Subcorneal pustular dermatosis an immnohisto-pathological perspective

Int J Clin Exp Pathol. 2011 Jun 20;4(5):526-9. Epub 2011 Jun 3.


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.

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

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antigens, CD / analysis
  • Antigens, Differentiation, Myelomonocytic / analysis
  • Chronic Disease
  • Diagnosis, Differential
  • Female
  • Fluorescent Antibody Technique, Direct
  • Groin / pathology
  • HLA Antigens / analysis
  • Humans
  • Immunoglobulins / analysis
  • Immunohistochemistry
  • Middle Aged
  • Skin / immunology
  • Skin / pathology*
  • Skin Diseases, Vesiculobullous / diagnosis*
  • Skin Diseases, Vesiculobullous / immunology
  • Skin Diseases, Vesiculobullous / pathology
  • Tryptases / analysis
  • ZAP-70 Protein-Tyrosine Kinase / analysis


  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD68 antigen, human
  • HLA Antigens
  • Immunoglobulins
  • ZAP-70 Protein-Tyrosine Kinase
  • Tryptases