Papular mucinosis. A clinicopathologic study of four patients

Abstract

Four patients with papular mucinosis were studied. Each patient had clinically characteristic cutaneous lesions, increased dermal deposition of mucin, and a serum monoclonal lgG paraprotein. One patient had sclerodermoid features consistent with the scleromyxedema variant of papular mucinosis. Associated findings in the patients included pachydermoperiostosis (one case), adenocarcinoma of the stomach (one case), carcinoma of the pancreas (one case), bizarre neurologic symptoms (one case), and emphysema (two cases). Autopsies were performed in two cases and no increased mucin deposition was observed in internal organs. Immunofluorescence microscopic study of involved skin showed no immunoreactant deposition in the three patients studied. Our observations support the hypothesis that papular mucinosis is a disorder of skin fibroblasts without internal organ involvement.

Scleromyxedema: A Case Report and Review of the Literature

“…PUVA, Grenz ray, and electron beam therapy have also been used with mixed results [19]. Variable results have been obtained with systemic steroids [20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32] and daily cyclophosphamide [33, 34]. Pulse cyclophosphamide was effective in 1 patient [35].…”

Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin

“…13 It is a rare manifestation of scleromyxedema with only 10 documented cases. [6][7][8]12,13,[39][40][41][42][43] Typically, dermatoneuro syndrome has been treated with intensive care monitoring, [39][40][41] plasmapheresis, 42 IV steroids, 8,12 or combinations of these treatments with variable outcomes. 13,43 Improved mental status has been observed in patients treated with plasmapheresis alone 42 and patients given combined treatment with both plasmapheresis and steroids.…”

Severe lung involvement in systemic scleromyxoedema: a highly unusual finding

“…FARMER et al [10] reported two scleromyxoedema patients with pulmonary emphysema in a post-mortem examination, but found no myxoedematous deposits in the pulmonary vessels. FUDMAN et al [5] first reported a diminished DL,CO in pulmonary function tests.…”