To the Editor,
Kaposi´s sarcoma (KS) is a malignant neoplasm of endothelial cells with low
proliferation, described by Moritz Kaposi in 1872. It is classified into four groups:
endemic, reported in African regions; epidemic, which is acquired immune deficiency
syndrome(AIDS)-related; classic, at first reported primarily in Ashkenazi Jews and
Mediterranean peoples, indicating a genetic relationship; and iatrogenic, which affects
immunosuppressed patients of autoimmune and malignant diseases, and transplanted
A 36-year-old female patient was diagnosed with lepromatous leprosy in 2008. After
five-month multibacillary treatment (dapsone, rifampicin and clofazimine), erythematous
and painful nodules disseminated throughout her body. Type 2 reaction was diagnosed
treated with prednisone 40 mg/day, ranging from 20-60 mg/day according to the patient's
response. Treatment lasted between 2008 and 2013 due to persistent lesions. However,
new exam in 2013 showed violaceous and erythematous nodules and plaques, ranging from
1.0-10cm, distributed on the upper and lower limbs, pubis, and left breast.
Non-depressible edema was also found on the lower limbs (Figure 1). No lesions were
found on oral and genital mucosae.
Violaceous and erythematous papules and plaques with irregular and well-defined
edges, and smooth and bright surface on the upper and lower limbs
The lesion was asymptomatic, and the patient showed no improvement, despite the continued
use of high doses of prednisone. SK was considered and confirmed after an
anatomopathological examination (Figure 2A).
Immunohistochemistry for human herpesvirus-8 (HHV-8) was positive (Figure 2B).
A: Proliferation of fusiform cells, band-forming cells with nuclear atypia and
mitotic activity, and extravasation of lymphocytes in the dermis (40x H&E).
B: Immunohistochemistry positive for human herpesvirus-8
Blood count, liver and kidney exams, serological tests for syphilis, hepatitis B and
and HIV, chest X-ray, and abdominal ultrasound were normal or non-reactive.
The lesion was diagnosed as iatrogenic KS induced by prolonged use of corticosteroids
As a critical therapeutic measure, due to the large number of lesions on different
of the body, the patient had to stop taking the inducing medication. CS dose was
gradually reduced, but the patient showed new erythematous and painful nodules on
upper and lower limbs, accompanied by malaise. It was interpreted as type 2 reaction;
clofazimine 300 mg/day was prescribed for 30 days to control the reaction, with gradual
dose reduction and the previous slow withdrawal of CS. After three months, the lesions
of type 2 reaction disappeared. The medication was fully withdrawn; six months after
KS diagnosis, these lesions improved with residual brownish stain (Figure 3). A three-year
follow-up showed persisting residual
Residual brownish macules on the upper and lower limbs
KS in immunosuppressed patients was first reported in transplant recipients under
cyclosporine. However, it has also been reported in other haematological,
rheumatological, and pulmonary diseases caused by immunosuppressive treatment.
This CS-related disease was found in the treatment of different diseases, such as
idiopathic thrombocytopenic purpura, temporal arteritis, Sjogren's syndrome, pemphigus
vulgaris, bullous pemphigoid, dermatomyositis, systemic lupus erythematosus, primary
biliary cirrhosis, rheumatoid arthritis, and lymphoproliferative disorders.
This case reports a patient under treatment of leprosy type 2 reaction. The patient's
medical records failed to reveal the time between the beginning of the CS treatment
the emergence of the first KS lesions. In the reviewed literature, the KS may appear
between one month and 20 years after the immunosuppressive drug is introduced.
The presence of HHV-8 was first associated with the KS in 1994. The herpesvirus DNA
sequence was identified in KS fragments from patients with AIDS
and has subsequently been reported in
all KS types. It infects the endothelial cells and remains there in a latent state.
reactivated, it causes aberrant angiogenesis, which is an increase in production and
secretion of pro-angiogenic factors.
KS clinical lesions may vary from brownish macules and plaques to violaceous exophytic
tumors. Some lesions may ulcerate or invade surrounding tissues, such as the bones.
Different stages may coexist, as seen in this case report, but adjacent tissues and
inner organs remain intact.
The severe lesions and the different body regions affected required the withdrawal
immunosuppressive drug prescribed in the earlier therapy. Such measured has been
reported by other authors and was successfully reproduced in this case.
KS is a HHV-8-associated tumor and must be taken into account for all cases of patients
undergoing immunosuppresseve therapy, regardless of the drug used. As far as it could
assessed, this is the first case report of KS in immunosuppressed patients by CS during
leprosy reaction treatment.