Systemische Therapien des Ulcus cruris Dissemond, Joachim; Cornelia Erfurt‐Berge; Goerge, Tobias ...
Journal der Deutschen Dermatologischen Gesellschaft,
07/2018, Letnik:
16, Številka:
7
Journal Article
Recenzirano
Odprti dostop
Zusammenfassung Für eine erfolgreiche Behandlung von Patienten mit Ulcus cruris ist es essentiell, die zugrundeliegende Ätiologie zu klären und, wenn möglich, eine kausale Therapie einzuleiten. ...Begleitend ist die an den Phasen der Wundheilung orientierte Wundbehandlung, meist in Verbindung mit einer Kompressionstherapie, die Therapie der Wahl. Der Stellenwert der systemischen Therapien wird in Abhängigkeit der zugrundeliegenden Genese sehr unterschiedlich und oft kontrovers beurteilt. In diesem Übersichtsartikel werden die aktuellen Aspekte der systemischen medikamentösen Therapien für Patienten mit Ulcus cruris bei den häufigen Genesen chronische venöse Insuffizienz, periphere arterielle Verschlusskrankheit, Livedovaskulopathie, Vaskulitis, Necrobiosis lipoidica, Calciphylaxie und Pyoderma gangraenosum differenziert vorgestellt und kritisch diskutiert. Zusammenfassend kann festgestellt werden, dass es sich bei vielen der hier vorgestellten Therapien um einen Off‐Label‐Use handelt. Bei den häufiger vorkommenden Wundarten wie beispielsweise Ulcus cruris venosum, mixtum und arteriosum sind Medikamente für die Therapie zwar hoffnungsvolle Optionen, bisher aber in der Regel kein Behandlungsstandard. Im Gegensatz hierzu haben systemische Therapien bei den anderen hier vorgestellten Krankheitsbildern eine meist zentral wichtige Bedeutung. Es sind in erster Linie immunmodulierende und rheologisch wirksame Medikamente, die eingesetzt werden, um die Wundheilung zu beschleunigen.
Summary
Background
The lack of objective diagnostic criteria renders pyoderma gangrenosum (PG) a diagnosis of exclusion. The diagnostic approaches proposed to date have not been systematically ...evaluated. Thus, PG remains a challenging and frequently misdiagnosed disorder.
Objectives
To develop and assess a comprehensive, yet clinically practicable, sensitive diagnostic scoring system for PG.
Methods
Clinical history and images of a total of 60 participants with previously confirmed PG located on the lower extremity and a control cohort of 50 patients with venous leg ulcers were retrospectively evaluated by expert teams at two tertiary dermatological centres specializing in wound care using a newly developed diagnostic scoring system composed of 10 criteria.
Results
The three major diagnostic criteria are rapidly progressing disease, assessment of relevant differential diagnoses and a reddish‐violaceous wound border (prevalent in 98% of patients with PG). Minor criteria (evident in 61–95% of patients with PG) include amelioration by immunosuppressant drugs, characteristically irregular shape of ulceration, extreme pain > 4/10 on a visual analogue scale and localization of lesion at the site of the trauma. Three additional criteria (observed in up to 60% of patients with PG) encompass suppurative inflammation in histopathology, undermined wound borders and systemic disease associated. A total score value of 10 points or higher indicates a high likelihood of PG and differentiates PG from venous leg ulcers. The initial letters of the above‐listed criteria form the acronym PARACELSUS.
Conclusions
The PARACELSUS score represents a novel, easily implementable, effective and sensitive diagnostic tool for PG.
What's already known about this topic?
Pyoderma gangrenosum (PG) represents a diagnosis of exclusion.
Objective and systematically evaluated diagnostic approaches for patients with suspected PG are lacking.
What does this study add?
A comprehensive and practicable diagnostic scoring system for PG was developed and evaluated by expert teams at two leading tertiary dermatological centres specializing in wound care. The initial letters of the score criteria form the acronym PARACELSUS.
A total score of 10 points or higher indicates a high likelihood of PG and differentiates PG from venous leg ulcers.
The PARACELSUS score is an effective and easily implementable diagnostic tool for PG.
Respond to this article
Linked Editorial: Schreml. Br J Dermatol 2019; 180:438–439.
Plain language summary available online
IMPORTANCE: Pyoderma gangrenosum is a rare inflammatory skin condition that is difficult to diagnose. Currently, it is a “diagnosis of exclusion,” a definition not compatible with clinical decision ...making or inclusion for clinical trials. OBJECTIVE: To propose and validate diagnostic criteria for ulcerative pyoderma gangrenosum. EVIDENCE REVIEW: Diagnostic criteria were created following a Delphi consensus exercise using the RAND/UCLA Appropriateness Method. The criteria were validated against peer-reviewed established cases of pyoderma gangrenosum and mimickers using k-fold cross-validation with methods of multiple imputation. FINDINGS: Delphi exercise yielded 1 major criterion—biopsy of ulcer edge demonstrating neutrophilic infiltrate—and 8 minor criteria: (1) exclusion of infection; (2) pathergy; (3) history of inflammatory bowel disease or inflammatory arthritis; (4) history of papule, pustule, or vesicle ulcerating within 4 days of appearing; (5) peripheral erythema, undermining border, and tenderness at ulceration site; (6) multiple ulcerations, at least 1 on an anterior lower leg; (7) cribriform or “wrinkled paper” scar(s) at healed ulcer sites; and (8) decreased ulcer size within 1 month of initiating immunosuppressive medication(s). Receiver operating characteristic analysis revealed that 4 of 8 minor criteria maximized discrimination, yielding sensitivity and specificity of 86% and 90%, respectively. CONCLUSIONS AND RELEVANCE: This Delphi exercise produced 1 major criterion and 8 minor criteria for the diagnosis of ulcerative pyoderma gangrenosum. The criteria may serve as a guideline for clinicians, allowing for fewer misdiagnoses and improved patient selection for clinical trials.
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