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Eosinophilic dermatosis

Andreas Benedikt Weins, Tilo Biedermann, Tina Weiss, Johannes Martin Weiss
A rare eosinophilic dermatosis, Wells syndrome, also referred to as eosinophilic cellulitis, is characterized by great clinical variability. Typical findings include infiltrated erythematous plaques arising on the extremities. Lesions initially resemble erysipelas/cellulitis, however, they do not improve with antibiotic treatment. Eosinophilic cellulitis is a diagnosis of exclusion that may only be made over the course of the disease, taking into account clinical and characteristic histological findings (flame figures)...
October 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
B Lozano-Masdemont, L Gómez-Recuero-Muñoz, A Pulido-Pérez, I Molina-López, R Suárez-Fernández
Hepatitis B virus (HBV) infection in pregnant women is very rare in western countries, thus, cutaneous manifestation of HBV infection may be confused with a dermatosis specific of pregnancy. We report a 39-year-old woman who presented in her 20th week of pregnancy with a pruritic rash, which consisted of generalized erythematous plaques, some of them with a purple centre. Serology testing showed acute HBV infection, and a biopsy revealed a superficial and interstitial perivascular inflammatory infiltrate of lymphocytes and eosinophils...
October 20, 2016: Clinical and Experimental Dermatology
S Goetze, A K Dumke, D Zillikens, U C Hipler, P Elsner
Anti-laminin γ1-pemphigoid (formerly known as anti-p200 pemphigoid) is a rare subepidermal autoimmunobullous disorder first described by Zillikens et al. in 1996 (1,2). Clinically, it mimics bullous pemphigoid, linear IgA dermatosis, dermatitis herpetiformis, pompholyx or the inflammatory variant of epidermolysis bullosa acquisita, respectively (1,3). The laminin γ1-chain has been identified as the major targeting antigen (1,4,5). Histologically, a subepidermal split is seen accompanied by neutrophils and eosinophils as well as linear deposits of IgG and/or C3 along the dermoepidermal junction in direct immunofluorescence microscopy (1)...
September 29, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
P S Jayasekera, A Bakshi, A Al-Sharqi
No abstract text is available yet for this article.
August 2016: Clinical and Experimental Dermatology
André Castro Pinho, José Carlos Cardoso, Miguel Gouveia, Hugo Oliveira
Elastosis perfurans serpiginosa is a rare perforating dermatosis found primarily in adolescents and young adults, characterized by transepidermal elimination of abnormal elastic fibers. The only drug known capable of inducing elastosis perfurans serpiginosa is D-penicillamine. We report the case of a 52 year-old woman with keratotic papules arranged in an annular pattern with central clearing and centrifugal growth, located in the anterior cervical region. The patient was chronically treated with D-penicillamine for Wilson disease...
March 2016: Acta Médica Portuguesa
Valerie H Chen, Hans E Grossniklaus, John M DelGaudio, Hee Joon Kim
Granuloma faciale is an eosinophilic dermatosis that typically presents as red-brown facial plaques of middle-aged Caucasian men, while eosinophilic angiocentric fibrosis is a histologically similar fibrotic condition affecting the respiratory mucosa. Both are very uncommon conditions and pose a therapeutic challenge. While a handful of reports have reported concurrent presentation, the authors present the first case of eyelid granuloma faciale with eosinophlilic angiocentric fibrosis.
June 8, 2016: Ophthalmic Plastic and Reconstructive Surgery
Christopher W Soon, Ilan R Kirsch, Andrew J Connolly, Bernice Y Kwong, Jinah Kim
The presence of eosinophils within the neutrophilic infiltrates of acute febrile neutrophilic dermatosis (Sweet syndrome) is documented in the literature. Here, the authors describe a case of eosinophil-rich acute febrile neutrophilic dermatosis in the setting of new onset enteropathy-associated T-cell lymphoma (EATL), type 1. Histopathologic evaluation of the skin biopsies demonstrated a mixed superficial perivascular and inflammatory infiltrate composed of neutrophils, lymphocytes, and abundant eosinophils...
September 2016: American Journal of Dermatopathology
Mohammad Ali Alshami
Juvenile colloid milium is an extremely rare degenerative dermatosis of the dermal connective tissue manifesting before puberty. This chronic skin condition, detected primarily in sun-exposed areas, is clinically characterized by the development of translucent, yellowish papules and plaques containing gelatinous material and by the presence of eosinophilic, fissured material in the papillary dermis. We present two cases of juvenile colloid milium, in a 13-year-old girl and her 10-year-old brother; the condition emerged in both at approximately 6 years of age and progressively worsened...
May 2016: Pediatric Dermatology
Takashi Nomura, Mayumi Katoh, Yosuke Yamamoto, Yoshiki Miyachi, Kenji Kabashima
Eosinophilic pustular folliculitis (EPF) is a sterile inflammatory dermatosis of unknown etiology. In addition to classic EPF, which affects otherwise healthy individuals, an immunocompromised state can cause immunosuppression-associated EPF (IS-EPF), which may be referred to dermatologists in inpatient services for assessments. Infancy-associated EPF (I-EPF) is the least characterized subtype, being observed mainly in non-Japanese infants. Diagnosis of EPF is challenging because its lesions mimic those of other common diseases, such as acne and dermatomycosis...
March 30, 2016: Journal of Dermatology
Lauren Penn, Ian Ahern, Adnan Mir, Shane A Meehan
Eosinophilic dermatosis of hematologic malignancy is a rare, paraneoplastic phenomenon that presents as a pruritic papular or vesicular eruption that is clinically and histopathologically similar to insect bites. We present a 56-year-old man with multiple relapses of diffuse large B cell lymphoma with a typical presentation of pruritic papules and vesicles on the extremities that correlate with a recent relapse of his lymphoma.
December 2015: Dermatology Online Journal
Takashi Nomura, Mayumi Katoh, Yosuke Yamamoto, Yoshiki Miyachi, Kenji Kabashima
Eosinophilic pustular folliculitis (EPF) is a non-infectious inflammatory dermatosis of unknown etiology that principally affects the hair follicles. There are three variants of EPF: (i) classic EPF; (ii) immunosuppression-associated EPF, which is subdivided into HIV-associated (IS/HIV) and non-HIV-associated (IS/non-HIV); and (iii) infancy-associated EPF. Oral indomethacin is efficacious, especially for classic EPF. No comprehensive information on the efficacies of other medical management regimens is currently available...
August 2016: Journal of Dermatology
Keisuke Goto, Sumiko Ishizaki, Keiji Tanese, Yoshimi Oshitani, Yo Kaku, Rie Kaneko, Hiromichi Takama, Misawo Ishikawa, Masaru Tanaka
Nonepisodic angioedema with eosinophilia (NEAE) is a rare condition characterized with monoepisodic angioedema, a nonfebrile state, eosinophilia, normal serum IgM levels, and lack of internal organ involvement. The histology of this disease is not yet well known. The purpose of this study was to characterize the histopathologic features of NEAE. Twelve cases of clinically confirmed NEAE were retrieved from 6 institutions, and these cases were reviewed regarding the clinical data and histopathology, particularly regarding granulomatous lesions...
February 2016: American Journal of Dermatopathology
Raquel Sucupira Andrade Lima, Gustavo Ávila Maquiné, Renato Cândido da Silva Junior, Antonio Pedro Mendes Schettini, Mônica Santos
Granuloma faciale is a rare dermatosis of chronic course, benign, usually asymptomatic, first described in 1945 by Wingley. It is characterized by the appearance of well-defined, single or multiple papules, plaques and nodules, predominantly located in sun-exposed areas, especially the face. In this work we report the case of a female patient, 58 years old, evolving for ten years with multiple erythematous-brownish and asymptomatic papules on the face, whose histological examination confirmed the diagnosis of granuloma faciale...
September 2015: Anais Brasileiros de Dermatologia
A M Manganoni, C Farisoglio, L Pavoni, M Chiudinelli, G Stillitano, M Ungari, P Calzavara-Pinton
We report a case of a healthy 26-year-old male with multiple asymptomatic reddish papules and papule-nodules on the central area of the face, persisting from more than 2 months and gradually increasing in number. An incisional skin biopsy revealed a confluent dense granulomatous infiltrate centred by large areas of eosinophilic necrosis consistent with the diagnosis of lupus miliaris disseminatus faciei. This is a rare dermatosis first described in 1878 by Fox, that often poses a clinical challenge as it is a disease process which is difficult to diagnose...
September 8, 2015: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Maxence Cormerais, Florence Poizeau, Laure Darrieux, Laurent Tisseau, Gilles Safa
Wells' syndrome (WS), or eosinophilic cellulitis, is an uncommon inflammatory dermatosis of unknown etiology that typically presents with pruritic cellulitis-like plaques on the extremities. Therefore, WS is often misdiagnosed as bacterial cellulitis due to its similarity in presentation. Here, we report two cases of WS that masqueraded as bacterial facial cellulitis. Under treatment with oral prednisone and/or a combination therapy with levocetirizine and hydroxyzine, both patients showed a dramatic improvement of the skin lesions...
May 2015: Case Reports in Dermatology
Antonio Torrelo, Isabel Colmenero, Luis Requena, Amy S Paller, Yuval Ramot, Chyi-Chia Richard Lee, Angel Vera, Abraham Zlotogorski, Raphaela Goldbach-Mansky, Heinz Kutzner
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a newly characterized autoinflammatory disorder, caused by mutations in PSMB8. It is characterized by early-onset fevers, accompanied by a widespread, violaceous, and often annular cutaneous eruption. Although the exact pathogenesis of this syndrome is still obscure, it is postulated that the inflammatory disease manifestations stem from excess secretion of interferons. Based on preliminary blood cytokine and gene expression studies, the signature seems to come mostly from type I interferons, which are proposed to lead to the recruitment of immature myeloid cells into the dermis and subcutis...
July 2015: American Journal of Dermatopathology
Burhan Engin, Zekayi Kutlubay, Emine Erkan, Yalçın Tüzün
Darier disease, also known as Darier-White disease, is characterized by yellow to brown, oily keratotic papules and plaques in the seborrheic areas of the face and chest. This disorder may show different clinical manifestations, such as palmoplantar pits and nail abnormalities. The trigger factors are mechanical trauma, heat, humidity, ultraviolet B, and pyogenic infections. The disease usually becomes apparent in the second decade of life. The ATP2 A2 (SERCA2) gene mutation was detected in all patients. Histopathologic changes include epidermal adhesion loss, acantholysis, abnormal keratinization, eosinophilic dyskeratotic cells in the spinous layer known as corps ronds, and the presence of grains in the stratum corneum...
July 2015: Clinics in Dermatology
L Thomas, S Fatah, S Nagarajan, S Natarajan
Eosinophilic annular erythema (EAE) is a rare and relatively newly described eosinophil-rich dermatosis. Debate still exists as to whether it represent a subtype of Well syndrome or a separate disease entity. We report an 8-year-old boy with a 4-year history of recurrent, asymptomatic annular lesions, which were diagnosed after clincopathological correlation as EAE. This condition usually runs a relapsing and remitting course with resistance to multiple treatments. Prednisolone and hydroxychloroquine have been reported as successful but the response to these was limited in this case...
December 2015: Clinical and Experimental Dermatology
Sabrina M Smith, Elizabeth A Kiracofe, Lindsey N Clark, Alejandro A Gru
IMPORTANCE: Wells syndrome (WS) (eosinophilic cellulitis) is an uncommon eosinophilic dermatitis that has been rarely described in association with, but distinct from, hypereosinophilic syndrome (HES). OBSERVATIONS: We report a case of an eosinophilic dermatosis with flame figures in association with idiopathic HES, manifested by inflammatory myocarditis, asthma, and peripheral blood eosinophilia. CONCLUSIONS AND RELEVANCE: The diagnoses of WS and HES, rather than being distinct findings, may represent 2 entities on a spectrum of hypereosinophilic diseases...
December 2015: American Journal of Dermatopathology
A Nouchi, G Hickman, M Battistella, E Estève, M Bagot, D Vignon-Pennamen, A Petit
BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare disease, currently considered a benign vascular proliferation of unknown etiology, and whose treatment is still unclear and challenging. PATIENTS AND METHODS: Two women in their thirties consulted for itchy lesions of the right ear. Both presented with a reddish bleeding papulonodular infiltration of the auricle, with a nodule at the entrance to the external auditory canal in the first patient...
May 2015: Annales de Dermatologie et de Vénéréologie
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