Lichen Myxedematosus (Papular Mucinosis): A Brief Review of the Literature and Report of a Case Which Failed to Respond to Acth and Cortisone

Abstract

CASES liave been reported of a skin disease resembling diffuse scleroderma, but in whicli subcutaneous nodules have been present. Under the name of " nodular scleroderma " several such cases have been recorded. Dubreuilh (1906) described a ^foman of 44 years with an almost generalized eruption of small nodules wliich were almost confluent on the extremities, shoulders and neck, discrete on the upper part of the trunk and absent on the abdomen and back. The individual lesions were white or yellowish-white, round, smooth nodules from one to two millimetres in diameter, firm to the touch and exhibiting no signs of inflammation. Histologieally there was an increase in the fibrous tissue accompanied by a slight round cell infiltration, which was most marked about hair follieles and gradually faded into the surrounding tissues.Eeitman (1908) reported a Avoman, aged 40 years, in whom there was a dill'use thickening of a ijeculiar alabaster white nature a,bout the nose and neighbouring parts of the cheeks, whicli had been present for several years. There were tiny papules on the nose and ears. The lips w~ere sclerotie, so that opening of the month was difficult. The skin of the neck and hands was infiltrated and studded with tiny follicular papules, Avhile on the lower limbs the skin w-as indnrated but no papules w-ere seen. The mucous membrane covering the right side of the soft palate was sclerotic to palpation, and on the right cheek the impressions of the teeth conld be seen. Histologieally there was a thickening of the cutis with an increased amonnt of fibrous connective tissue. In some of the larger blood vessels there w^as proliferation of the intima and endarteritis. The sweat glands were surrounded by a eellnlar infiltration. Lipschntz (1913) reported a case of selerodaetylia with several tender bean-shaped nodnles. Biopsy of the nodnles showed hypertrophy of the connective tissue, endothelial proliferation and adventitial eellnlar infiltration in the vessels and the deeper parts of the dermis. Lipsehutz considered the case one of selerodaetylia with circmnscribed fibrons nodosities, possibly analogous to those appearing in idiopathic atrophy of the skin.Brnhn's (1921) patient with nodnlar scleroderma was a boy aged 13 years. The skin of the chest and abdomen w^as stippled by browaiish-blne, lentil-sized nodnles, which w^ere raised above the level of the snrronnding skin. The left thigh was involved by a typieal band of scleroderma, roniid >vhieh were lesions, similar to those on the chest. On the left calf, right biittoek and in the right seapular region w^ere nodnlar lesions which were not raised appreciably above

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].…”

Papular mucinosis with myopathy, arthritis, and eosinophilia

“…Many authors have stated that this disease is an example of a benign monoclonal gammopathy and is basically a cutaneous disease with no systemic features. There have been suggestions, however, of associated systemic pathology-specifically, accelerated coronary artery (7,10, 19,21) or cerebrovascular (19)(20)(21)(22)(23) disease. In this patient the electrocardiogram revealed an old inferior wall myocardial infarction.…”

“…Two patients with papular mucinosis have been described previously with an associated myopathy (24,25), and significant weakness was mentioned in 1 other patient (26), but was not further characterized. Dysphagia has been noted more frequently (22,(27)(28)(29). but associated proximal limb-girdle weakness was not mentioned.…”

“…This patient also emphasized the clinical resemblance to systemic sclerosis. Papular mucinosis frequently leads to acrosclerosis, facial induration with decrease in mouth aperature (1 ), associated dysphagia (22,(27)(28)(29), Raynaud's phenomenon (1, 26,33), and rarely cutaneous ulcerations (24), telangiectasia (24,31), and subcutaneous calcinosis (5). The similarity is further increased in this patient because of the polyarthritis, myopathy, and mild pulmonary interstitial fibrosis, all of which may occur in systemic sclerosis.…”