Reactive angioendotheliomatosis in a patient with Myelodysplastic Syndrome presenting as a cellulitis-like plaque

ARTICLE

Auteur(s) : Ingrid Aguayo-Leiva¹, Sergio Vano-Galván¹, Irene Salguero¹, Rosario Carrillo-Gijón², Ana Vallés³, Pilar Herrera³, Ernesto Muñoz-Zato¹

¹Department of Dermatology, Hospital Ramón y Cajal, Carretera Colmenar Viejo Km 9100, Madrid, Spain
²Department of Pathology, Hospital Ramón y Cajal, Carretera Colmenar Viejo Km 9100, Madrid, Spain
³Department of Haematology, Hospital Ramón y Cajal, Carretera Colmenar Viejo Km 9100, Madrid, Spain

Reactive angioendotheliomatosis (RAE) is a rare condition characterized by cutaneous vascular proliferation that usually occurs in patients with diverse types of coexistent systemic disease. The first generally accepted cases of RAE were reported in the late 1950s by Gottron et al. [1] who described a female patient who presented with self-limited but impressive purpuric plaques. An association with coexistent systemic disease has been described in about 75% of the reported cases of RAE [2]. We describe a case of RAE presenting as a plaque on the right forearm of a patient with myelodysplastic syndrome (MDS). To our knowledge the only case of RAE associated with MDS previously reported in the literature presented as crusted necrotic ear lesions after cold exposure [3]. Our patient developed a cellulitis-like plaque on the forearm, supporting the heterogeneous clinical presentation of this entity.

An 80-year-old man had been diagnosed by the haematology department with MDS two years before. The patient presented in our service with a 2-month slow-growing asymptomatic violaceous plaque on his right forearm. He referred neither pain nor fever. He was diagnosed as having cellulitis but failed to respond to antibiotics (meropenem and vancomycin). Physical examination revealed a large violaceous purpuric plaque involving the entire circumference of the forearm that extended to the mid phalanx of his right hand (figure 1A). Laboratory evaluation revealed pancytopenia and increased levels of lactate dehydrogenase, creatinine, total bilirubin and haptoglobin. Tumour markers including PSA, CEA, alphafetoprotein, calcium and phosphorus were normal or negative. The inmunological study revealed low levels of cryoglobulins. All other tests, including rheumatoid factor, antinuclear antibodies and antiphospholipid antibodies, were in normal ranges or negative. Doppler ultrasonography did not detect stenotic lesions of the arteries or arteriovenous fistulas. A cutaneous biopsy showed minimal focal intravascular proliferation of endothelial cells and diffuse proliferation of these cells interstitially between collagen bundles of the reticular dermis (figure 1B). CD31-immunostaining demonstrated the endothelial origin of this proliferation (figure 1C), leading us to the diagnosis of diffuse dermal angiomatosis, a variant of RAE [4]. The immunostaining for B-cells markers was negative, excluding malignant angioendotheliomatosis. Oral methylprednisolone 10 mg/d was administered to the patient for 4 months without clinical improvement.

Reactive angioendotheliomatosis is a rare disorder seen in association with numerous systemic conditions. The clinical presentation described is heterogeneous, including erythematous macules, purpuric plaques, papules, ulcerated lesions, depressed plaques, angioma-like lesions, nodules, Kaposi sarcoma-like and tumors. The prognosis in reported cases has generally been good, characterized by self-limited disease. No specific treatment for RAE is available [2].

Although the exact stimulus for the intravascular endothelial cell proliferation is uncertain, occlusion of vascular lumen by different causes seems to be the common denominator of many cases of RAE. This occlusion increases tissue ischaemia, with the possible development of necrosis and additional liberation of local proangiogenic factors [3, 5]. MDS may promote a pro-coagulative status and the release of angiogenic circulating factors. In these patients, the mean values of thrombin antithrombin complexes and prothrombin fragment are high, indicating chronic coagulation activation. Recent studies have documented that angiogenesis plays a significant role in haematological malignancies, including MDS, with increased serum levels of angiogenic factors in these patients such as: basic fibroblast growth factor, hepatocyte growth factor and tumor necrosis factor-alpha which are multifunctional cytokines that potently stimulate angiogenesis [6].

Our case supports the association between RAE and haematological disorders, specifically MDS. RAE is an entity with a low clinical index of suspicion that is usually diagnosed through histological findings. Our patient presented with a cellulitis-like plaque but histological examination was concordant with RAE. Therefore, this entity should be ruled out in cellulitis-like plaques which do not respond to antibiotics, especially in patients with systemic diseases.

Acknowledgements

References

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