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Poikiloderma a varied presentation - Huriez syndrome.

Kharge P, Fernendes C, Jairath V, Mohan M, Chandra S - Indian Dermatol Online J (2015 Jan-Feb)

Bottom Line: Huriez syndrome is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma (PPK) and hypoplastic nails.We report the case of a 25 year old male, with nonfamilial Huriez syndrome.

View Article: PubMed Central - PubMed

Affiliation: Departments of Dermatology, Venereology and Leprosy, Dr. B.R Ambedkar Medical College, Bengaluru, Karnataka, India.

ABSTRACT
Huriez syndrome is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma (PPK) and hypoplastic nails. We report the case of a 25 year old male, with nonfamilial Huriez syndrome.

No MeSH data available.


Related in: MedlinePlus

Genralised atrophy, dyschromia and xerosis; the hallmark features of poikiloderma
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Figure 2: Genralised atrophy, dyschromia and xerosis; the hallmark features of poikiloderma

Mentions: A 25-year-old male, born out of a nonconsanguineous marriage presented to the outpatient department of our hospital with complaints of pigmentation of skin, painful fissures of the hands and feet, with hypohidrosis since birth. There was tightening of skin of the distal extremities since 15 years. His family members were unaffected. There was no history of Raynaud's phenomenon, photosensitivity, growth or mental retardation, exposure to chemicals or any drugs that would be responsible for his sclerotic skin. On examination, both palms and soles had diffuse scleroatrophy with fissuring and hyperpigmentation [Figure 1]. The skin had reduced pinchability on the distal extremities with generalized atrophy, dyschromia, telangiectasia, and xerosis [Figure 2]. There was ulnar deviation at the left wrist due to contracture along the ulnar border of the palm. A raised tender nodular lesion measuring around 1 cm × 1.5 cm was present on the left thenar eminence; there was no ulceration or bleeding from the lesion [Figure 3].


Poikiloderma a varied presentation - Huriez syndrome.

Kharge P, Fernendes C, Jairath V, Mohan M, Chandra S - Indian Dermatol Online J (2015 Jan-Feb)

Genralised atrophy, dyschromia and xerosis; the hallmark features of poikiloderma
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4314883&req=5

Figure 2: Genralised atrophy, dyschromia and xerosis; the hallmark features of poikiloderma
Mentions: A 25-year-old male, born out of a nonconsanguineous marriage presented to the outpatient department of our hospital with complaints of pigmentation of skin, painful fissures of the hands and feet, with hypohidrosis since birth. There was tightening of skin of the distal extremities since 15 years. His family members were unaffected. There was no history of Raynaud's phenomenon, photosensitivity, growth or mental retardation, exposure to chemicals or any drugs that would be responsible for his sclerotic skin. On examination, both palms and soles had diffuse scleroatrophy with fissuring and hyperpigmentation [Figure 1]. The skin had reduced pinchability on the distal extremities with generalized atrophy, dyschromia, telangiectasia, and xerosis [Figure 2]. There was ulnar deviation at the left wrist due to contracture along the ulnar border of the palm. A raised tender nodular lesion measuring around 1 cm × 1.5 cm was present on the left thenar eminence; there was no ulceration or bleeding from the lesion [Figure 3].

Bottom Line: Huriez syndrome is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma (PPK) and hypoplastic nails.We report the case of a 25 year old male, with nonfamilial Huriez syndrome.

View Article: PubMed Central - PubMed

Affiliation: Departments of Dermatology, Venereology and Leprosy, Dr. B.R Ambedkar Medical College, Bengaluru, Karnataka, India.

ABSTRACT
Huriez syndrome is a rare autosomal dominant genodermatosis characterized by the triad of congenital scleroatrophy of the distal extremities, palmoplantar keratoderma (PPK) and hypoplastic nails. We report the case of a 25 year old male, with nonfamilial Huriez syndrome.

No MeSH data available.


Related in: MedlinePlus