Mucocutaneous histoplasmosis in HIV with an atypical ecthyma like presentation
Published Web Locationhttps://doi.org/10.5070/D30r47h950
Mucocutaneous histoplasmosis in HIV with an atypical ecthyma like presentationFrom the Dept of Skin & STD, Kasturba Medical College, Manipal, Karnataka, India. email@example.com
Vandana Mehta, Abhishek De, C Balachandran, Puja Monga
Dermatology Online Journal 15 (4): 10
Pulmonary and disseminated forms of histoplasmosis are very common in AIDS, but primary cutaneous histoplasmosis is rare. We report a case of primary mucocutaneous histoplasmosis in the setting of HIV.
Histoplasmosis, also known as Darling disease, is caused by a dimorphic saprophytic fungus Histoplasma capsulatum, commonly found in the soil contaminated with bird or bat droppings. It has two variants viz. Histoplasma capsulatum var. capsulatum and Histoplasma capsulatum var. duboisii. Primary infection occurs through inhalation of spores, causing a self-limiting flu-like illness in immunocompetent hosts. However, in immunosuppressed individuals, H.capsulatum can produce a disseminated disease affecting other organ systems such as the liver, spleen, bone marrow, lymphoreticular system, gastrointestinal tract, and less commonly, the skin .
A 43-year-old male agriculturist, HIV positive for about 8 years and on HAART therapy, presented with multiple irregular, painful, and hemorrhagic ulcers on the upper and lower extremities of 2 months duration (Fig. 1). He attributed the lesions to trauma that occurred while working in the fields. There was no history of fever, cough, dyspnea, abdominal pain, or dysphagia, but the patient reported nasal regurgitation while taking liquids. His past medical history was eventful for having received treatment for disseminated tuberculosis and cryptococcal meningitis; he was on maintanance treatment with oral fluconazole 200 mg. On examination he was found to be anemic and his ESR was elevated. There was a mild elevation of liver enzymes, however the renal parameters, chest X-ray, and abdominal ultrasound were normal. Initially, bacterial infection was assumed and we started our patient on oral and intravenous antibiotics over a period of 2 months. First, augmentin was given intravenously for two weeks, after which he was switched to oral augmentin. Once a lack of substantial benefit was noted, the combination of cefoperazone injection along with sulbactum was also tried for about 2 weeks. The lesions failed to heal and in addition to the cutaneous ulcers, he developed crusted plaques on his ears, forehead, and the philtrum of the upper lip and nose (Fig. 2). There was near complete destruction of the nasal architecture with oozing of blood and serosanguineous fluid. His oral cavity showed a 1 x 1 cm palatal perforation. At this visit his HIV viral load was 716976 copies and CD4 count was 15.
|Figure 2||Figure 3|
A biopsy for histopathology from the forearm revealed a diffuse lymphocytic infiltrate in the dermis, coupled with a striking number of yeast forms that demonstrated narrow neck buds (Fig. 3). PAS stain confirmed the presence of spores consistent with Histoplasma capsulatum. Other conditions demonstrating parasitized histiocytes such as leishmaniasis, cryptococcosis, donovanosis, and rhinoscleroma were excluded based on the presence of a negative PAS. A bone marrow aspiration and biopsy performed subsequently to rule out reticuloendothelial involvement with histoplasma was negative. We started our patient on oral itraconazole, 400 mg daily. HAART (Lamivudine, stavudine, efavirenz) was continued and all the skin lesions healed after one month, with scarring and depigmentation.
The infection resolved, but unfortunately, our patient had destruction of his nasal cartilage and the nasal pyramid was lost with a part of the left nasal ala. There was also scarring alopecia involving a part of his moustache area along with a palatal perforation (Figs. 4, 5, 6).
|Figure 5||Figure 6|
Histoplasmosis is caused by either Histoplasma capsulatum var. capsulatum, which is endemic in the central and eastern USA and South America, or Histoplasma capsulatum var. duboisii, which is restricted to the African subcontinent. The African form differs from the American form in its varied clinical presentation. In the African form cutaneous and osseous lesions predominate, whereas in the American form pulmonary changes dominate the clinical picture. The infection is acquired by inhalation of conidia from soil contaminated by bird or bat excreta. Three forms of histoplasmosis exist: acute pulmonary, chronic cavitary, and disseminated forms. Whether the HIV-associated disseminated histoplasmosis is a manifestation of progressive primary infection or whether it represents reactivation of a latent focus of infection is not known .
Disseminated histoplasmosis (DH) is a relatively common manifestation of AIDS, occurring in 10 percent of patients living in endemic areas; it may be the first clinical expression of a patient's HIV infection . A myriad of clinical lesions have been described such as erythematous macules, papules, nodules, and pustules. Herpetic, acneiform, erythema multiforme-like, molluscum contagiosum-like, vasculitic, and exfoliative forms have been reported. Cutaneous histoplasmosis frequently localizes to the face, chest, arms and mucosal involvement is known to occur in 40 percent cases .
The histopathological findings in AIDS-associated DH depend on the degree of immunosuppression. In patients with higher CD4 cell counts, there is usually a granulomatous inflammation with necrotizing and non-necrotizing tuberculoid-type granulomas that demonstrate a small number of intrahistiocytic microorganisms. In patients with lower CD4 cell counts, like our index case, there is usually a diffuse dermal histiocytic infiltration with heavily parasitized epitheloid histiocytes. Rarely leukocytoclastic vasculitis, or collagen necrosis with prominent extracellular microorganisms may occur .
Histoplasmosis is sporadically reported from India, perhaps owing to its varied clinical presentation. Panja and Sen first reported histoplasmosis from India in 1959 . Hsitoplasma capsulatum is considered to be endemic in certain north Indian states like West Bengal, where the prevalence of skin positivity to histoplasmin antigen was determined to be 9.4 percent . Our patient presented with cutaneous lesions resembling bacterial infection on the face and extremities. A trial with intravenous and oral antibiotics for about 2 months (Amoxicillin with clavulinic acid, cefadroxil, levofloxacin, cotrimoxazole) failed to produce resolution and prompted us to undertake a biopsy. Oral involvement in histoplasmosis may occur as hyperplastic gingivitis and oral ulceration [8, 9]. Naso-sinus involvement is rare in disseminated histoplasmosis is rare . However, a recent report documented oral histoplasmosis as the presenting sign of disseminated histoplasmosis and the initial suggestion of HIV infection . In that patient destruction of the hard palate was found along with significant involvement of the right maxillary sinus and right nasal cavity. In addition to the cutaneous ulcers, our patient also had palatal perforation and nasal cartilage destruction. Furthermore, our patient attributed his lesions to trauma. He had no suggestive pulmonary shadows on chest X-ray, however a remote possibility of his having an earlier self-limited pulmonary histoplasmosis infection prior to the mucocutaneous presentation cannot be excluded. A recent report of 23 patients with AIDs and disseminated histoplasmosis found pulmonary involvement in 65 percent . Our case developed cutaneous disseminated histoplasmosis while already on oral fluconazole 200 mg. Therefore, treatment was initiated with itraconazole 400 mg daily; all the lesions healed with depigmentation and scarring in 1 month's time. .
To conclude, DH in HIV may present with many clinical manifestations. Skin biopsy and culture is therefore of pivotal importance in the rapid and reliable confirmation of the clinical diagnosis and timely institution of appropriate therapy.
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