Cutaneousvasculitis in a Patient with Dermatomyositis Ithout Muscle Involvement

CASE REPORT

CutaneousVasculitis in a Patient with Dermatomyositis Akifumi Kadoya, Tohruw ithoutAkahoshi, MuscleNaho Sekiyama,InvolvementShigeru 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 the skin ulceration indicated vascular occlusion without cellular infiltration. Cutaneous ulceration is a very rare manifestation of adult-onset DMpatients without inflammatory myopathy. (Internal Medicine 33: 809-812, 1994) Key words: skin ulceration, amyopathic dermatomyositis

Introduction purplish erythematous eruptions on proximal interphalangeal (PIP) joints, metacarpophalangeal (MCP) joints and extensor Dermatomyositis (DM) is a connective tissue disorder char- surfaces of the elbows (Fig. 1) and knees. A faint purple, scaly acterized by prominent cutaneous features and inflammatory and macular eruption was noted on the clavicular and V-area of myopathy. A DMpatient may not necessarily have both cuta- anterior part of the chest. There was no indication of muscle neous and muscle disease at the initial presentation. Cutaneous weakness. Levels of muscle enzymes such as serum aspartate manifestation of DM frequently precedes the development of aminotransferase,creatine kinase and aldolase were normal. muscle disease. The absence of muscle disease in patients with Serum lactic dehydrogenase was 55 1 IU/1 (normal range; 1 80- the classical skinrash ofDM persisting over aperiod of 10 years 350) on admission, but it decreased to normal ranges without has been reported (1). Amyopathic DM is the term used to treatment. The results of an electromyogram were negative. A describe patients with typical cutaneous manifestation of DM muscle biopsy was performed from right biceps brachii muscle. ith no or minimal muscle disease. Muscle specimens did not exhibit atrophy, degeneration or Cutaneous vasculitis characterized by periungual infarcts fibrosisof the muscle fiber. Distribution of type I and type II and digital ulcerations has been noted frequently in child-onset muscle fibers were indicated to be normal. Infiltration of inflam- M.However, its occurrence in adult-onset DM is rare. matory cells and vasculitis were absent in the specimens.

w D The patient in this study had the typical skin rash of DM Histological examination of skin specimens obtained from the without apparent muscle disease; subsequently digital and fingers and elbows indicated hyperkeratosis of epidermis, thick- foot ulcerations developed. The rare association of skin ulcera- ness and edematous changes in collagenous connective tissue tion and amyopathic DM in adults is discussed. ofthe dermis, basal keratinocyte liquefaction degeneration and mild infiltration of mononuclear cells around small blood Case Report vessels (Fig. 2). These findings were consistent with the fea- tures of skin eruption of DM. A diagnosis of DM without A74-year-old Japanese female wasfirst admitted to Kitasato prominent muscle disease was made. The patient was treated University Hospital in April 1993 with a two-month history of with prednisolone 20 mg/day; the skin eruptions showed good fatigue, swelling of the ringers and scaly and erythematous response. eruptions on proximal interphalangeal joints, metacarpo- At one month following discharge from the hospital, a skin phalangealjoints, elbows and knees. She had no past or family ulceration of the right fifth finger and left sole developed during history of collagen disease nor had she experienced Raynaud's prednisolone treatment of 17.5 mg/day. The right fifth finger phenomenon or arthralgia. then became edematous and erythematous with subsequent Examination indicated an edematous and very faint spreading over her right forearm. In August 1993, she was erythematous eruption on the upper eyelids. There were scaly admitted again. She had Gottron's papules on PIPjoints, MCP

From the Department of Internal Medicine, Kitasato University, School of Medicine, Sagamihara Received for publication March 25, 1994; Accepted for publication August 24, 1994 Reprint requests should be addressed to Dr. Akifumi Kadoya, the Department of Internal Medicine, Kitasato University, 1 - 1 5- 1 , Kitasato, Sagamihara, Kanagawa 228

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joints and typical rash ofDM onthe elbows and knees. Periungual erythema, multiple nail fold thrombi on fingers and scattered papular erythema on the fingers and soles were noted. A small digital ulceration 3 mm in diameter on the dorsum of the right fifth PIP joint, deeper skin ulcerations 12 mm in diameter on the left sole and 6 mm in diameter on the left fifth toe were etected (Fig. 3). The tip of the left fourth toe was gangranous. Swelling and tenderness of the right arm due to cellulitis wereevident. No sclerodactyly or muscle weakness could be

detected.d Muscle enzymes such as aspartate aminotransferase, creatine kinase, aldolase and lactic dehydrogenase showed normal range levels. The erythrocyte sedimentation rate (ESR) had increased to 72 mm/h and C-reactive protein (CRP) was 5,048 jLXg/dl. Serum IgG and IgA were increased to 1 ,880 mg/dl and 5 14 mg/dl, respectively. Antinuclear antibody was positive at x80 with a speckled and homogenous pattern, but the anti- body to extractable nuclear antigen was negative. Antineutrophil cytoplasmic antibody (ANCA) and anticardiolipin antibody werenot detected. The results of the electromyogram were also negative. To analyze for occult malignancy, X-ray examina- tion, abdominal echogram, chest and abdominal CT scan, gallium radioisotope scanning, gastroflberscope and barium Fig. 1. Gottron's papules on the elbow. enema were performed. No associated malignancy was de- tected. Pathological examination of skin biopsy specimens from the right sole demonstrated dilation of vessels in the dermis with endothelial hyperplasia, vascular occlusion due to intraluminal thrombi; there was no cellular infiltration around the vessels (Fig. 4). Immuno-histochemical examination dis- closed the deposition of IgG, IgA, IgM, C3, C4, Clq and ibrinogen on the vessels.

f The patient was treated with heparin (10,000 U/day) and lipo-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 and warfarin. The skin ulcerations were resistant to the therapy, but significant improvement was noted at 4 months.

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

Fig. 4. Microscopic section of the biopsy specimen taken from sole exhibiting skin ulceration, showing endothelial swelling and vascular occlu- sion due to intraluminal thrombi but no cellular infiltration is revealed around Fig. 3. Cutaneous ulceration on the right fifth toe. the vessels (HE stain, x40).

810 InternalMedicineVol. 33, No. 12 (December 1994) Skin Ulceration in Amyopathic DM

Discussion feature of child-onset DM.Cutaneous vasculitic ulceration has been observed frequently in one-fourth of child-onset DM Dermatomyositis is the designation for patients who have patients, but it is rarely noted in adult-onset DM. The clinical both inflammatory myopathy and typical skin eruption. The features of adult-onset DM showing cutaneous vasculitis have onset ofa skin disorder does not necessarily occur at the time of notbeen well characterized. Feldman et al observed seven cases muscleinvolvement. Bohan et al found that 93% of 45 patients (9.2%) with cutaneous vasculitis. They detected subcutaneous with DMhad a skin rash at the time of presentation but muscle nodulesin two, periungual infarcts in three and digital ulcera- weakness was present in only 53% (2). Rockerbie et al observed tionin two, of 76 patients with adult-onset DM (9). Increased that skin rash preceded muscle weakness in 56% of DM association of cutaneous vasculitis and malignancy was noted patients. Skin rash preceded muscle disease by more than 1.75 intheir work. In the Japanese literature, four cases of DM years in 12% of this group (3). Krain reported six cases ofDM associated with cutaneous ulcerations are reported (10-13). showing typical cutaneous changes without apparent muscle However, associated malignancy was not found in these cases. involvement at the time of presentation (1). All these patients No patient with adult-onset DM without muscle involvement, eventually developed inflammatory myopathy. It is thus appar- such as amyopathic dermatomyositis, manifesting cutaneous nt that skin rash of DM commonly precedes muscle involve- ulcerationas a feature of skin disease, has been reported. ment. Various pathological changes in cutaneous vessels have Pearson demonstrated five cases of DM with typical rash been noted in child-onset DM (14). Endothelial swelling,

bute no indication of muscle weakness (4). One of these patients vascular occlusion and infarction and necrotizing vasculitis had rash for 13 years without muscle weakness. Thus, associated with cellular infiltration have been shown. The amyopathic dermatomyositis is a designation applicable to deposition of immunoglobulin, complement and fibrin on ves- patients showing typical cutaneous manifestation of DM with sel walls has also been observed. In the present case and in three noor minimal muscle disease. Euwer and Sontheimer reported reported cases of adult-onset DM with cutaneous ulceration, six cases of amyopathic dermatomyositis in whom the skin vascular occlusion or lymphocytic infiltration around subcuta- manifestations occurred two years prior to muscle disorder; eous vessels were demonstrated.

they maintained that this term was only a provisional diagnosis n The present patient was treated with methylprednisolone for some form ofDM (5). Four cases ofDM demonstrated only pulse therapy followed by prednisolone and anti-coagulant skin eruption after 4 to ll years; these may be true cases of therapy. Although skin ulceration was quite resistant to therapy, amyopathic dermatomyositis (6). Thus, not only does skin rash significant improvement was noted at 4 months. Cutaneous frequently precede muscle involvement, but skin manifesta- ulceration was reported resistant to steroid therapy in one report ions alone are expressed in some patients. (9). Thus, cutaneous ulceration due to vasculopathy in DM Clinical evidence for muscle disease was not found until 1 patients should be treated carefully due to the resistance to the

yeart and 5 months after the onset of the typical skin rash ofDM therapy. in the present patient. The systemic administration of glucocorticoid hormone may have caused delayed develop- References ment of inflammatory myopathy in this case. The patient 1 ) Krain LS. Dermatomyositis in six patients without initial muscle involve- developed severe cutaneous vasculitis during therapy, and thus ment. Arch Dermatol 111: 241, 1975. may belong to a subset ofDM showing cutaneous manifestation 2) Bohan A, Peter JB, Bowman RL, Pearson CM. A computer-assisted ofDM with no muscle disease. There is no established criteria analysis of 153 patients with polymyositis and dermatomyositis. Medi- for amyopathic dermatomyositis. Therefore, the patient may be cine 56: 255, 1977. tentatively diagnosed as amyopathic dermatomyositis. Muscle 3) Rockerbie NR, Woo TY, Callen JP, Giustina T. Cutaneous change of dermatomyositis precede muscle weakness. J Am Acad Dermatol 20: involvement should be evaluated carefully in this case to 629, 1989. determine the applicability of this term. The limited number of 4) Pearson CM. Polymyositis and dermatomyositis. in: Arthritis and Allied amyopathic DM patients makes it difficult to confirm the Conditions, 9th ed. McCarty DJ, Ed. Lea & Febiger, Philadelphia, 1979, clinical characteristics of the disease. Stonecipher et al reported p.742. four cases of amyopathic DM with skin manifestation alone 5) Euwer RL, Sontheimer RD. Amyopathic dermatomyositis showing good prognosis. However, two cases with malignancy (dermatomyositis sine myositis). J Am Acad Dermatol 24: 959, 1991. 6) StonecipherMR, Jorizzo JL, White WL, WalkerFO, Prichard E. Cutane- were found among nine amyopathic DM patients in whom ous change of dermatomyositis in patients with normal muscle enzymes: developed myositis subsequently (6). Fudman and Schnitzer dermatomyositis sine myositis? J Am Acad Dermatol 28: 951, 1993. also demonstrated three patients with malignancy and two 7) Fudman EJ, Schnitzer TJ. Dermatomyositis without creatine kinase patients with severe interstitial pneumonitis among seven DM elevation. AmJ Med 80: 329, 1986. patients who had cutaneous changes and muscle involvement 8) Tokiyama K, Tagawa H, Yokota E, et al. Two cases of amyopathic dermatomyositis with fatal rapidly progressive interstitial pneumonitis. but normal creatine kinase levels (7). Two patients with Ryumachi 30: 204, 1990 (Abstract in English). amyopathic DM in Japan have been noted to exhibit fatal 9) Feldman D, Hochberg MC,Zizic TM, Stevens MB. Cutaneous vasculitis interstitial pneumonitis (8). Thus, some patients with amyopathic in adult polymyositis/dermatomyositis. J Rheumatol 10: 85, 1983. M have been demonstrated to have a poor prognosis. 10) Hattori T, Kitade K, ShidaY, Mizutani H, Shiraisi Y. An autopsy case of dermatomyositis with intractable skin ulcer dead of acute interstitial D Systemic vascular injury is the characteristic pathognomonic

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pneumonitis. Rinsho Dermatol 38: 533, 1984 (in Japanese). 3) Ohsako S, Kamatani N, Kashiwazaki S, Endo H, Kondo H. A case of 1 1) Hatakenaka K, Kitagawa N, Arata J. Dermatomyositis associated with dermatomyositis associated with digital gangrene and eruption improved

vasculitis and steatitis. Jpn J Clin Dermatol 28: 703, 1986 (in Japanese). 1 by vitamin A. Jpn J Clin Immun 15: 297, 1992 (Abstract in English). 12) Niizawa M, Maie O, Asanuma Y, Saito T. Adult dermatomyositis with 14) Cassidy JT, Petty RE. Juvenile dermatomyositis. in: Pediatric angiopathy and cecum perforation. Jpn J Dermatol 101: 447, 1991 Rheumatology, 2nd Ed, Churchill Livingstone, New York, 1990, p.33 1. (Abstract in English).

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