IgA pemphigus associated with diffuse large B-cell lymphoma showing unique reactivity with desmocollins: unusual clinical and histopathological features

Antidesmocollin 1 autoantibody negative subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma

“…Additional immunological and molecular studies in our patient ruled out IgA/IgG autoantibodies against other epidermal antigens. These findings are in contrast with those related to other lymphoproliferative disorders …”

“…The immunostaining observed by DIF and IIF in IgA pemphigus may show different patterns: IgA deposition to the cell surfaces of the uppermost epidermis in Dsc1‐related (SPD) type, to cell surfaces of the entire epidermis in the intraepidermal neutrophilic (IEN) type, or to the entire epidermis and stronger in the upper epidermis depending on whether anti‐Dsc2/Dsc3 antibodies are positive . Those IgA pemphigus cases associated with anti‐Dsg1 or anti‐Dsg3 antibodies show IgA deposits in cell surfaces of only the uppermost or lower epidermis, respectively.…”

The expanding spectrum ofIgApemphigus: a case report and review of the literature

“…Similar to our patient, subcorneal pustular dermatoses without anti-Dsc1 IgA have been previously reported. [10][11][12] A review of the literature indicates that SPD-like features can be associated with IgA antibodies targeting autoantigens other than Dsc1, whereas IgA reactivity against Dsc1 does not necessarily result in subcorneal pustule formation. Further underscoring the complexity of IGAP, several cases have been reported with positive direct and indirect immunofluorescence evidence for IgA autoantibodies, but no reactivity to either Dsc or Dsg, [13][14][15] suggesting the existence of other, not yet identified, cell surface autoantigens in IGAP.…”

Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus

“…49 Other cases showed haematological malignancies including those of B-cell origin, while some cases were associated with solid tumours, such as lung cancer. 76,77 Gastrointestinal diseases may also be associated with IgA pemphigus: one case each of Crohn's disease and gluten-sensitive enteropathy have been reported. 49 …”