Carbon dioxide laserabrasion: a new approach to management of familial benign chronic pemphigus (Hailey-Hailey disease). (medscape.com)
Kartamaa M, Reitamo S. Familial benign chronic pemphigus (Hailey-Hailey disease). (medscape.com)
Hailey-Hailey disease (HHD), or familial benign chronic pemphigus: 559 or familial benign pemphigus,: 622 was originally described by the Hailey brothers (Hugh Edward and William Howard) in 1939. (wikipedia.org)
Hailey-Hailey disease, or benign familial chronic pemphigus, is a rare autosomal dominant disease that can be difficult to diagnose due to wide variation in clinical features. (vohrawoundcare.com)
A chronic and relatively benign subepidermal blistering disease usually of the elderly and without histopathologic acantholysis. (uchicago.edu)
low mitotic rate TYPES ▪ Embryonic origin of GI tract (e.g. foregut, midgut, hindgut) Foregut tumors (e.g. stomach) ▪ Type I ▫ Most common ▫ Originates from enterochromaffin-like (ECL) cells ▫ In association with high gastrin levels secondary to chronic atrophic gastritis ▫ Small, usually benign ▪ Type II ▫ Originates from ECL cells ▫ In association with high gastrin levels induced by gastrinomas (e.g. (osmosis.org)
Hailey-Hailey9
Familial benign pemphigus (Hailey-Hailey disease) originally was described by the Hailey brothers in 1939. (medscape.com)
Hailey-Hailey disease, or familial benign pemphigus, is hypothesized to result from a genetic defect in a calcium pump protein. (medscape.com)
Acantholytic dermatosis of the crural folds may be a variant of Hailey-Hailey disease (familial benign pemphigus) and is also associated with ATP2C1 mutation. (medscape.com)
In addition to the primary gene defect in Hailey-Hailey disease (familial benign pemphigus), contributing factors are known that exacerbate the disease. (medscape.com)
Ruiz-Rodriguez R, Alvarez JG, Jaen P, Acevedo A, Cordoba S. Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease). (medscape.com)
citation needed] While the term pemphigus typically refers to "a rare group of blistering autoimmune diseases" affecting "the skin and mucous membranes", Hailey-Hailey disease is not an autoimmune disorder and there are no autoantibodies. (wikipedia.org)
Axillae: Consider inverse psoriasis, erythrasma, seborrheic dermatitis, irritant or allergic contact dermatitis from deodorants/shaving, benign familial pemphigus (Hailey-Hailey disease) , or axillary granuloma parakeratosis. (medscape.com)
Inframammillae: Consider inverse psoriasis, candidal infection, inflammatory metastatic breast cancer, Paget disease, or benign familial pemphigus (Hailey-Hailey disease). (medscape.com)
Other skin disorders that may be associated with eczema herpeticum include Darier disease, pemphigus foliaceus, and benign familial pemphigus (Hailey-Hailey disease). (racgp.org.au)
Pancreatic endocrine tumor, also known as pancreatic neuroendocrine tumor (PNET), describes a group of endocrine tumors originating in the pancreas that are usually indolent and benign, but may have the potential to be malignant. (findzebra.com)
Both benign and malignant tumors, which are not cancerous, can damage your peripheral nervous system. (ontariohomeopath.ca)
A group of disorders having a benign course but exhibiting clinical and histological features suggestive of malignant lymphoma. (lookformedical.com)
Pseudolymphoma is characterized by a benign infiltration of lymphoid cells or histiocytes which microscopically resembles a malignant lymphoma. (lookformedical.com)
Lesions3
Through ultrastructural studies of familial benign pemphigus lesions, characteristic changes in keratinocyte morphology have been described, including retracted tonofilaments, elongated membrane microvilli, and reduced numbers of desmosomes. (medscape.com)
Benign familial pemphigus lesions often begin during the teenage years and manifest as itchy and malodorous plaques. (medscape.com)
The lack of oral lesions and intercellular antibodies distinguishes familial benign pemphigus from other forms of pemphigus. (wikipedia.org)
Many hypotheses exist concerning the pathogenesis of familial benign pemphigus, but the cause remains uncertain. (medscape.com)
Vulgaris1
Characterization of skin cytokines in bullous pemphigoid and pemphigus vulgaris. (uchicago.edu)
Eczema1
Botulinum toxin has also been used experimentally in the treatment of many other dermatological conditions with positive results, including persistent facial flushing, gustatory sweating, anal fissures, familial benign pemphigus, dyshidrotic eczema, and surgical wound closures. (jcadonline.com)
Fabry1
Fabry disease and familial amyloidosis can both be treated. (ontariohomeopath.ca)
Topical1
Benign familial pemphigus Topical antibiotics such as or are used short term for localised infection but are best arcea-pradettes.com where Can I Get Triamcinolone Cheap a sexually transmitted disease std Velocity Max Legends Nightclub You must destroy all this Kingdom. (arcea-pradettes.com)
Tumors1
Solitary or multiple benign hepatic vascular tumors, usually occurring in women of 20-50 years of age. (lookformedical.com)
Botulinum1
Lopez-Ferrer A, Alomar A. Botulinum toxin A for the treatment of familial benign pemphigus. (medscape.com)
Disease2
I was diagnosed in 1991 with an immune system disease called " Pemphigus Benign Familial" - which in simple terms is a condition brought on by stress and causes blistering of the skin folds in most areas of your body. (algaecal.com)
Familial AN, drug-induced AN, AN occurring in hyperinsulinemic states (eg, diabetes, obesity), AN associated with polycystic ovary disease, and AN associated with a spectrum of autoimmune disease in women should be considered before AN is determined to represent a paraneoplastic syndrome. (medscape.com)
Patients2
Approximately two thirds of patients with familial benign pemphigus have a family history of the disorder. (medscape.com)
Patients with familial benign pemphigus live long and productive lives. (medscape.com)
Malignancies1
6] Case reports have described AN associated with hematologic malignancies, including acute myeloid leukemia, and even benign gastrointestinal neoplasms. (medscape.com)
Cases2
[ 4 ] . Study of familial and sporadic cases of familial benign pemphigus in the Chinese population has revealed a number of mutations scattered throughout the ATP2C1 gene. (medscape.com)
He collaborated on the identification, characterization and inheritance patterns of the first cases of Canine Benign Familial Pemphigus. (lancasterpetemergency.com)
Treatment1
Improvement of familial benign pemphigus after treatment with pulsed-dye laser: a case report. (medscape.com)
Multiple1
Solitary or multiple benign cutaneous nodules comprised of immature and mature vascular structures intermingled with endothelial cells and a varied infiltrate of eosinophils, histiocytes, lymphocytes, and mast cells. (lookformedical.com)
Vulgaris19
Differential histological characteristics of benign hereditary familial pemphigus, Darier's disease and chronic pemphigus vulgaris]. (nih.gov)
discussion on the relation of this disease to Darier's disease and chronic pemphigus vulgaris]. (nih.gov)
Pemphigus vulgaris in two MHC-haploidentical brothers. (nih.gov)
NEW YORK (Reuters Health) Jul 03 - Methotrexate allows discontinuation of steroids in many patients with pemphigus vulgaris, a retrospective study suggests. (medscape.com)
Methotrexate should be considered as a first-line adjuvant treatment option for patients with pemphigus vulgaris," Dr. Kathleen D. Tran from New York University Medical Center, New York told Reuters Health. (medscape.com)
Although methotrexate has been used for pemphigus vulgaris at least since 1968, only a few small studies have evaluated it, and none of them were randomized controlled trials. (medscape.com)
Dr. Tran and colleagues reviewed their experience in 23 patients with pemphigus vulgaris who were treated with methotrexate between 2001 and 2012, using its steroid-sparing effect as a marker of clinical improvement. (medscape.com)
I would like other physicians to understand that this inexpensive, widely-available drug is an effective, underutilized, underappreciated treatment option for pemphigus vulgaris" Dr. Tran said. (medscape.com)
Other systemic immunomodulators such as mycophenolate mofetil, dapsone, (and) azathioprine have all shown efficacy in pemphigus vulgaris," Dr. Tran added. (medscape.com)
It is unrelated to PEMPHIGUS VULGARIS though it closely resembles that disease. (nih.gov)
Welcome to the website of the Pemphigus Vulgaris Network, the United Kingdom support group for people living with Pemphigus and Mucous Membrane Pemphigoid. (pemphigus.org.uk)
Pemphigus Vulgaris (PV) is one of a group of rare, relapsing auto-immune diseases causing blistering of the skin and mucous membranes (eg mouth, nose, throat and genitals). (pemphigus.org.uk)
The Pemphigus Vulgaris Network is a voluntary, not-for-profit, group started in 1997 to provide information and support for people living with pemphigus and their relatives, friends and carers. (pemphigus.org.uk)
Cheques should be made out to the Pemphigus Vulgaris Network and sent c/o 14 Lime Court, Gayton Rd, Harrow HA1 2YD. (pemphigus.org.uk)
Pemphigus Vulgaris is rare and most general practitioners (GPs) have never seen it. (pemphigus.org.uk)
Direct immunofluorescence microscopy performed on epithelial biopsy specimen obtained from a patient with pemphigus vulgaris detects immunoglobulin G deposits at the epithelial cell surfaces. (medscape.com)
Erythematosus13
Pérez-Pérez et al hypothesized that pemphigus erythematosus is a multiple autoimmune disease. (medscape.com)
The lupus component of pemphigus erythematosus is demonstrated by circulating antinuclear antibodies (ANA) and sometimes by immunoglobulin and complement deposits at the dermoepidermal junction. (medscape.com)
[ 2 ] Patients with classic pemphigus erythematosus present with small, flaccid bullae with scaling and crusting. (medscape.com)
On the face, pemphigus erythematosus presents on the bridge of the nose and on the malar areas as in the butterfly distribution seen in LE. (medscape.com)
With extensive involvement, pemphigus erythematosus patients may present with an exfoliative erythroderma. (medscape.com)
Patients with pemphigus erythematosus do not typically develop mucous membrane involvement. (medscape.com)
In pemphigus erythematosus, select an early vesicle or bulla for skin biopsy. (medscape.com)
With direct immunofluorescence (see the image above) in pemphigus erythematosus, linear deposits of immunoglobulin G (IgG) and C3 are present in the intercellular space of the epidermis. (medscape.com)
With immunoelectron microscopy in pemphigus erythematosus, IgG and C3 deposits are localized to the epidermal cell membranes and the upper dermis. (medscape.com)
Enzyme-linked immunosorbent assay (ELISA) has also been suggested as a useful diagnostic method in suspected cases of pemphigus erythematosus with equivocal immunofluorescence results. (medscape.com)
Topical corticosteroids are useful for pemphigus erythematosus patients with limited disease or as an adjunct to systemic therapy. (medscape.com)
Griffies et al reported promising results in treating discoid lupus and pemphigus erythematosus in dogs with topical application of 0.1% tacrolimus, an immunomodulator produced by a fungus. (medscape.com)
Disorder2
Approximately two thirds of patients with familial benign pemphigus have a family history of the disorder. (medscape.com)
Pelger-Hüet anomaly is a benign inherited disorder. (remixeducation.in)
Pemphigoid1
In general, we find the best way we can help is if you also have a look at the International Pemphigus and Pemphigoid Foundation website and then see if you have any further questions or if there is anything you want clarified. (pemphigus.org.uk)
Sporadic1
[ 4 ] . Study of familial and sporadic cases of familial benign pemphigus in the Chinese population has revealed a number of mutations scattered throughout the ATP2C1 gene. (medscape.com)
Incidence1
No precise data are available on the incidence of familial benign pemphigus. (medscape.com)
Manifests1
Familial benign pemphigus often manifests in the late teenage years or in adulthood (30s and 40s). (medscape.com)
Mucous1
In pemphigus disorders the immune system makes a mistake, views cells in the skin and mucous membranes as foreign and an immune response is triggered. (pemphigus.org.uk)
Primary hyperparathyroidism is also a feature of several familial endocrine disorders: Multiple endocrine neoplasia type 1 and type 2A (MEN type 1 and MEN type 2A), and familial hyperparathyroidism. (the-medical-dictionary.com)
Primary1
Primary hyperparathyroidism causes hypercalcemia (elevated blood calcium levels) through the excessive secretion of parathyroid hormone (PTH), usually by an adenoma (benign tumors) of the parathyroid glands. (the-medical-dictionary.com)
A pemphigus that is characterized by recurring blistering most commonly occurring in the folds of the skin and has_material_basis_in mutations in the ATP2C1 gene that result in loss of adhesion within the skin. (jax.org)
Summary Background Epithelioid haemangioma (EH) arising from the skin is a benign vascular tumour with marked inflammatory. (cn1699.cn)
Share1
Sometimes people want to talk to another person with pemphigus just to share a common experience. (pemphigus.org.uk)