Hypergammaglobulinemic Purpura in Cystic Fibrosis

Nielsen, H. E.; Lundh, Søren; Jacobsen, Søren; Høiby, Niels

doi:10.1111/j.1651-2227.1978.tb16351.x

Cited by 24 publications

(14 citation statements)

References 14 publications

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“…6 Hypergammaglobulinaemia and high serum complement have also been reported in association with these cutaneous lesions in cystic fibrosis6 7 and were seen in some of our patients as well. Whether these and other phenomena such as the presence of circulating immune complexes play a direct role in the pathogenesis of joint and skin lesions in cystic…”

Section: Patientssupporting

confidence: 74%

Arthritis in cystic fibrosis.

Schidlow¹,

Goldsmith²,

Palmer³

et al. 1984

Archives of Disease in Childhood

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Cushing's syndrome and bronchial carcinoid tumour 377 costeroids are unaffected by high dose dexamethasone and metyrapone.In our patient, the adrenal function tests were so consistent with pituitary disease that the pulmonary lesion was initially thought to be coincidental. However, the resolution of hypercortisolism after excision of a tumour, known in adults3 to be associated with the ectopic adrenocorticotrophic hormone syndrome, and the finding of adrenocorticotrophic hormone and related peptides in the tumour, confirmed the diagnosis of ectopic adrenocorticotrophic hormone secretion.Serum

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Section: Patientssupporting

confidence: 74%

Arthritis in cystic fibrosis.

Schidlow¹,

Goldsmith²,

Palmer³

et al. 1984

Archives of Disease in Childhood

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“…Purpura has been reported in CF and has been described as cutaneous necrotizing vasculitis or hypergammaglobulinaemic purpura, although not all the patients had hypergammaglobulinaemia [16,17]. The skin biopsy in our patient showed mild oedematous changes of the endothelium of the capillaries, thickening of arteriolar walls and deposition of IgA, C3 and fibrinogen by immunofluorescence.…”

Section: Discussionsupporting

confidence: 43%

Nephrotic syndrome and fibrillary glomerulonephritis

Laufer¹,

Augarten

Szeinberg

et al. 1997

Journal of Internal Medicine

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A 28-year-old female with cystic fibrosis presented with nephrotic syndrome and progressive renal failure. In addition, she complained of blurred vision and there was a purpuric skin eruption localized to her legs. A renal biopsy revealed fibrillary glomerulonephritis. Skin biopsy demonstrated swelling of capillary endothelium, thickening of arteriolar walls and deposition of IgA, C3 and fibrinogen by immunofluorescence. Opthalmoscopy and fluorescein angiography disclosed cotton wool spots with intraretinal haemorrhages and ischaemia of the macula. Albumin infusions resulted in worsening of eye symptoms and signs. The presence of these three clinicopathologic entities in a patient with CF may indicate the possibility of systemic involvement related to continued exposure to chronic bacterial lower lung infection.

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“…The most common clinical presentation of CF‐associated vasculitis includes macular purpura that progresses to palpable purpura. Urticarial vasculitis and bullae secondary to vasculitis have also been reported (21,22–26,28). Vasculitic lesions tend to be located on the lower extremities (21–23).…”

Section: Vascular Damage—vasculitismentioning

confidence: 99%

“…One study noted that the appearance of CF‐associated hypergammaglobulinemic purpura was associated with an average survival of 10 months (23), although patients are often reported late in the disease course (24). Data regarding the relationship between elevated circulating immune complexes and the severity of the CF disease process is conflicting (61,62,65,66).…”

Section: Vascular Damage—vasculitismentioning

confidence: 99%

Cutaneous Manifestations of Cystic Fibrosis

Bernstein

McCusker

Grant‐Kels

2008

Pediatric Dermatology

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Cystic fibrosis is an autosomal recessive disease reported in 1 in 2500 live births in Northern American and Northern European Caucasian populations. Classic disease findings include chronic bacterial infection of airways and sinuses, malabsorption of fat, infertility in men, and elevated concentrations of chloride in sweat. Less well-recognized findings associated with cystic fibrosis include cutaneous findings, which can be primary or secondary manifestations of the disease process. Patients demonstrate more atopic and drug hypersensitivity reactions than the general population, but have similar rates of urticaria compared with the general population. In atypical presentations of cystic fibrosis, the nutrient deficiency dermatitis of the disease may aid with diagnosis, and notably can be the presenting sign. Other dermatologic manifestations of cystic fibrosis include early aquagenic skin wrinkling and cutaneous vasculitis, which can be associated with arthralgias. Familiarity with the nutrient deficiency dermatitis of this entity may play a role in the timely diagnosis of the disease, and the other cutaneous findings add to our understanding of the protean nature of its manifestations.

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Hypergammaglobulinemic Purpura in Cystic Fibrosis

Cited by 24 publications

References 14 publications

Arthritis in cystic fibrosis.

Arthritis in cystic fibrosis.

Nephrotic syndrome and fibrillary glomerulonephritis

Cutaneous Manifestations of Cystic Fibrosis

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