We present a 35-year-old Woman, which presented with Flexural Hyperpigmentation considerate as acanthosis nigricans [19]. [gulfdoctor.net]

Figure 1: (a) Symmetrically distributed hyper- and hypopigmented macules present over limbs of three siblings. [pigmentinternational.com]

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]

Initially, the lesions were present only over the fingers and gradually they increased in size as well as number to attain the present status. [indianpediatrics.net]

However, in our patient, lesions were present in the unexposed sites as well. Moreover, the lesions did not show any atrophy or telangiectasia as seen in xeroderma pigmentosum. [jmgims.co.in]

• Nail Abnormality

  There was no abnormality of oral mucosa or nails. Abdominal examination was normal. No mass could be palpated by bimanual palpation. Neurological examination was also normal. [karger.com]

  Apart from a history of associated hyperhidrosis, the patient showed no other abnormalities in palms, soles, nails and mucous membranes. Systemic examination revealed neither deafness, cataract, neurological abnormalities nor history of seizures. [clinical-pediatrics-dermatology.imedpub.com]

• Normal Stature

  On examination she was of normal stature and average build. [jcdr.net]

• Normal Hearing

  Complete blood count, liver and renal function tests, anti-thyroid antibody levels and anti-nuclear antibody titre were normal. Hearing and vision was normal. [jcdr.net]

• Suggestibility

  Collectively, these observations suggest that DUH is a heterogeneous disorder of increased production and transport of melanosomes caused by SASH1 mutation together with melanocyte maldistribution. [precedings.nature.com]

  It has been suggested in the past that DUH is a disorder of melanocyte number. [ijdvl.com]

  A second locus for dyschromatosis was mapped on chromosome 6q24.2-q25.2 in two Chinese families initially reported to be affected with DSH, but later suggested to have autosomal dominant DUH. [eprints.hud.ac.uk]

  Our results suggest that DUH may be a disorder of melanosome production and distribution in epidermal melanin units rather than a a disorder of melanocyte number [6]. [wikigenes.org]

  We report a rare case of Dyschromatosisuniversalishereditaria in a 23 year old male patient with no affected family members suggesting the possibility of sporadic mutation. [scopemed.org]

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  No specific treatment exists  Palmoplantar keratoderma: Topical keratolytics 45. [slideshare.net]

The main treatment is by reassuring the patient. Camouflage, miniature-skin graft, and excimer laser therapy have all been tried for treatment of this condition. References [1] Muller CS, Tremezaygues L, Pfohler C, Vogt T. [article.sapub.org]

Broaden your knowledge with updated information on serological diagnosis of pemphigus, TNF-I for hidradenitis suppurativa, the use of immunosuppressives for atopic dermatitis, excimer laser for the treatment of vitiligo and much more. [books.google.de]

WY Al-Saeed, KM Al-Dawood, IA Bukhari, AA Bahnassy Saudi Medical Journal 27 (2), 227-234, 2006 33 2006 Impetigo herpetiformis in a primigravida: successful treatment with etretinate. [scholar.google.com]

The heterozygosity for the DSRAD knockout causes embryonic lethality in mice, whereas patients with DSH, which is localized specifically on the backs of the hands and tops of the feet, have a good prognosis. 23 Miyamura et al 11 speculated that when melanoblasts [jamanetwork.com]

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]

Etiology A mutation has been identified in the double-stranded RNA-specific adenosine deaminase ( ADAR ) gene. Genetic counseling Transmission is autosomal dominant. [orpha.net]

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]

The etiology of DUH is still unclear, but it has been hypothesized to be a disorder of melanosome production and distribution in epidermal melanin units rather than a disorder of melanocyte number [2]. [ejdv.eg.net]

This chapter covers the epidemiology, pathophysiology, clinical presentation, histopathology, differential diagnosis, and treatment of both common and rare genetic hyperpigmentation disorders. [link.springer.com]

Amyloidosis cutis dyschromica in two siblings and review of the epidemiology, clinical features and management in 48 cases. Australas J Dermatol 2016; 57: 307–11. DOI: 10.1111/ajd.12342. PubMed Qiao J, Fang H, Yao H. Amyloidosis cutis dyschromica. [dermnetnz.org]

Epidemiology  Rare group of disorders  Incidence and prevalence: Not known  Most cases: Japan  Paucity of literature might be due to underreporting of cases  Mostly autosomal dominant with variable degrees of penetrance 6. [slideshare.net]

This chapter covers the epidemiology, pathophysiology, clinical presentation, histopathology, differential diagnosis, and treatment of both common and rare genetic hyperpigmentation disorders. [link.springer.com]

The TJEM also covers the fields of disaster-prevention science, including earthquake archeology. Submitted manuscripts will be screened for plagiarism with Similarity Check ( https://www.crossref.org/services/similarity-check/ ). [jstage.jst.go.jp]

The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer. [gulfdoctor.net]

Patient was started on cyclical hormonal supplements for development of secondary sexual characters and prevention of osteoporosis after consultation with an endocrinologist and gynaecologist. Genetic counselling was given. [jcdr.net]

Treatment  Exposure to heat should be limited, and sufficient hydration is recommended  Sports-related exercise should be restricted  Tooth care is required to prevent early dental caries 52. [slideshare.net]

Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public [emedicine.medscape.com]