Allergy Asthma Immunol Res.  2011 Apr;3(2):138-140. 10.4168/aair.2011.3.2.138.

A Rare Association Between Leukocyte Adhesion Deficiency Type I and Psoriasis in Humans

Affiliations
  • 1Department of Pediatrics, Ain Shams University, Cairo, Egypt. zeinabawad@gmail.com
  • 2Department of Dermatology, Ain Shams University, Cairo, Egypt.
  • 3Clinical Pathology, Ain Shams University, Cairo, Egypt.

Abstract

The beta2 integrins are expressed exclusively on leukocytes and participate in many immune and inflammatory processes. This subfamily comprises four heterodimeric glycoproteins with a common beta-subunit, designated beta2 (CD18). Spontaneous mutations of the CD18 gene result in leukocyte adhesion deficiency type I (LAD-I). Low level of CD18 expression has also been implicated in the pathogenesis of psoriasis. We here describe a child with recurrent skin infections without pus formation, persistent gingivitis and periodontitis. His blood counts showed persistent leukocytosis (neutrophilia). CD11b expression was defective on neutrophils, while that of CD18 was normal. So, our patient represents a mild variant of LAD-I with possible dysfunctional CD18. Moreover, he developed psoriasis with reduced CD18 expression on CD4+ T-cells. Psoriasiform dermatitis has been described before in association with LAD-I, however, clinically and histologically confirmed psoriasis in association with LAD-I has been described only in CD18 hypomorphic mice. Therefore, our patient represents the first clinically and histopathologically documented association between LAD-I and psoriasis in humans. It lends support to the role of beta2 integrins in the etiopathogenesis of psoriasis.

Keyword

beta2 integrins; LAD-I; psoriasis

MeSH Terms

Animals
Antigens, CD18
Child
Dermatitis
Gingivitis
Glycoproteins
Humans
Leukocytes
Leukocytosis
Mice
Neutrophils
Periodontitis
Psoriasis
Skin
Suppuration
T-Lymphocytes
Antigens, CD18
Glycoproteins

Figure

  • Fig. 1 Gingivitis, periodontitis and caries of upper central incisors.

  • Fig. 2 Psoriatic skin lesions on the upper limb; a scar is also seen.

  • Fig. 3 Skin biopsy findings. The epidermis shows hyperkeratosis, parakeratosis, hypogranulosis and acanthosis with regular elongation of the rete ridges (psoriatic epidermal hyperplasia). The dermis shows edema and mixed dermal inflammatory infiltrate together with dilation of the papillary capillaries.


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