Intercellular IgA dermatosis with clinical features of subcorneal pustular dermatosis

We report the case of a 51-year-old Japanese man with a unique pustulosis. He had multiple erythematous plaques and numerous pinpoint pustules on the trunk and extremities resembling pustular psoriasis. Histologic features revealed a fully developed intraepidermal abscess filled with neutrophils and disrupted epidermal keratinocytes. Mild leukocytosclastic vasculitis was seen in the underlying dermis. A direct immunofluorescence study revealed IgM, C1q, C3 and fibrinogen deposits in the dermal vessels. The patient had also sternoclavicular hyperostosis. We think that this represents a unique type of pustular vasculitis distinct from pustular psoriasis.

Section: Discussioncontrasting

confidence: 43%

Pustular Vasculitis with Clinical Feature of Pustular Psoriasis and Sternoclavicular Hyperostosis

Demitsu¹,

Ishiharajima²,

Miyamoto³

et al. 1993

“…Hashimoto et al [7], Saurai et al [8] and Piette et al [9] presented cases in which IgA bound in vivo to the intercel lular spaces of the epidermis and circulating IgA antibodies were found. Immuno-electrophoresis does not always reveal an alteration of IgA level.…”

mentioning

confidence: 99%

Intraepidermal Neutrophilic IgA Dermatosis: Pemphigus-Like IgA Deposits

Gengoux¹,

Tennstedt²,

Lachapelle³

1992

“…Clinically, he presented a hcrpetiform eruption, and direct immunofluorescence revealed intercellular antibodies of both the IgG and IgA class. Previously, cases with intercellular IgA deposits and subcorneal pustules were usually considered to be rare manifestations of subcorneal pustular dermatosis [1][2][3][4]. Indeed, subcorneal pustular dermatosis and pem phigus foliaceus may be very similar both clinically and pathologically [16].…”

Section: Discussionmentioning

confidence: 99%

IgA Pemphigus foliaceus: A Case Report

Zillikens

Miller²,

Hartmann³

et al. 1990

An 84-year-old male with a 4-week history of vesiculobullous eruptions was diagnosed clinically and pathologically to suffer from pemphigus foliaceus. Amazingly, direct immunofluorescence demonstrated intercellular IgA deposits in the whole epidermis. The patient did not respond to a regimen of fluocortolone and azathioprine but rapidly improved under dapsone. The few reported dermatoses with intraepidermal IgA deposits do not form a homogeneous group. In the following, their spectrum will be outlined. It is important to distinguish these dermatoses from IgG pemphigus, since they require a different therapy.