Bullous lesions of the skin and mucous membranes in primary amyloidosis

Northover, J. M. A.; Presbury, D. G. C.; Pickard, J. D.; Haskell, R.; Murray‐Lyon, Iain M.; Keith, David A.

doi:10.1136/pgmj.48.560.351

Cited by 29 publications

(5 citation statements)

References 13 publications

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“…Our patient had oesophagopharyngeal blisters and erosions, which were associated with subepithelial amyloid deposition. Although extension of bullous lesions to involve oral mucosa has previously been noted in two patients with bullous amyloidosis, 10 to our knowledge there has been no prior description of an oesophagopharyngeal disease. Vesicobullous oesophagitis is known to occur in several types of bullous dermatoses, including bullous pemphigoid, mucous membrane pemphigoid, various forms of pemphigus and epidermolysis bullosa, Hailey–Hailey disease, Darier disease, Stevens–Johnson syndrome and graft‐versus‐host disease 11–14 .…”

Section: Discussionmentioning

confidence: 67%

Mucocutaneous bullous amyloidosis with an unusual mixed protein composition of amyloid deposits

Rekhtman

Hash

Moresi

2005

British Journal of Dermatology

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We describe a case of fatal systemic amyloidosis presenting with mucocutaneous bullous lesions in a patient with IgA kappa monoclonal gammopathy. The amyloid plaques were composed of an unusual mixture of immunoglobulin kappa light chain and amyloid A proteins. Whereas oesophageal and oropharyngeal blisters are known to occur in several types of bullous dermatoses, to our knowledge this is the first report of oesophagopharyngeal blisters complicating bullous amyloidosis.

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Section: Discussionmentioning

confidence: 67%

Mucocutaneous bullous amyloidosis with an unusual mixed protein composition of amyloid deposits

Rekhtman

Hash

Moresi

2005

British Journal of Dermatology

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“…History and the presence of bullous skin lesions differentiate the oral blood blisters found in ABH from those observed in epidermolysis bullosa (inherited disorder). Another form of epidermolysis bullosa, called acquired oral non‐dystrophic epidermolysis bullosa, may begin later in life, but this form is accompanied by dermal lesions, lesions of the masticatory mucosa and by bullae on the gingivae which can be provoked by gentle friction (Northover et al , 1972).…”

Section: Discussionmentioning

confidence: 99%

Angina bullosa haemorrhagica: presentation of eight new cases and a review of the literature

et al. 2002

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Angina bullosa haemorrhagica (ABH) describes the acute and sometimes painful onset of oral blood-filled vesicles and bullae not attributable to blood dyscrasia, vesiculo-bullous disorders, systemic diseases or other known causes. The haemorrhagic bullae spontaneously burst after a short time resulting in ragged, often painless, superficial erosions that heal spontaneously within 1 week without scarring. Although the pathogenesis is still unclear, ABH seems to be a multifactorial phenomenon: dental or functional trauma seems to be the major provoking factor. The lesions of ABH can be easily confused with other mucosal diseases. It is important that the presentation of this benign disorder is distinguished from other more serious disorders with similar presenting features. The aim of this paper is to report the clinical features of eight cases of ABH, in an attempt to distinguish ABH from other blistering diseases of oral mucosa and to describe their management.

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“…Nail changes, including brittleness, crumbling, subungual striations and partial or complete anonychia, may be the initial and sometimes the sole presenting physical abnormality, and are reviewed elsewhere in the current issue of this journal (Breathnach, Wilkinson & Black, 1979). Bullous lesions consequent upon shearing within extensive intradermal amyloid deposits may occur on skin and mucous membranes (Chow & Burns, 1967;Northover et al, 1972), or be confined to oral mucous membrane. In some cases of systemic amyloidosis bullae were largely confined to the dorsum of the hands and closely simulated porphyria cutanea tarda (Hunter, 1976).…”

Section: Clinical Presentationmentioning

confidence: 99%

Systemic amyloidosis and the skin: a review with special emphasis on clinical features and therapy

Breathnach

Black

1979

Clinical and Experimental Dermatology

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Summary This report is a review of the literature on systemic amyloidosis and concentrates largely on primary and plasma cell dyscrasia‐related disease. Recent developments in the fields of amyloid structure and pathogenesis arc discussed and patterns of clinical presentation are described. It is emphasized that a combination of the carpal tunnel syndrome, macroglossia and specific mucocutaneous lesions necessitates an intensive search for an underlying plasma cell dyscrasia, and that immunodectrophoresis of serum and concentrated urine is essential to exclude a paraproteinaemia. Other diagnostic measures, including cutaneous histopathology, are considered. Despite the advances in our understanding of the pathogenesis of amyloid, there has been little progress in terms of the success of therapy, and the ultimate prognosis of patients with systemic amyloidosis remains poor.

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Bullous lesions of the skin and mucous membranes in primary amyloidosis

Abstract: SummaryFour cases of primary amyloidosis are described in which bullous lesions of the skin or oral mucosa were a prominent clinical feature. Biopsy of such lesions and demonstration of amyloid by special staining may be of great diagnostic help.

Cited by 29 publications

References 13 publications

Mucocutaneous bullous amyloidosis with an unusual mixed protein composition of amyloid deposits

Mucocutaneous bullous amyloidosis with an unusual mixed protein composition of amyloid deposits

Angina bullosa haemorrhagica: presentation of eight new cases and a review of the literature

Systemic amyloidosis and the skin: a review with special emphasis on clinical features and therapy

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