Immunosuppressive Kaposi’s sarcoma with bullous pemphigoid associated with hepatitis B virus infection

ARTICLE

Auteur(s) : Ting Xiao¹, Hong-Guang Lu², Guang-Yu Jin¹, Jian Wu¹, Zhi-Hua Wang¹, Hong-Hui Xu¹, Xing-Hua Gao¹, Hong-Duo Chen¹

¹Department of Dermatology, No. 1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang 110001, China
²Department of Dermatology, Affiliated Hospital of Guiyang Medical Collage, Guiyang, China

Bullous pemphigoid (BP) is an autoimmune blistering disease commonly seen in the elderly. There have been few cases of immunosuppressive Kaposi’s sarcoma (KS) that occurred in BP [1-3]. We report a case of KS in a BP patient with hepatitis B virus (HBV) infection.

A 62-year-old Chinese woman presented with a 3-week history of vesicles and bullae. Physical examination revealed multiple tense vesicles or bullae on the trunk (figure 1A) and limbs. Nikolsky sign was negative. Laboratory tests revealed total white blood cell count 18.4 × 10⁹/L with 17% eosinophils, serum total IgE 1,000 IU/mL, positive serum hepatitis B surface antibody (HBsAb) and hepatitis B e antibody (HBeAb), and negative serum HBV DNA. The liver enzyme levels were normal. Serum antibodies against human immunodeficiency virus, herpes simplex virus and cytomegalovirus were negative. Histopathology revealed a subepidermal blister containing eosinophils (figure 1B). Direct immunofluorescence (IF) showed linear IgG and C3 deposits along the basement membrane zone (BMZ). Indirect IF on 1-mol/L sodium chloride-separated normal human skin showed IgG anti-BMZ antibodies bound to the epidermal side (figure 1C). A diagnosis of BP and HBV infection was made.

Oral prednisolone 80 mg and azathioprine 100 mg daily were administered. On Day 15, diabetes mellitus occurred and was controlled by oral metformin, glipizide and glibenclamide. On Day 23, the lesions resolved. On Day 35, thrush occurred and was cured by oral nystatin. On Day 49, pulmonary infection was diagnosed by X-ray and cured by intravenous ciprofloxacin 800 mg daily for 10 days. On Day 59, multiple bean-sized bluish-purple colored painless nodules appeared on her hands, arms, feet (figures 1D-F) and legs. Histopathology showed aggregates of blood vessels with proliferation of spindle-shaped cells peripheral to the layer of endothelial cells, as well as dermal hemosiderin deposition (figure 1G). KS was diagnosed. Human herpes virus 8 (HHV-8) DNA was negative in the serum, peripheral blood mononuclear cells (PBMCs), KS and BP lesional skin of the patient using a nested polymerase chain reaction (PCR) method [4, 5]. HHV-8-specific IgG antibody in the serum of the patient was negative using an enzyme-linked immunosorbent assay [5].

Azathioprine was discontinued. On Day 72, tinea corporis occurred and was cured by topical terbinafine. On Day 73, she was discharged. Prednisolone was discontinued one year later. All KS lesions spontaneously cleared after another year. Annual serum HBV tests revealed no HBV reactivation. No relapse of BP or KS occurred and the liver enzyme levels were all normal in a 7-year follow-up period.

To date, the etiology of KS is unknown. The most important finding is HHV-8 [4]. HHV-8 DNA was detected in PBMCs of 18/230 (7.8%) normal controls in northeast China; HHV-8 IgG antibodies were positive in sera of 8/109 (7.3%) normal controls in the same region [5]. Although the seropositive rate of HHV-8 in the general population of our region is high, the incidence of KS in our region is low. To date, only 9 cases of KS associated with BP have been reported. HHV-8 DNA, proteins or antibodies were identified in three cases [1-3]. These reports may support the HHV-8 hypothesis. Interestingly, HHV-8 DNA and HHV-8 IgG antibodies were not detected in this case. This may imply that factors other than HHV-8 may account for the occurrence of KS in this case. An immuno-compromised status caused by corticosteroids, azathioprine, HBV infection and aging may be possible triggering factors for the KS in this BP patient.

Acknowledgements

References

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