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Occlusive cutaneous vasculopathies as cause of chronic ulcers
Moritz Ronicke1,2, Carola Berking1,2, Cornelia Erfurt-Berge1,2
1Department of Dermatology, Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany.
Insights
Occluding vasculopathies, conditions causing skin ulcers, require consideration for leg ulcers. Differentiating these from other causes like hypertonic leg ulcers is crucial for proper diagnosis and treatment.
Area of Science:
- Dermatology
- Pathology
- Vascular Medicine
Background:
- Occluding vasculopathies are a diverse group of conditions characterized by the obliteration of small cutaneous vessels.
- These conditions frequently present as skin ulcers, particularly on the legs, necessitating their inclusion in differential diagnoses.
Purpose of the Study:
- To highlight livedoid vasculopathy and calciphylaxis as key differential diagnoses for leg ulcers.
- To differentiate these conditions from other causes of leg ulceration, including hypertonic leg ulcers.
Main Methods:
- Review of literature focusing on occluding vasculopathies, livedoid vasculopathy, antiphospholipid syndrome, and calciphylaxis.
- Discussion of diagnostic criteria, including clinical presentation (livedo racemosa, retiform purpura) and histopathological evaluation.
- Emphasis on differentiating from inflammatory dermatoses, vasculitides, and hypertonic leg ulcers.
Main Results:
- Occluding vasculopathies, including livedoid vasculopathy and calciphylaxis, are significant considerations in leg ulcer differential diagnosis.
- Clinical signs like livedo racemosa and retiform purpura suggest reduced cutaneous perfusion.
- Histopathology and specific laboratory tests, especially coagulation parameters, are vital for accurate diagnosis.
Conclusions:
- Occluding vasculopathies, particularly livedoid vasculopathy and calciphylaxis, are important causes of leg ulcers that require careful diagnostic consideration.
- Distinguishing these from other leg ulcer etiologies, such as hypertonic leg ulcers and vasculitides, is essential for effective management.
- A combination of clinical findings, histopathological examination, and laboratory tests aids in diagnosing these complex vascular conditions.
Abstract:
The term occluding vasculopathies covers a large number of different conditions. These often manifest as skin ulcers. Occluding vasculopathies should be considered in the differential diagnosis of leg ulcers. The term "occlusive vasculopathies" encompasses pathophysiologically related entities that share structural or thrombotic obliteration of small cutaneous vessels. In this article, we will focus on livedoid vasculopathy with and without antiphospholipid syndrome and calciphylaxis with differentiation from hypertonic leg ulcer as the most relevant differential diagnoses of leg ulcer. The term also includes vascular occlusion, for example due to oxalate or cholesterol embolism, and septic vasculopathy. This often leads to acral ulceration and is therefore not a differential diagnosis with classic leg ulcers. It will not be discussed in this article. Occlusive vasculopathy may be suspected in the presence of the typical livedo racemosa or (non-inflammatory) retiform purpura as a sign of reduced cutaneous perfusion in the wound area. Inflammatory dermatoses, especially vasculitides, must be differentiated. This is achieved by histopathological evaluation of a tissue sample of sufficient size and depth taken at the appropriate time. In addition, specific laboratory parameters, particularly coagulation parameters, can support the diagnosis.
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