Cutaneous Leukocytoclastic Vasculitis and Myelodysplastic Syndrome With Little or No Evidence of Associated Autoimmune Disorders—A Case Report and a Brief Review of the Literature

Das, Monisha; Chhabra, Rajiv; Hinton, Stuart

doi:10.1097/maj.0b013e31815e8c0b

Cited by 16 publications

(15 citation statements)

References 15 publications

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“…5 However, the definition used for this group of disorders varies from one study to another. [1][2][3]6 We prefer to use the term NLV, which we believe better defines the clinical condition of our case.…”

Section: Discussionmentioning

confidence: 99%

“…1 NLV may be associated with bacterial, viral or fungal infections, drug ingestion, inflammatory chronic systemic diseases, rheumatic diseases and haematological malignancies, including leukaemia, lymphoma, myeloma, cryoglobulinemia and myelodysplastic syndrome. 1,2 However, the etiology is unknown in approximately 60% of cases. 3 The clinical presentation of NLV varies and may consist of macules, pustules, vesicles, nodules, ulcerative lesions and papulonodular necrotic lesions on the extensor surfaces of the lower limbs and granulomatous lesions.…”

Section: Introductionmentioning

confidence: 99%

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Necrotizing leukocytoclastic vasculitis mimicking necrotizing fasciitis: a case report

et al. 2016

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<p>There are several subtypes of necrotizing leukocytoclastic vasculitis, which are classified according to their morphological features in biopsy specimens using immunofluorescence microscopy. Necrotizing leukocytoclastic vasculitis is limited to the skin, predominantly that of the lower extremities, and usually spares the palms and soles. The most common skin manifestation is palpable purpura. Other skin manifestations include maculopapular rash, bullae, papules, nodules, ulcers and livedo reticularis. There is no specific laboratory test to determine the diagnosis. There are various diseases presenting with these nonspecific symptoms, and a rapid differential diagnosis must be conducted, because the appropriate differentiation and diagnosis markedly influence the treatment strategy and survival of patients. In this study, we report a case of necrotizing leukocytoclastic vasculitis<strong> </strong>presenting with internal organ involvement and symptoms of necrotizing fasciitis, with emphasis on the clinical differentiation.</p>

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Necrotizing leukocytoclastic vasculitis mimicking necrotizing fasciitis: a case report

et al. 2016

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“…CSVV seems to be an immune complex-mediated process caused by an antigen excess that deposits on postcapillary venules and activates complement, as well as the inflammation cascade leading to vascular wall damage 2 3 6. The trigger is the excess of circulating antigens that seems to be tumour-associated;2–7 despite this, other causes such as infections and drug-exposure should be considered in patients with cancer 2 4 8 9.…”

Section: Discussionmentioning

confidence: 99%

“…The cutaneous manifestations can appear previously, simultaneously, or after the diagnosis of malignancy, most frequently as palpable purpura of the lower limbs 3 4. If there is a history of solid organ malignancy, a new episode of cutaneous vasculitis may be linked to recurrence of the disease 2.…”

Section: Discussionmentioning

confidence: 99%

“…There is no evidence that the timing of cutaneous lesion appearance has a prognostic value 5. Prognosis seems to be more dependent on the underlying neoplasm than the presence of CSVV 3 9Learning points

Cutaneous small vessel vasculitis (CSVV) typically presents with palpable purpura of the lower limbs.

CSVV is most frequently classified as primary or idiopathic but can also be secondary to other processes such as infection or inflammatory diseases, or may be drug-related.

Two to five per cent of all CSVV cases are associated with neoplasms, most commonly of haematological origin.

…”

Section: Discussionmentioning

confidence: 99%

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Primary mediastinal (thymic) B-cell lymphoma presenting as cutaneous vasculitis

et al. 2015

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Cutaneous small vessel vasculitis is a severe neutrophilic vascular inflammation mediated by immune complexes that involves the dermal postcapillary venules. Neoplasms represent 2-5% of all causes of secondary cutaneous vasculitis. We present a case of a 52-year-old man who was admitted due to a 10-day history of respiratory symptoms and myalgia. From the third day of symptoms onwards, the patient noticed the appearance of cutaneous lesions in the lower limbs with palpable purpura and erythematous papules. Additionally, he reported of asthenia, anorexia and weight loss during the prior month. Chest radiography showed an enlarged mediastinum and thoracoabdominal-pelvic CT scan revealed a bulky left hilar mass. Biopsy of the left superior lobar bronchus' mucosa allowed for the histological diagnosis of diffuse large B-cell lymphoma (DLBL) of the thymus. The patient received symptomatic treatment with improvement of the purpuric lesions and a multiagent chemotherapy regimen was initiated.

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The burden of autoimmunity in myelodysplastic syndromes

Fozza

2017

Hematological Oncology

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The clinical history of patients with myelodysplastic syndromes (MDS) is characterised by bone marrow insufficiency as well as by the possible evolution into acute leukaemia. However a number of reports highlight the frequent occurrence of autoimmune manifestations involving different sites and organs. The present review will first describe the clinical pictures most often observed in MDS patients. The actual burden of autoimmunity will be then addressed by focusing on the few available registry studies. Finally, the potential collateral impact of specific treatments for MDS on the evolution of autoimmune disorders will be considered.

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Cutaneous Leukocytoclastic Vasculitis and Myelodysplastic Syndrome With Little or No Evidence of Associated Autoimmune Disorders—A Case Report and a Brief Review of the Literature

Cited by 16 publications

References 15 publications

Necrotizing leukocytoclastic vasculitis mimicking necrotizing fasciitis: a case report

Necrotizing leukocytoclastic vasculitis mimicking necrotizing fasciitis: a case report

Primary mediastinal (thymic) B-cell lymphoma presenting as cutaneous vasculitis

The burden of autoimmunity in myelodysplastic syndromes

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