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Simultaneous co-occurrence of porokeratosis of Mibelli with disseminated superficial actinic porokeratosis.

Mehta V, Balachandran C - Indian J Dermatol (2009)

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Sir, Porokeratosis is a group of hereditary or acquired disorders of epidermal keratinization, characterized by keratotic lesions with an atrophic center and a prominent peripheral ridge, with a typical histologic hallmark, the cornoid lamella... Classically, five clinical variants are recognized: classic porokeratosis of Mibelli (CPM), disseminated superficial actinic porokeratosis (DSAP), disseminated superficial porokeratosis (DSP), porokeratosis palmaris et plantaris disseminata, and linear porokeratosis... A 43-year-old lady presented with a pruritic hyperpigmented annular keratotic plaque with raised thready borders on the flexor aspect of the right forearm since childhood [Figure 1]... Similarly, such annular pigmented lesions appeared over the years on the face, neck, back and extensor forearms associated with photosensitivity [Figure 2]... DSAP is the most common of the five clinical variants of PK and usually develops in the third and fourth decades of life, with a female preponderance... There is good evidence that ultraviolet light can precipitate the development of new lesions or exacerbate preexisting DSAP, and this observation is consistent with its common clinical presentation in the sun-exposed areas... Unlike other forms of PK, DSAP does not appear to have significant risk of malignant change... Our case had lesions that were typical of DSAP on the face, neck, back and porokeratosis of Mibelli on the right forearm... Interestingly, she also gave history of a familial occurrence of such lesions in her mother and sister, which is quite rare... Reported cases of mixed PK in literature mostly involve DSAP and linear PK... Only three cases of Porokeratosis of Mibelli have been described in association with DSAP... The rarity of this association along with a familial occurrence prompted us to report this case... Our patient was commenced on topical retinoic acid along with sun-protective measures following which she was lost to follow up.

No MeSH data available.


40× photomicrograph showing the close up of the cornoid lamella
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Figure 0004: 40× photomicrograph showing the close up of the cornoid lamella

Mentions: A 43-year-old lady presented with a pruritic hyperpigmented annular keratotic plaque with raised thready borders on the flexor aspect of the right forearm since childhood [Figure 1]. Similarly, such annular pigmented lesions appeared over the years on the face, neck, back and extensor forearms associated with photosensitivity [Figure 2]. Her past medical history was noncontributory; however, she gave a history of similar lesions in her mother and sister. Histopathological examination of a representative lesion on the face and forearm revealed features typical of porokeratosis [Figures 3 and 4].


Simultaneous co-occurrence of porokeratosis of Mibelli with disseminated superficial actinic porokeratosis.

Mehta V, Balachandran C - Indian J Dermatol (2009)

40× photomicrograph showing the close up of the cornoid lamella
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2807725&req=5

Figure 0004: 40× photomicrograph showing the close up of the cornoid lamella
Mentions: A 43-year-old lady presented with a pruritic hyperpigmented annular keratotic plaque with raised thready borders on the flexor aspect of the right forearm since childhood [Figure 1]. Similarly, such annular pigmented lesions appeared over the years on the face, neck, back and extensor forearms associated with photosensitivity [Figure 2]. Her past medical history was noncontributory; however, she gave a history of similar lesions in her mother and sister. Histopathological examination of a representative lesion on the face and forearm revealed features typical of porokeratosis [Figures 3 and 4].

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Sir, Porokeratosis is a group of hereditary or acquired disorders of epidermal keratinization, characterized by keratotic lesions with an atrophic center and a prominent peripheral ridge, with a typical histologic hallmark, the cornoid lamella... Classically, five clinical variants are recognized: classic porokeratosis of Mibelli (CPM), disseminated superficial actinic porokeratosis (DSAP), disseminated superficial porokeratosis (DSP), porokeratosis palmaris et plantaris disseminata, and linear porokeratosis... A 43-year-old lady presented with a pruritic hyperpigmented annular keratotic plaque with raised thready borders on the flexor aspect of the right forearm since childhood [Figure 1]... Similarly, such annular pigmented lesions appeared over the years on the face, neck, back and extensor forearms associated with photosensitivity [Figure 2]... DSAP is the most common of the five clinical variants of PK and usually develops in the third and fourth decades of life, with a female preponderance... There is good evidence that ultraviolet light can precipitate the development of new lesions or exacerbate preexisting DSAP, and this observation is consistent with its common clinical presentation in the sun-exposed areas... Unlike other forms of PK, DSAP does not appear to have significant risk of malignant change... Our case had lesions that were typical of DSAP on the face, neck, back and porokeratosis of Mibelli on the right forearm... Interestingly, she also gave history of a familial occurrence of such lesions in her mother and sister, which is quite rare... Reported cases of mixed PK in literature mostly involve DSAP and linear PK... Only three cases of Porokeratosis of Mibelli have been described in association with DSAP... The rarity of this association along with a familial occurrence prompted us to report this case... Our patient was commenced on topical retinoic acid along with sun-protective measures following which she was lost to follow up.

No MeSH data available.