Livedo racemosa as a cutaneous manifestation of polycythemia vera

ARTICLE

Auteur(s) : A. SHIMIZU, A. TAMURA, O. ISHIKAWA

Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan
<ashimizu@med.gunma-u.ac.jp>

In January of 2005, a 68-year-old woman visited our department complaining of discoloration of her foot. She had first noticed violaceous lesions of her toes 6 months previously. Then the skin change spread to her lower legs and became painful. She had been medicated for hypertension from 2002, and received intermittent phlebotomy as a therapy for polycythaemia vera (PV) which was diagnosed in 2003. Physical examination of both her lower legs revealed dark reddish erythema in a reticulate pattern (figure 1A). There were some infiltrated areas in the lesion. Clinical appearance of her lower legs was consistent with livedo racemosa (LR). Acrocyanosis was additionally seen in her bilateral toes (figure 1B). Raynaud’s phenomenon was only observed in the right big toe during the examination. Her face and both hands showed diffuse erythema and her conjunctiva were congested. Histological examination taken from the infiltrated lesion of LR showed eosinophilic-stained vessels located in the subcutaneous fat tissues (figure 1C). High-power magnification disclosed a thrombus within a small vessel and secondary vasodilations around the vessel and in the dermis (figure 1D). Laboratory studies revealed the following values: haematocrit, 58% (normal 34-46); haemoglobin, 17.1 g/dL (normal 11.8-15.1); erythrocyte count, 871 × 10⁴ /μL (normal 400-500); white blood cell count, 16,000 /μL (normal 4,000-9,600); platelet count, 55.4 × l0⁴ /μL (normal 16-35); erythrocyte sedimentation rate, 1 mm/hr (normal 1-10); uric acid, 7.4 mg /dL (normal 2.6-5.8). Serum anticardiolipin antibodies, cryoglobulins and gammaglobulins were within normal limits. Taking clinical and histological findings into consideration, we supposed that LR in this patient was caused by the stasis condition of blood flow, complicated with PV. Treatment with aspirin, 100 mg daily, and sarpogrelate hydrochloride, 300 mg daily, was started. The pain was dramatically decreased, although the skin change was only slightly improved. One month later, a therapeutic phlebotomy of 400 ml was performed. This treatment dramatically resolved her skin symptoms including LR, Raynaud’s phenomenon, and acrocyanosis. Because there was an increased risk of hemorrhage, we immediately stopped the administration of sarpogrelate hydrochloride after the improvement of LR. Thereafter, she remained symptom-free for 4 months.

Discussion

PV, a myeloproliferative disease, develops various clinical manifestations. Although it has been poorly mentioned in the literature, cutaneous lesions in PV display diverse morphology. Most frequent cutaneous changes are cyanosis and purple coloration of the face [1]. Aquagenic pruritus [2, 3] and erythromelalgia [4] have been also reported. These cutaneous manifestations are considered to be at least partly due to the microcirculatory disturbances resulting from the increased blood viscosity, secondary to an enlarged red cell volume, thrombocytosis, and occasional spontaneous platelet aggregation.
LR is a symptomatic terminology, and can be observed in multiple pathological conditions. However, in our review of the literature published between 1975 and 2004, only 2 cases of PV with LR were found [1, 5]. The first case was a 75-year-old woman with LR and acrocyanosis in bilateral soles. Her cutaneous changes had completely resolved [5] with hydroxyurea in addition to aspirin and phlebotomy. The second case was a 68-year-old man with LR in the buttocks and right thigh. After four phlebotomies the cutaneous lesions disappeared [1]. There was no patient with LR located in the lower leg like our patient.
In our patient, histological examination demonstrated a thrombus in a small vessel and vasodilatation around it as well as in the overlying dermis. These findings suggest that stasis condition of blood flow or subsequent thrombus formation due to increased blood viscosity may account for the development of LR in PV. This hypothesis is supported by the fact that phlebotomy was effective on LR in our patient as well as reported cases. n

1. Filo V, Brezova D, Hlavcak P, Filova A. Livedo reticularis as a presenting symptom of polycythaemia vera. Clin Exp Dermatol 1999; 24: 428.

2. Abdel-Naser MB, Gollnick H, Orfanos CE. Aquagenic pruritus as a presenting symptom of polycythemia vera. Dermatology 1993; 187: 130-3.

3. Bircher AJ, Meier-Ruge W. Aquagenic pruritus. Arch Dermatol 1988; 124: 84-9.

4. Ongenae K, Janssens A, Noens L, Wieme N, Geerts ML, Beele H, Naeyaert JM. Erythromelalgia: a clue to the diagnosis of polycythemia vera. Dermatology 1996; 192: 408-10.

5. Maroon M. Polycythemia rubra vera presenting as livedo reticularis. J Am Acad Dermatol 1992; 26: 264-5.