CASE REPORT

Cutaneous Vasculitis in a Patient with Dermatomyositisw ithout Muscle InvolvementAkifumi Kadoya, Tohru Akahoshi, Naho Sekiyama, Shigeru Hosaka and Hirobumi Kondo

A 74-year-old female patient with cutaneous ulcerations and typical dermatomyositis (DM)skin rash had no muscle disease for a 1-year and 5 months period. Histological examination of theskin ulceration indicated vascular occlusion without cellular infiltration. Cutaneous ulceration isa very rare manifestation of adult-onset DMpatients without inflammatory myopathy.(Internal Medicine 33: 809-812, 1994)

Key words: skin ulceration, amyopathic dermatomyositis

Introduction

Dermatomyositis (DM) is a connective tissue disorder char-acterized by prominent cutaneous features and inflammatorymyopathy. A DMpatient may not necessarily have both cuta-neous and muscle disease at the initial presentation. Cutaneousmanifestation of DM frequently precedes the development ofmuscle disease. The absence of muscle disease in patients withthe classical skinrash ofDM persisting over aperiod of 10 yearshas been reported (1). Amyopathic DM is the term used todescribe patients with typical cutaneous manifestation of DM

w

ith no or minimal muscle disease.Cutaneous vasculitis characterized by periungual infarcts

and digital ulcerations has been noted frequently in child-onset

D

M.However, its occurrence in adult-onset DM is rare.

The patient in this study had the typical skin rash of DMwithout apparent muscle disease; subsequently digital and

foot ulcerations developed. The rare association of skin ulcera-tion and amyopathic DM in adults is discussed.

Case Report

A 74-year-old Japanese female was first admitted to KitasatoUniversity Hospital in April 1993 with a two-month history of

fatigue, swelling of the ringers and scaly and erythematouseruptions on proximal interphalangeal joints, metacarpo-

phalangealjoints, elbows and knees. She had no past or family

history of collagen disease nor had she experienced Raynaud'sphenomenon or arthralgia.

Examination indicated an edematous and very fainterythematous eruption on the upper eyelids. There were scaly

purplish erythematous eruptions on proximal interphalangeal(PIP) joints, metacarpophalangeal (MCP) joints and extensorsurfaces of the elbows (Fig. 1) and knees. A faint purple, scalyand macular eruption was noted on the clavicular and V-area ofanterior part of the chest. There was no indication of muscleweakness. Levels of muscle enzymes such as serum aspartateaminotransferase,creatine kinase and aldolase were normal.

Serum lactic dehydrogenase was 55 1 IU/1 (normal range; 1 80-350) on admission, but it decreased to normal ranges withouttreatment. The results of an electromyogram were negative. Amuscle biopsy was performed from right biceps brachii muscle.

Muscle specimens did not exhibit atrophy, degeneration orfibrosisof the muscle fiber. Distribution of type I and type IImuscle fibers were indicated to be normal. Infiltration of inflam-matory cells and vasculitis were absent in the specimens.

Histological examination of skin specimens obtained from thefingers and elbows indicated hyperkeratosis of epidermis, thick-ness and edematous changes in collagenous connective tissueofthe dermis, basal keratinocyte liquefaction degeneration andmild infiltration of mononuclear cells around small bloodvessels (Fig. 2). These findings were consistent with the fea-tures of skin eruption of DM. A diagnosis of DM withoutprominent muscle disease was made. The patient was treatedwith prednisolone 20 mg/day; the skin eruptions showed good

response.At one month following discharge from the hospital, a skin

ulceration of the right fifth finger and left sole developed duringprednisolone treatment of 17.5 mg/day. The right fifth finger

then became edematous and erythematous with subsequentspreading over her right forearm. In August 1993, she wasadmitted again. She had Gottron's papules on PIPjoints, MCP

From the Department of Internal Medicine, Kitasato University, School of Medicine, SagamiharaReceived for publication March 25, 1994; Accepted for publication August 24, 1994Reprint requests should be addressed to Dr. Akifumi Kadoya, the Department of Internal Medicine, Kitasato University, 1 - 1 5- 1 , Kitasato, Sagamihara, Kanagawa

228

InternalMedicineVol. 33, No. 12 (December 1994) 809

Kadoya et al

Fig. 1. Gottron's papules on the elbow.

Fig. 2. Microscopic section of the biopsy specimen obtained from theelbow, showing hyperkeratosis of the epidermis, basal keratinocyte liquefac-tion degeneration and sparse mononuclear cellular infiltration around smallvessels (HE stain, x40).

Fig. 3. Cutaneous ulceration on the right fifth toe.

Fig. 4. Microscopic section of the biopsy specimen taken from soleexhibiting skin ulceration, showing endothelial swelling and vascular occlu-sion due to intraluminal thrombi but no cellular infiltration is revealed aroundthe vessels (HE stain, x40).

joints and typical rash ofDM on the elbows and knees. Periungualerythema, multiple nail fold thrombi on fingers and scatteredpapular erythema on the fingers and soles were noted. A smalldigital ulceration 3 mm in diameter on the dorsum of the rightfifth PIP joint, deeper skin ulcerations 12 mm in diameter onthe left sole and 6 mm in diameter on the left fifth toe were

d

etected (Fig. 3). The tip of the left fourth toe was gangranous.

Swelling and tenderness of the right arm due to cellulitiswereevident. No sclerodactyly or muscle weakness could bedetected. Muscle enzymes such as aspartate aminotransferase,

creatine kinase, aldolase and lactic dehydrogenase showednormal range levels. The erythrocyte sedimentation rate (ESR)had increased to 72 mm/h and C-reactive protein (CRP) was5,048 jLXg/dl. Serum IgG and IgA were increased to 1 ,880 mg/dland 5 14 mg/dl, respectively. Antinuclear antibody was positiveat x80 with a speckled and homogenous pattern, but the anti-body to extractable nuclear antigen was negative. Antineutrophil

cytoplasmic antibody (ANCA) and anticardiolipin antibodywerenot detected. The results of the electromyogram were alsonegative. To analyze for occult malignancy, X-ray examina-tion, abdominal echogram, chest and abdominal CT scan,gallium radioisotope scanning, gastroflberscope and bariumenema were performed. No associated malignancy was de-tected. Pathological examination of skin biopsy specimens

from the right sole demonstrated dilation of vessels in thedermis with endothelial hyperplasia, vascular occlusion due tointraluminal thrombi; there was no cellular infiltration aroundthe vessels (Fig. 4). Immuno-histochemical examination dis-

closed the deposition of IgG, IgA, IgM, C3, C4, Clq and

f

ibrinogen on the vessels.

The patient was treated with heparin (10,000 U/day) andlipo-PGEl (10 jig/day), but without significant effect.

Methylprednisolone pulse therapy (500 mg/day for 3 days) was

followed by oral administration prednisolone 40 mg/day andwarfarin. The skin ulcerations were resistant to the therapy, butsignificant improvement was noted at 4 months.

810 InternalMedicineVol. 33, No. 12 (December 1994)

Skin Ulceration in Amyopathic DM

Discussion

Dermatomyositis is the designation for patients who haveboth inflammatory myopathy and typical skin eruption. Theonset ofa skin disorder does not necessarily occur at the time ofmuscleinvolvement. Bohan et al found that 93% of 45 patients

with DMhad a skin rash at the time of presentation but muscleweakness was present in only 53% (2). Rockerbie et al observedthat skin rash preceded muscle weakness in 56% of DMpatients. Skin rash preceded muscle disease by more than 1.75years in 12% of this group (3). Krain reported six cases ofDMshowing typical cutaneous changes without apparent muscleinvolvement at the time of presentation (1). All these patientseventually developed inflammatory myopathy. It is thus appar-

e

nt that skin rash of DM commonly precedes muscle involve-ment.

Pearson demonstrated five cases of DM with typical rashbut no indication of muscle weakness (4). One of these patients

had rash for 13 years without muscle weakness. Thus,amyopathic dermatomyositis is a designation applicable to

patients showing typical cutaneous manifestation of DM withnoor minimal muscle disease. Euwer and Sontheimer reportedsix cases of amyopathic dermatomyositis in whom the skinmanifestations occurred two years prior to muscle disorder;

they maintained that this term was only a provisional diagnosisfor some form ofDM (5). Four cases ofDM demonstrated onlyskin eruption after 4 to ll years; these may be true cases ofamyopathic dermatomyositis (6). Thus, not only does skin rashfrequently precede muscle involvement, but skin manifesta-

t

ions alone are expressed in some patients.Clinical evidence for muscle disease was not found until 1

year and 5 months after the onset of the typical skin rash ofDMin the present patient. The systemic administration of

glucocorticoid hormone may have caused delayed develop-

ment of inflammatory myopathy in this case. The patientdeveloped severe cutaneous vasculitis during therapy, and thusmay belong to a subset ofDM showing cutaneous manifestationofDM with no muscle disease. There is no established criteriafor amyopathic dermatomyositis. Therefore, the patient may betentatively diagnosed as amyopathic dermatomyositis. Muscle

involvement should be evaluated carefully in this case todetermine the applicability of this term. The limited number ofamyopathic DM patients makes it difficult to confirm theclinical characteristics of the disease. Stonecipher et al reportedfour cases of amyopathic DM with skin manifestation aloneshowing good prognosis. However, two cases with malignancywere found among nine amyopathic DM patients in whomdeveloped myositis subsequently (6). Fudman and Schnitzeralso demonstrated three patients with malignancy and twopatients with severe interstitial pneumonitis among seven DMpatients who had cutaneous changes and muscle involvement

but normal creatine kinase levels (7). Two patients withamyopathic DM in Japan have been noted to exhibit fatalinterstitial pneumonitis (8). Thus, some patients with amyopathic

D

M have been demonstrated to have a poor prognosis.Systemic vascular injury is the characteristic pathognomonic

feature of child-onset DM. Cutaneous vasculitic ulceration hasbeen observed frequently in one-fourth of child-onset DM

patients, but it is rarely noted in adult-onset DM. The clinicalfeatures of adult-onset DM showing cutaneous vasculitis havenotbeen well characterized. Feldman et al observed seven cases(9.2%) with cutaneous vasculitis. They detected subcutaneousnodulesin two, periungual infarcts in three and digital ulcera-tionin two, of 76 patients with adult-onset DM (9). Increasedassociation of cutaneous vasculitis and malignancy was notedintheir work. In the Japanese literature, four cases of DMassociated with cutaneous ulcerations are reported (10-13).However, associated malignancy was not found in these cases.No patient with adult-onset DM without muscle involvement,such as amyopathic dermatomyositis, manifesting cutaneousulcerationas a feature of skin disease, has been reported.

Various pathological changes in cutaneous vessels havebeen noted in child-onset DM (14). Endothelial swelling,

vascular occlusion and infarction and necrotizing vasculitisassociated with cellular infiltration have been shown. Thedeposition of immunoglobulin, complement and fibrin on ves-sel walls has also been observed. In the present case and in threereported cases of adult-onset DM with cutaneous ulceration,vascular occlusion or lymphocytic infiltration around subcuta-

n

eous vessels were demonstrated.The present patient was treated with methylprednisolone

pulse therapy followed by prednisolone and anti-coagulanttherapy. Although skin ulceration was quite resistant to therapy,significant improvement was noted at 4 months. Cutaneousulceration was reported resistant to steroid therapy in one report(9). Thus, cutaneous ulceration due to vasculopathy in DMpatients should be treated carefully due to the resistance to the

therapy.

References1 ) Krain LS. Dermatomyositis in six patients without initial muscle involve-

ment. Arch Dermatol 111: 241, 1975.2) Bohan A, Peter JB, Bowman RL, Pearson CM. A computer-assisted

analysis of 153 patients with polymyositis and dermatomyositis. Medi-cine 56: 255, 1977.

3) Rockerbie NR, Woo TY, Callen JP, Giustina T. Cutaneous change ofdermatomyositis precede muscle weakness. J Am Acad Dermatol 20:629, 1989.

4) Pearson CM. Polymyositis and dermatomyositis. in: Arthritis and AlliedConditions, 9th ed. McCarty DJ, Ed. Lea & Febiger, Philadelphia, 1979,p.742.

5) Euwer RL, Sontheimer RD. Amyopathic dermatomyositis(dermatomyositis sine myositis). J Am Acad Dermatol 24: 959, 1991.

6) StonecipherMR, Jorizzo JL, White WL, WalkerFO, Prichard E. Cutane-ous change of dermatomyositis in patients with normal muscle enzymes:dermatomyositis sine myositis? J Am Acad Dermatol 28: 951, 1993.

7) Fudman EJ, Schnitzer TJ. Dermatomyositis without creatine kinaseelevation. AmJ Med 80: 329, 1986.

8) Tokiyama K, Tagawa H, Yokota E, et al. Two cases of amyopathicdermatomyositis with fatal rapidly progressive interstitial pneumonitis.Ryumachi 30: 204, 1990 (Abstract in English).

9) Feldman D, Hochberg MC, Zizic TM, Stevens MB. Cutaneous vasculitisin adult polymyositis/dermatomyositis. J Rheumatol 10: 85, 1983.

10) Hattori T, Kitade K, ShidaY, Mizutani H, Shiraisi Y. An autopsy case ofdermatomyositis with intractable skin ulcer dead of acute interstitial

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pneumonitis. Rinsho Dermatol 38: 533, 1984 (in Japanese).1 1) Hatakenaka K, Kitagawa N, Arata J. Dermatomyositis associated with

vasculitis and steatitis. Jpn J Clin Dermatol 28: 703, 1986 (in Japanese).12) Niizawa M, Maie O, Asanuma Y, Saito T. Adult dermatomyositis with

angiopathy and cecum perforation. Jpn J Dermatol 101: 447, 1991(Abstract in English).

1

3) Ohsako S, Kamatani N, Kashiwazaki S, Endo H, Kondo H. A case ofdermatomyositis associated with digital gangrene and eruption improvedby vitamin A. Jpn J Clin Immun 15: 297, 1992 (Abstract in English).

14) Cassidy JT, Petty RE. Juvenile dermatomyositis. in: PediatricRheumatology, 2nd Ed, Churchill Livingstone, New York, 1990, p.33 1.

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