Childhood linear IgA disease in association with autoimmune lymphoproliferative syndrome

Wong, Chun-Shu; Arkwright, Peter D.; Rieux-Laucat, Frédéric; Cant, Andrew J.; Stevens, Robert G.; Judge, M. R.

doi:10.1111/j.1365-2133.2004.05850.x

Cited by 19 publications

(12 citation statements)

References 15 publications

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“…7,11 Childhood LABD has been mostly associated with infections or drug ingestion and rarely with other conditions such as ulcerative colitis and autoimmune lymphoproliferative disease. 2,9 The most frequently reported drugs that can induce LABD in adults are antibiotics such as vancomycin, penicillin G, ampicillin, cefamandole, TMP-SMX, rifampin and sulfisoxazole, NSAID such as piroxicam, naproxen and diclofenac, interferon-gamma, interleukin-2, phenytoin, amiodarone, captopril, iodine contrast agent, somatostatin, lithium, furosemide and cyclosporine. 1,4,5 Drug-induced LABD is not as well documented in childhood as it is in adults.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 LABD and chronic bullous disease of childhood are now considered to be a single entity. 3 The diagnosis of LABD is confirmed by detecting a linear band of IgA deposit at the basement membrane.…”

Section: Introductionmentioning

confidence: 99%

“…3 The diagnosis of LABD is confirmed by detecting a linear band of IgA deposit at the basement membrane. 2 As many as two-thirds of LABD are related to drug ingestion, particularly certain antibiotics, non-steroidal anti-inflammatory drugs (NSAID) and diuretics. 1 In this case report, we describe a drug-induced LABD secondary to trimethoprim-sulfamethoxazole (TMP-SMX) which was possibly triggered by a CMV infection.…”

Section: Introductionmentioning

confidence: 99%

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Childhood linear IgA bullous disease induced by trimethoprim-sulfamethoxazole

Nantel-Battista¹,

Dhaybi²,

Hatami³

et al. 2010

J Dermatol Case Rep

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Background: Linear IgA bullous disease (LABD) is a rare mucocutaneous autoimmune subepidermal blistering disease that can affect children mostly of pre-school age. As many as two-thirds of LABD are related to drug ingestion, particularly certain antibiotics, non-steroidal anti-inflammatory drugs and diuretics.Main observation: We describe a 3-year-old boy who presented a CMV infection followed by LABD induced by trimtheporim-sulfametoxazole. To our knowledge, this is the first reported case of trimethoprim-sulfamethoxazole that was confirmed by a rechallenge.Conclusions: Most cases of drug-induced LABD are patients being treated with multiple systemic drugs that could induce the LABD. In the lack of suitable alternative treatment, the identification of the causative drug can be achieved by a rechallenge under close medical surveillance.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Childhood linear IgA bullous disease induced by trimethoprim-sulfamethoxazole

Nantel-Battista¹,

Dhaybi²,

Hatami³

et al. 2010

J Dermatol Case Rep

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“…In a case, as suspected in our patient, ALPS was associated with linear disease, an autoimmune subepithelial disorder characterized, at all ages from the first month of life, by onset of blisters on skin and mucosal membranes, pain and swallowing difficulties, raised serum IgA, and linear IgA deposits at the basement membrane zone. 24 Wu et al 25 reported on a case of lupus and lymphoproliferative disease, due to a FAS-ligand gene mutation and consequent FAS-ligand deficiency, with positive lupus test, raised T-cells after activation, normal CD4 -CD8 -T cells in blood (ALPS type 1b). Dianzani et al 26 reported on a series of male patients with a familial syndrome characterized by thrombocytopenia, enlarged lymph nodes and/or splenomegaly, neutropenia in which TCD4 -and TCD8 -cells were not expanded, PHA test was normal as in controls, and hyper γ-globulinemia (2 cases), hypo-γ-globulinemia (2 cases), and γ-globulinemia fluctuations (3) were present.…”

Section: Discussionmentioning

confidence: 99%

Association of severe myoclonic epilepsy of infancy (SMEI) with probable autoimmune lymphoproliferative syndrome-variant

2014

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The paper reported on a case of severe myoclonic epilepsy of infancy (SMEI) associated with a probable autoimmune lymphoproliferative syndrome variant (Dianzani autoimmune lymphoproliferative disease) (DALD). A male patient with typical features of SMEI and a SCN1A gene variant presented in the first year of life with multiple lymph nodes, palpable liver at 2 cm from the costal margin, neutropenia, dysgammaglobulinemia, relative and sometimes absolute lymphocytosis. Subsequently the patient presented with constantly raised IgA in serum and positive antinuclear and thyroid antimicrosomal antibodies. The diagnosis of probable autoimmune lymphoproliferative syndrome was made; arthritis, skin and throat blisters, which appeared subsequently led to the diagnosis of linear IgA disease. On the basis of these unique associations, the Authors hypothesized that autoimmunity may be partly responsible of the severe epileptic symptomatology, perhaps mediated by autoantibodies against sodium channels or by accompanying cytotoxic T-lymphocytes. Corticosteroid treatment ameliorated the epilepsy and laboratory tests. Future studies will be necessary to evaluate the relevance of autoimmunity in SMEI. RiassuntoGli Autori riportano un paziente con epilessia mioclonica severa dell'infanzia (SMEI) ed una variante del gene SCN1A, associata con una probabile sindrome autoimmune linfoproliferativa, (variante malattia autoimmune linfoproliferativa di Dianzani). Un paziente con il quadro clinico tipico della SMEI, presentò nel primo anno di vita multipli linfonodi, margine epatico palpabile a 2 cm dall'arco costale, neutropenia, disgammaglobulinemia, linfocitosi relativa e talvolta assoluta. Successivamente il paziente presentò IgA nel siero costantemente elevate ed anticorpi antinucleo ed antimicrosomi tiroidei positivi. Fu posta diagnosi di probabile variante della sindrome autoimmune linfoproliferativa (DALD); la comparsa di artrite, vescicole cutanee ed in faringe, che apparvero successivamente, portarono alla diagnosi di possibile malattia lineare ad IgA. Sulla base di queste associazioni, segnalate per la prima volta, gli Autori hanno ipotizzato che, oltre alla turba genetica, l'autoimmunità possa essere in parte responsabile della grave sintomatologia epilettica, forse mediata da autoanticorpi contro i canali del sodio o da T-linfociti citotossici. Il trattamento con cortisone migliorò il quadro epilettico, clinico e di laboratorio. Ulteriori studi diranno se è consigliabile ricercare sistematicamente la presenza di autoimmunità in ogni caso di SMEI.

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“…Lebensjahr auf und zeigt in der Regel einen selbstlimitierenden Verlauf. Bei der LAD wurden assoziierte Erkrankungen wie lymphoproliferative Syndrome [10] und Colitis ulcerosa [4] beschrieben. Paige et al berichten über eine Inzidenz der Colitis ulcerosa von 7,1% bei Patienten mit einer LAD [7], diese liegt somit bedeutend höher, als sie bei der Gesamtbevölkerung mit 0,2-0,5% angegeben wird [1,6].…”

Section: Diskussionunclassified