Papular mucinosis with rapid spontaneous regression in an HIV-infected patient

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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