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Dyschromatosis Universalis Hereditaria


Presentation

  • DUH presents in a fashion similar to that of a variety of reticulate and pigmentary dermatoses, some of which are associated with precancerous entities and other comorbidities.[ncbi.nlm.nih.gov]
  • They presented to our department with asymptomatic progressive mottled pigmentation over the trunk and limbs, which had been noted since birth and had become more noticeable with age. Palms and soles were also affected in the first case.[dermatology.cdlib.org]
  • Figure 1: (a) Symmetrically distributed hyper- and hypopigmented macules present over limbs of three siblings.[pigmentinternational.com]
  • We report a case of DUH in a 13-year-old boy presenting with oral leukokeratosis, with a review of the literature exploring the differential diagnoses.[pesquisa.bvs.br]
Nail Abnormality
  • Except for skin lesions, there is no other common associated condition in DUH; however, there have been various reports of an association with short stature and high-tone deafness, seizure, mental retardation, hair and nail abnormalities, ocular abnormalities[karger.com]
  • abnormalities [ 10 ].[clinical-pediatrics-dermatology.imedpub.com]
Hyperpigmentation in Sun-exposed Areas
  • , poikiloderma sun-exposed areas skin cancers, photophobia, keratitis, deafness, mental retardation Chronic arsenic toxicity acquired guttate hypopigmentation superimposed on hyperpigmentation any part of the body skin cancers, internal malignancy AD[docksci.com]
Incontinence
  • Histopathology revealed a variable degree of pigmentary incontinence. Although the precise aetiology of this disorder is not yet known, the clinicopathological findings implicate an inherent abnormality of melanosomes or melanin processing.[ncbi.nlm.nih.gov]
  • Histological exam revealed basal layer hypermelanosis with pigmentary incontinence in some areas.[dermatology.cdlib.org]
  • , there was no evidence of increased epidermal pigmentation or pigmentary incontinence.[idoj.in]

Workup

  • The possibility of primary immunodeficiency was kept and baseline immunodeficiency workup was done. His serum immunoglobulin assay was normal and human immunodeficiency virus one and two tests (ELISA) were negative.[e-ijd.org]
Lymphocytic Infiltrate
  • The dermis showed a few pilosebaceous units surrounded by sparse lymphocytic infiltrate. There was no evidence of increased epidermal pigmentation or pigment incontinence [Figure 5] , [Figure 6] , [Figure 7] .[idoj.in]

Treatment

  • Stay at the forefront of your field with updated treatment methods throughout, as well as an increased focus on patients with skin of color.[books.google.de]
  • It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice .[uniprot.org]

Prognosis

  • Prognosis - Dyschromatosis universalis hereditaria Not supplied. Treatment - Dyschromatosis universalis hereditaria Not supplied. Resources - Dyschromatosis universalis hereditaria Not supplied.[checkorphan.org]
  • Although, each condition possesses unique phenotypic characteristics and the prognosis for each is somewhat different, these disorders are highly overlapped and difficult to differentiate in the clinical setting.[bioportfolio.com]
  • Summary Background Subtypes of inherited epidermolysis bullosa (EB) vary significantly in their clinical presentation and prognosis. Establishing an accurate diagnosis is important for genetic counselling and patient management.[scinapse.io]
  • Tacrolimus has been reported as speeding resolution. [16] In exceptionally severe cases PUVA therapy may be considered. [17] Prognosis The patches of pityriasis alba may last from 1 month to about one year, but commonly on the face last a year.[medbox.iiab.me]
  • Development of cell morphologic and molecular variables in melanoma prognosis (H.R. Byers, M.C. Mihm Jr.). A videomicroscopic approach to malignant melanoma (R Akasu et al. ). Treatment of malignant melanoma.[espcr.org]

Etiology

  • Although the precise etiology of this disorder is not yet known, the clinicopathological findings implicate an inherent abnormality of melanosomes or melanin processing. We describe a case in a young Nigerian girl.[ncbi.nlm.nih.gov]
  • The exact etiology of this disorder is unknown. However, studies seem to point towards an inherent abnormality of melanosomes or a defect in melanocyte processing. DUH occurs most commonly among the Japanese.[cags.org.ae]
  • The etiology of this disorder is not yet known. DUH is characterized by mixtures of hyperpigmented- and hypopigmented macules all over the body. Skin lesions are usually present in the first years of life.[sparrho.com]
  • Only genetic counselling is advised because of the recent reports of genetic etiology. This case of DUH is reported because of its rarity.[jmgims.co.in]
  • The etiology is unknown.[pigmentinternational.com]

Epidemiology

  • PubMed EJERCICIO Y LA DETECCION DEL MAL AGUDO DE MONTAÑA GRAVE EJERCICIO Y LA DETECCION DEL MAL AGUDO DE MONTAÑA GRAVE NIHPA Author Manuscripts. 2010; 70(1)3 Epidemiological and clinical aspects of certain venomous animals of Spain.[ncbi.nlm.nih.gov]
  • Vitiligo on black skin: epidemiological and clinical aspects in dermatology, Cotonou (Benin).[science.gov]
  • This chapter covers the epidemiology, pathophysiology, clinical presentation, histopathology, differential diagnosis, and treatment of both common and rare genetic hyperpigmentation disorders.[link.springer.com]
  • Differential Diagnosis and Management of Dermal Melanocytosis View Table Favorite Table Download (.pdf) Table 75-1 Differential Diagnosis and Management of Dermal Melanocytosis Nevus of Ota Nevus of Ito Mongolian Spot Nevus of Hori Dermal Melanocyte Hamartoma Epidemiology[accessmedicine.mhmedical.com]
  • "Epidemiological survey of skin diseases in schoolchildren living in the Purus Valley (Acre State, Amazonia, Brazil)". Dermatologica . 163 (1): 78–93. doi: 10.1159/000250144 . PMID 7274519 . Abdel-Hafez K, Abdel-Aty MA, Hofny ER (2003).[medbox.iiab.me]
Sex distribution
Age distribution

Pathophysiology

  • This chapter covers the epidemiology, pathophysiology, clinical presentation, histopathology, differential diagnosis, and treatment of both common and rare genetic hyperpigmentation disorders.[link.springer.com]
  • In: The Pigmentary System: Physiology and Pathophysiology, 2nd ed, Nordlund J, Boissy RE, Hearing VJ, et al (Eds), Blackwell Publishing Ltd, Malden, MA 2006. p.924. Qiao J, Fang H, Yao H. Amyloidosis cutis dyschromica.[menopause.medicclub.info]
  • Melanogenesis and the Malignant Melanoma: Biochemistry, Cell Biology, Molecular Biology, Pathophysiology, Diagnosis and Treatment Edited by: Y. Hori, V.J. Hearing, J. Nakayama.[espcr.org]
  • Recognitionof further cases may help elucidate the aetiology of thiscondition and give insight into the pathophysiology of bothpigmentation disorders and epileptic encephalopathies.Keywords Epileptic encephalopathy .[docslide.us]

Prevention

  • […] universalis hereditaria * Aplasia cutis congenita - epibulbar dermoids - skin atrophy * Arthrogryposis - atrophic tracts of skin * Atrophoderma vermiculata - skin atrophy * Barber-Say syndrome - atrophic skin * Blegvad-Haxthausen syndrome - skin atrophy Prevention[checkorphan.org]
  • The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer.[gulfdoctor.net]
  • "Force majeure" term will be regarded as outside of the concerned party's reasonable control and any situation that "Turkiye Klinikleri" cannot prevent even though it shows due diligence.[turkiyeklinikleri.com]
  • The c.3203-2A G mutation is located in the acceptor splice site and could prevent proper splicing of the transcript.[vdocuments.mx]
  • Probiotic Supplementation for Prevention of Atopic Dermatitis in Infants and Children: ... Preview American Journal of Clinical Dermatology; Li L, Han Z et. al.[allmedx.com]

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