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Tuberous sclerosis.

George A, Kanish B, Bhatia A - Indian Dermatol Online J (2015 Mar-Apr)

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Christian Medical College, Ludhiana, Punjab, India.

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She had no neurological symptoms or family history of similar disease... She was found to have skin-colored to slightly hyperpigmented papules 2-4 mm in diameter all over her central face including over the eyelids and vermillion border of lips [Figure 1]... She also had few leafy white macules scattered over her trunk [Figure 2], apart from numerous confetti-like white freckles [Figure 3], rubbery hypopigmented plaques [Figure 4] on her lower back and lower limbs, and subungual tumors [Figure 5]... The cutaneous manifestations of this phakomatosis include the ash-leaf macules, confetti skin lesions, adenoma sebaceum, shagreen patches and Koenen's tumors... Confetti skin lesions are white freckles, seen less commonly, but are most specific... These hypomelanotic macules on histopathology reveal a normal number of melanocytes, however, the melanocytes have poorly developed dendritic processes with smaller and fewer melanosomes. “Adenoma sebaceum” was the old misnomer for facial angiofibromas, seen in a butterfly pattern and giving a ruddy appearance to the cheeks... These are small red to hyperpigmented, dome-shaped, discrete, smooth, bilaterally symmetrical papules seen over the central face, with relative sparing of the upper lip and lateral face... This dome-shaped lesion on biopsy shows large spindle-shaped fibroblasts in the dermis and a characteristic perifollicular concentric fibrosis giving an “onion-skin pattern. ” Shagreen patch, also called collagenoma, is named after a type of leather; felt as a rubbery plaque with a firm, irregular, knobby surface similar to shark skin... The prominence of slightly depressed hair follicles is characteristic, giving it an “orange peel” or pigskin-like appearance... Koenen's tumors are ungual fibromas, usually the last cutaneous manifestation, at puberty... They are seen more often over the toe nails rather than fingernails and may be periungual or subungual.

No MeSH data available.


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Facial angiofibromas
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Figure 1: Facial angiofibromas

Mentions: A 16-year-old girl presented with asymptomatic lesions on her face for 5 years, which began as reddish lesions. She had no neurological symptoms or family history of similar disease. She was found to have skin-colored to slightly hyperpigmented papules 2-4 mm in diameter all over her central face including over the eyelids and vermillion border of lips [Figure 1]. She also had few leafy white macules scattered over her trunk [Figure 2], apart from numerous confetti-like white freckles [Figure 3], rubbery hypopigmented plaques [Figure 4] on her lower back and lower limbs, and subungual tumors [Figure 5]. She was diagnosed to have tuberous sclerosis.


Tuberous sclerosis.

George A, Kanish B, Bhatia A - Indian Dermatol Online J (2015 Mar-Apr)

Facial angiofibromas
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4375767&req=5

Figure 1: Facial angiofibromas
Mentions: A 16-year-old girl presented with asymptomatic lesions on her face for 5 years, which began as reddish lesions. She had no neurological symptoms or family history of similar disease. She was found to have skin-colored to slightly hyperpigmented papules 2-4 mm in diameter all over her central face including over the eyelids and vermillion border of lips [Figure 1]. She also had few leafy white macules scattered over her trunk [Figure 2], apart from numerous confetti-like white freckles [Figure 3], rubbery hypopigmented plaques [Figure 4] on her lower back and lower limbs, and subungual tumors [Figure 5]. She was diagnosed to have tuberous sclerosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Christian Medical College, Ludhiana, Punjab, India.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

She had no neurological symptoms or family history of similar disease... She was found to have skin-colored to slightly hyperpigmented papules 2-4 mm in diameter all over her central face including over the eyelids and vermillion border of lips [Figure 1]... She also had few leafy white macules scattered over her trunk [Figure 2], apart from numerous confetti-like white freckles [Figure 3], rubbery hypopigmented plaques [Figure 4] on her lower back and lower limbs, and subungual tumors [Figure 5]... The cutaneous manifestations of this phakomatosis include the ash-leaf macules, confetti skin lesions, adenoma sebaceum, shagreen patches and Koenen's tumors... Confetti skin lesions are white freckles, seen less commonly, but are most specific... These hypomelanotic macules on histopathology reveal a normal number of melanocytes, however, the melanocytes have poorly developed dendritic processes with smaller and fewer melanosomes. “Adenoma sebaceum” was the old misnomer for facial angiofibromas, seen in a butterfly pattern and giving a ruddy appearance to the cheeks... These are small red to hyperpigmented, dome-shaped, discrete, smooth, bilaterally symmetrical papules seen over the central face, with relative sparing of the upper lip and lateral face... This dome-shaped lesion on biopsy shows large spindle-shaped fibroblasts in the dermis and a characteristic perifollicular concentric fibrosis giving an “onion-skin pattern. ” Shagreen patch, also called collagenoma, is named after a type of leather; felt as a rubbery plaque with a firm, irregular, knobby surface similar to shark skin... The prominence of slightly depressed hair follicles is characteristic, giving it an “orange peel” or pigskin-like appearance... Koenen's tumors are ungual fibromas, usually the last cutaneous manifestation, at puberty... They are seen more often over the toe nails rather than fingernails and may be periungual or subungual.

No MeSH data available.


Related in: MedlinePlus