Alles wat je moet weten over cutane vasculitis bij huidziekten

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.

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]

How to Confirm If You Have Vasculitis Rash

If you think you have a vasculitis rash, it's important to see a healthcare provider and, if possible, a dermatologist (a physician specializing in skin diseases).

During the diagnostic process, a dermatologist will:

• Analyze your symptoms
• Look for abnormalities in a physical examination
• Evaluate the results of various tests, including blood work and a skin biopsy

Medical History and Physical Exam

When evaluating for possible cutaneous vasculitis, a dermatologist will ask about skin symptoms, drug exposures, family medical histories, and past or present health conditions, such as a recent viral infection.

They will also inquire about other non-skin symptoms, which, if present, can suggest systemic vasculitis (affecting other areas of the body) or an underlying disease.

Systemic vasculitis indicates that blood vessels in organs other than the skin—for example, the liver, kidney, joints, or nerves—are inflamed.

Symptoms of Systemic Vasculitis

Systemic vasculitis is a more serious, potentially dangerous condition than vasculitis limited to the skin. Symptoms of systemic vasculitis can include:

• Fever, chills, and night sweats
• Unintended weight loss
• Fatigue and malaise (generalized feeling of being unwell)
• Joint and muscle aches
• Abdominal pain
• Blood in the urine or stool
• Numbness and weakness
• Cough or sinus inflammation
• Vision changes

Next, a dermatologist will carefully inspect the affected skin area and perform a complete body examination.

Skin Biopsy

A skin biopsy is necessary to confirm or exclude a diagnosis for any skin finding resembling a vasculitis rash.

Though there are different types of skin biopsy techniques, a punch biopsy is often used when a vasculitis rash is suspected because it can go deep into the skin's layers.

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).

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

10. Khetan P, Sethuraman G, Khaitan BK, Sharma VK, Gupta R, Dinda AK, et al. An aetiological & clinicopathological study on cutaneous vasculitis. Indian J Med Res. (2012) 135:107–13. doi: 10.4103/0971-5916.93432