Hyperkeratotic variant of porokeratosis Mibelli with dermal amyloid deposits

Uenishi, Toshiaki; Teramura, Kazuya; Kitamura, Masato; Fujii, Norikazu; Nakanishi, Gen; Tanaka, Toshihiro; Uehara, Masami

doi:10.1111/j.1346-8138.2010.00803.x

Cited by 15 publications

(17 citation statements)

References 16 publications

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“…A reported unique feature of porokeratosis ptychotropica is the presence of multiple cornoid lamellae on histopathological examination. In addition, amyloid deposition has been reported in several cases of porokeratosis ptychotropica, as well as in disseminated superficial actinic porokeratosis 4,5,7–9 . The lack of amyloid in our patient may be attributed to the limited amount of friction in the scrotal region, as compared to the intertriginous areas where it has been more frequently reported 10 .…”

Section: Discussionsupporting

confidence: 51%

Genitogluteal porokeratosis involving the scrotum: an unusual presentation of an uncommon disease

et al. 2011

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Porokeratosis represents a heterogeneous group of keratinization disorders typified by the presence of annular plaques with distinct, raised borders that include cornoid lamellae. Histopathologically, a cornoid lamella is a column of parakeratotic scale overlying an epidermal invagination that displays nearby dyskeratotic keratinocytes and loss of the granular layer. Porokeratosis ptychotropica constitutes a rare variant that classically presents as a plaque in the gluteal cleft that mimics a dermatitis and microscopically contains numerous cornoid lamellae. We report a 28 year-old man with a two-month history of scrotal burning and itching associated with the development of multiple thin red plaques with distinct elevated borders and a pebbled appearance. Histopathological examination revealed psoriasiform acanthosis and multiple cornoid lamellae, which is consistent with a diagnosis of porokeratosis ptychotropica. Our patient's presentation may represent a distinct variant with clinical features of verrucous porokeratosis and histopathological features of porokeratosis ptychotropica which may suggest that the finding of multiple cornoid lamellae is not unique to porokeratosis ptychotropica.

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Section: Discussionsupporting

confidence: 51%

Genitogluteal porokeratosis involving the scrotum: an unusual presentation of an uncommon disease

et al. 2011

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“…The present case was characterized by hyperkeratotic erythematous papules with severe itching. Verrucous papular and/or pruritic inflammatory lesions have been previously described as rare variants of porokeratosis 1–9 . In addition to the above‐noted macroscopic features, our case was histopathologically characterized by a prominent lichenoid tissue reaction.…”

supporting

confidence: 54%

“…Histopathologically, the present case also featured an extensive deposition of keratinocyte‐derived amyloid in the dermis, which might have been due to a persistent lichenoid tissue reaction‐mediated destruction of the epidermal cells. Although uncommon and not prominent as in the present case, dermal amyloid deposition has been reported to occur in porokeratosis 4,10,11 …”

contrasting

confidence: 38%

Hyperkeratotic variant of inflammatory disseminated superficial porokeratosis with lichenoid reaction and extensive amyloid deposition

2013

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“…Eosinophilic amorphous depositions positive for Dylon staining4 that were resistant to potassium permanganate treatment5 and positive for amyloid P component were found 6. These results strongly suggested AL amyloidosis.…”

Section: Patientmentioning

confidence: 84%

18F-fluorodeoxyglucose positron emission tomography might be useful for diagnosis of hepatic amyloidosis

Tawada¹,

Oide²,

Tsuyuguchi³

et al. 2014

IMCRJ

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We report on a woman with hepatic involvement of primary systemic (immunoglobulin light chain, AL) amyloidosis. Her diagnosis was confirmed by liver biopsy. Clinical symptoms of hepatic amyloidosis are generally mild at its first stage, with most frequent findings being hepatomegaly and alkaline phosphatase elevation. Recent advances in the understanding of the pathophysiology of systemic amyloidosis have made several treatments available. However, its prognosis is occasionally poor. Because liver biopsy is not always safe, other modalities for the diagnosis are needed. Of interest was that fluorodeoxyglucose (FDG) uptake into the liver was observed, compared with that into the spleen, in this patient, indicating that FDG positron emission tomography and computed tomography might be useful for the diagnosis of hepatic amyloidosis with mild liver dysfunction.

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Hyperkeratotic variant of porokeratosis Mibelli with dermal amyloid deposits

Cited by 15 publications

References 16 publications

Genitogluteal porokeratosis involving the scrotum: an unusual presentation of an uncommon disease

Genitogluteal porokeratosis involving the scrotum: an unusual presentation of an uncommon disease

Hyperkeratotic variant of inflammatory disseminated superficial porokeratosis with lichenoid reaction and extensive amyloid deposition

18F-fluorodeoxyglucose positron emission tomography might be useful for diagnosis of hepatic amyloidosis

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