Cutaneous Colesional Acquired Immunodeficiency Syndrome Associated Kaposi Sarcoma and Cryptococcosis

Abstract

The clinicopathologic features of 4 AIDS patients with cutaneous colesional Kaposi sarcoma (KS) and cryptococcosis, a rare phenomenon, are described. Biopsies from 3 patients who were highly active antiretroviral therapy (HAART)-naive demonstrated predominant KS with a conspicuous spindle cell component and small aggregates of cryptococcal yeasts in 2 biopsies and predominant gelatinous cryptococcosis with attenuated KS spindle cells in 1 biopsy. One patient was HAART exposed. He had childhood pulmonary tuberculosis, was treated for disseminated cutaneous cryptococcosis 18 months earlier and presented with cutaneous lesions, odynophagia and massive cervical lymphadenopathy in the eighth week of HAART, after achieving viral suppression and a CD4 cell increase from 28 to 184 cells/μL. His skin biopsy demonstrated a dense lymphoplasmacytic infiltrate, neutrophils, and granulomas with admixed aggregates and single Cryptococcus neoformans and focal aggregation of human herpes virus 8-immunopositive spindle cells. Acid fast bacilli were not identified and mycobacterial molecular studies were negative. The features were compatible with cutaneous cryptococcal immune reconstitution inflammatory syndrome. His nodal and oropharyngeal biopsies demonstrated dense mixed, including granulomatous, inflammation with few cryptococcal yeasts and acid fast bacilli, confirmed to be Mycobacterium tuberculosis on polymerase chain reaction testing, without KS. These features were also compatible with immune reconstitution inflammatory syndrome, but the exact role of each infection in the extracutaneous sites was unconfirmed. Colesional KS and cryptococcosis served as the sentinel lesion of AIDS in 3 patients and of immune reconstitution inflammatory syndrome in 1 patient.

Microscopic diversity in oral Kaposi sarcoma

“…28 The occurrence of co-existent infections, dermatoses and neoplasms has been described in cutaneous KS and to a lesser extent in OKS. [28][29][30][31][32][33] Twenty-five cases in this series showed co-existent pathology, most commonly in the form of superficially invasive candidiasis. This is not unexpected, considering oral candidiasis represents the most frequent intra-oral manifestation of HIV, regardless of stage.…”

Recognition of Dual or Multiple Pathology in Skin Biopsies from Patients with HIV/AIDS

“…The detection of tuberculous granulomas or Cryptococcus organisms in KS has similar implications [3, 4, 36]. Although the patients whose biopsies are illustrated herein were largely HAART-naïve, awareness that colesional pathology may be a rare manifestation of IRIS is appropriate in the era of HAART.…”

“…Failure to recognise the opportunistic pathogen, whose presence is often masked by the more obvious background spindle cell proliferation, could have grave prognostic implications. To date there have been only nine recorded cases of AIDS-associated KS with colesional cryptococcosis, one of whom had oral lesions exclusively [4, 23–27]. The author has encountered three additional examples of KS with incidental Cryptococcus neoformans infection, including the case illustrated in Figure 7.…”

“…In this context, H. capsulatum yeasts should be distinguished from infection with capsule deficient forms of C. neoformans occurring in association with KS [3, 4]. There are two documented cases of KS with apparent intralesional Candida organisms.…”

“…Consequently, any given skin biopsy from an individual with cutaneous lesions in the presence of underlying HIV/AIDS could potentially reveal more than one pathological process. In severely immunosuppressed patients who have not received highly active antiretroviral therapy (HAART), subtle features of a coincidental potentially lethal opportunistic pathogen (e.g., cryptococcosis) can easily be overlooked in a specimen predominated by the primary pathological lesion (e.g., Kaposi sarcoma (KS)) [1, 3, 4]. Although dual or multiple pathological processes are seldom encountered in single skin biopsies in everyday practise, it is always wise to maintain a high index of suspicion when examining specimens from HIV/AIDS patients.…”