225 results match your criteria Acrokeratosis Verruciformis of Hopf
Acta Derm Venereol 2018 Nov 14. Epub 2018 Nov 14.
Deparment of Dermatology, University of Oulu, FIN-90029 Oulu, Finland.
Hautarzt 2018 Oct;69(Suppl 1):42-44
Klinik und Poliklinik für Dermatologie und Allergologie der Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland.
JAAPA 2018 Jun;31(6):30-33
Megan L. Adelman practices at the Ohio State University's Comprehensive Cancer Center, Arthur G. James Cancer Hospital and Richard J. Solove Research Institute, in Columbus, Ohio. Quoc-Anh Ho is a medical student at the University of California at Davis in Sacramento, Calif. Amit Agrawal is an associate professor in the Department of Otolaryngology at the Ohio State University Hospital in Columbus. The authors have disclosed no potential conflicts of interest, financial or otherwise.
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. Read More
Intern Med 2018 May 27;57(10):1501-1502. Epub 2017 Dec 27.
Department of General Internal Medicine, Nerima Hikarigaoka Hospital, Japan.
Ear Nose Throat J 2017 Oct-Nov;96(10-11):413-414
Department of Otolaryngology and Head and Neck Surgery, RHMS Baudour, EpiCURA Hospital, Rue Louis Cathy, Baudour, Belgium.
QJM 2018 Jan;111(1):63-64
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Cutis 2017 08;100(2):E3-E5
Department of Dermatology, Hospital Universitario Gregorio Marañón, Madrid, Spain.
Rev Med Interne 2017 Sep 21. Epub 2017 Sep 21.
Service de médecine interne, CHU Carémeau Nîmes, place du Professeur-Robert-Debré, 30000 Nîmes, France.
J Am Acad Dermatol 2017 08;77(2):e33-e35
Department of Pathology, JIPMER, Puducherry, India.
Am J Med Genet A 2017 May 12. Epub 2017 May 12.
University of Newcastle, NSW, Australia.
Darier disease and Acrokeratosis Verruciformis of Hopf (AKV) are rare disorders of keratinization with autosomal dominant inheritance and very distinct clinical pictures. Both have been shown to be caused by mutations in ATP2A2 (ATPase, Ca transporting, cardiac muscle, slow-twitch) a gene encoding one of the SERCA (sarcoplasmic/endoplasmic reticulum calcium ATPase2) intracellular pumps with a crucial role in cell-to-cell adhesion in both skin and heart. While hundreds of different missense and nonsense mutations cause Darier disease, only one missense mutation, p. Read More
Am J Dermatopathol 2017 May;39(5):370-373
*Divisions of Dermatopathology and Dermatology, Department of Pathology, Albany Medical College, Albany, NY; †Division of Dermatology, Upper Hudson Valley Dermatology, Castleton, NY; and ‡Departments of Dermatology, Microbiology/Medical Genetics, and Internal Medicine, University of Texas Health Science Center, Houston, TX.
The co-existence of Darier disease (DD) and acrokeratosis verruciformis of Hopf (AKV) has been noted for decades and the relationship between the 2 entities remains controversial. Although, it has been shown that both diseases are associated with mutations in ATPA2 gene, it is yet to be determined if they are the same disease, or separate but allelic, or interlinked in some other fashion. Herein, the authors report the case of a 13-year-old girl presenting with shiny flat-topped verruca plana-like papules, on the dorsal hands and feet and red-brown crusted papules on her forehead and along the sides of her neck. Read More
J Eur Acad Dermatol Venereol 2017 Jul 19;31(7):1119-1136. Epub 2017 Apr 19.
Klinik für Hautkrankheiten, Universitätsklinikum, Jena, Deutschland.
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. Read More
Case Rep Dermatol Med 2016 22;2016:7137691. Epub 2016 Dec 22.
Karmanos Cancer Institute, Wayne State University, 4100 John R St., Detroit, MI 48201, USA.
. 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. Read More
Case Rep Dermatol 2016 Sep-Dec;8(3):358-362. Epub 2016 Dec 8.
Department of Dermatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
An 82-year-old Japanese man was referred for detailed examination of hyperkeratotic erythematous plaques on his palms and soles for 6 months. Two weeks before his first visit, he had undergone lung lobectomy for right lung squamous cell carcinoma (SCC). Laboratory findings showed elevations of eosinophil counts, serum IgE, thymus and activation-regulated chemokine, SCC antigen, and soluble interleukin-2 receptor levels. Read More
Hum Mutat 2017 04 15;38(4):343-356. Epub 2017 Feb 15.
Departments of Dermatology, Maastricht University Medical Centre, Maastricht, The Netherlands.
The two disorders of cornification associated with mutations in genes coding for intracellular calcium pumps are Darier disease (DD) and Hailey-Hailey disease (HHD). DD is caused by mutations in the ATP2A2 gene, whereas the ATP2C1 gene is associated with HHD. Both are inherited as autosomal-dominant traits. Read More
Clin Dermatol 2017 Jan - Feb;35(1):50-54. Epub 2016 Sep 10.
Department of Dermatology, Akdeniz University, Faculty of Medicine, Antalya, Turkey. Electronic address:
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. Read More
An Bras Dermatol 2016 Sep-Oct;91(5):639-641
Instituto Lauro de Souza Lima (ILSL) - Bauru, SP, Brazil.
A 54 year-old woman with a 3-year history of rheumatoid arthritis (RA) consulted us because of weight loss, fever and skin eruption. On physical examination, erythematous plaques with a pseudo-vesicular appearance were seen on the back of both shoulders. Histological examination was consistent with rheumatoid neutrophilic dermatosis (RND). Read More
Indian J Dermatol 2016 Sep-Oct;61(5):482-6
Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal, India.
A pigmented variant of lichen planus (LP) was first reported from India in 1974 by Bhutani et al. who coined the term LP pigmentosus (LPP) to give a descriptive nomenclature to it. LP has a number of variants, one of which is LPP. Read More
Clin Exp Dermatol 2016 Oct;41(7):761-3
Department of Pathology, Dicle University, Diyarbakir Faculty of Medicine, Turkey.
The relationship between acrokeratosis verruciformis (AVH; also known as Hopf disease) and Darier disease (DD) has been debated for several decades. There is still substantial controversy over the characterization and association of AVH with DD. Certain histopathological features overlapping with those of DD have been demonstrated in patients with AVH. Read More
Semin Oncol 2016 06 23;43(3):366-9. Epub 2016 Feb 23.
University of Louisville, Division of Dermatology, Louisville, KY. Electronic address:
Skin findings can serve as a clue to internal disease. In this article, cutaneous manifestations of underlying lung malignancy are reviewed. Paraneoplastic dermatoses are rare, but when recognized early, can lead to early diagnosis of an underlying neoplasm. Read More
Indian J Dermatol 2016 Mar-Apr;61(2):237
Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad, Gujarat, India. E-mail:
Intern Med J 2016 Jan;46(1):119-20
Flinders Medical Centre, Adelaide, South Australia, Australia.
Dermatol Online J 2016 Sep 15;22(9). Epub 2016 Sep 15.
1The Ohio State University Department of Internal Medicine, The Ohio State University College of Medicine, Columbus.
BACKGROUNDAcrokeratosis paraneoplastica (Bazex Syndrome) is a rare paraneoplastic syndrome and dermatosis that only arises in patients with underlying malignancy and uncommonly resolves with systemic therapy.OBJECTIVE/METHODSWe present a patient with acrokeratosis paraneoplastica that improved significantly with acitretin. We present evidence to justify costs of therapy for insurance purposes. Read More
N Engl J Med 2015 Nov;373(22):2161
Maastricht University Medical Center, Maastricht, the Netherlands
BMC Cancer 2015 Jul 8;15:507. Epub 2015 Jul 8.
Department of Gynaecology, University Hospital Zurich, Frauenklinikstrasse, 8091, Zurich, Switzerland.
Background: 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. Read More
Indian Dermatol Online J 2015 Mar-Apr;6(2):110-2
Department of Skin and V.D., Pramukhswami Medical College, Karamsad, Anand, Gujarat, India.
Acrokeratosis verruciformis (AKV) of Hopf is an autosomal dominant genodermatosis with unknown etiology. It is characterized by multiple flat-topped keratotic papules resembling planar warts located mainly on the dorsum of hands and feet. Superficial ablation is the treatment of choice. Read More
JAMA Dermatol 2015 Jun;151(6):677-8
Division of Hematology and Oncology, Department of Medicine, Medical College of Wisconsin, Milwaukee.
J Eur Acad Dermatol Venereol 2016 Apr 26;30(4):695-7. Epub 2015 Jan 26.
Academy of Scientific and Innovative Research (AcSIR), CSIR-IGIB South Campus, Delhi, 110020, India.
Hautarzt 2015 Jul;66(7):542-4
Dermatologische Ordination, Thurnfeldgasse 3a, 6060, Hall in Tirol, Österreich,
Background: Bazex's syndrome is a rare paraneoplasia that is usually associated with cancer.
Diagnosis: Characteristic for the disease are diffuse, psoriasis-like, acral erythema, distal hyperkeratosis and onychodystrophy. Differential diagnoses comprise psoriasis, eczema and tinea. Read More
Indian Dermatol Online J 2014 Nov;5(Suppl 1):S17-9
Department of Dermatology, Dr. Panjabrao Deshmukh Memorial Medical College, Amravati, Maharashtra, India.
Acrokeratosis verruciformis of Hopf (AKV) is a rare disorder of keratinization inherited in an autosomal dominant fashion. A 15-year-old female presented with numerous skin-colored papular lesions over the neck as well as dorsa of the hands and feet of 7 years duration. Similar lesions were noted in her mother. Read More
Case Rep Med 2014 11;2014:351065. Epub 2014 Sep 11.
Department of Internal Medicine, Tzaneio General Hospital of Piraeus, 1 Zanni & Afentouli Street, 185 36 Piraeus, Greece.
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. Read More
Clin Exp Dermatol 2014 Dec 28;39(8):955-6. Epub 2014 Jul 28.
St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Cutis 2014 Jun;93(6):E3-5
Changi General Hospital, 2 Simei St 3, Singapore 529889.
N Z Med J 2014 Feb 14;127(1389):86-7. Epub 2014 Feb 14.
General Medicine, Flinders Private Hospital, Flinders Drive, Bedford Park, South Australia 5042, Australia.
An Bras Dermatol 2013 Nov-Dec;88(6 Suppl 1):209-11
Instituto de pesquisa e ensino medico, Rio de JaneiroRJ, Brazil.
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. Read More
Rev Clin Esp (Barc) 2014 Jan-Feb;214(1):52-3. Epub 2013 Nov 23.
Servicio de Medicina Interna, Hospital Comarcal de La Axarquía, Vélez-Málaga, Málaga, España.
Presse Med 2013 Dec 21;42(12):1582-95. Epub 2013 Oct 21.
UMR 1352, université François-Rabelais, INRA, infectiologie et santé publique, CHRU de Tours, service de dermatologie, 37044 Tours cedex, France. Electronic address:
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. Read More
Indian J Dermatol 2013 Sep;58(5):406
Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad, Gujarat, India.
Acrokeratosis verruciformis of Hopf (AKV) is a rare genodermatosis presenting as multiple plane wart-like lesions symmetrically distributed on dorsum of hands and feet. Its pathogenesis is unknown. A case of 24 years old female is described with multiple hyperpigmented, hyperkeratotic papules on the left side of body since 5 years along the lines of Blaschko. Read More
Acta Otorrinolaringol Esp 2014 Mar-Apr;65(2):138-9. Epub 2013 Jan 4.
Servicio de Otorrinolaringología, Complejo Hospitalario Universitario, A Coruña, España.
J Dermatol 2013 Apr 5;40(4):280-1. Epub 2013 Jan 5.
Dermatol Online J 2012 Dec 15;18(12):12. Epub 2012 Dec 15.
The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, USA.
We present a case of acrokeratosis verruciformis of Hopf in a 58-year-old woman with multiple, skin-colored, flat-topped, hyperkeratotic papules on the dorsal aspects of the hands and feet. Her lesions appeared at age 13 and her son developed similar lesions at about the same age. Acrokeratosis verruciformis of Hopf is a rare genodermatosis, which is distinct from keratosis follicularis; sporadic cases have also been described. Read More
Eur J Dermatol 2012 Nov-Dec;22(6):809-11
Am J Dermatopathol 2012 Aug;34(6):597-601
Department of Dermatology, Rambam Medical Center, Bruce Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel.
The relationship between acrokeratosis verruciformis (AKV) of Hopf and Darier disease (DD) has been debated for several decades. Both diseases are now thought to result from mutations in the same gene, that is, the ATP2A2 gene encoding the sarco (endo) plasmic reticulum Ca ATPase2 pump (SERCA2), although their histopathological features are different. We sought to detect possible overlapping histopathological features between AKV and DD. Read More
Singapore Med J 2012 Jan;53(1):e12-4
Department of Dermatology, Changi General Hospital, 2 Simei Street 3, Singapore.
Acrokeratosis paraneoplastica of Bazex is a rare but important paraneoplastic dermatosis, usually manifesting as psoriasiform rashes over the acral sites. It often precedes diagnosis of the associated malignancy, usually that of upper aerodigestive tract squamous cell carcinoma. We present the case of a patient with a newly diagnosed pyriform sinus tumour and associated acrokeratosis paraneoplastica. Read More
Hautarzt 2012 Jun;63(6):477-9
Klinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland.
Acrokeratosis paraneoplastica Bazex is a rare, obligate paraneoplasia initially presenting with palmoplantar hyperkeratosis. Later stages show acral psoriasiform lesions on other parts of the body. Common associated malignancies are laryngeal cancer and other tumors of the head or neck region or neck lymph node metastases. Read More
Ann Dermatol 2011 Sep 30;23 Suppl 1:S61-3. Epub 2011 Sep 30.
Department of Dermatology, The Catholic University of Korea School of Medicine, Seoul, Korea.
Acrokeratosis verruciformis of Hopf is a rare genodermatosis with an autosomal dominant mode of inheritance. It is a disorder of keratinization, characterized by multiple, flat-topped, skin-colored keratotic lesions resembling plane warts typically observed on the dorsum of the hands and feet. Histopathologically, the lesion shows considerable hyperkeratosis, acanthosis, and papillomatosis, mimicking a "church spire", and a thickened granular layer. Read More
Int J Dermatol 2011 Nov;50(11):1393-6
Department of Dermatology, Nihon University School of Medicine, Tokyo, Japan.
Am J Clin Dermatol 2011 Aug;12(4):217-31
The University of Houston Health Center, Texas, USA.
Longitudinal erythronychia is a linear red band on the nail plate that originates at the proximal nail fold, traverses the lunula, and extends to the free edge of the nail plate. Longitudinal erythronychia is classified based upon the number of nails affected and the number of red streaks present on each nail as follows: type Ia (monodactylous - single band), type Ib (monodactylous - bifid bands), type IIa (polydactylous - single band), and type IIb (polydactylous - multiple bands). Associated morphologic findings that can be present at the distal tip of the nail with longitudinal erythronychia include fragility, onycholysis, splinter hemorrhage, splitting, subungual keratosis, thinning, and V-shaped nick. Read More
J Radiol Case Rep 2011 1;5(7):1-6. Epub 2011 Jul 1.
Department of Radiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
Acrokeratosis paraneoplastic (Bazex syndrome) is a rare, but distinctive paraneoplastic dermatosis characterized by erythematosquamous lesions located at the acral sites and is most commonly associated with carcinomas of the upper aerodigestive tract. We report a 58-year-old female with a history of a pigmented rash on her extremities, thick keratotic plaques on her hands, and brittle nails. Chest imaging revealed a right upper lobe mass that was proven to be small cell lung carcinoma. Read More