Fulminant cranio-cerebral invasive aspergillosis in a patient with phytophotodermatitis

Cranio-cerebral invasive aspergillosis of sino-nasal involving the skull base is rare and associated with a very high mortality due to its peculiarly fulminant clinical course. We present an unusual case of 55-year-old diabetic female who initially treated for phytophotodermatitis, but later on she developed massive invasive cerebral aspergillosis. Advances in diagnosis and management of aspergillosis with improved and early detection and more effective therapy have promised some success in the management of these devastating infections.

Keywords: Aspergillosis, fungus, phytophotodermatitis, skull base

Cranio-cerebral invasive aspergillosis of sino-nasal involving the skull base is rare and associated with a very high mortality due to its peculiarly fulminant clinical course. ^{[1],[2],[3],[4]} We present an unusual case of a diabetic female who initially treated for phytophotodermatitis, but later on she developed massive invasive cerebral aspergillosis.

A 55-year-old female managed for erythematous, vesicular, pruritic, and bullous skin lesions on her scalp, face, upper chest, abdomen, and extremities, which were developed over 24 h [Figure 1]a-d]. With a diagnosis of phytophotodermatitis, she received steroid and antihistaminics at a peripheral hospital. She was a known diabetic. She was apparently alright for next 7 days that she noticed rapid deterioration of vision in both the eyes, and when she presented to us, she was completely blind. Neurological examination was suggestive of bilateral cortical blindness. Fundus was normal. Higher mental functions, motor, and sensory examination was normal. A magnetic resonance imaging of the brain showed extensive infarction of both the occipital lobes, and diffuse signal changes in the clivus and occipital bone and extensive lesions involving basal cisterns and encasing the major vessel [Figure 2]a-c]. There was diffuse enhancement of the blood vessels [Figure 3]. Magnetic resonance venogram showed diffuse narrowing of the major vessels that resulted in the diffuse cerebral infarcts [Figure 4]. A diagnosis of fungal infection was suspected. Biopsy from the sphenoid sinus was negative however the tests for immunoglobulin E specific for Aspergillus was positive. The patient was started on antifungal therapy (amphotericin B and metronidazole) without any improvement in the clinical condition.

Phytophotodermatitis is a phototoxic reaction characterized by skin eruption that results from the interaction of radiant energy from the sun (ultraviolet-A radiation-wavelengths of 320-400 nm) with photosensitizing compounds found in many plant varieties, including several isomers of psoralens (furocoumarins). ^[5],[6] Phytophotodermatitis is a clinical diagnosis and typical lesions with unusual configurations suggest the diagnosis. ^[6] The acute phase of phytophotodermatitis is characterized by erythematous plaques, vesicles, and bullae, and over a period the lesions may become erythematous or appear as hyperpigmented patches. The skin eruptions may appear hours to days after exposure, and the exposed parts of the body are, usually, involved. ^[6] There is no specific treatment for this reaction however in severe cases, analgesics, antihistamines for itching, wet compresses and topical steroids may be used. Blistered areas need to be kept clean to prevent secondary infection. The hyperpigmentation fades over time as it does in sunburned skin. ^[5],[6] As the patient was a farmer she would have been exposed to furocoumarins as well as fungus through handling of plants while working in the fields and also in present case administration of steroids in a known diabetic resulted in further immnocompromised state and infection with Aspergillus. The temperate climate of our country and constant exposure to the high spore content of pathogenic Aspergillus species in the “mouldy” work environment ^[7] posed a high risk to the patient. Cerebral infarction associated with skull base aspergillosis can mimic like any other causes of cerebrovascular disease. ^[2],[3],[4] Vascular invasion in cerebral aspergillosis can lead to thrombosis, infarction, hemorrhage, or mycotic aneurysms. ^[8],[9] In its severe form, the hyphal elements of the organisms can occlude the veins (frequently in basal ganglia, thalamus, corpus callosum, and cortical or subcortical localizations) ^{[9],[10],[11]} or lenticulostriate and thalamoperforating arteries ^[12] leading to infarction. Imaging is helpful to suspect a diagnosis of aspergillosis. ^[1] As in present case detection of the fungus may not be possible in biopsy; however, the diagnosis can be suspected based on serology. ^[2] Radical surgical excision followed by aggressive antifungal chemotherapy is the mainstay of treatment for invasive aspergillosis. ^[3] However, once the intracranial invasion of infection occurs; it is difficult to control with conventional antifungal (possibly due to difficulties in the transport of antifungal agents through the hyphal element into the brain tissues) and surgical therapies and the mortality is very high. ^[13] Advances in diagnosis and management of aspergillosis with improved and early detection and more effective therapy have promised some success in the management of these devastating infections. ^[14],[15]

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1755-6783.150113

[Figure 1], [Figure 2], [Figure 3], [Figure 4]