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VASCULITIS Suppl. 52 Review Cutaneous vasculitis and their differential diagnoses N. Kluger1, C. Francès2 1Université Montpellier I, Service de ABSTRACT depth and distribution. Even though Dermatologie, Hôpital Saint-Eloi, CHU de Vasculitis is defined as an inflammatory a certain number of syndromes have Montpellier, Montpellier, Paris, France; cell infiltration and destruction of blood been described, a patient may present 2Université Pierre et Marie Curie, Paris 6, vessels identified upon histologic exam- with symptoms that overlap with an- Service de Dermatologie, Hôpital Tenon, Assistance Publique – Hôpitaux de Paris, ination. Cutaneous manifestations are other clinical diagnosis making a diag- Paris, France. frequent during the course of many sys- nosis “at first sight” impossible. Vas- Nicolas Kluger, MD temic vasculitis. Lesions are often not culitis has a histopathologic definition, Camille Francès, MD specific, the most frequent being palpa- therefore its confirmation comes only Please address correspondence to: ble purpura. They may be the first and from the microscopic examination of Camille Francès, only manifestation of the disease or be the lesion. Université Pierre et Marie Curie, a part of a systemic condition. Histolo- The diagnosis of CV is made by mi- Paris 6, Service de Dermatologie, gy is mandatory to confirm the diagno- croscopic examination of hematoxy- Hôpital Tenon, sis of vasculitis to avoid a delayed and lin-eosin stained biopsies (2). A list of Assistance Publique - Hôpitaux de Paris, inappropriate diagnosis that could lead criterias allows a trained pathologist 4, rue de la Chine, to improper management. Cutaneous Paris, France. to diagnose and distinguish an active E-mail: [email protected] histology gave some data that may help vasculitis, from chronic and healed le- Received on May 2, 2009; accepted in to classify the vasculitis without deter- sions of vasculitis and changes that are revised form on June 24, 2009. mining precisely its type. A histological adjacent to vasculitis and may help to Clin Exp Rheumatol 2009: 27 (Suppl. 52): examination of all other skin lesions is define a subtype or the etiology of the S124-S138. necessary. The result of the biopsy has CV (2). Inflammatory infiltrates within © Copyright CLINICAL AND to be correlated to DIF data, medical and around the vessel walls associated EXPERIMENTAL RHEUMATOLOGY 2009. history, physical examination, labora- by intramural and/or intraluminal fibrin tory and radiological findings leading deposition (fibrinoid necrosis) con- Key words: Vasculitis, skin, to the correct diagnosis and effective firm the diagnosis of vasculitis. Some pathology. treatment. changes are suggestive of active vascu- In this review, we discuss the diagnosis litis such as red blood cell extravasation, of cutaneous vasculitis (CV) and the pit- perivascular nuclear dust (leukocyto- falls related to the cutaneous pathology. clasia), eccrine gland necrosis, ulcera- We also describe the essential features of tion, necrosis/infarction. In the absence the major categories of skin vasculitis. of fibrinoid necrosis, the diagnosis of CV becomes more difficult. Lamination Introduction of the adventia, media and/or intima; Vasculitis is defined as an inflamma- perivascular nuclear dust (leukocyto- tory cell infiltration and destruction of clasia) without fibrinoid necrosis; loss blood vessels identified upon histologic of the elastic lamina with acellular scar examination. Cutaneous manifestations tissue; or, subendothelial intramuscular may be the first and only manifestation and/or advential inflammatory cells in of the disease or be a part of a systemic large vessels are all other argues for condition (1). vessel wall damages (2). In this review, we discuss the diagno- A direct immunofluorescence examina- sis of cutaneous vasculitis (CV) and tion (DIF) is also mandatory in case of the pitfalls related to the cutaneous pa- CV. It does not confirm the diagnosis thology. We also describe the essential of CV but allows to orienter for one or features of the major categories of skin another diagnosis. Absence of immune vasculitis. complex is in favor for pauci-immune vasculitis: Wegener’s granulomatosis Diagnosis of cutaneous vasculitis (WG), Churg-Strauss syndrome (CSS), Physical cutaneous signs of vasculitis Microscopic Polyangiitis (MPA). Im- are wide and non-specific. Vasculitis munoglobulin (Ig) G, IgM, IgA and/or Competing interests: none declared. affects the skin with varying intensity, C3 in or around the vessels may be S-124 Cutaneous vasculitis / N. Kluger & C. Francès REVIEW found in immune mediated vasculitis Table I. Differential diagnoses of vasculitis: lesion must include the epidermis, der- like cryglobulinemia. In all case of CV, cutaneous pseudovasculitis, modified from mis and hypodermis to precise the size immune depositions of Ig and comple- (4). of the affected vessels. Some CV affect ment may be found especially C3 and HEMORRHAGE typically the upper part of the dermis IgM. However, the older the biopsied Thrombopenia like HSP. Therefore, a punch skin bi- lesion is, the less immunoglobulins are Congenital and acquired thrombopathy opsy will permit to show the lesions. In found. After 72h, only C3 is detected. Scurvy the case of polyarteritis nodosa, deep Solar/senile purpura Therefore, a negative DIF does not rule Pigmented purpuric dermatoses muscular vessels of the dermis-hypo- out the diagnosis of CV. Predominance Arthropod dermis and the hypodermis are affected of IgA is highly in favor for Henoch- Viral and drug reactions which imply a deep incisional biopsy. Ehler Danlos syndrome Schönlein purpura (HSP) without be- Gardner-Diamond syndrome Similarly, a livedo should be biopsied ing constant or specifi c (3). IgM depo- on its infliltrated or necrotic areas. sitions are observed, specially in case EMBOLISM In some specific cases, an incidental vas- of circulating rheumatoid factor or cry- Cardiac myxoma culitis may be found on the skin biopsy. Fibrinocruroric emboli oglobulinemia. IgA deposits are absent Cholesterol Emboli This pathologic statement should not in case of cryoglobulinemia. Of note, Other emboli (gazous, fat, neoplastic…) mislead to diagnose a vasculitis. Thus, positive DIF without pathological as- neutrophilic small vessel vasculitis may THROMBOSIS sessment of CV is not relevant. Purpura Fulminans be observed if a biopsy is performed on After confirmation of the diagnosis of Intravascular coagulation an ulcer (2), while the surrounding ves- CV itself, vasculitis may be defined Vitamin K antagonists and heparin induced skin sels are normal. Besides, vessel damage more accurately by vessel size involve- necrosis induced by neutrophils is observed in Antiphospholipid Syndrome ment (small; small and medium vessel Cryoglobulinemia type I lesions related to neutrophilic derma- and medium to large vessel), the extent Thrombocytaemia toses such Sweet’s syndrome (5). of the lesions (superficial perivascular Livedoid vasculopathy/atrophie blanche to dermal and/or subcutaneous) and the Other coagulation disorder (protein C, protein S Clinical pathologic correlation deficiencies…) predominant inflammatory cell infiltra- Calcyphylaxis The cutaneous lesions correlate some- tion. The finding of small-vessel vascu- times with the size of the affected ves- litis with predominance of neutrophilic VASOSPAMS sels. Palpable purpura, infiltrated ery- Drug induced infiltrate and positive DIF is indicative Cocaine thema, urticaria, vesicules, blisters are of cutaneous leukocytoclasic vasculitis, mainly related to small vessel vasculitis HSP, urticarial vasculitis or erythema VASCULAR TRAUMA of the dermis, while subcutaneous nod- elevatum diutinum. More rarely, other ules, ulceration, gangrene are related cells may predominate such as eosino- cin deposition is associated with lupus frequently to medium sized vessel vas- phils or lymphocytes. Presence of both erythematosus and dermatomyositis. culitis located at the dermo-hypoder- small and medium sized vasculitis fa- Intraepidermal or dermal pustules with mal junction or in the subcutaneous fat. vors ANCA associated/pauci immune neutrophils small vessel vasculitis is re- Necrosis and livedo occur when either vasculitis (with negative DIF): CSS, lated to an infectious related vasculitis small and/or larger vessels are involved. MPA, WG or cryoglobulinemia, con- (2). Skin biopsy allows to exclude pseu- nective tissue disease (lupus, rheuma- dovasculitic disorder, a wide group of Clinical manifestation toide arthritis) or hypocomplemental heteregenous diseases that may mimic Cutaneous vasculitis displays a wide vasculitis if DIF is positive. Polyarteri- cutaneous vasculitis (Table I) (4). range of elementary lesions that may tis nodosa is characterized by a neutro- be associated and lead to a pleomor- philic infiltration associated with a me- Pifalls phic appearance of the eruption. CV dium vessel arteries vasculitis. In order to enable the diagnosis of vas- may manifest variously as urticaria, Some extravascular histologic pattern culitis, the choice of the “best” lesion is purpura, infiltrated erythema, hemor- found in the surrounding tissue may be crucial. A lesion of cutaneous vasculi- rhagic vesicles, ulcers, nodules, livedo, helpful to indicate a specific disease. tis should be analyzed withing the first infarcts, digital gangrene (1). Lesions Thus, palisading granulomatous der- 48h after its appearance, otherwise typ- affect primarly the lower limbs. Up- matitis (“Winkelmann granuloma”) is ical signs of vasculitis may be absent. A per extremity, trunk,
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