Chronic disseminated cutaneous histoplasmosis in an immunocompetent individual - a case report

A 45-year-old woman from central India reported to clinic with multiple swellings on the face and neck. She had red patches on her forearms and trunk, but there was a predominance of lesions on the face and neck. On examination, her face showed multiple, succulent, erythematous plaques which were mildly pruritic ( Fig. 1). There was no discharge. There were also some scattered erythematous papules and nodules on the face ( Fig. 1). Examination of the neck revealed multiple erythematous plaques, many of them with a linear orientation and central ulceration and crusting ( Fig. 2). The upper extremities showed multiple erythematous plaques, most of which were ulcerated ( Fig. 3). Plaques without ulceration had been present for the past 2 years. The patient had been treated in various centers around her village and in Baroda as a case of reactional leprosy. The patient was apparently in good health and was a farmer living in a rural area. She had no other complaints. She was afebrile. There was no anesthesia. No nerves were palpable. There were no palpable lymph nodes. Her spleen and liver were not palpable. The mucosae appeared normal, but she complained of recurrent superficial ulceration, which resolved spontaneously. There was no cough. There were no joint pains. She had a steady weight. Her vital signs were normal. Routine investigations revealed a hemoglobin of 10 g%, an erythrocyte sedimentation rate (ESR) of 30 mm/h, and mildly elevated aspartate aminotransferase (AST) and alanine aminotransferase (ALT). X-Ray of the chest showed mild widening of the superior mediastinum with a right paratracheal lobular shadow, signifying right paratracheal lymphadenopathy. Ultrasonography (USG) showed mild splenomegaly with enlarged pre- and para-aortic nodes. Computed tomography (CT) scan of the chest and abdomen revealed paratracheal, mediastinal, and pre- and para-aortic lymphadenopathy, mild splenomegaly, and a horseshoe kidney. There was nothing suggestive of any overt immune suppression, namely leukemia, lymphoma, cancer, human immunodeficiency virus (HIV), or any collagen vascular disorders. Her CD4 count was normal at 581/mu L ( range, 290 - 2600/mu L). Diabetes, which could have been the cause of the immune suppression, was found only later during the course of treatment and is now under control with oral hypoglycemic agents. Her renal profile was normal. She had no central nervous system (CNS) signs or symptoms. Skin biopsy was performed from two plaques. It showed a granulomatous infiltrate in the dermis with numerous parasitized macrophages. The macrophages contained small round to ovoid yeast-like organisms with a surrounding clear halo ( Fig. 4). Lymphocytes, plasma cells, etc. were very few in number. The picture was essentially dominated by parasitized macrophages. The overlying epidermis was unremarkable. A diagnosis of histoplasmosis was made. Additional stains, including periodic acid - Schiff ( PAS) and silver methanamine, were found to be positive at an advanced dermatology department in New Delhi, substantiating the diagnosis of cutaneous histoplasmosis. Whilst awaiting the confirmation of the diagnosis, the patient was empirically given itraconazole 200 mg twice daily, and she has now been on the drug for the past 6 months. On her first follow-up, her lesions were slightly flattened, but distinctly scaly, unlike the initial appearance. She reported subjective improvement. Fifteen days later the patient reported with severe erythema and a flare up of the plaques and nodules on the face and neck. All the plaques were very scaly. The lesions on her arms were ulcerated. Hypersensitivity to destruction of the organisms was suspected and she was given 40 mg of triamcinolone acetonide intramuscularly. She telephoned in to say that she was much better on the 10th day. Six weeks after the telephone call she was seen again and her lesions showed remarkable improvement. All the lesions had dried up and the plaques and nodules had flattened dramatically ( Figs 5 and 6). There was no scaling. The erythema was markedly reduced. Her lesions had almost disappeared at the third monthly follow-up ( Figs 5 and 6). The patient was feeling well. The patient continues on itraconazole, now at a dose of 100 mg twice daily. Her laboratory parameters are normal. Her hemoglobin has improved to 12 g%. Her ALT and AST values remain unchanged.