ARTICLE
Papular mucinosis (lichen myxoedematosus), is a rare disease characterized
by infiltrative skin lesions, due to the accumulation of mucin in the
dermis. Its course is usually chronic and slowly progressive, but rarely,
spontaneous regression has been described [1, 2]. Paraproteinaemia is
often identified, but an unusual polyclonal pattern has occasionally been
reported. Recently, papular mucinosis has been reported in association
with HIV seropositivity [3-8].
We report a new case of a patient with acquired immunodeficiency syndrome
in whom papular mucinosis resolved rapidly.
Case report
The patient was a 37-year-old man, known to be HIV-infected since 1990.
He had a past history of pulmonary and ganglionary tuberculosis, and was
hospitalized for visceral leishmaniosis and CMV infection. He was seen
in our department because of an eruption which had started 3 months earlier.
Examination revealed multiple, asymptomatic and symmetrical, 2-4 mm
skin-colored papules involving only the dorsal aspect of hands, wrists
and forearms without tending to coalesce (Fig.
1).
Histopathological findings included diffuse
mucin deposits, stained with alcian blue (pH 2.5), between the upper dermal
collagen bundles, with associated spindle shaped cells but no inflammatory
infiltrate (Fig. 2).
Laboratory studies disclosed the following results; leucocytes 3,200/mm3;
lymphocytes 288/mm3 with a CD4 cell count of 2/mm3
and a CD8 cell count of 114/mm3 (ratio 0.02); platelets 40,000/mm3;
red blood cells 2.106/mm3; haemoglobin 7 g/dl; positive
HIV P24 antigen (58 pg/ml); ß2 microglobulin 4 mg/l (normal range:
0.8-1.8); gamma-glutamyltransferase 157 u/l (n.r; 7-35).
Serum protein electrophoresis and immunoelectrophoresis showed polyclonal
hypergammaglobulinaemia but no paraprotein spike. Bone marrow biopsy showed
medullary leishmaniosis.
No treatment was proposed, and complete regression was noted 1 month
later.
Comment
Cutaneous mucinosis associated with HIV infection has been recently
described. Ten patients [3-8] with papular mucinosis and one with reticular
erythematous mucinosis [9] have been reported in the literature. All were
male, six were homosexual and three were intravenous drug abusers. Apart
from one case [7], the CD4 cell counts were below 100/mm3,
as in our patient. The cutaneous lesions were mainly observed on the trunk
and limbs. In our case, persistent acral papular mucinosis was ruled out
on the basis of the clinical course and the histological examination (no
grenz zone in the upper dermis). The discrete papular form of the disease
has already been described in HIV patients in association with polyclonal
hypergammaglobulinaemia, as in our case, and in two cases [3, 8] with
a paraproteinaemia. This finding could be related to the HIV infection
and may reflect B cell overactivation as a result of abnormal cytokine
production [10, 11]. The course is unpredictable and there is no effective
treatment. Spontaneous regression is rarely reported [2] and to the best
of our knowledge only in one case has been reported in an HIV patient
[8]. We thus report the second case with a rapid, complete and spontaneous
regression in a patient with acquired immunodeficiency syndrome.
Papular mucinosis, despite the small number
of cases published, could represent another cutaneous marker of human
immunodeficiency virus disease. Although the pathogenesis of papular mucinosis
is unknown, its clinical and laboratory data and the occasional association
with immunologic disorders, such as paraproteinaemia and HIV infection,
suggests that an immune dysregulation might play a pathogenetic role.
Possible factors in serum that could cause an upregulation of glycosaminoglycan
synthesis include immunoglobulins ond cytokines. It has been shown that
even when gamma globulin was removed, serum from patients with Graves'
disease still showed a stimulatory effect on glycosaminoglycan production
[12], suggesting that factors other than autoantibodies stimulate mucin
production. Cytokines in serum constitute another factor that could stimulate
mucin production in skin.
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