Nodular lichen myxoedematosus-a rare presentation

SK Uniyal, RK Jain, KR Beena

 Department of Skin & STD and Institute of Pathology (ICMR) Safdarjang Hospital, New Delhi, India

Correspondence Address:
S K Uniyal
A-56, Delhi Citizen Society, Sector-13, Rohini, Delhi-110085
India

Introduction

Lichen myxoedomatosus is a rare chronic mucinoses characterised by infiltrative skin lesions because of proliferation of fibroblast and subsequent excessive deposition of acid mucopolysachrides in the skin.[1] Four classical clinical types are recognised. 1) generalised lichenoid papular eruption (commonest type). 2) Discrete papular form. 3) localised to generalised lichenoid plaque and 4) urticarial plaque and nodular eruption. The rare predominantly nodular presentation of mucinosis with acute onset in association with pregnancy in a young patient prompted us to report this case.

Case Report

a 21-year-old housewife presented to skin OPD with multiple asymptomatic nodular swellings over face, scalp, upper limbs and feet along with thickening of skin over nape of neck and anterior part of chest of four months duration. Patient was apparently well four months back when she developed low grade fever and malaise followed by appearance of nodular swellings over face, scalp, forearms and feet which gradually became stationary after a period of one month. This was followed by development of generalised itching associated with thickening of skin overlying upper chest and nape of neck. Patient was having ammenorrhoea for 12 weeks.

Examination revealed multiple nontender firm subcutaneous nodulocystic swelling varying from 5mm to 4cm distributed over face scalp, neck, bilateral upper arms, posterior axillary folds and dorsum of hands and feet. Skin coloured shiny tense plaques were also present over nape of neck [Figure - 1] anterior chest above right breast, right inframammary area, medical aspect of bilaterial arms and axillae. Some plaque on trunk had a peau-d-orange appearance.

Routine haematological investigations, thyroid function tests and x-rays were normal. Serum electrophoresis showed normal pattern. Ultrasonography whole abdomen showed uterus to be enlarged by a gestation sac with a single live foetus=12 weeks. FNAC done from nodules over forehead and nape of neck showed scattered and loose cohesive clusters of round to oval cells against myxoid material in the back ground. Two skin biopsy taken from early nodule and infiltrative plaques showed identical features of hyperkeratosis with acanthosis. Superficial dermis showed chronic inflammatory infiltrate. Mid and deep dermis showed separation of collagen bundles which suggest mucinoses. Special stain for mucin with alcian-blue was negative at ph 7 but stained positive at ph 2.5. Patient was lost to follow-up since no treatment was advised view of pregnancy.

Discussion

The original description of lichen myxoedematosus is attributed to dubreuith in 1906.[1] In 1953, montgomery and underworld clasified it into 4 clinical types[1]-1) generalised lichenoid eruption with discrete papules covering the entire body but especially the hands, forearms, upper part of trunk, face and neck, 2) A discrete papular eruption on the trunk and extremities, 3) localised to generalised lichenoid plaques and 4) Urticarial plaques and nodular eruption. A few new entities have been added since then.[2]

Lichen myxoedematosus is uncommon. On review of literature approximately 115 cases have been reported in the world. Of these reports presentation with plaques and nodule are rare. Cutaneous mucinoses is a disease of unknown etiology, which usually affects adults of middle age without any sex predilection.[1] A paraproteinemia of usually IgG class has been found on serum electrophoresis but their levels have not been found to correlate with either extent or progression of the disease.[3] Bone marrow studies in this disease has shown a mild plasmacytic infiltration in some cases but no radiological skeletal abnormalities have been detected.[4] No endocrine abnormalities have either been demonstrated.[3] Slight to severe proximal muscle weakness occasionally associated with slight elevation of muscle enzymes have been reported.[4] Occasionally systemic involvement have been reported in other internal organs.[4]

The classical histopathology and histochemistry of mucinoses have been reported to show separation of collagen bundles into loose fiber network, fibroblast proliferation, fibrosis and mucin deposition. Acid mucropolysachrides stain with toluidine blue, colloidal iron and alcian blue at pH 2.5.[1]

The diseases have been found to be capable of long term regression and treatment is empiric. Topical and intralesional hyaluronidase, topical, intralesional and systemic corticosteroids, PUVA, electron beam therapy, retinoids, plasmapheresis, extracorporeal photochemotherapy, dermabrasion have all produced some improvement.[1],[2],[3] Low dose melphalan is considered drug of choice but has also resulted in deaths due to hematological malignancies and septicemia.[4]

To the best of our knowledge this is the first case of plaque & nodular type of lichen myxoedematosus in a pregnant female with normal thyroid functions. The sudden onset, preceded by fever, rapid progression without any systemic manifestation were other interesting observations. It is also stressed that alcian blue stain at pH 2.5 is essential for accurate diagnosis since diagnosis may be missed as stain may come negative at a routine higher pH as had happened in the case reported.

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