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Extremely low levels of epidermal skin-derived antileucoproteinase/elafin in a patient with impetigo herpetiformis.
Br J Dermatol. 1997 Jul; 137(1):123-9.BJ

Abstract

Impetigo herpetiformis (IH) is a rare pustular dermatosis with unknown aetiology, typically occurring during pregnancy. Based upon a similar clinical and histological presentation, i.e. spongiform accumulation of polymorphonuclear leucocytes in the stratum corneum, several authors consider IH as a variant of generalized pustular psoriasis (GPP), while others state that IH is a separate entity. Skin-derived antileucoproteinase (SKALP) is a strong and specific inhibitor of human leucocyte elastase (HLE) and proteinase 3, two neutral proteinases that have been implicated in leucocyte migration and tissue destruction. Previously, we reported decreased SKALP activity in pustular forms of psoriasis compared with plaque psoriasis. In this study we present a case study of a patient with IH, where SKALP activity was measured using biochemical and immunochemical techniques. Epidermal scales and sera were collected during the course of the disease. Comparison was made with three patients with GPP and six patients with plaque psoriasis. Initially, anti-HLE activity in epidermal scales of the patient with IH was comparable with values in patients with GPP, i.e. decreased compared with plaque psoriasis. During the course of the disease, anti-elastase activity dropped to undetectable levels, concomitant with the appearance of free elastase activity. This finding suggests a total saturation of epidermal anti-HLE activity. Low levels of SKALP, presumably complexed with HLE, could be measured immunochemically in scale extracts. Serum levels of total SKALP correlated with the disease activity. We suggest that a reduced amount of epidermal SKALP contributes to an imbalance between elastase and its inhibitor, resulting in the formation of epidermal pustules. This mechanism of pustule formation could apply both to GPP and IH, suggesting a final common pathway in the pathogenic mechanisms of IH and GPP.

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Authors+Show Affiliations

Kuijpers AL
Department of Dermatology, University Hospital Nijmegen, The Netherlands.
Schalkwijk J
No affiliation info available
Rulo HF
No affiliation info available
Peperkamp JJ
No affiliation info available
van de Kerkhof PC
No affiliation info available
de Jong EM
No affiliation info available

MeSH

AdultEnzyme-Linked Immunosorbent AssayEpidermisFemaleHumansImpetigoIsotretinoinKeratolytic AgentsPregnancyPregnancy ComplicationsPregnancy Trimester, ThirdProteinase Inhibitory Proteins, SecretoryProteinsPsoriasisSerine Proteinase Inhibitors

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

9274639

Citation

Kuijpers, A L., et al. "Extremely Low Levels of Epidermal Skin-derived Antileucoproteinase/elafin in a Patient With Impetigo Herpetiformis." The British Journal of Dermatology, vol. 137, no. 1, 1997, pp. 123-9.
Kuijpers AL, Schalkwijk J, Rulo HF, et al. Extremely low levels of epidermal skin-derived antileucoproteinase/elafin in a patient with impetigo herpetiformis. Br J Dermatol. 1997;137(1):123-9.
Kuijpers, A. L., Schalkwijk, J., Rulo, H. F., Peperkamp, J. J., van de Kerkhof, P. C., & de Jong, E. M. (1997). Extremely low levels of epidermal skin-derived antileucoproteinase/elafin in a patient with impetigo herpetiformis. The British Journal of Dermatology, 137(1), 123-9.
Kuijpers AL, et al. Extremely Low Levels of Epidermal Skin-derived Antileucoproteinase/elafin in a Patient With Impetigo Herpetiformis. Br J Dermatol. 1997;137(1):123-9. PubMed PMID: 9274639.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Extremely low levels of epidermal skin-derived antileucoproteinase/elafin in a patient with impetigo herpetiformis. AU - Kuijpers,A L, AU - Schalkwijk,J, AU - Rulo,H F, AU - Peperkamp,J J, AU - van de Kerkhof,P C, AU - de Jong,E M, PY - 1997/7/1/pubmed PY - 1997/7/1/medline PY - 1997/7/1/entrez SP - 123 EP - 9 JF - The British journal of dermatology JO - Br J Dermatol VL - 137 IS - 1 N2 - Impetigo herpetiformis (IH) is a rare pustular dermatosis with unknown aetiology, typically occurring during pregnancy. Based upon a similar clinical and histological presentation, i.e. spongiform accumulation of polymorphonuclear leucocytes in the stratum corneum, several authors consider IH as a variant of generalized pustular psoriasis (GPP), while others state that IH is a separate entity. Skin-derived antileucoproteinase (SKALP) is a strong and specific inhibitor of human leucocyte elastase (HLE) and proteinase 3, two neutral proteinases that have been implicated in leucocyte migration and tissue destruction. Previously, we reported decreased SKALP activity in pustular forms of psoriasis compared with plaque psoriasis. In this study we present a case study of a patient with IH, where SKALP activity was measured using biochemical and immunochemical techniques. Epidermal scales and sera were collected during the course of the disease. Comparison was made with three patients with GPP and six patients with plaque psoriasis. Initially, anti-HLE activity in epidermal scales of the patient with IH was comparable with values in patients with GPP, i.e. decreased compared with plaque psoriasis. During the course of the disease, anti-elastase activity dropped to undetectable levels, concomitant with the appearance of free elastase activity. This finding suggests a total saturation of epidermal anti-HLE activity. Low levels of SKALP, presumably complexed with HLE, could be measured immunochemically in scale extracts. Serum levels of total SKALP correlated with the disease activity. We suggest that a reduced amount of epidermal SKALP contributes to an imbalance between elastase and its inhibitor, resulting in the formation of epidermal pustules. This mechanism of pustule formation could apply both to GPP and IH, suggesting a final common pathway in the pathogenic mechanisms of IH and GPP. SN - 0007-0963 UR - https://www.unboundmedicine.com/medline/citation/9274639/Extremely_low_levels_of_epidermal_skin_derived_antileucoproteinase/elafin_in_a_patient_with_impetigo_herpetiformis_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0007-0963&date=1997&volume=137&issue=1&spage=123 DB - PRIME DP - Unbound Medicine ER -