Cutaneous manifestations in patients with essential thrombocythemia

doi:10.1016/0190-9622(91)70010-Y

Journal of the American Academy of Dermatology

Volume 24, Issue 1, January 1991, Pages 59-63

https://doi.org/10.1016/0190-9622(91)70010-Y Get rights and content

Abstract

In a retrospective study of 268 patients with essential thrombocythemia, related cutaneous manifestations were found in 58 (22%). In 27 cases ( 10%) the related skin lesions were present at the time of the primary diagnosis of essential thrombocythemia. Hematomas, ecchymoses, petechiae, or purpura occurred in 24 (41%) of the 58 patients. Fifteen patients (26%) had erythromelalgia, in 11 of whom it was the initial complaint. Livedo reticularis, recurrent superficial thrombophlebitis, ischemic complications with gangrene, leg ulcers, or ulcers on the toes were other manifestations. Urticaria occurred in two patients, and Raynauďs phenomenon and necrotizing vasculitis were each observed in one patient. Platelet function may be abnormal, and this was found in 13 of 19 patients studied, 9 of whom had spontaneous platelet aggregation. Knowledge of the cutaneous manifestations in essential thrombocythemia may lead to its earlier detection. Prompt diagnosis of essential thrombocythemia is important because treatment may prevent severe hemorrhagic or thrombotic events.

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Erythromelalgia: Diagnosis and therapeutic approach
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L’érythromélalgie est un acrosyndrome paroxystique rare, associant douleur, augmentation de la chaleur locale et rougeur des extrémités. Elle est considérée dans ses formes familiales comme une neuropathie des petites fibres à transmission autosomique dominante en rapport avec des mutations du gène SCN9a codant pour le canal sodique voltage dépendant Nav1. On distingue les formes secondaires associées aux syndromes myéloprolifératifs, à la prise de certains médicaments (inhibiteurs calciques, bromocritine), ou plus rarement, à certaines situations (infection virale, maladies systémiques) et les formes primitives familiales d’origine génétique ou sporadiques proches des neuropathies des petites fibres. Le traitement des formes secondaires associées aux syndromes myéloprolifératifs repose sur l’aspirine. Celui des formes primitives est plus difficile et justifie une approche physiopathologique en utilisant des molécules agissant sur les canaux sodiques tels la lidocaïne, la mexilétine ou la phénytoïne.
Erythromelalgia is a rare intermittent vascular acrosyndrome characterized by the combination of recurrent burning pain, warmth and redness of the extremities. It is considered in its primary form as an autosomal dominant neuropathy related to mutations of SCN9A, the encoding gene of a voltage-gated sodium channel subtype Nav1.7. Secondary erythromelalgia is associated with myeloproliferative disorders, drugs (bromocriptine, calcium channel blockers), or clinical conditions such as rheumatic diseases or viral infection. Primary familial erythromelalgia include genetics and sporadic forms associated with small fibers neuropathy. Aspirin is a useful treatment of erythromelagia associated with myeloproliferative disorders. Treatment of primary erythromelalgia is difficult, individualized, with sodium channel blockers such as lidocaine, carbamazepine and mexiletine.
Essential Thrombocythemia: The Dermatologic Point of View
2015, Clinical Lymphoma, Myeloma and Leukemia
Citation Excerpt :
Not many studies have been conducted on patients with ET. Erythromelalgia developed in 6% of 268 patients according to Itin and Winkelmann19 and in 6% of 977 patients according to Gugliotta (Gugliotta L, for the RIT; personal communication). However, in a smaller study by Michiels et al,20 the incidence was significantly different, with 26 of 40 patients (65%) reporting erythromelalgia.
Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by an increase in blood platelets and dominated by a predisposition to vascular events. Cutaneous manifestations can complicate its course. itching has been the most common symptom reported; however, the percentage has ranged from 3% to 46%, depending on the survey. Erythromelalgia is found in 6% of cases, and livedo reticularis, minor bleeding, acrocyanosis, and Raynaud's phenomenon are rare manifestations. It is important to recognize and treat these events, because they can affect patients' quality of life and could worsen the prognosis. In addition to skin involvement as a possible sign of ET, the treatment of ET can be associated with cutaneous complications. Hydroxycarbamide, interferon-alfa, and anagrelide can induce different skin lesions. Hydroxycarbamide has been associated with major complications, including painful leg ulcers and actinic keratoses. Minor events include alopecia and hyperpigmentation. Xerosis, pruritus, and photosensitivity are some of the complications reported by patients treated with interferon-alfa. Anagrelide has proved to be associated with fewer dermatologic effects, only detected in single cases. Knowledge of the ET cutaneous manifestations, together with the clinical examination findings, can result in an earlier diagnosis and the start of effective treatment.
Urticarial vasculitis associated with essential thrombocythaemia progressing to myelofibrosis
2014, Annales de Dermatologie et de Venereologie
La vascularite urticarienne (VU) est une entité anatomo-clinique caractérisée par des lésions urticariennes atypiques associées à une image histologique compatible avec une vasculite leucocytoclasique. On distingue les VU normo-complémentémiques et hypo-complémentémiques. Quatre cas de VU normo-complémentémiques associées à des syndromes myéloprolifératifs ont déjà été décrits : trois thrombocytémies essentielles (TE) et une polyglobulie primitive. Nous en rapportons une nouvelle observation.
Un homme de 59 ans était suivi en hématologie pour une TE avec présence de la mutation V617F de JAK2. Une évolution de la TE en myélofibrose (MF) secondaire était suspectée en raison d’une anémie, d’une altération de l’état général et d’une augmentation du volume de la rate, puis confirmée par une biopsie médullaire. Le patient présentait des lésions cutanées urticariennes fixes. La biopsie cutanée était compatible avec une vascularite urticarienne (VU), avec une dilatation modérée des vaisseaux capillaires, la présence de neutrophiles dans leur lumière, dissociant leur paroi, et des zones de leucocytoclasie. Un traitement par ruxolitinib (inhibiteur de JAK2) était initialement efficace sur l’anémie et la splénomégalie mais n’empêchait pas une poussée de la VU. La prednisone per os (20 mg/jour) permettait une rémission cutanée complète.
Chez ce patient, la VU est très probablement associé au syndrome myéloprolifératif en raison de l’apparition/aggravation des signes cutanés au cours des poussées évolutives de la maladie hématologique, et de l’absence d’autre cause. Il s’agit à notre connaissance du premier cas publié de VU associé à une TE avec MF secondaire.
Urticarial vasculitis (UV) is a rare form of leukocytoclastic vasculitis in which skin lesions resemble urticaria. UV comprises hypocomplementemic and normocomplementemic subtypes. To date, only 4 cases of UV associated with myeloproliferative disorders have been described, including 3 cases with essential thrombocythaemia (ET) and one case with polycythaemia vera.
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To our knowledge, this is the fourth reported case of UV associated with ET and the first case associated with MF.

View all citing articles on Scopus

^†: Supported by the Swiss National Science Foundation.

¹: From the Department of Dermatology, Mayo Clinic and Mayo Foundation.

View full text

Cutaneous manifestations in patients with essential thrombocythemia†

Abstract

J Am Acad Dermatol

J Am Acad Dermatol

Lancet

Essential thrombocythemia: clinical characteristics and course of 61 cases

Cancer

Primary thrombocythemia in the young patient

Mayo Clin Proc

Clinical manifestations of essential thrombocythemia in young adults

Am J Hematol

Essential thrombocythemias: clinical evolutionary and biological data

Cancer

Thrombocythaemia

Clin Haematol

Erythromelalgia caused by platelet-mediated arteriolar inflammation and thrombosis in thrombocythemia

Ann Intern Med

Thrombocythemia as a cause of erythermal-gia

Arch Dermatol

Histopathology of erythromelalgia in thrombocythaemia

Histopathology

Erythermalgia: review of 51 cases

Circulation

Microvascular occlusive lesions in primary thrombocythaemia

Br J Haematol

Erythermalgia: a clue to the early diagnosis of myeloproliferative disorders

Arch Intern Med

Thrombocythaemic erythromelalgia in chronic myeloproliferative and myelodysplastic disorders

Neth J Med

Idiopathic thrombocythemia: cutaneous manifestations

Arch Dermatol

Livedo révélateur ďune thrombocytémie

Ann Dermatol Venereol

Les manifestations vascu-laires des hyperplaquettoses

Lyon Med