanying skin changes, as a consequence of immunosuppression, or by the development of specific lesions that occur as a paraneoplastic syndromes The most frequently diagnosed dermatologic conditions include acrokeratosis paraneoplastica, Sweet’s syndrome and paraneoplastic pemphigus. The purpose of this article is to describe the most popular in internal clinical practice neurologic, endocrine, haematologic, rheumatologic and dermatologic paraneoplastic syndromes associated with cancer.

Breast cancer presenting as paraneoplastic erythroderma: an extremely rare case.

The skin may exhibit the first clinical evidence of a systemic disease and may provide the first clues to a diagnosis in malignancies. Erythroderma is defined as generalized redness and scaling and it is a clinical manifestation of a variety of underlying diseases including, rarely, solid tumors. Breast cancer is associated with a variety of skin paraneoplastic manifestations like acanthosis nigricans, erythromelalgia, thrombotic thrombocytopenic purpura, acrokeratosis paraneoplastica, dermatomyositis, systemic sclerosis, and scleroderma. However, in the literature, the correlation of erythroderma with breast cancer is quite infrequent. Here, we describe a case of a 76-year-old woman who presented with a paraneoplastic manifestation of erythroderma due to breast cancer.

Bazex Syndrome

Acrokeratosis Paraneoplastica or Bazex syndrome is a dermatologic manifestation usually associated with the diagnosis of squamous cell carcinoma of the upper aerodigestive tract. We report a case with exuberant clinical manifestations, exemplifying the typical cutaneous lesions in this rare syndrome, in a patient with squamous cell carcinoma of the esophagus.

Examen dermatologique des mains

There are four different areas to consider: palms, back of the hands, fingers, periungual folds (and nails). Palmoplantar keratodermas are a group of inherited or acquired disorders. Dysidrosis is a peculiar form of eczema on the palms and lateral aspects of the fingers. SAPHO syndrome (Synovitis - Acne - Pustulosis - Hyperostosis - Osteomyelitis) presents pustules on palms. Photo-ageing is frequently noticed on the back of the hands. Paraneoplastic acrokeratosis (Bazex syndrome) affects the nose, ears, and periungual folds of fingers and toes. Spontaneous blue finger syndrome can be a benign process that resolves rapidly.

Acute vesiculobullous hand dermatitis and anagen effluvium in Ganoderma lucidum-induced aplastic crisis

Dear Editor, A 66-year-old male patient presented to our department due to vesiculobullous skin lesions over the entire palms and soles that had rapidly progressed over three days. He was treated with topical corticosteroids and oral antihistamines at a private clinic; however, the skin lesions were refractory to the treatments, and the patient developed additional symptoms of fever and myalgia. His medical history revealed that he had been treated with daily olmesartan 20 mg and amlodipine 5 mg for hypertension, which had not been changed for over a year. During the previous two weeks, he had been ingesting Ganoderma lucidum, which his wife and son had also ingested. On physical examination, his body temperature was 38.4 °C, and extensive vesiculobullous lesions with sloughing epidermis were noted on the erythematous palms and soles (Fig. 1). In addition, diffuse alopecic patches over the entire scalp and a positive hair pull test with numerous anagen hairs were noted. Routine laboratory tests were performed, and a biopsy specimen was obtained from an erythematous, but nonvesiculobullous, hand lesion. Complete blood count revealed severe leucopenia of 190 cells/μL (normal range 4,000–10,800 cells/μL; neutrophils 13.6 %, lymphocytes 82.7 %, monocytes 1 %, eosinophils 1 %, and basophils 0.6 %), thrombocytopenia of 36,000 cells/μL (normal range 150,000–400,000 cells/μL), mild anemia of 12.2 g/dL (normal range 13.0–17.0 g/dL), and reticulocyte count of 0.98 % (normal range 0.5–2.31 %). Other laboratory tests were unremarkable. Skin biopsy of the palm revealed mildly infiltrated inflammatory cells adjacent to the dilated capillaries in the upper dermis (Fig. 2a, b). Further investigation for the evaluation of pancytopenia revealed nearly acellular marrow and panhypoplasia without remarkable abnormal cells or blasts (Fig. 2c, d). Klebsiella pneumoniae was isolated from a blood culture. Additional tests, including computed tomography, were performed to rule out Bazex acrokeratosis paraneoplastica. Although there were no remarkable findings suggestive of malignancy, pulmonary angioinvasive aspergillosis forming multifocal areas of lobular and subsegmental consolidation was detected. The patient was diagnosed with G. lucidum-induced aplastic crisis, and pancytopenia was treated with a transfusion of platelet concentrates and subcutaneous filgrastim injection. For the skin lesions of the palms and soles, topical corticosteroid and emollient creams were applied to alleviate the burning sensation. The patient was discharged after five weeks of systemic antifungal and antibacterial therapy, and his complete blood count was normalized at follow up. His hands and feet completely recovered and his scalp hair grew back completely. Routine laboratory tests were also performed for his wife and son, who had also been ingesting G. lucidum. His wife complained of fever and myalgia; however, no skin lesions of the palms or soles or hair loss were noted. Her body temperature was 39.0 °C and complete blood count revealed leucopenia of 830 cells/μL (neutrophils 4.2 %, lymphocytes 79.1 %, monocytes 0.7 %, eosinophils 8.8 %, and basophils 0.4 %), thrombocytopenia of 43,000 cells/μL, anemia of 8.8 g/dL, and reticulocyte count of 0.34 %. Other laboratory tests were unremarkable, with the exception of elevated liver enzyme levels with AST of 213 IU/L (normal range: M. J. Choi :D.-Y. Kim : S. B. Cho (*) Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, Korea e-mail: drsbcho@gmail.com

Re-exacerbation of cutaneous lesions in Bazex syndrome (acrokeratosis paraneoplastica) without tumor recurrence

ejd.2012.1877 Auteur(s) : Sachiko Ono1, Kenzo Takahashi2, Yoshiki Miyachi1, Kenji Kabashima1 kaba@kuhp.kyoto-u.ac.jp 1 Department of Dermatology, Kyoto School of Medicine 54 Shogoin-Kawara, Sakyo, Kyoto 606-8507, Japan 2 Department of Dermatology, Ryukyu School of Medicine, Okinawa 903-0215, Japan Bazex syndrome (acrokeratosis paraneoplastica) is a dermatosis characterized by acral psoriasiform lesions and palmoplantar keratoderma associated with an underlying neoplasm. The majority of associated [...]

Felsô légúti carcinomához társuló acrokeratosis paraneoplastica Bazex

Felsô légúti carcinomához társuló acrokeratosis paraneoplastica Bazex

Coexistence of papuloerythroderma of Ofuji and acrokeratosis paraneoplastica (Bazex syndrome) preceding the diagnosis of primary hepatocellular carcinoma

Coexistence of papuloerythroderma of Ofuji and acrokeratosis paraneoplastica (Bazex syndrome) preceding the diagnosis of primary hepatocellular carcinoma