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A rare odontogenic tumor characterized by aggressive clinical course and local destruction, occurring in mandible more often than in maxilla. The most common symptom is a rapidly progressing painful swelling, but it may present as a benign cystic lesion or as a large, rapidly growing mass with ulceration, bone resorption and teeth mobility, as well. The tumor may metastasize, most commonly to the cervical lymph nodes and the lungs.
Advances in Experimental Medicine and Biology. 589 . pp. 213–34 . doi : 10.1007/978-0-387-46954-6_14 . ISBN 978-0-387-35136-0 . ... Noack; Trainor, Paul A. (2014), "Neurocristopathies", Neural Crest Cells , Elsevier, pp. 361–394, doi : 10.1016/b978-0-12-401730-6.00018-1 , ISBN 9780124017306 ^ Cerrizuela, Santiago; Vega‐Lopez, Guillermo A.; Aybar, Manuel J. (2020). ... Inflammopharmacology . 18 (6): 265–90. doi : 10.1007/s10787-010-0054-4 . PMID 20862553 . S2CID 11711382 .
A number sign (#) is used with this entry because pachyonychia congenita-4 (PC4) is caused by heterozygous mutation in the KRT6B gene (148042) on chromosome 12q13. Description Pachyonychia congenita (PC) is an autosomal dominant genodermatosis with the main clinical features of hypertrophic nail dystrophy, painful and highly debilitating plantar keratoderma, oral leukokeratosis, and a variety of epidermal cysts. Although the condition had previously been subdivided clinically into Jadassohn-Lewandowsky PC type 1 and Jackson-Lawler PC type 2, patients with PC were later found to have a mixed constellation of both types, leading to a classification of PC based on genotype (summary by Sybert, 2010; Eliason et al., 2012; McLean et al., 2011). For a discussion of genetic heterogeneity of pachyonychia congenita, see 167200. Historical Classification of Pachyonychia Congenita Gorlin et al. (1976) suggested that 2 distinct syndromes are subsumed under the designation pachyonychia congenita.
Pachyonychia congenita (PC) is a rare genodermatosis predominantly featuring painful palmoplantar keratoderma, thickened nails, cysts and whitish oral mucosa. Epidemiology The prevalence is not known but approximately 1000 patients have been registered to date worldwide. Clinical description PC presents clinically as a spectrum of conditions. PC onset is variable with most cases manifesting soon after birth, others becoming clinically apparent only in late childhood and rarely in adulthood. The first signs of the disease usually are thickened nails or neonatal teeth.
Pachyonychia congenita (PC) is a rare inherited condition that primarily affects the nails and skin. The fingernails and toenails may be thickened and abnormally shaped . Affected people can also develop painful calluses and blisters on the soles of their feet and less frequently on the palms of their hands ( palmoplantar keratoderma ). Additional features include white patches on the tongue and inside of the mouth (leukokeratosis); bumps around the elbows, knees, and waistline (follicular hyperkeratosis); and cysts of various types including steatocystoma. Features may vary among affected people depending on their specific mutation.
For a phenotypic description and a discussion of genetic heterogeneity of pachyonychia congenita, see 167200. Inheritance Chong-Hai and Rajagopalan (1977) suggested autosomal recessive inheritance of pachyonychia congenita in a 4-year-old Malaysian girl with first-cousin parents, although they recognized new dominant mutation as a possibility. See also Sivasundram et al. (1985). INHERITANCE - Autosomal recessive HEAD & NECK Mouth - No oral leukoplakia SKIN, NAILS, & HAIR Skin - Horny papules (face, leg, buttocks) - No palmoplantar hyperkeratosis - No hyperhidrosis Nails - Episodic inflammatory swelling of nail bed - Recurrent shedding of nails - Hard,thickened nails (pachyonychia) - Subungual hyperkeratosis MISCELLANEOUS - See also pachyonychia congenita, type 3 (PC1, 167200 ) ▲ Close
A number sign (#) is used with this entry because pachyonychia congenita-3 (PC3) is caused by heterozygous mutation in the keratin-6a gene (KRT6A; 148041) on chromosome 12q13. Description Pachyonychia congenita (PC) is an autosomal dominant genodermatosis with the main clinical features of hypertrophic nail dystrophy, painful and highly debilitating plantar keratoderma, oral leukokeratosis, and a variety of epidermal cysts. Although the condition had previously been subdivided clinically into Jadassohn-Lewandowsky PC type 1 and Jackson-Lawler PC type 2, patients with PC were later found to have a mixed constellation of both types, leading to a classification of PC based on genotype (summary by Sybert, 2010; Eliason et al., 2012; McLean et al., 2011). For a discussion of genetic heterogeneity of pachyonychia congenita, see 167200. Historical Classification of Pachyonychia Congenita Gorlin et al. (1976) suggested that 2 distinct syndromes are subsumed under the designation pachyonychia congenita.
A number sign (#) is used with this entry because of evidence that pachyonychia congenita-2 (PC2) is caused by heterozygous mutation in the KRT17 gene (148069) on chromosome 17q21. Description Pachyonychia congenita (PC) is an autosomal dominant genodermatosis with the main clinical features of hypertrophic nail dystrophy, painful and highly debilitating plantar keratoderma, oral leukokeratosis, and a variety of epidermal cysts. Although the condition had previously been subdivided clinically into Jadassohn-Lewandowsky PC type 1 and Jackson-Lawler PC type 2, patients with PC were later found to have a mixed constellation of both types, leading to a classification of PC based on genotype (summary by Sybert, 2010; Eliason et al., 2012; McLean et al., 2011). For a discussion of genetic heterogeneity of pachyonychia congenita, see 167200. Historical Classification of Pachyonychia Congenita Gorlin et al. (1976) suggested that 2 distinct syndromes are subsumed under the designation pachyonychia congenita.
A number sign (#) is used with this entry because pachyonychia congenita-1 (PC1) is caused by heterozygous mutation in the keratin-16 gene (KRT16; 148067) on chromosome 17q21. Description Pachyonychia congenita (PC) is an autosomal dominant genodermatosis with the main clinical features of hypertrophic nail dystrophy, painful and highly debilitating plantar keratoderma, oral leukokeratosis, and a variety of epidermal cysts. Although the condition had previously been subdivided clinically into Jadassohn-Lewandowsky PC type 1 and Jackson-Lawler PC type 2, patients with PC were later found to have a mixed constellation of both types, leading to a classification of PC based on genotype (summary by Sybert, 2010; Eliason et al., 2012; McLean et al., 2011). Historical Classification of Pachyonychia Congenita Gorlin et al. (1976) suggested that 2 distinct syndromes are subsumed under the designation pachyonychia congenita. PC type 1, the Jadassohn-Lewandowsky type, shows oral leukokeratosis.
Diabetes mellitus is the most common risk factor involved. [2] Contents 1 Types 2 Signs and symptoms 3 Pathophysiology 4 Diagnosis 5 Treatment 6 Epidemiology 7 See also 8 References 9 Further reading 10 External links Types [ edit ] Granuloma [3] The types of fungal sinusitis are based on invasive and non-invasive as follows: [4] [5] Invasive Acute fulminant Chronic invasive Granulomatous Non Invasive Saprophytic infection Sinus fungal ball Eosinophil related FRS including AFRS Signs and symptoms [ edit ] Individuals with the condition of fungal sinusitis mostly present with features that include facial pain and pain around the eyes, nasal congestion , rhinorrhea (running nose), headache , later there may be ophthalmoplegia (paralysis of ocular muscles). [1] Pathophysiology [ edit ] The mechanism of fungal sinusitis depends on which form, such as: Acute fulminant form – the fungus invades into vessels causing thrombosis, necrosis with minimum inflammation [3] Chronic invasive – fungal hyphae invades tissue leaving necrosis with minimal inflammation [3] Granulomatous form – invasive hyphae invades tissue with inflammation and non-caseating granuloma (with foreign bodies). [3] Saprophytic infection – growth of fungus seen on mucous crusts within sinus cavity. [3] Sinus fungal ball – sequestration of fungal hyphae as densely tangled, and has gritty matted appearance. [3] Eosinophil related Allergic fungal sinusitis – though not completely understood, a possible mechanism sees the protein component of fungus elicits IgE mediated allergic mucosal inflammation. [6] Diagnosis [ edit ] In terms of diagnosis, the clinical examination gives an idea about fungal sinusitis, [5] as well as: MRI Suggestive clinical features include - multiple recurrent episodes, persistent pathology , and absent ability to smell (the Eustachian tube may also be affected). [5] X Ray - can be done if the diagnosis is not certain. [5] CT – can document the presence of sinusitis, in the coronal views [1] MRI – used to find the CNS spread (extent of the disease), to evaluate individuals who demonstrate signs of invasive fungal sinusitis [1] Histology studies [1] Treatment [ edit ] Voriconazole Treatment for fungal sinusitis can include surgical debridement; helps by slowing progression of disease thus allowing time for recovery [7] additionally we see the options below: In cases where the fungus has invaded the sinus tissue, echinocandins , oral voriconazole , and I.V amphoterecin may be used [8] For allergic fungal sinusitis, systemic corticosteroids like prednisolone , methylprednisolone are added for their anti-inflammatory effect, bronchodilators and expectorants help to clear secretions in the sinuses. [ medical citation needed ] Epidemiology [ edit ] Though it is widely held that fungal infections of the nose and paranasal sinuses are not common, most agree that their frequency has been increasing over past decades. [9] See also [ edit ] Granuloma References [ edit ] ^ a b c d e f g h i "Fungal Sinusitis: Background, History of the Procedure, Problem" . eMedicine . 28 June 2016 . ... Indian Journal of Otolaryngology Head and Neck Surgery . 62 (4): 381–5. doi : 10.1007/s12070-010-0062-0 . PMC 3266098 . PMID 22319697 . ... Indian Journal of Otolaryngology and Head & Neck Surgery . 62 (4): 381–385. doi : 10.1007/s12070-010-0062-0 . ISSN 2231-3796 . PMC 3266098 . ... External links [ edit ] Classification D ICD - 10 : J30.89 External resources eMedicine : Sinusitis/863062-overview Fungal Sinusitis/863062 Scholia has a topic profile for Fungal sinusitis . v t e Medicine Specialties and subspecialties Surgery Cardiac surgery Cardiothoracic surgery Colorectal surgery Eye surgery General surgery Neurosurgery Oral and maxillofacial surgery Orthopedic surgery Hand surgery Otolaryngology ENT Pediatric surgery Plastic surgery Reproductive surgery Surgical oncology Transplant surgery Trauma surgery Urology Andrology Vascular surgery Internal medicine Allergy / Immunology Angiology Cardiology Endocrinology Gastroenterology Hepatology Geriatrics Hematology Hospital medicine Infectious disease Nephrology Oncology Pulmonology Rheumatology Obstetrics and gynaecology Gynaecology Gynecologic oncology Maternal–fetal medicine Obstetrics Reproductive endocrinology and infertility Urogynecology Diagnostic Radiology Interventional radiology Nuclear medicine Pathology Anatomical Clinical pathology Clinical chemistry Cytopathology Medical microbiology Transfusion medicine Other Addiction medicine Adolescent medicine Anesthesiology Dermatology Disaster medicine Diving medicine Emergency medicine Mass gathering medicine Family medicine General practice Hospital medicine Intensive care medicine Medical genetics Narcology Neurology Clinical neurophysiology Occupational medicine Ophthalmology Oral medicine Pain management Palliative care Pediatrics Neonatology Physical medicine and rehabilitation PM&R Preventive medicine Psychiatry Addiction psychiatry Radiation oncology Reproductive medicine Sexual medicine Sleep medicine Sports medicine Transplantation medicine Tropical medicine Travel medicine Venereology Medical education Medical school Bachelor of Medicine, Bachelor of Surgery Bachelor of Medical Sciences Master of Medicine Master of Surgery Doctor of Medicine Doctor of Osteopathic Medicine MD–PhD Related topics Alternative medicine Allied health Dentistry Podiatry Pharmacy Physiotherapy Molecular oncology Nanomedicine Personalized medicine Public health Rural health Therapy Traditional medicine Veterinary medicine Physician Chief physician History of medicine Book Category Commons Wikiproject Portal Outline
Greenwood Publishing Group. ISBN 978-0-313-31520-6 , p. 402. ^ Miller, Laura. (2006). ... University of California Press. ISBN 978-0-520-24509-9 ^ Latteier 1998 ^ Buss, David (2019). ... Dazed . Archived from the original on 10 August 2017 . Retrieved 20 November 2014 . ... El Rio, TX: Hauck Pub Co. ISBN 978-0-9621797-2-3 . ^ McDonough, Jimmy (2005). ... The Breast Fetish (pg. 29). Palgrave Macmillan. ISBN 0-312-22129-0 . Glazier, Stephen D. ; Flowerday, Charles (2003).
Pearson Longman. ISBN 978-1-4058-8118-0 . ^ https://medical-dictionary.thefreedictionary.com/epicanthus . ... Granby, Mass: Bergin & Garvey. ISBN 978-0-89789-166-0 . ^ Angel, J. Lawrence (1963). ": The Physical Anthropology of Ceylon. ... PMID 13905256 . ^ Lang, Berel (ed.) (2000) Race and Racism in Theory and Practice , Rowman & Littlefield, p. 10 ^ Lang, Berel (ed.) (2000) Race and Racism in Theory and Practice , Rowman & Littlefield, pp. 10-11 ^ Park JI (1 January 2000). ... New York: McGraw-Hill Medical. pp. Chapter 2. ISBN 978-0-07-162167-0 . CS1 maint: extra text: authors list ( link ) ^ Pham, V. (2010). ... Indian Journal of Pediatrics . 77 (10): 1151–1152. doi : 10.1007/s12098-010-0199-6 .
Wheeless' Textbook of Orthopedics @ wheelessonline.com . Retrieved 2009-10-11 . ^ Cary D. Alberstone; Michael Steinmetz; Edward C. ... Anatomic Basis of Neurologic Diagnosis . Thieme. pp. 105–. ISBN 978-0-86577-976-1 . Retrieved 4 November 2010 . ^ Charles A. ... HSS J . 6 (2): 199–205. doi : 10.1007/s11420-010-9176-x . PMC 2926354 . PMID 21886536 . ^ a b "NINDS Brachial Plexus Injuries: Information Page" . ... September 29, 2008 . Retrieved 2009-10-11 . ^ Beghi E, Kurland LT, Mulder DW, Nicolosi A (1985). ... Lippincott Williams & Wilkins. pp. 1–. ISBN 978-0-7817-6135-2 . Retrieved 4 November 2010 . ^ a b Frank Gaillard MD.
Some patients have shown that their relatives suffered from migraines as well and even some from migralepsy, forming the possibility that migralepsy is genetic in origin and forms only rarely as both, generally resulting in only one condition or the other. [8] [9] [10] Diagnosis [ edit ] Because epileptic seizures may occur with a side effect that resembles migraine aura, it is complicated to diagnose whether a patient is having a normal epileptic episode or if it is a true migraine that is then being followed by a seizure, which would be a true sign of migralepsy. ... The Journal of Headache and Pain . 11 (3): 235–40. doi : 10.1007/s10194-010-0191-6 . PMC 3451908 . PMID 20112041 . ^ Jha, Sanjeev; Kumar, Rajesh (2007). ... Migraine and other headache disorders . Informa Healthcare . ISBN 0-8493-3695-3 . Retrieved February 4, 2012 . ^ "Epilepsy Migraine - More than just a headache" - .docstoc ^ Milligan, Tracey A.; Bromfield, Edward (2005). ... John Libbey Eurotext. pp. 288–291. ISBN 0-86196-577-9 . Retrieved February 4, 2012 . ^ "Migralepsy" and the Significance of Differentiating Occipital Seizures from Migraine - InterScience ^ "The EEG: Differential diagnosis of migraine and epilepsy" - Epilepsy.com External links [ edit ] Sances, Grazia; Guaschino, Elena; Perucca, Piero; Allena, Marta; Ghiotto, Natascia; Manni, Raffaele (2009).
More pronounced regurgitation that is noticed through a routine physical examination is a medical sign of disease and warrants further investigation. [ medical citation needed ] If it is secondary to pulmonary hypertension it is referred to as a Graham Steell murmur . [7] Contents 1 Signs and symptoms 2 Causes 3 Pathophysiology 4 Diagnosis 5 Treatment 6 See also 7 References 8 Further reading 9 External links Signs and symptoms [ edit ] Because pulmonic regurgitation is the result of other factors in the body, any noticeable symptoms are ultimately caused by an underlying medical condition rather than the regurgitation itself. [3] However, more severe regurgitation may contribute to right ventricular enlargement by dilation, and in later stages, right heart failure . [8] A diastolic decrescendo murmur can sometimes be identified,( heard best) over the left lower sternal border. [ medical citation needed ] Causes [ edit ] Rheumatic heart disease, Among the causes of pulmonary insufficiency are: Pulmonary hypertension [1] Infective endocarditis [1] Rheumatic heart disease [1] Connective tissue disease [8] Carcinoid syndrome [8] Congestive abnormalities [9] Tetralogy of Fallot , [10] Prosthetic heart valve [11] Pathophysiology [ edit ] The pathophysiology is due to diastolic pressure variations between the pulmonary artery and right ventricle , differences are often very small, but increase regurgitation. ... Philadelphia: Lippincott Williams & Wilkins. p. 430. ISBN 978-0-7817-5175-9 . Retrieved 30 August 2015 . ... New York, NY: Lange Medical Books/McGraw-Hill. p. 269. ISBN 978-0-07144-010-3 . CS1 maint: extra text: authors list ( link ) ^ T. ... Lippincott Williams & Wilkins. 2009-10-01. p. 83. ISBN 9781605474540 . ^ a b c "Pulmonic Regurgitation Clinical Presentation" . ... Sydney [etc.]: Elsevier, Churchill Livingstone. p. 108. ISBN 978-0-72954-147-3 . Retrieved 29 August 2015 . ^ Chaturvedi, Rajiv R; Redington, Andrew N (2007-07-01).
According to the Histiocytosis Association of America , 1 in 200,000 children in the United States are born with histiocytosis each year. [2] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10, although the disease can afflict adults. ... Information concerning histiocytosis and clinicians located in European countries may be found in many languages at the web portal of Euro Histio Net (EHN). This is a project funded by the European Union, coordinated by Jean Donadieu, APHP , Paris, France. ... The Society has instituted several clinical trials and treatment plans. [9] [10] References [ edit ] ^ Histiocytosis Archived 2016-10-09 at the Wayback Machine at eMedicine Dictionary ^ Disease information at the Histiocytosis Association of America ^ "Histiocytosis - Signs and Symptoms" . ... Andrews' Diseases of the Skin: clinical Dermatology . Saunders Elsevier. ISBN 0-7216-2921-0 . ^ Goldberg, J; Nezelof, C (1986), "Lymphohistiocytosis: a multi-factorial syndrome of macrophagic activation clinico-pathological study of 38 cases", Hematol Oncol , 4 (4): 275–289, PMID 3557322 . ^ Egan, Caoimhe; Jaffe, Elaine S. (2018). ... Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997" . Ann Oncol . 10 (12): 1419–32. doi : 10.1023/A:1008375931236 .
New York: W. W. Norton. p. 389 . ISBN 0-393-97777-3 . ^ Robert H. Brookshire. ... Neurobiology of Language . pp. 913–22. doi : 10.1016/B978-0-12-407794-2.00073-0 . ISBN 978-0-12-407794-2 . ^ Damasio, Hanna; Damasio, Antonio R (1980). ... Hillsdale, N.J: L. Erlbaum. pp. 40–42. ISBN 0-8058-0681-4 . ^ Manasco, Hunter (2017). ... Current Neurology and Neuroscience Reports . 10 (6): 499–503. doi : 10.1007/s11910-010-0142-2 . ... Hillsdale, N.J: L. Erlbaum. pp. 28–29. ISBN 0-8058-0681-4 . ^ Feinberg, T. E; Rothi, L.
Lippincott Williams & Wilkins. pp. 254–256. ISBN 0-7817-3905-5 . ^ a b c d e f g h i Smith, Melanie N. (2006-05-10). ... Cambridge University Press . p. 77. ISBN 1-900151-51-0 . ^ Papadakis, Maxine A.; Stephen J. ... McGraw-Hill Professional. p. 60. ISBN 0-07-145892-1 . ^ a b Bosze, Peter; David M. ... Informa Health Care. p. 66. ISBN 963-00-7356-0 . ^ "Cervical Polyps" (PDF) . ... . Medscape.com . Retrieved 2007-10-21 . ^ Tillman, Elizabeth. "Short Instructor Materials" (PDF) .
Nutritional supplementation with Vitamin E in some studies has been shown to be effective in controlling nail changes. [3] Prognosis [ edit ] People with yellow nail syndrome have been found to have a moderately reduced lifespan compared to people without the condition. [5] Epidemiology [ edit ] The condition is thought to be rare, with approximately 150 cases described in the medical literature. [5] History [ edit ] The condition was first described in 1964 by London physicians Peter Samman and William White. [10] Earlier cases may have been recorded in 1927 and 1962. [5] References [ edit ] ^ a b James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology . Saunders Elsevier. ISBN 978-0-7216-2921-6 . ^ "Yellow nail syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program" . rarediseases.info.nih.gov . ... Fitzpatrick's Dermatology in General Medicine . (6th ed.). McGraw-Hill. ISBN 0-07-138076-0 . ^ a b c d e f g h i j k l Maldonado, Fabien; Ryu, Jay H (July 2009). ... Biological Trace Element Research . 143 (1): 1–7. doi : 10.1007/s12011-010-8828-5 . PMC 3176400 . PMID 20809268 . ^ Hoque SR, Mansour S, Mortimer PS (June 2007). ... External links [ edit ] Classification D ICD - 10 : L60.5 OMIM : 153300 MeSH : D056684 SNOMED CT : 400211001 External resources MedlinePlus : 003247 eMedicine : article/109403 Orphanet : 662 v t e Disorders of skin appendages Nail thickness: Onychogryphosis Onychauxis color: Beau's lines Yellow nail syndrome Leukonychia Azure lunula shape: Koilonychia Nail clubbing behavior: Onychotillomania Onychophagia other: Ingrown nail Anonychia ungrouped: Paronychia Acute Chronic Chevron nail Congenital onychodysplasia of the index fingers Green nails Half and half nails Hangnail Hapalonychia Hook nail Ingrown nail Lichen planus of the nails Longitudinal erythronychia Malalignment of the nail plate Median nail dystrophy Mees' lines Melanonychia Muehrcke's lines Nail–patella syndrome Onychoatrophy Onycholysis Onychomadesis Onychomatricoma Onychomycosis Onychophosis Onychoptosis defluvium Onychorrhexis Onychoschizia Platonychia Pincer nails Plummer's nail Psoriatic nails Pterygium inversum unguis Pterygium unguis Purpura of the nail bed Racquet nail Red lunulae Shell nail syndrome Splinter hemorrhage Spotted lunulae Staining of the nail plate Stippled nails Subungual hematoma Terry's nails Twenty-nail dystrophy Hair Hair loss / Baldness noncicatricial alopecia : Alopecia areata totalis universalis Ophiasis Androgenic alopecia (male-pattern baldness) Hypotrichosis Telogen effluvium Traction alopecia Lichen planopilaris Trichorrhexis nodosa Alopecia neoplastica Anagen effluvium Alopecia mucinosa cicatricial alopecia : Pseudopelade of Brocq Central centrifugal cicatricial alopecia Pressure alopecia Traumatic alopecia Tumor alopecia Hot comb alopecia Perifolliculitis capitis abscedens et suffodiens Graham-Little syndrome Folliculitis decalvans ungrouped: Triangular alopecia Frontal fibrosing alopecia Marie Unna hereditary hypotrichosis Hypertrichosis Hirsutism Acquired localised generalised patterned Congenital generalised localised X-linked Prepubertal Acneiform eruption Acne Acne vulgaris Acne conglobata Acne miliaris necrotica Tropical acne Infantile acne / Neonatal acne Excoriated acne Acne fulminans Acne medicamentosa (e.g., steroid acne ) Halogen acne Iododerma Bromoderma Chloracne Oil acne Tar acne Acne cosmetica Occupational acne Acne aestivalis Acne keloidalis nuchae Acne mechanica Acne with facial edema Pomade acne Acne necrotica Blackhead Lupus miliaris disseminatus faciei Rosacea Perioral dermatitis Granulomatous perioral dermatitis Phymatous rosacea Rhinophyma Blepharophyma Gnathophyma Metophyma Otophyma Papulopustular rosacea Lupoid rosacea Erythrotelangiectatic rosacea Glandular rosacea Gram-negative rosacea Steroid rosacea Ocular rosacea Persistent edema of rosacea Rosacea conglobata variants Periorificial dermatitis Pyoderma faciale Ungrouped Granulomatous facial dermatitis Idiopathic facial aseptic granuloma Periorbital dermatitis SAPHO syndrome Follicular cysts " Sebaceous cyst " Epidermoid cyst Trichilemmal cyst Steatocystoma simplex multiplex Milia Inflammation Folliculitis Folliculitis nares perforans Tufted folliculitis Pseudofolliculitis barbae Hidradenitis Hidradenitis suppurativa Recurrent palmoplantar hidradenitis Neutrophilic eccrine hidradenitis Ungrouped Acrokeratosis paraneoplastica of Bazex Acroosteolysis Bubble hair deformity Disseminate and recurrent infundibulofolliculitis Erosive pustular dermatitis of the scalp Erythromelanosis follicularis faciei et colli Hair casts Hair follicle nevus Intermittent hair–follicle dystrophy Keratosis pilaris atropicans Kinking hair Koenen's tumor Lichen planopilaris Lichen spinulosus Loose anagen syndrome Menkes kinky hair syndrome Monilethrix Parakeratosis pustulosa Pili ( Pili annulati Pili bifurcati Pili multigemini Pili pseudoannulati Pili torti ) Pityriasis amiantacea Plica neuropathica Poliosis Rubinstein–Taybi syndrome Setleis syndrome Traumatic anserine folliculosis Trichomegaly Trichomycosis axillaris Trichorrhexis ( Trichorrhexis invaginata Trichorrhexis nodosa ) Trichostasis spinulosa Uncombable hair syndrome Wooly hair nevus Sweat glands Eccrine Miliaria Colloid milium Miliaria crystalline Miliaria profunda Miliaria pustulosa Miliaria rubra Occlusion miliaria Postmiliarial hypohidrosis Granulosis rubra nasi Ross’ syndrome Anhidrosis Hyperhidrosis Generalized Gustatory Palmoplantar Apocrine Body odor Chromhidrosis Fox–Fordyce disease Sebaceous Sebaceous hyperplasia
Yellow nail syndrome is a very rare disorder characterized by three features: yellow nail discoloration, respiratory problems, and lower limb swelling ( lymphedema ). It usually occurs in people over age 50, but can occur in younger people. In addition to being yellow, nails may lack a cuticle, grow very slowly, and become detached ( onycholysis ). Respiratory problems may include chronic cough, bronchiectasis , and pleural effusion . Chronic sinusitis may also occur. The cause of yellow nail syndrome remains unknown.
A rare, syndromic nail anomaly disease characterized by the variable triad of characteristic yellow nails, chronic respiratory manifestations, and primary lymphedema. Epidemiology Prevalence and incidence rates are not known, but Yellow nail syndrome (YNS) is considered a rare condition. To date, approximately 400 cases have been reported, mainly as single case reports. The disorder affects males and females equally. Clinical description Most cases of YNS are of late onset, after 50 years of age, but the disease has also occasionally been observed in neonates and children. Patients generally complain of slowed or arrested nail growth: the nails become thickened and opaque, with yellowish or green discoloration.
In Dogger Bank itch, sensitivity is acquired after repeated handling of the sea chervils that become entangled in fishing nets. [ citation needed ] The specific toxin responsible for the rash was determined to be the sulfur -bearing salt (2-hydroxyethyl) dimethylsulfoxonium chloride. [3] This salt is also found in some sea sponges and has potent in vitro activity against leukemia cells. [4] Treatment [ edit ] A study of two cases in 2001 suggests that the rash responds to oral ciclosporin . ... The sea chervil, abundant in the area, frequently came up with the fishing nets and had to be thrown back into the water. ... Andrews' Diseases of the Skin: clinical Dermatology . Saunders Elsevier. ISBN 978-0-7216-2921-6 . ^ Bonnevie, P. (1948). ... Comparative Biochemistry and Physiology B . 128 (1): 27–30. doi : 10.1016/S1096-4959(00)00316-X . CS1 maint: multiple names: authors list ( link ) ^ a b Bowers PW, Julian CG., PW; Julian, CG (2001). ... "Dogger Bank Itch. 4. an eczema-causing sulfoxonium ion from the marine animal, Alcyonidium gelatinosum ". Toxicon . 20 (1): 307–10. doi : 10.1016/0041-0101(82)90232-X .
SeSAME syndrome is characterized by Sei zures , S ensorineural deafness , A taxia (lack of muscle coordination), intellectual ( M ental) disability , and E lectrolyte imbalance (low levels of potassium and magnesium in the blood, hypokalemia and hypomagnesemia, and metabolic alkalosis ). It may also be known as EAST syndrome ( E pilepsy , A taxia, S ensorineural deafness, and T ubulopathy ( kidney problems in the structures known as tubules)). Seizures tend to start in early childhood. The seizures are typically of the generalized tonic-clonic seizure type (also known as grand mal seizures), but they usually respond well to medication. Non-progressive, cerebellar ataxia and hearing loss start later. The ataxia seems to be the most debilitating feature of the syndrome. It is caused by mutations in the KCNJ10 gene, and inherited in an autosomal recessive pattern.
SeSAME syndrome is characterized by seizures, sensorineural deafness, ataxia, intellectual deficit, and electrolyte imbalance (hypokalemia, metabolic alkalosis, and hypomagnesemia). Epidemiology It has been described in five patients from four families. Etiology The disease is caused by homozygous or compound heterozygous mutations in the KCNJ10 gene, encoding a potassium channel expressed in the brain, spinal cord, inner ear and kidneys. Genetic counseling Transmission is autosomal recessive.
Actual rates of hypoglycemia associated with a fibrous tumor are quite rare (a 1981 study of 360 solitary fibrous tumors of the lungs found that only 4% caused hypoglycemia [8] ), and are linked to large tumors with high rates of mitosis . [9] Removal of the tumor will normally resolve the symptoms. [1] [9] Tumors causing DPS tend to be quite large; [10] in one case a 3 kg (6.6 lb), 23×21×12 cm (9.1×8.3×4.7 in) mass was removed, sufficiently large to cause a collapsed lung . [5] In X-rays , they appear as a single mass with visible, defined borders, appearing at the edges of the lungs or a fissure dividing the lobes of the lungs. [10] Similar hypoglycemic effects have been related to mesenchymal tumors. [6] References [ edit ] ^ a b Balduyck B, Lauwers P, Govaert K, Hendriks J, De Maeseneer M, Van Schil P (July 2006). ... Surg . 119 (1): 185–7. doi : 10.1016/S0022-5223(00)70242-X . PMID 10612786 . Archived from the original on 2013-01-12. ^ a b Shields, TW; LoCicero J; Ponn RB; Rusch VW (2005). ... Hagerstwon, MD: Lippincott Williams & Wilkins. pp. 893 . ISBN 0-7817-3889-X . ^ Ellorhaoui M, Graf B (February 1976). ... Hagerstwon, MD: Lippincott Williams & Wilkins. pp. 172–3 . ISBN 978-0-7817-6957-0 . v t e Paraneoplastic syndromes Endocrine Hypercalcaemia SIADH Zollinger–Ellison syndrome Cushing's syndrome Hematological Multicentric reticulohistiocytosis Nonbacterial thrombotic endocarditis Neurological Paraneoplastic cerebellar degeneration Encephalomyelitis Limbic encephalitis Opsoclonus Polymyositis Transverse myelitis Lambert–Eaton myasthenic syndrome Anti-NMDA receptor encephalitis Musculoskeletal Dermatomyositis Hypertrophic osteopathy Mucocutaneous reactive erythema Erythema gyratum repens Necrolytic migratory erythema papulosquamous Acanthosis nigricans Ichthyosis acquisita Acrokeratosis paraneoplastica of Bazex Extramammary Paget's disease Florid cutaneous papillomatosis Leser-Trélat sign Pityriasis rotunda Tripe palms Other Febrile neutrophilic dermatosis Pyoderma gangrenosum Paraneoplastic pemphigus v t e Tumours of endocrine glands Pancreas Pancreatic cancer Pancreatic neuroendocrine tumor α : Glucagonoma β : Insulinoma δ : Somatostatinoma G : Gastrinoma VIPoma Pituitary Pituitary adenoma : Prolactinoma ACTH-secreting pituitary adenoma GH-secreting pituitary adenoma Craniopharyngioma Pituicytoma Thyroid Thyroid cancer (malignant): epithelial-cell carcinoma Papillary Follicular / Hurthle cell Parafollicular cell Medullary Anaplastic Lymphoma Squamous-cell carcinoma Benign Thyroid adenoma Struma ovarii Adrenal tumor Cortex Adrenocortical adenoma Adrenocortical carcinoma Medulla Pheochromocytoma Neuroblastoma Paraganglioma Parathyroid Parathyroid neoplasm Adenoma Carcinoma Pineal gland Pinealoma Pinealoblastoma Pineocytoma MEN 1 2A 2B
A rare photodermatosis characterized by the development of pruritic or painful vesicles in a photodistributed pattern in response to sunlight exposure. The lesions heal with permanent varioliform scarring. Ocular involvement, deformities of ears and nose, or contractures of the fingers may occasionally be observed. Systemic signs and symptoms are absent. The condition typically occurs in childhood and regresses spontaneously in adolescence or young adulthood.
