A boy in his teens with large ulcerations of the head and neck
BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, has a predilection for the upper airways, lungs and kidneys. However, any other organ can be affected. Although cutaneous lesions are common, they have only rarely been reported as a primary manifestation of the disease.
CASE PRESENTATION: We present a case of a teenage boy with pyoderma gangrenosum-like ulcerations of the neck and face. Anti-neutrophil cytoplasmic antibody with antigen specificity for proteinase 3 (PR3-ANCA) was detected. In the absence of other symptoms and organ manifestations, the ulcerations were still considered to be pyoderma gangrenosum. The ulcers started to heal during treatment with corticosteroids and infliximab. One month later the patient developed sinusitis, and eventually lost vision in his left eye. The diagnosis was changed to GPA and he started treatment with methylprednisolone, rituximab and cyclophosphamide with good response on vision, sinusitis and ulcerations.
INTERPRETATION: Recognition of this rare skin presentation of GPA is essential, to prevent delays in diagnosis and treatment that can lead to organ damage.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2019 |
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| Erschienen: |
2019 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:139 |
|---|---|
| Enthalten in: |
Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke - 139(2019), 7 vom: 09. Apr. |
| Sprache: |
Norwegisch |
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| Weiterer Titel: |
Gutt i tenårene med store ulcerasjoner i hodet og nakken |
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| Beteiligte Personen: |
Moen, Bjørnar Halsør [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 29.10.2019 Date Revised 29.10.2019 published: Electronic-Print Citation Status MEDLINE |
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| doi: |
10.4045/tidsskr.18.0647 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM29590433X |
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| 100 | 1 | |a Moen, Bjørnar Halsør |e verfasserin |4 aut | |
| 245 | 1 | 2 | |a A boy in his teens with large ulcerations of the head and neck |
| 246 | 3 | 3 | |a Gutt i tenårene med store ulcerasjoner i hodet og nakken |
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| 500 | |a Date Completed 29.10.2019 | ||
| 500 | |a Date Revised 29.10.2019 | ||
| 500 | |a published: Electronic-Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, has a predilection for the upper airways, lungs and kidneys. However, any other organ can be affected. Although cutaneous lesions are common, they have only rarely been reported as a primary manifestation of the disease | ||
| 520 | |a CASE PRESENTATION: We present a case of a teenage boy with pyoderma gangrenosum-like ulcerations of the neck and face. Anti-neutrophil cytoplasmic antibody with antigen specificity for proteinase 3 (PR3-ANCA) was detected. In the absence of other symptoms and organ manifestations, the ulcerations were still considered to be pyoderma gangrenosum. The ulcers started to heal during treatment with corticosteroids and infliximab. One month later the patient developed sinusitis, and eventually lost vision in his left eye. The diagnosis was changed to GPA and he started treatment with methylprednisolone, rituximab and cyclophosphamide with good response on vision, sinusitis and ulcerations | ||
| 520 | |a INTERPRETATION: Recognition of this rare skin presentation of GPA is essential, to prevent delays in diagnosis and treatment that can lead to organ damage | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Necrotising ulceration | |
| 650 | 4 | |a PR3-ANCA | |
| 650 | 4 | |a face ulcer neck ulcer. | |
| 650 | 4 | |a granulomatosis with polyangiitis | |
| 650 | 4 | |a malignant pyoderma | |
| 650 | 4 | |a pyoderma gangrenosum | |
| 650 | 4 | |a vasculitis | |
| 650 | 7 | |a Dermatologic Agents |2 NLM | |
| 650 | 7 | |a Glucocorticoids |2 NLM | |
| 650 | 7 | |a Immunologic Factors |2 NLM | |
| 650 | 7 | |a Rituximab |2 NLM | |
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| 650 | 7 | |a Prednisolone |2 NLM | |
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| 700 | 1 | |a Barrett, Torill Myklestad |e verfasserin |4 aut | |
| 700 | 1 | |a Sandvik, Lene Frøyen |e verfasserin |4 aut | |
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