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Zygomycosis is a rare opportunistic fungal infection, that often complicates patients with uncontrolled Diabetes Mellitus, primary and acquired immunodeficiencies as defects of the cell-mediated immunity, myelodisplastic syndrome, hematological malignancies undergoing chemotherapy , HIV and long term therapy with steroid [1]. Mortality rate of these patient is very high, until 85% [2].

The causative organism is an aerobic saprophytic fungus, releasing spores, belonging to the order of Mucorales of the class Zygomycetes [3].  Inhalation and direct contamination of skin lesions are the major causes of infection, so the sporangiospores can migrate up to the  lungs, nasal cavity and paranasal sinuses, gut and cutaneous tissues causing primary infection, hyphae invade vassels producing thrombi. Rhinocerebral Zygomycosis occurs with cranial nerve palsies, eye proptosis, pain and often blindness. Zygomicetes can invade central nervous system (CNS) from paranasal sinuses or remote site of infecton [1].

This life-threatening infection could be defeat through early detection, surgical excision and appropriate debridement, aggressive antifungal therapy, and control of risk factors, like diabetes mellitus like in our cases.

We reported the follow up after long term therapy with Posaconazole in two Italian girls with Diabetes Mellitus Type 1 previously published [1,4].