Vasculitis in childhood – a dermatological approach

Abstract

Summary Vasculitis, an inflammatory condition affecting the blood vessels, may be restricted to a single organ or involve several organ systems. The size of the involved vessels is an important criterion for categorization of vasculitides, which is a prerequisite for rapid diagnosis and initiation of treatment. In pediatric patients, this particularly applies to Kawasaki disease. However, making the diagnosis can be challenging for dermatologists as skin involvement may be variable and non‐specific. In contrast, Henoch‐Schönlein purpura (IgA vasculitis) presents with the classic picture of palpable purpura. It predominantly affects postcapillary venules frequently following upper respiratory tract infections. Severe organ involvement is relatively rare in children and the prognosis is good. As renal involvement may occur during the course of disease, continuous monitoring is required. Acute hemorrhagic edema of infancy is considered as a distinct type of immune complex vasculitis and is characterized by a triad of fever, edema and rosette‐shaped purpura. The clinical course of this rare disease is usually benign and self‐limited. Due to the variability of clinical symptoms and manifestations, management of childhood vasculitides represents a special challenge requiring interdisciplinary collaboration. Dermatologists should be aware of their important role especially for making an early diagnosis.

Vasculitic wheel – an algorithmic approach to cutaneous vasculitides

“…Both annular erythema and urticarial vasculitis may be early manifestations in patients with lupus erythematosus. Acute hemorrhagic purpura (cockade purpura of Seidlmayer‐Finkelstein) (Figure ) is a deep‐seated dermal to subcutaneous form of leukocytoclastic vasculitis, most commonly presenting as hemorrhagic nodules on the trunk and extremities in infants and children . Eosinophil‐rich leukocytoclastic vasculitis (Figure ) may show flame figures and there is overlap with both Wells’ syndrome and eosinophilic granulomatosis with polyangiitis (EGPA, Churg‐Strauss syndrome).…”

Erythema multiforme in a 14‐year‐old girl with Henoch‐Schönlein purpura and pancolitis: a relationship with mesalazine?

“…The most common vasculitis in childhood is Henoch-Schönlein purpura (HSp), a type of small vessel inflammation leading to leukocyte rupture mediated by immunoglobulin A-dominant immune complexes, mostly involving the skin, gastrointestinal tract, joints and kidneys [1,2]. Due to the variety of clinical manifestations, management of HSp represents a special challenge requiring interdisciplinary collaboration [3,4]. Mesalazine (5-aminosalicylic acid) can be useful in managing specifically abdominal distress with HSp due to its anti-inflammatory role in the treatment of chronic inflammatory bowel disease [5].…”

Das Vaskulitis‐Rad – ein algorithmischer Ansatz für kutane Vaskulitiden

“…Sowohl das anuläre Erythem als auch die urtikarielle Vaskulitis können frühe Manifestationen bei Patienten mit Lupus erythematodes sein. Die akute hämorrhagische Purpura (Kokarden‐Purpura Seidlmayer‐Finkelstein) (Abbildung ) ist eine tief reichende dermale bis subkutane leukozytoklastische Vaskulitis, die sich meist in Form hämorrhagischer Knoten an Rumpf und Extremitäten bei Kleinkindern und Kindern bemerkbar macht . Bei der eosinophilenreichen leukozytoklastischen Vaskulitis (Abbildung ) können sich Flammenfiguren zeigen und es gibt Überlappungen sowohl mit dem Wells‐Syndrom als auch mit der eosinophilen Granulomatose mit Polyangiitis (EGPA, Churg‐Strauss‐Syndrom).…”