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Int J Clin Exp Pathol 2011;4(5):526-529

Case Report
Subcorneal pustular dermatosis an immune-histopathological perspective

Ana Maria Abreu Velez, J Graham Smith, Jr., Michael S Howard

Georgia Dermatopathology Associates, Atlanta, Georgia, USA; Diagnostic and Medical Clinic/Dermatology, Mobile, Alabama, USA.

Received March 28, 2011; accepted May 25, 2011; Epub June 3, 2011; published June 20, 2011

Abstract: Subcorneal pustular dermatosis (SPD) represents a chronic, relapsing sterile pustular eruption, involving the trunk and flexoral
proximal extremities. A 54-year-old female presented with recurrent, flaccid pustules measuring several millimeters in diameter, on normal and
mildly erythematous skin of the groin and submammary areas. Biopsies for hematoxylin and eosin (H&E) examination, direct
immunofluorescence (DIF) and immunohistochemistry (IHC) analysis were performed. The H&E staining demonstrated typical features of
SPD, including some damage within dermal pilosebaceous units subjacent to the subcorneal blistering process. DIF revealed strong deposits
of immunoreactants IgG, IgM, fibrinogen and complement/C3, present in a shaggy pattern within the subcorneal disease areas; in focal, areas
of the basement membrane junction and in focal pericytoplasmic areas of epidermal keratinocytes. IHC revealed strong positivity to
HLA-DPDQDR, mast cell tryptase, CD68, and ZAP-70 in the subcorneal inflammatory infiltrate, and surrounding dermal blood vessels.
Myeloperoxidase was also positive. Positive staining with the anti-ribosomal protein S6-pS240 at the edges of hair follicles and sebaceous
glands subjacent to the subcorneal blisters was also noted.  Conclusions: We conclude that this disorder may have several components in its
etiopathology, including a possible restricted immune response and a possible genetic component; these possibilities warrant further
investigation. (IJCEP1103008)

Keywords: Subcorneal pustular dermatosis, anti-ribosomal protein S6-pS240, mast cell tryptase, HLA-DPDQDR, ZAP-70

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Address all correspondence to:
Ana Maria Abreu-Velez, MD, PhD
Georgia Dermatopathology Associates
1534 North Decatur Road. NE
Suite 206, Atlanta, GA 30307-1000, USA.
Tel: (404) 371-0027, Fax: (404) 371-1900