A 32-year-old man presented with a 1-week history of several erythematous papules on the lower back. They had started as tiny skin-colored papules, and these progressively enlarged to form confluent papules. During this time, several new lesions developed. There were no subjective symptoms, and the patient denied any history of injection or trauma. The physical examination showed multiple, relatively well-demarcated, variable sized erythematous papules scattered over his lower back (Fig. 1).
Two years earlier, he had begun to develop arthralgia in his knees and shoulders. He had also noticed joint stiffness in the morning, Raynaud’s phenomenon and oral ulcers. He had showed no skin lesions, including malar rash, discoid rash or photosensitivity during the previous 2 years. The significant laboratory values included leukocyto- penia (2,200/mm3), an antinuclear antibody titer of 1 : 1280 (a speckled pattern), hypocomplementemia (C3: 53.1 mg/dl [normal range: 88 ~201 mg/dl], C4: 10.0 mg/dl [normal range: 16~47 mg/dl]) and positivity for antibodies against Ro/SSA, La/SSB and Scl-70. However, the anti-ds DNA antibodies, the hemoglobin level, the erythrocyte sedimentation rate, the kidney and liver function tests and urinalysis were within normal limits. He had been diagnosed with systemic LE and then treated with an antimalarial agent (hydroxychloroquine 100 mg/day) and prednisolone (7.5 ~ 10 mg/day) for 2 years, which resulted in substantial improvement.
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Fig. 1. Multiple, relatively well-demarcated, flesh or erythematous colored papules on the lower back.
A 4 mm punch biopsy of skin from a papule on the patient’s lower back showed deposition of bluish materials around the sweat glands and between the collagen bundles in the dermis (Fig. 2A, B). The mucinous material was stained with alcian blue at pH 2.5 (Fig. 2C). A scant perivascular lymphocytic infiltrate was observed. The number of fibroblasts in the dermis was not significantly increased. Any histopathologic findings of cutaneous LE were not detected, such as epidermal involvement and va- cuolar degeneration at the dermoepidermal junction.
Fig. 2. A skin biopsy specimen showing (A) intact epidermis, deposition of basophilic mucinous materials and scant infiltration of mononuclear cells in the dermis (H&E, x40). (B) Deposition of mucinous materials between the collagen bundles and around the sweat glands (H&E, x100). (C) Alcian blue staining (pH 2.5) showing the deposits of mucin in the dermis (x40).
Based on these findings, the skin lesions were diagnosed as papular mucinosis in a patient with systemic LE. He was treated with topical steroids in addition to receiving systemic antimalarials and prednisolone. After 2 weeks treatment with the medications, he achieved remission of his PNM without recurrence for 5 months.
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