Annular Flaccid Pustules on the Trunk

Kiritsi, Dimitra; Hoch, M.; Kern, Johannes S.

doi:10.1001/jamadermatol.2017.0901

Cited by 2 publications

(1 citation statement)

References 8 publications

(17 reference statements)

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“…IgA pemphigus is an autoimmune blistering disorder, caused by IgA autoantibodies against keratinocyte cell surface antigens, desmocollins 1–3 and sometimes desmogleins 1 and 3. Based on clinical and histopathological characteristics it can be subdivided into subcorneal dermatosis type (SPD) and intraepidermal neutrophilic type (IEN) [ 1 , 2 ] The patients present with flaccid pustules solely or on scaly erythematous plaques, often arranged in an annular or circinate pattern [ 3 , 4 ], which are commonly itchy and located on the trunk. No treatment option has been found to be universally effective.…”

Section: Introductionmentioning

confidence: 99%

Systemic retinoids for treatment of recalcitrant IgA pemphigus

Schauer

Kern

Kiritsi

2018

Orphanet J Rare Dis

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IgA pemphigus is an exceedingly rare autoimmune blistering disorder, caused by IgA autoantibodies against desmosomal proteins. No treatment option has been found to be universally effective. The disease is often recalcitrant to oral steroids and immunosuppressants. Here, we describe the use of systemic retinoids for the treatment of recalcitrant IgA pemphigus in 3 cases. Although the use of acitretin has been reported before, we present for the first time the positive effects of alitretinoin in treatment of 2 patients with IgA pemphigus. Besides hyperlipoproteinaemia requiring use of hypolipidemic agents in one case, alitretinoin was well-tolerated and has generally a more favorable side effect spectrum than immunosuppressants.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0899-y) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Systemic retinoids for treatment of recalcitrant IgA pemphigus

Schauer

Kern

Kiritsi

2018

Orphanet J Rare Dis

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Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

et al. 2021

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The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.

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Annular Flaccid Pustules on the Trunk

Cited by 2 publications

References 8 publications

Systemic retinoids for treatment of recalcitrant IgA pemphigus

Systemic retinoids for treatment of recalcitrant IgA pemphigus

Anti-Desmocollin Autoantibodies in Autoimmune Blistering Diseases

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