Photodermatol Photoimmunol Photomed 2000; 16: 229–231 Copyright C Munksgaard 2000Printed in Denmark ¡ All rights reservedMunksgaard ¡ Copenhagen

ISSN 0905-4383

Brief communication

Successful treatment of lichen myxoedematosus with PUVA photochemotherapy

Y. Adachi, S. Iba, T. HorioDepartment of Dermatology, Kansai Medical University, Osaka, Japan

Lichen myxoedematosus is an unusual disorder of un-known etiology and pathogenesis. Although severaltreatments have reportedly been tried, therapeutic ef-ficacies are variable and unsatisfactory. A patient withsevere skin changes of this disease was successfullytreated with PUVA photochemotherapy. PUVA ther-apy was carried out using 30 mg of 8-methoxypsoral-en orally, and subsequent exposure to UVA starting at4 J/cm2. The eruption disappeared almost completely

Lichen myxoedematosus (LM), or papular mucinosis, isan uncommon disease characterized by papular li-

chenoid eruption and mucin deposition in the dermis. Theprognosis is poor, and the clinical course is chronic. Anumber of treatment modalities have been attempted withunsatisfactory results. We reported remarkable improve-ment of the eruption in a patient with LM followingPUVA photochemotherapy. Scleromyxoedema, which is avariant form of LM, has been treated with photo-chemotherapy in a few patients. However, this is the firstreport of a successful treatment of LM with PUVAtherapy.

Case reportA 64-year-old Japanese man was referred to our clinic be-cause of multiple papules and nodules on various parts ofthe body. His disease had started 2 years earlier with pru-ritus and subsequent development of nodules on the napeof the neck. The eruption gradually extended and did notrespond to topical corticosteroid therapy.

Physical examination during his initial visit showed nu-merous flesh-colored or brownish papules and nodules onthe nape, back, chest and buttocks (Fig. 1). Individualnodules were solid, 1–8 mm in diameter, and coalesced toform plaques in certain parts of the lesions, where theskin became indulated and thickened. The patient did notshow the diffuse thickening of the skin that is usuallyseen in scleromyxoedema. Lymph nodes were not pal-pable. The complete blood cell counts were within normal

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after 35 treatments at a cumulative dose of 202 J/cm2.Histologically, mucin deposition was greatly dimin-ished after the therapy. The photochemotherapy mayexert its effect directly by inhibiting proliferation offibroblasts and synthesis of mucopolysaccharides, andalso indirectly by immunomodulating action.

Key words: lichen myxoedematosus; PUVA photo-chemotherapy; fibroblast; mucopolysaccharide.

levels. Total protein was elevated (8.4 g/dl) and polyclonalgammopathy was observed (IgG: 3020 mg/dl, IgA: 675mg/dl, IgM: 203 mg/dl). Neither paraproteinemia norBence-Jones proteinuria was found. Other serologicaltests and the rest of the physical examination revealed noabnormalities.

A skin biopsy from the papules on the neck showed anincrease in fibroblasts and collagen fibers in the dermis.The collagen bundles showed an irregular arrangementseparated in parts by a mucoid material, which wasstained with Alcian blue. A focal lymphocytic infiltrationwas present surrounding blood vessels. PUVA photo-chemotherapy was started with oral 8-methoxypsoralen at30 mg (0.5 mg/kg). Two hours after the drug administra-tion, the patient was exposed in a UV cabinet containingfluorescent black lights (M-DMR-TS, II-type,FLR100H,BL/A/DMR, Toshiba Clinical Supply, Tokyo,Japan) at 4 J/cm2 as an initial dose of UVA. The treatmentwas given three times a week without any other therapies.The UVA dose was gradually increased at 1 J/cm2 in-crements up to a maximum dose of 7 J/cm2. The pruritusdisappeared completely after five treatments. The cu-taneous changes began to improve after seven of PUVAtherapy treatments (total UVA dose 28 J/cm2), and hadalmost completely subsided after 35 treatments at a cumu-lative UVA dose of 202 J/cm2 (Fig. 2) when PUVA photo-chemotherapy was completed.

Histological examination after the completion ofPUVA therapy revealed no abnormal findings. The poly-

Adachi et al.

Fig. 1. Before PUVA photochemotherapy, solid papulesand nodules were found on the nape of the neck (a) andback (b).

clonal gammopathy was not significantly altered after thetherapy. Two years after the discontinuation of PUVAtherapy, several papules recurred on the nape of the neck.

DiscussionLichen myxoedematosus is the primary mucinosis of theskin without associated endocrine dysfunction. Treatmentof LM still remains unsatisfactory, although several mod-alities have been reported to be beneficial in sporadiccases. Among them, cyclophosphamide (1) and melphalan(2) with or without prednisone (1) may be the treatmentof choice in patients with widespread disease such as scler-omyxoedema, a generalized variant of this disorder. How-ever, these chemotherapeutic agents have adverse effectswhich may cause morbidity and mortality (3). SuperficialX-ray therapy (4) or electron beam (5) irradiation hasbeen reported to be useful each in a single patient. Photo-

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chemotherapy has been used with satisfactory results inpatients with scleromyxoedema. One patient was treatedwith PUVA photochemotherapy (3) and two cases withextracorporeal photopheresis (6, 7). The photopheresiswas done on 2 consecutive days every month, and signifi-cantly or completely cleared the skin changes after 12months of therapy in both patients (6, 7).

The precise pathogenesis of LM and scleromyxedemaremains unknown. The presence of paraproteinemia isoften found in patients, although its significance is notclear. Histologically, the proliferation of fibroblasts is en-hanced in the dermis, and collagen bundles are split bymucinous infiltration. Harper & Risper reported thatserum from a patient with LM stimulated proliferationand DNA synthesis of cultured fibroblasts (8). It is wellknown that PUVA treatment has a suppressive effect onDNA synthesis and cell proliferation. This may explain inpart the mechanisms of action of PUVA for the treatmentof LM. However, it seems unlikely that UVA radiationcan penetrate deep enough to exert its effect in the lowerdermis, which was affected in the present patient with se-vere skin changes. In extracorporeal photopheresis, circu-

Fig. 2. After PUVA photochemotherapy, skin lesions im-proved significantly on the nape (a), and completely onthe back (b).

Lichen myxoedematosus

lating leukocytes are exposed to UVA radiation, but der-mal fibroblasts are not directly irradiated. It is widely ac-cepted that PUVA therapy is an immunomodulatingtreatment, effective for skin diseases in which immunolog-ic or allergic processes are involved in the pathogenesis.Therefore, PUVA photochemotherapy may activate animmune response against pathogenic clones of T lympho-cytes (9).

In conclusion, the numbers of patients with LM orscleromyxoedema successfully treated with PUVA photo-chemotherapy are gradually increasing. This modalityshould be included in the treatments options for the dis-ease. Therapeutic effects of photochemotherapy suggestthat an immunologic process may be involved in the devel-opment of LM.

References1. Dirneen AM, Dicken CH. Scleromyxoedema. J Am Acad

Dermatol 1995: 33: 37–43.2. Feldman P, Shapino L, Pick AI, Slatkin MH. Scleromyxoedema.

Arch Derm 1969: 99: 51–56.3. Farr PM, Ive FA. PUVA treatment of scleromyxoedema. Br J

Dermatol 1984: 110: 347–350.

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4. Hill TG, Crawford JN, Rogers CC. Successful management oflichen myxoedematosus. Arch Dermatol 1976: 112: 67–69.

5. Lowe NJ, Dufton PA, Hunter RD, Vickers CFH. Electron-beamtreatment of scleromyxoedema. Br J Dermatol 1982: 106: 449–453.

6. Krasagakis K, Zouboulis CC, Owsianowski M, Ramaker J,Trautmann G, Tebbe B, Orfanos CE. Remission of scleromyxoe-dema following treatment with extracorporeal photopheresis. BrJ Dermatol 1996: 135: 463–466.

7. Berkson M, Lazarus GS, Benz MV, Rook AH. Extracorporealphotochemotherapy: a potentially useful treatment for sclero-myxoedema. J Am Acad Dermatol 1991: 25: 724.

8. Harper RA, Risper J. Lichen myxoedematosus serum stimulateshuman skin fibroblast proliferation. Science 1978: 199: 545–547.

9. Edelson RL. Photopheresis: A new therapeutic concept. Yale JBiol Med 1989: 62: 565–577.

Accepted for publication March 23, 2000

Corresponding author:Yukari Adachi, MDDepartment of DermatologyKansai Medical University10-15 Fumizono-choMoriguchi-cityOsaka570-8506 Japan