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A case of dyschromatosis universalis hereditaria with adermatoglyphia: A rare association.

Kumar S, Bhoyar P, Mahajan BB - Indian Dermatol Online J (2015 Mar-Apr)

Bottom Line: Dyschromatosis universalis hereditaria (DUH) is a rare, autosomal dominant genodermatosis with a peculiar reticulate pigmentary change, consisting of hyperpigmented macules mingled with hypopigmented lesions to give an overall impression of mottling.We herein report a case of DUH with adermatoglyphia in a young male with family history of the disorder.

View Article: PubMed Central - PubMed

Affiliation: Department of Skin and VD, Guru Gobind Singh Medical College, Faridkot, Punjab, India.

ABSTRACT
Dyschromatosis universalis hereditaria (DUH) is a rare, autosomal dominant genodermatosis with a peculiar reticulate pigmentary change, consisting of hyperpigmented macules mingled with hypopigmented lesions to give an overall impression of mottling. We herein report a case of DUH with adermatoglyphia in a young male with family history of the disorder.

No MeSH data available.


Related in: MedlinePlus

Dystrophic changes in fingernails
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Figure 6: Dystrophic changes in fingernails

Mentions: A 31-year-old unmarried male presented with progressively increasing, generalized mottled pigmentation since the age of 3 years. The lesions had started over neck and trunk that spread centrifugally to the extremities over a period of 10 years. There was no history of photosensitivity. There was no history of handling any chemical directly or any significant history of drug intake. There was history of similar lesions in the younger brother. His younger sister was normal. There was no history of consanguinity among the parents who were also normal. On dermatological examination, multiple hyper- and hypo-pigmented macules of varying size ranging from 2 to 30 mm were present in a reticulate pattern without atrophy over the forehead [Figure 1], trunk [Figures 2 and 3], upper and lower extremities [Figure 4]. Reticulate pigmentation was present also on palms with absent dermatoglyphics distal to the distal creases [Figure 5]. Dystrophic changes in the fingernails including longitudinal striations, ragged cuticles and pterygium in one fingernail were seen [Figure 6]. Mucous membranes and soles were normal. Systemic examination did not reveal any abnormality. Routine investigations, including complete blood counts, urinalysis, liver function tests, renal function tests, and serum electrolytes were within normal limits. Venereal Disease Research Laboratory test and enzyme-linked immunosorbent assay for human immunodeficiency virus were both nonreactive. Histopathological examination from hyperpigmented lesions showed mild hyperkeratosis. The basal layer showed pigmentation with some coarse melanocyte granules, predominantly in the lower 2–3 layers of the epidermis. There was prominent melanin incontinence with melanophages in the dermis [Figure 7]. Sparse lymphohistiocytic infiltrates were seen in the perivascular areas. This confirmed the diagnosis of DUH.


A case of dyschromatosis universalis hereditaria with adermatoglyphia: A rare association.

Kumar S, Bhoyar P, Mahajan BB - Indian Dermatol Online J (2015 Mar-Apr)

Dystrophic changes in fingernails
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4375753&req=5

Figure 6: Dystrophic changes in fingernails
Mentions: A 31-year-old unmarried male presented with progressively increasing, generalized mottled pigmentation since the age of 3 years. The lesions had started over neck and trunk that spread centrifugally to the extremities over a period of 10 years. There was no history of photosensitivity. There was no history of handling any chemical directly or any significant history of drug intake. There was history of similar lesions in the younger brother. His younger sister was normal. There was no history of consanguinity among the parents who were also normal. On dermatological examination, multiple hyper- and hypo-pigmented macules of varying size ranging from 2 to 30 mm were present in a reticulate pattern without atrophy over the forehead [Figure 1], trunk [Figures 2 and 3], upper and lower extremities [Figure 4]. Reticulate pigmentation was present also on palms with absent dermatoglyphics distal to the distal creases [Figure 5]. Dystrophic changes in the fingernails including longitudinal striations, ragged cuticles and pterygium in one fingernail were seen [Figure 6]. Mucous membranes and soles were normal. Systemic examination did not reveal any abnormality. Routine investigations, including complete blood counts, urinalysis, liver function tests, renal function tests, and serum electrolytes were within normal limits. Venereal Disease Research Laboratory test and enzyme-linked immunosorbent assay for human immunodeficiency virus were both nonreactive. Histopathological examination from hyperpigmented lesions showed mild hyperkeratosis. The basal layer showed pigmentation with some coarse melanocyte granules, predominantly in the lower 2–3 layers of the epidermis. There was prominent melanin incontinence with melanophages in the dermis [Figure 7]. Sparse lymphohistiocytic infiltrates were seen in the perivascular areas. This confirmed the diagnosis of DUH.

Bottom Line: Dyschromatosis universalis hereditaria (DUH) is a rare, autosomal dominant genodermatosis with a peculiar reticulate pigmentary change, consisting of hyperpigmented macules mingled with hypopigmented lesions to give an overall impression of mottling.We herein report a case of DUH with adermatoglyphia in a young male with family history of the disorder.

View Article: PubMed Central - PubMed

Affiliation: Department of Skin and VD, Guru Gobind Singh Medical College, Faridkot, Punjab, India.

ABSTRACT
Dyschromatosis universalis hereditaria (DUH) is a rare, autosomal dominant genodermatosis with a peculiar reticulate pigmentary change, consisting of hyperpigmented macules mingled with hypopigmented lesions to give an overall impression of mottling. We herein report a case of DUH with adermatoglyphia in a young male with family history of the disorder.

No MeSH data available.


Related in: MedlinePlus