Cutaneous granulomas associated with primary immunodeficiency disorders
Identifieur interne : 001E84 ( Main/Exploration ); précédent : 001E83; suivant : 001E85Cutaneous granulomas associated with primary immunodeficiency disorders
Auteurs : A. Mitra ; B. Pollock ; J. Gooi ; J. C. Darling ; A. Boon [Royaume-Uni] ; J. A. Newton-BishopSource :
- British Journal of Dermatology [ 0007-0963 ] ; 2005-07.
English descriptors
- Teeft :
- Acad, Acad dermatol, Antibiotic, Ataxia, Ataxia telangiectasia, British association, Buttock, Cutaneous, Cutaneous granulomas, Cutaneous granulomatous lesions, Dermatol, Dermatologist, Dermatologists british journal, Dermatology, Dermis, Disease control, Eosin, Epithelioid, Epithelioid cells, Erythematous, Facial lesion, Granuloma, Granulomatous, Granulomatous process, Haematoxylin, Immunoglobulin, Intravenous, Lesion, Mitra, Necrobiosis, Oral prednisolone, Oral steroids, Prednisolone, Primary disorders, Sarcoidosis, Steroid, Subcutis, Systemic steroids, Telangiectasia, Visceral granulomas.
Abstract
Cutaneous granulomas are uncommon in primary immunodeficiency disorders. We report cutaneous granulomas in a child with ataxia telangiectasia (AT) and compare the clinical course with similar lesions in an adult with common variable immunodeficiency (CVI). A 4‐year‐old female with AT developed cutaneous granulomas as erythematous plaques. The largest lesion appeared on her left cheek and continued to progress despite treatment with topical and intralesional steroids. Disease control was obtained initially with oral antibiotics and low‐dose oral steroids. On cessation of oral steroids, significant relapse of the facial granuloma occurred. Pulsed and then oral steroids were required to stop the disease process leaving significant scarring. The second case is of cutaneous granulomas in a 66‐year‐old man, with CVI, who presented with an erythematous reticulate rash on the legs. We consider it useful to report this patient here as disease control was obtained in a similar way with systemic immunosuppression. In this patient a combination of oral steroids and azathioprine was used. These cutaneous granulomas are thought to be a manifestation of immune dysregulation. No infectious cause has been found so far. We recommend the use of broad‐spectrum antibiotics in conjunction with systemic steroids for progressive granulomas, as these patients are immunosuppressed and infection with an unidentified organism cannot be excluded.
Url:
DOI: 10.1111/j.1365-2133.2005.06619.x
Affiliations:
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We report cutaneous granulomas in a child with ataxia telangiectasia (AT) and compare the clinical course with similar lesions in an adult with common variable immunodeficiency (CVI). A 4‐year‐old female with AT developed cutaneous granulomas as erythematous plaques. The largest lesion appeared on her left cheek and continued to progress despite treatment with topical and intralesional steroids. Disease control was obtained initially with oral antibiotics and low‐dose oral steroids. On cessation of oral steroids, significant relapse of the facial granuloma occurred. Pulsed and then oral steroids were required to stop the disease process leaving significant scarring. The second case is of cutaneous granulomas in a 66‐year‐old man, with CVI, who presented with an erythematous reticulate rash on the legs. We consider it useful to report this patient here as disease control was obtained in a similar way with systemic immunosuppression. In this patient a combination of oral steroids and azathioprine was used. These cutaneous granulomas are thought to be a manifestation of immune dysregulation. No infectious cause has been found so far. We recommend the use of broad‐spectrum antibiotics in conjunction with systemic steroids for progressive granulomas, as these patients are immunosuppressed and infection with an unidentified organism cannot be excluded.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country></list><tree><noCountry><name sortKey="Darling, J C" sort="Darling, J C" uniqKey="Darling J" first="J. C." last="Darling">J. C. Darling</name><name sortKey="Gooi, J" sort="Gooi, J" uniqKey="Gooi J" first="J." last="Gooi">J. Gooi</name><name sortKey="Mitra, A" sort="Mitra, A" uniqKey="Mitra A" first="A." last="Mitra">A. Mitra</name><name sortKey="Newton Ishop, J A" sort="Newton Ishop, J A" uniqKey="Newton Ishop J" first="J. A." last="Newton-Bishop">J. A. Newton-Bishop</name><name sortKey="Pollock, B" sort="Pollock, B" uniqKey="Pollock B" first="B." last="Pollock">B. Pollock</name></noCountry><country name="Royaume-Uni"><noRegion><name sortKey="Boon, A" sort="Boon, A" uniqKey="Boon A" first="A." last="Boon">A. Boon</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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