Alles wat je moet weten over cutane vasculitis bij huidziekten

Cutaneous vasculitis, An algorithmic approach to diagnosis

Vasculitides, characterized by inflammation and damage of blood vessels, encompass a broad spectrum of diseases. They can occur with different pathophysiological mechanisms and have a rich clinical heterogeneity depending on the vessel diameters they affect. Vasculitides may also present with a broad spectrum of severity, ranging from a mild self-limiting to a potentially life-threatening disease. The high prevalence of skin involvement in vasculitis, visible character and, finally, the easy accessibility of the skin for both physical examination and biopsy offers important advantages for prompt disease recognition and diagnosis. Thus, dermatologists are privileged to diagnose the disease earlier and more effectively than any other discipline. As a consequence, a detailed clinical and histopathological evaluation of the skin is one of the most critical steps in diagnosing vasculitis. Besides obtaining a good medical history, laboratory and radiological evaluation methods are used in the diagnosis. In this review, a practical and algorithmic approach is aimed to assist in the diagnosis of vasculitis. However, this approach should not be seen as strict rules. This stepwise algorithmic diagnostic approach for vasculitis was developed by combining the current literature knowledge and the author's experience in this field to provide a rational framework for selecting the most appropriate among various diagnostic approaches.

Vasculitis refers to a broad and heterogeneous disease spectrum characterized by inflammation and damage of the blood vessel. It may occur in any organ of the body. When skin vessels are affected, the term cutaneous vasculitis is used. In systemic vasculitis, blood vessels of at least one organ are affected in addition to the skin. Of note, besides being a component of systemic vasculitis, including the skin, cutaneous vasculitis can be a skin-limited or skin-dominant expression or variant of systemic vasculitis. Finally, it may be a single-organ vasculitis of the skin (1). The skin is one of the most frequently affected organs in vasculitis, and small-vessel vasculitis of the skin is the most common vasculitis dermatologists encounter in their clinical practice (2). While the disease affects both genders equally, its frequency increases with age at diagnosis. Cutaneous vasculitis may present at any age, but it is more common in adults than children. The clinical course of the disease is usually self-limiting in children, and IgA vasculitis is the most common vasculitis in this age group. Idiopathic etiology is more prominent in adults and infectious etiology in children. Compared with children, underlying systemic vasculitis, connective tissue disease, or malignancy are more common in adult patients (3, 4).

StatPearls [Internet].

Jose L. Zaldivar Fujigaki , Fatima Anjum .

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Last Update: August 8, 2023 .

Schamberg disease is the most frequent purpuric of the dermatoses, with a classical presentation characterized by petechiae, purpura, and increased skin pigmentation. It is a benign and chronic condition, although idiopathic in origin, the diagnosis is not a dilemma since it is mainly made by inspections and identification of classic morphology of the lesions. The biopsy is required to rule out mycosis fungoides. This activity highlights the role of the interprofessional team in the evaluation and treatment of Schamberg disease.

References

1. Sunderkötter CH, Zelger B, Chen KR, Requena L, Piette W, Carlson JA, et al. Nomenclature of cutaneous vasculitis: dermatologic addendum to the 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheumatol. (2018) 70:171–84. doi: 10.1002/art.40375

2. Goeser MR, Laniosz V, Wetter DA. A practical approach to the diagnosis, evaluation, and management of cutaneous small-vessel vasculitis. Am J Clin Dermatol. (2014) 15:299–306. doi: 10.1007/s40257-014-0076-6

3. Lakdawala N, Fedeles F. Vasculitis: Kids are not just little people. Clin Dermatol. (2017) 35:530–40. doi: 10.1016/j.clindermatol.2017.08.004

5. Frumholtz L, Laurent-Roussel S, Lipsker D, Terrier B. Cutaneous vasculitis: review on diagnosis and clinicopathologic correlations. Clin Rev Allergy Immunol. (2021) 61:181–93. doi: 10.1007/s12016-020-08788-4

6. Caproni M, Verdelli A. An update on the nomenclature for cutaneous vasculitis. Curr Opin Rheumatol. (2019) 31:46–52. doi: 10.1097/BOR.0000000000000563

7. Morita TCAB, Trés GFS, Criado RFJ, Sotto MN, Criado PR. Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis- part I. An Bras Dermatol. (2020) 95:355–71. doi: 10.1016/j.abd.2020.01.003

8. Gibson LE, Winkelmann RK. Cutaneous granulomatous vasculitis: its relationship to systemic disease. J Am Acad Dermatol. (1986) 14:492–501. doi: 10.1016/S0190-9622(86)70064-9

9. Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine. (1998) 77:403–18. doi: 10.1097/00005792-199811000-00006

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Introduction

Schamberg disease represents the most common type of pigmented purpuric dermatoses (PPDs), a chronic, benign, cutaneous eruptions characterized by petechiae, purpura, and increased skin pigmentation (brown, red, or yellow patchy). The PPDs are grouped into five clinical entities: Schamberg's purpura, Majocchi purpura, lichen aureus, Gougerot-Blum purpura and, eczematoid-like purpura of Doucas and Kapetanakis.[1]

The exact cause of Schamberg disease is not known. It is caused by extravasation of blood vessels particularly capillaries which allow red blood cells to get deposited into the skin which then releases their iron from hemoglobin. This iron causes a rust color accounting for the orange or brown tint of the rash.[4]

The exact cause of capillary inflammation is unknown and can be multifactorial. Potential contributors in the etiology can be:

Drugs such as acetaminophen, aspirin, adalin, amlodipine, carbromal, chlordiazepoxide, glipizide, glybuzole, hydralazine, meprobamate, nitroglycerin, persantin, reserpine, thiamine, interferon-alfa, injection medroxyprogesterone acetate, topical fluorouracil, and sildenafil[7][8][9][10][11]

Vasculitis Symptoms by Body Part

The symptoms of vasculitis may differ depending on the cause and type. Additionally, different types of vasculitis can affect other parts of the body. For example, Takayasu arteritis is a type of vasculitis that affects about one or two people per million and has symptoms of low blood pressure, low pulse, fever, and weight loss. Other types can lead to symptoms in different body parts.

Legs

Vasculitis can prevent blood flow to areas farther away from the core of the body. For example, blood flow to the legs may be reduced, causing tingling, pain, or swelling. The same symptoms can also affect the arms.

Some types of vasculitis that involve leg symptoms include:

• Anti-GBM disease or Goodasture's syndrome
• IgA vasculitis or Henoch-Schonlein Purpura (HSP)
• Polyarteritis nodosa (PAN)
• Takayasu's arteritis (TAK)

Hands

Types of vasculitis affecting the hands, such as Buerger's disease or thromboangiitis obliterans, cause tingling in the hands and fingers. In some cases, it can lead to gangrene, a condition that causes discoloration and tissue death. People with vasculitis symptoms in their hands may also experience swelling.

Internal Organs

Vasculitis can lead to symptoms that affect internal organs. This may include chest, abdomen, or lower back pain if the heart, lungs, digestive tract, or kidneys are affected. Lung and respiratory symptoms may also include shortness of breath, coughing up blood, nasal congestion, and nosebleeds. Additionally, blood in the urine can be a symptom of vasculitis that has affected the kidneys, and blood in the stool can be a symptom that it has affected the stomach or digestive tract.

Ears

Vasculitis can lead to symptoms related to the ears, including tinnitus (ringing in the ears) or hearing loss.

Dizziness is another symptom that can happen if vasculitis affects the ears. This is because parts of the inner ears send signals to the brain about balance based on the movements of the head. The swelling or inflammation of vasculitis in the ears can affect this balance system, leading to dizziness.