Sweet's Syndrome Associated with Mycobacteria Chelonae Cervical Lymphadenitis and Herpes Genitalis - A Case ReportBULLETIN FOR MEDICAL PRACTITIONERS Dr Colin Theng, Registrar Introduction
Six months after completion of her anti-tuberculous treatment, she presented with a 4-month history of painful vulval ulcerations. She was married with a stable monogamous relationship. On examination, there were 2 tender erosions, 3cm and 0.5cm in diameter, over the right labia minora. Biopsy showed an eroded lesion with epidermal hyperplasia and a moderately dense infiltrate of lymphocytes and plasma cells in the dermis. The epidermis also showed a mild degree of dysplasia with no features of giant cells or inclusion bodies. The histology was interpreted as showing inflammatory dysplasia. Culture was positive for herpes simplex virus type II. Shortly thereafter, she presented with an acute cutaneous eruption of erythematous plaques with blister formation. The cutaneous eruption was associated with fever and a 1-month history of painful neck lumps. She had erythematous plaques and tense blisters on the face, neck and limbs, with bilateral firm cervical lymph nodes. Her white cell count was raised at 26.0x109/I, with 80% neutrophils. The rest of her biochemistry was unremarkable. Her CD4 and CD8 counts were normal, as was her chest X-ray (CXR). She refused HIV serological testing. Based on initial clinical impression of bullous erythema multiforme secondary to herpes simplex virus infection, she was given oral acyclovir 200 mg 5 times a day for a week, followed by maintenance therapy of 400mg bd upon which there was clearance of vulval and skin lesions. However, biopsy of the cutaneous lesion showed dense diffuse dermal infiltrates of neutrophils. There were no features of interface dermatitis to suggest erythema multiforme, and acid-fast bacilli and fungal organisms were not demonstrated. She was then diagnosed as Sweet's syndrome, based on the histological features and clinical presentation of fever and neutrophilia. Direct immunoflourescence was negative. Biopsy of a cervical lymph node showed reactive lymphadenopathy and was negative for acid-fast bacilli.
One month later, she presented to the clinic with a 4-day history of a blistering eruption involving the palms, face and neck with swelling of her knees and hands. On examination, she was afebrile and had multiple oedematous tender red plaques studded with pustules over the face, neck, upper limbs, back and knees (Figs. 1 and 2). She had bilateral knee effusion, swelling of the finger joints and dusky erythematous patches over both shins, which were warm and tender. She had multiple bilateral enlarged cervical lymph nodes. Mycobacterium chelonae was isolated from the lymph node culture sent previously. Initial investigation revealed elevated total white cell count of 30.9x109/dl with 80% polymorphs, and ESR of 108 mm/h. Her CD4 and CD8 counts were normal and the ratio was not reversed. She was investigated further to rule out connective tissue disease and underlying immunodeficiency. ANA, anti-dsDNA, anti-Ro, anti-La and rheumatoid factor were negative. Myeloma screen was negative and nitroblue tetrazolium (NBT) test was normal. A repeat biopsy showed similar features of dense diffuse infiltrates of neutrophils throughout the dermis, mixed with few histiocytes, lymphocytes and eosinophils, consistent with Sweet's syndrome (Fig. 3).
The final diagnosis was Sweet's syndrome associated with Mycobacterium chelonae infection and reactive arthritis. The patient was started on clarithromycin and ethambutol, and in view of progressive skin lesions, prednisolone 30 mg/day was also added. Her skin lesions, cervical lymphadenopathy and arthritis resolved rapidly and totally to the treatment. The prednisolone dose was gradually tapered off over 3 months. She was continued on ethambutol and clarithromycin for a total of 6 months. She remained well 4 months after discontinuation of prednisolone and 1 month after completing the anti-microbial therapy.
Our patient presented with a bullous eruption and genital herpes. The initial clinical impression was that of bullous erythema multiforme associated with genital herpes, as this is a more common association. Sweet's syndrome was diagnosed later on review of the histological findings. The association between Sweet's syndrome and herpes simplex virus infections is rare but has been reported.[12] The initial improvement of her skin condition may have been a result of the use of acyclovir. There have also been previous reports of spontaneous resolution of Sweet's syndrome without treatment [13,14] and the improvement in our patient may have been the natural progression of her disease at that time. She presented again with tender oedematous plaques, arthritis and leukocytosis. Based on the constellation of clinical and histological features, our patient thus fulfilled all the proposed major and minor criteria of Sweet's syndrome. The culture from her cervical lymph node biopsy grew Mycobacterium chelonae. It is possible that her previous subglottic mass and lung nodule were a result of Mycobacterium chelonae infection, which may also respond to anti-tuberculosis treatment. Development of cervical lymphadenitis and positive culture of Mycobacterium chelonae from the cervical lymph nodes may have resulted from recurrence of infection. In view of her susceptibility to atypical mycobacterial infection, we had investigated the patient for underlying immunodeficiency and connective tissue disease, which were negative. Her total white and lymphocyte counts were normal and her CD4 to CD8 ratio was not reversed, making HIV infection highly unlikely, though not excluded unequivocally as she had refused HIV serological testing. There were 3 previous reports of the rare association of Sweet's syndrome and atypical mycobacterial infection. In 1992, Kramers et al [15] reported a case of Sweet's syndrome as the presenting symptom of hairy cell leukaemia, in a patient with concomitant Mycobactenium kansasii. In 1995, Hsiao and Chiu [16] reported a case of Sweet's syndrome in a patient with cervical lymphadenitis caused by Mycobactenium fortuitum. Choonhakarn at al [17] reported 5 cases of Sweet's syndrome associated with non-tuberculous mycobacteria infection in 1998. In this series, 3 cases were associated with Mycobactenium chelonae, one with Mycobactenium avium intracellulare and one with Mycobactenium scrofulaceum. All cases were treated with prednisolone with improvement. The simultaneous resolution of cervical lymphadenitis and the bullous tumid skin lesions in our patient seems to suggest Mycobactenium chelonae as the cause of Sweet's syndrome. A hypersensitive response to infections has been postulated as a possible cause of Sweet's syndrome. This case report lends further credence to this hypothesis. In the search for an underlying cause of Sweet's syndrome, infections including non-tuberculous mycobacterial infections and herpes simplex virus infections should be looked for, in addition to internal malignancies.
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