Extensive Macular Amyloidosis Associated With Poikiloderma

Serna-Pérez, M.J.; Vázquez-Doval, E.J.; Idoate, Miguel A.; Casas, M. A. Sola; Quintanilla, E.

doi:10.1111/j.1365-4362.1992.tb03571.x

Cited by 9 publications

(6 citation statements)

References 4 publications

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“…Early onset is considered as an important criterion for distinguishing PCA syndrome from ordinary PCA. Serna-Perez et al [7]described a patient with extensive macular amyloidosis with poikiloderma. The 49-year-old white woman had poikilodermatous appearance on her back, chest, arms and legs and an asymptomatic uniformly brown plaque on the internal aspect of her right arm.…”

Section: Discussionmentioning

confidence: 99%

Unusual Skin Manifestation of Cutaneous Amyloidosis

Hung¹,

Wang²,

Hong³

et al. 2003

Dermatology

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A 76-year-old man with a 20-year history of extensive cutaneous amyloidosis is reported. He had asymptomatic symmetric brownish reticulated pigmented patches with well-demarcated borders on his thighs, lower legs, dorsal feet and both arms. The trunk and popliteal fossae were not affected. A skin biopsy specimen showed abundant amyloid deposits in the papillary dermis and reticular dermis. Despite the extensive cutaneous involvement and large amount of amyloid in the deep dermis, no evidence of systemic amyloidosis could be found. Various manifestations of cutaneous amyloidosis are reviewed. We report this case to remind dermatologists of the protean presentations of cutaneous amyloidosis.

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Section: Discussionmentioning

confidence: 99%

Unusual Skin Manifestation of Cutaneous Amyloidosis

Hung¹,

Wang²,

Hong³

et al. 2003

Dermatology

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“…Sometimes pruritus may be absent. Unusual variants of PLCA include macular forms with diffuse hyperpigmentation,6 simulating nevoid hyperpigmentation7 and a poikilodermatous form 8. Amyloidosis cutis dyschromica is assumed to be a congenital disorder with hypersensitivity to UVB radiation, with possible DNA repair defects; hyperpigmented and hypopigmented xerotic lesions with deposits of amyloid in papillary dermis occur in sun-exposed skin 9.…”

Section: Discussionmentioning

confidence: 99%

Unusual presentation of generalized macular amyloidosis in a young adult

et al. 2008

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Macular amyloidosis is a common problem seen dermatology out-patient department. Generalized macular amyloidosis presenting with a poikilodermatous appearance is rare. In our case, an 18-year-old male presented with generalized hypopigmented macules with a poikilodermatous appearance of 10-year duration. His developmental milestones were normal with negative family history of similar complaints. Histopathology of hyperpigmented lesions revealed hyperkeratosis and acanthosis of epidermis and hypopigmented lesion showing only hyperkeratosis. Both lesions were showing the deposition of amorphous, hazy material in the tips of papillary dermis with perivascular inflammatory infiltrate. Congo red staining of the amorphous material was positive for amyloid.

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“…Furthermore, hypersensitivity to UVB with possible DNA repair defects has been suggested to be the cause of amyloidosis cutis dyschromica, a congenital form of primary cutaneous amyloidosis presenting as poikiloderma [19]. Amyloidosis cutis dyschromica is thought to be distinct from macular amyloidosis, a noncongenital form of primary cutaneous amyloidosis which can present as poikiloderma [20]. Thus, the relationship between poikilodermatous SCLE and macular amyloidosis should also be discussed.…”

Section: Discussionmentioning

confidence: 99%

“…Thus, the relationship between poikilodermatous SCLE and macular amyloidosis should also be discussed. Indeed, macular amyloidosis usually involves the lower extremities, although it can also be associated with generalized poikiloderma [20]; histologically, deposits of amyloid are limited to the papillary dermis, but there are few or no epidermal changes. In contrast, in poikilodermatous SCLE, ‘classic’ SCLE lesions are preexisting or coexisting with extensive poikilodermatous features, and histology shows both findings consistent with SCLE and amyloid deposits in poikilodermic skin.…”

Section: Discussionmentioning

confidence: 99%

Poikilodermatous Subacute Cutaneous Lupus erythematosus

2003

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Background: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of lupus erythematosus with unique clinical, immunological and genetic features. Among the unusual variants of SCLE, there is a poikilodermic presentation. However, to date, only 1 case of poikilodermatous SCLE has been reported. Objective: Our goal was to summarize the clinical characteristics and course as well as the pathological, laboratory and immunofluorescence findings of 4 patients with poikilodermatous SCLE. Methods: A retrospective study was conducted including 54 patients diagnosed as having SCLE between 1980 and 2002. Results: Four patients (7.4%) had SCLE. All patients were alive, and none developed severe systemic involvement in up to 36 years (median, 24 years) after the onset of disease. The most noteworthy laboratory finding was the cutaneous deposition of amyloid. Conclusion: Poikilodermatous SCLE represents an uncommon variant within the clinicopathological spectrum of SCLE following a favorable course, in spite of extensive cutaneous involvement. Photosensitivity is the pathomechanism explaining, theoretically, the development of both poikiloderma and cutaneous amyloidosis in such cases.

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Extensive Macular Amyloidosis Associated With Poikiloderma

Cited by 9 publications

References 4 publications

Unusual Skin Manifestation of Cutaneous Amyloidosis

Unusual Skin Manifestation of Cutaneous Amyloidosis

Unusual presentation of generalized macular amyloidosis in a young adult

Poikilodermatous Subacute Cutaneous Lupus erythematosus

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