PNM is a distinctive, although unusual cutaneous manifestation of LE and PNM is due to a diffuse deposit of mucin in the dermis. It is clinically characterized by asymptomatic flesh-colored papules and nodules, which occur in the presence or absence of the typical cutaneous lesions of LE. PNM has a predilection for the trunk and upper extremities, but the face and other areas of the body such as the neck and thighs may also be affected. This condition must be distinguished from other cutaneous mucinoses, such as myxedema, reticular erythematous mucinosis, scleredema, alopecia mucinosa and cutaneous focal mucinosis. PNM is histopathologically characterized by mucin deposition between the collagen bundles in the papillary and mid-reticular dermis and there is also mild superficial perivascular lymphocytic infiltrates, whereas the epidermis is normal and it is without basal vaculopathy. The classic epidermal and dermal inflammatory changes characteristic of LE are not typically seen in PNM.
To the best of our knowledge, of the 33 reported cases of PNM in the English medical literature, 28 were accompanied by other typical cutaneous eruptions of LE, such as discoid lesions, macular erythema, annular atropic lesions, papulosquamous eruptions, vasculopathic lesions or alopecia. It is interesting that our patient had no skin lesions for 2 years after being diagnosed with systemic LE, and papular mucinosis was the only cutaneous manifestation of his systemic LE. PNM may be a presenting sign of LE or it may be the first cutaneous manifestation of LE. Yet for our patient, PNM did not precede the onset of LE, but the PNM occurred after the clinical manifestations of systemic LE.
Several factors may contribute to the pathogenesis of this condition. UV light may aggravate skin lesions. In addition, androgens or other sex- related factors may have a role in the pathogenesis of PNM because the male-to-female ratio of patients with PNM is much greater than that of patients with systemic LE. Furthermore, serum factors, such as cytokines and circulating antibodies, have been found to upregulate the synthesis of mucin from fibroblasts.
Many therapeutic modalities have been proposed to treat this condition, including glucocorticoids, antimalarial agents, retinoids, cyclophosphamide and methotrexate; plasmapheresis and surgical options such as dermabrasion, laser and excision have also been tried, but these are usually unsatisfactory. However, the skin lesions on our patient responded to medication comprised of topical steroids, systemic antimalarials and prednisolone.
In conclusion, we describe here a patient with papular mucinosis associated with systemic LE, and the patient had no LE-specific skin lesions after being diagnosed with systemic LE.
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