ARTICLE
A 64-year-old woman presented with eruptive,
purpuric lesions, some of them palpable, ranging in size from pinpoint
to a few centimeters, symmetrically distributed on the legs. The recent
medical history was unremarkable. In particular, the patient had neither
had infectious diseases nor received medication in the last 6 months.
Routine investigations were normal, as well as abdominal ultrasound and
chest x-rays; circulating immune complexes were not detected. The histologic
examination showed angiocentric inflammation of venules, endothelial swelling,
fibrinoid degeneration of the vessel wall, and nuclear debris of neutrophils
(leukocytoclasia). Direct immunofluorescence showed perivascular IgM,
C3 and fibrinogen deposits. The diagnosis of acute cutaneous vasculitis
(ACV) was made. After a short course of systemic corticosteroids (prednisone
p.o. at the initial dosage of 1 mg/kg/die, with gradual tapering off and
discontinuation in 3 weeks), the lesions evolved into brownish spots.
Approximately 4 weeks after healing, the patient started to complain
about a prickling, burning sensation, involving the trunk and limbs and
sparing the face, scalp and hands. The symptoms were triggered by contact
with water at any temperature, started few minutes after contact, and
lasted for about 30-60 minutes. Neither familial nor personal history
of atopy nor familial history of water-related itching was present. Skin
examination was negative. Common heat or cold or water related-skin diseases
were excluded by performing proper tests. Therefore, the diagnosis of
aquagenic pruritus (AP) was made. The patient was treated with antihistamines
- anti-H1 (cetirizine) and anti-H2 (cimetidine) plus hydroxyzine
- and topical emollients for two months, with partial relief.
During the following two years, hemogram and blood chemistry were periodically
performed and were normal. In June 1997, blood tests showed a consistent
increase of haemoglobin level (19.2 g/dl), erythrocytes (7,100,000/mul),
and haematocrit (57%). MCV was 77, while MCHC, leucocyte alkaline phosphatase,
leukocytes and platelets were normal. A bone marrow biopsy showed the
typical features of polycythemia. The patient was treated weekly by venesection,
obtaining a considerable improvement of pruritus. She is currently followed
in the Dept. of Haematology, University of Florence Medical School, and
is in good general shape.
Discussion
The condition known as AP [1] is typically precipitated by contact with
water at any temperature without visible skin lesions. The symptoms are
variously described by the patients: prickling, stinging, itching, burning.
Three different forms have been reported: idiopathic AP [1, 2], AP of
the elderly [3] and AP associated with polycythemia rubra vera (PRV) [4].
Generalised pruritus and, particularly, AP are manifestations that not
rarely suggest the diagnosis of this typical haematological disorder.
Although AP is usually associated with PRV and is sometimes the presenting
symptom [5], it very seldom precedes - even by years - the development
of the disease [6, 7], as documented in our case.
In addition, the case presented here may also suggest a possible relationship
between ACV, the presenting skin disease, and PRV. The association between
ACV and PRV has not been reported to date. Conversely, PRV associated
with chronic leucocytoclastic vasculitis [8-10] has been previously described.
Although we cannot exclude that it was a coincidence, the sequential
development of the three diseases is highly suggestive of a common link
among them. Therefore, we suggest regarding ACV - in addition to
AP - as a possible warning sign of PRV.
Article accepted on 8/1/02
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