Metabolic and storage diseases

Weedon, David

doi:10.1016/b978-0-7020-3485-5.00019-x

Cited by 8 publications

(10 citation statements)

References 622 publications

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“…The main features consist of eosinophilic hyaline material in and around small vessels of the upper dermis, reduplication of vascular and sometimes epidermal basement membrane, and the deposition of fibrillar and amorphous material around the superficial vessels and at the dermoepidermal junction. 165 Peculiar, elongated, segmented eosinophilic structures in the roof of PCT, EPP, and pseudoporphyria bullae, called "caterpillar bodies," may be seen. 182 They are periodic acidSchiff (PAS) positive and contain collagen IV.…”

Section: Histopathologymentioning

confidence: 98%

“…173 There are 3 major forms of PCT: familial, sporadic, and toxic. 165 The familial form is inherited as an autosomal dominant trait and its onset is usually earlier than that of the sporadic form. The sporadic form usually has its onset in mid-life.…”

Section: Porphyriasmentioning

confidence: 99%

“…Type IV collagen and laminin are additional components of the vascular and perivascular hyaline material that may be detected using monoclonal antibodies. 165 Ultrastructurally, the hyaline deposits found in the basement membrane and upper dermal blood vessels consist of concentric basement membrane multiplications. 100 The "caterpillar bodies" appear to be a combination of degenerating keratinocytes, colloid bodies, and basement membrane bodies.…”

Section: Histopathologymentioning

confidence: 99%

“…The acute episodes are characterized by abdominal pain, neurological changes, and psychiatric disturbances. 165 The cutaneous manifestations are of 2 types: an acute flare pattern with rapidly evolving, painful, and burning lesions with some erythema and edema, and a second pattern of skin fragility with the development of blisters, erosions, and scars. 166 CEP or Gunther disease is a rare autosomal recessive disorder, in which the defective enzyme is uroporphyrinogen III synthase, which leads to an accumulation uroporphyrinogen I and III in the bone marrow, blood, and other organs.…”

Section: Porphyriasmentioning

confidence: 99%

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Cutaneous Deposits

Molina-Ruiz

Cerroni

Kutzner

et al. 2014

The American Journal of Dermatopathology

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: The cutaneous deposition disorders are a group of unrelated conditions characterized by the accumulation of either endogenous or exogenous substances within the skin. These cutaneous deposits are substances that are not normal constituents of the skin and are laid down usually in the dermis, but also in the subcutis, in a variety of different circumstances. There are 5 broad categories of cutaneous deposits. The first group includes calcium salts, bone, and cartilage. The second category includes the hyaline deposits that may be seen in the dermis in several metabolic disorders, such as amyloidosis, gout, porphyria, and lipoid proteinosis. The third category includes various pigments, heavy metals, and complex drug pigments. The fourth category, cutaneous implants, includes substances that are inserted into the skin for cosmetic purposes. The fifth category includes miscellaneous substances, such as oxalate crystals and fiberglass. In this article, the authors review the clinicopathologic characteristics of cutaneous deposition diseases, classify the different types of cutaneous deposits, and identify all the histopathologic features that may assist in diagnosing the origin of a cutaneous deposit.

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Section: Histopathologymentioning

confidence: 98%

Section: Porphyriasmentioning

confidence: 99%

Section: Histopathologymentioning

confidence: 99%

Section: Porphyriasmentioning

confidence: 99%

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Cutaneous Deposits

Molina-Ruiz

Cerroni

Kutzner

et al. 2014

The American Journal of Dermatopathology

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“…Diabetic microangiopathy, characterized by thickenning of the walls of small blood vessels by the deposition of PAs-positive material, together with sparse perivascular lymphocytic infiltrate may accompany. Direct immunofluorescence is negative (8). Rarely, in some autoimmune thyroid diseases, especially in Graves disease, immunobullous disorders have been reported in association with it (9).…”

Section: Bullous Diseasesmentioning

confidence: 99%

Skin manifestations of endocrine diseases

Demirkesen¹

2015

TJPATH

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Endocrine diseases may result in changes in cutaneous function and morphology, which cause various skin manifestations, including nonspecific or pathognomonic signs. some of these manifestations are already known dermatologic diseases with only increased frequency in this patient group. As a result the skin may the play role of a screen displaying endocrine disorders, either due to hormone excess or deficiency. Awareness of the skin manifestations may permit prompt and adequate approach to the patients, and therefore facilitate the early diagnosis of the endocrine disease and even be life saving. some of these manifestations may be recognized clinically, but sometimes they need to be confirmed histopathologically. In this article, many endocrine diseases and their associated skin lesions will be reviewed briefly.

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Atlas of Dermatology in Internal Medicine

Saanchez¹

2012

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Metabolic and storage diseases

Cited by 8 publications

References 622 publications

Cutaneous Deposits

Cutaneous Deposits

Skin manifestations of endocrine diseases

Atlas of Dermatology in Internal Medicine

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