Paraneoplastic dermatomyositis presenting as erythroderma

Journal of the American Academy of Dermatology Volume 39, Number 4, Part 1

Brief communications 653

Paraneoplastic dermatomyositis presenting as erythroderma Hossein C. Nousari, MD,a Arash Kimyai-Asadi, BA,a and Douglas J. Spegman, MDb Baltimore, Maryland Erythroderma is idiopathic in one third of patients. Its most common causes include a variety of pre-existing skin diseases such as atopic dermatitis and psoriasis, drug reactions, malignancies, and congenital dermatoses.1 To our knowledge there are only 4 well-documented cases in the literature of dermatomyositis presenting with erythroderma.2-5 Because none of these cases was associated with malignancy, this is the first report of paraneoplastic dermatomyositis presenting with erythroderma. CASE REPORT A 64-year-old Korean man had a 6-month history of a pruritic, scaling, photosensitive eruption that began on the face and then progressively generalized. The patient later experienced progressive proximal muscle From the Departments of Dermatologya and Internal Medicine,b The Johns Hopkins University School of Medicine. Reprint requests: Hossein C. Nousari, MD, Division of Immunodermatology, Ross Research Building, Room 771, 720 Rutland Ave, Baltimore, MD 21205. J Am Acad Dermatol 1998;39:653-4. Copyright © 1998 by the American Academy of Dermatology, Inc. 0190-9622/98/$5.00 + 0 16/54/92460

weakness. He also had anorexia, a 25-lb weight loss, and intermittent postprandial abdominal pain. He had a nasopharyngeal carcinoma 10 years earlier that was treated with surgery, chemotherapy, and radiotherapy. His father died of gastric cancer at 55 years of age. Examination revealed exfoliative erythroderma sparing the palmoplantar surfaces (Fig 1), a periorbital violaceous and edematous eruption, poikilodermatous changes of the scalp and upper trunk, periungual telangiectases, and a symmetric muscle weakness affecting the proximal upper extremities. A biopsy specimen of the back showed an interface dermatitis with scant perivascular mononuclear cell infiltrate. Direct immunofluorescence of the skin was negative. A deltoid muscle biopsy specimen and electromyographic examination were consistent with an inflammatory myopathy. A computed tomographic scan of the head as well as nasopharyngeal fiberoptic examination failed to reveal any evidence of recurrence of nasopharyngeal carcinoma. Esophagogastroduodenoscopy revealed an infiltrative antral ulcer; histologic examination revealed infiltrating poorly differentiated adenocarcinoma. He underwent hemigastrectomy. During surgery multiple small mesenteric and peripancreatic soft tissue areas were noted. One month after surgery,

654 Brief communications

Journal of the American Academy of Dermatology October 1998

he showed mild clinical improvement of his muscular and cutaneous findings. DISCUSSION

Despite the small number of reported cases, we believe that erythroderma may not be an uncommon cutaneous presentation of dermatomyositis. Many patients with dermatomyositis have been described as having a “diffuse rash,” raising the possibility of erythroderma in at least some of these patients.6 REFERENCES

Fig 1. Patient’s back, demonstrating erythroderma at presentation.

1. Adam JE. Exfoliate dermatitis (erythroderma). Curr Probl Dermatol 1971;4:1-23. 2. Miyagawa S, Okazaki A, Minowa R, Shirai T. Dermatomyositis presenting as erythroderma. J Am Acad Dermatol 1992;26:489-90. 3. Pedragosa JR, Peyri RJ, Palou J. Modalidad eritrodermica de dermatomiositis en transito a poiquilodermatomiositis. Actas Dermosifiliogr 1974;65:179-80. 4. Pierson JC, Taylor JS. Erythrodermic dermatomyositis. J Am Acad Dermatol 1993;28:136. 5. Ramirez G, Asgerson RA, Khamashta MA, Cervera R, D’Cruz D, Hughes GRV. Adult-onset polymyositis-dermatomyosis: description of 25 patients with emphasis on treatment. Semin Arthritis Rheum 1990;20:144-20. 6. Bohan A, Peter JB, Bowman RL, Pearson CM. A computer-assisted analysis of 153 patients with polymyositis and dermatomyositis. Medicine 1977;56:255-86.