Dermatomyositis with tuberculous fasciitis

ARTICLE

Auteur(s) : Yuichi YOSHIDA^{a, b}, Juichiro NAKAYAMA^a, Masutaka FURUE^c, Tetsuo MATSUDA^{b, c}

^a Department of Dermatology, School of Medicine, Fukuoka University, 7-45-1, Nanakuma, Jonan-ku, Fukuoka 814-0180, Japan 
^b Division of Dermatology, Hamanomachi Hospital, 3-5-27, Maizuru, Chuo-ku, Fukuoka 810-8539, Japan 
^c Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, 812-8582, Fukuoka, Japan

Article accepted on 19/12/2003

Although extrapulmonary tuberculosis and musculoskeletal involvement of tuberculosis is rare, several cases of tuberculous infection in immunocompromised hosts have been reported [1-4]. We report a case of dermatomyositis with tuberculous fasciitis that was treated with oral prednisolone. Immunosuppression caused by steroid therapy contributed to the development of tuberculous fasciitis, and delay in diagnosis was fatal. It is important for clinicians to rule out the possibility of tuberculous infection in an immunocompromised host.

Case Report

A 69-year-old Japanese man was referred to our department by his rheumatologist for evaluation of persistent swollen bilateral arms and forearms. He was diagnosed as having dermatomyositis 6 months before admission to our hospital based on the findings of erythema on the extensor surfaces of forearms, proximal muscle weakness, muscle pain, elevated level of creatine phosphokinase and presence of systemic inflammatory signs, and was treated with oral prednisolone at 50 mg daily. There was a prompt response to the treatment, and his muscle strength improved. As his condition became stable, the dosage of prednisolone was tapered to 10 mg daily. However, he noticed erythema and bilateral swelling of forearms 6 weeks before referral to our department. Although he was hospitalized and the dosage of prednisolone was increased by his rheumatologist to 50 mg daily for 5 weeks, the response was poor. Since his bilateral arms and forearms became diffusely erythematous and swollen, antibiotic therapy with piperacillin and meropenem was started. Despite this treatment, he became febrile with chills, anorexia, and general fatigue, and he was sent to our hospital for further evaluation. On admission, physical examination showed marked swelling of bilateral arms and forearms (Fig. 1) with a persistent spiking fever. Results of laboratory investigation were as follows: leukocyte count, 14,300/µl; hemoglobin, 11.3 g/dl; erythrocyte sedimentation rate, 104 mm/h; blood urea nitrogen, 25 mg/dl; asparate aminotransferase, 125 IU/l; alanine aminotransferase, 96 IU/l; creatine phosphokinase, 1014 IU/l; aldolase, 21.2 IU/l; lactate dehydrogenase, 1424 IU/l; and C-reactive protein, 25.4 mg/dl. A chest radiograph was unremarkable, and no pathogen (common bacteria) was isolated from blood culture. In addition, he had a past medical history of tuberculous spondylitis 44 years previously. We first suspected the lesion to be deep cellulitis or necrotizing fasciitis and performed a skin biopsy from a diffusely erythematous lesion. At surgery, the fascia of his forearm showed evidence of inflammatory changes, and continuous yellowish purulent discharge was seen. Histological examination of a specimen taken from the lesion showed dense, diffuse infiltrate of neutrophils and lymphocytes as well as hemorrhage without granuloma formation in the dermis, subcutaneous fat tissue, and fascial tissue (Fig. 2). Debridement revealed necrosis of the subcutaneous tissue and fascia. Moreover, acid-fast bacilli were identified by Ziehl-Neelsen’s staining (Fig. 3). Based on these findings, a diagnosis of tuberculous fasciitis was made. Although the dosage of prednisolone was tapered and we were just about to start anti-tuberculous therapy, he died suddenly from acute respiratory insufficiency at one week after the biopsy. Mycobacterium tuberculosis was identified from tissue cultures 7 weeks later.

Discussion

Although cutaneous manifestations of tuberculosis, e.g., lupus vulgaris, tuberculosis verrucosa cutis and scrofuloderma, have been well documented [5], the diagnosis of tuberculous infection is often delayed owing to its unusual presentations [6]. In addition, fasciitis due to tuberculosis is extremely rare, only three cases having been reported previously [1, 2, 4]. Curiously, all of the reported cases were in an immunosuppressed condition due to corticosteroid therapy and the mean delay in arriving at correct diagnosis was about 11 weeks, suggesting difficulty in early diagnosis. Our patient was treated with a high dose of steroids at first because exacerbation of dermatomyositis was suspected. However, the results of the initial treatment were not satisfactory. When he was referred to our department, we considered clinical differential diagnoses, including deep cellulitis, necrotizing fasciitis and other infectious dermatoses. Since the clinical course was subacute and unusual and since there was no response to antibiotic treatment, we performed a biopsy to rule out the possibility of mycobacterium infection. Histopathological examination and surgical debridement revealed features of tuberculous fasciitis. Soon after the diagnosis of tuberculous fasciitis had been made, we intended to initiate anti-tuberculous therapy. However, he died suddenly from acute respiratory insufficiency. It took 7 weeks after the appearance of bilateral swollen arms and forearms to reach a correct diagnosis. Since dissection was not performed, the cause of tuberculous infection was not clear. Although there was no evidence of scrofuloderma or recurrence of tuberculous spondylitis, it is most likely that mycobacterium tuberculosis, which had been dormant in his body, was activated due to immunosuppression caused by steroid therapy.
In conclusion, the findings in our case indicate that it is important for clinicians to pay attention to tuberculous infection in an immunocompromised host and perform a biopsy as soon as possible from a suspicious lesion. Delay in diagnosis might result in significant morbidity for patients. n

References

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