Dermatology Online Journal

Primary cutaneous nocardiosis with craniocerebral extension: A case report
Sharmila P Patil MD DDV¹, Manjyot M Gautam MD², Avani A Sodha MD³, Kaleem J Khan MD³
Dermatology Online Journal 15 (6): 8

1. Head of Department of Dermatology. drsharmilapatil@gmail.com
2. Lecturer
3. Resident
Department of Dermatology, D.Y.Patil Hospital and Research Centre, Navi Mumbai, India

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Abstract

A 26-year-old male, a fisherman by occupation, presented with boggy swelling of the scalp and multiple discharging sinuses over the fronto-parietal area for one year. He described a history of periodic trauma to the scalp beginning about one year prior to the onset of the scalp condition. Histopathology suggested a chronic granulomatous suppuration. Magnetic resonance imaging showed intracranial and intra-orbital involvement. Tissue cultures of the sample identified Nocardia brasilienses. The patient was successfully treated with a modified Welsh regimen, given for eight months.

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Introduction

Nocardial infection may be difficult to diagnose. The clinical manifestations, the site of involvement, and the severity are extremely variable [1]. The manifestations are mostly determined by certain factors such as the immune status of the patient and the route of infection. Although the lungs are the primary organs affected by nocardia species, cutaneous nocardiosis also occurs as a primary disease that is more often seen in apparently immunocompetent individuals [2, 3].

The authors report an interesting patient with primary cutaneous nocardiosis involving the scalp. He presented with multiple discharging sinuses, exopthalmos, and headache because of intraorbital and intracranial invasion by Nocardia brasilienses.

Case report

A 28-year-old male presented with a one-year history of multiple scalp nodules that discharged purulent material. He complained of fever and headache with blurred vision present for three months. There was a history of the discharge of white-colored particles from the sinuses. The patient had no other systemic complaints.

A fisherman by occupation, he described often being pricked or pierced by wooden twigs that he regularly carried on his head. Over the past year he had developed multiple abscesses over his scalp; individual nodules had temporarily regressed with incision and drainage of pus or total excision.

Cutaneous examination revealed multiple healed and discharging sinuses over the fronto-parietal area of the scalp. The discharge was serosanguinous with no evidence of grains. On ophthalmological examination the patient had diplopia, with left corneal reflex displaced. The right eye showed apparent enophthalmos. No motor or sensory deficit was noted.

Figure 1	Figure 2
Figure 1. Multiple discharging sinuses and old healed scars over the scalp Figure 2. Grains of clumped organisms surrounded by inflammatory infiltrate

The patient had a hemoglobin of 11 gm and ESR 140 mm/hr. All other serological and hematological parameters including ELISA for HIV were negative. A Mantoux test was within normal limits.

A skin biopsy showed normal epidermis. The dermis showed a lymphocytic infiltrate around clumps of bacilli-forming grains in the center suggestive of Actinomycetoma.

Figure 3	Figure 4
Figures 3 & 4. LJ Medium showing powdery yellow colonies. Gram positive branching filamentous bacteria are noted.

The tissue was sent for culture on Lowenstein Jensen medium. The dry, wrinkled yellow colonies, which showed gram positive branching filamentous bacilli on smears, were consistent with Nocardia brasilienses.

Figure 5	Figure 6
Figures 5 & 6. Moth-eaten appearance of the frontal bone with soft tissue mass in the orbital roof- proptosis of the left eye is noted

Magnetic resonance imaging of the scalp showed moth-eaten appearance of the frontal bone with dual enhancement, with a soft tissue mass in the left eyelid area. The orbital roof was also involved which was causing proptoses of the left eye.

A final diagnosis of primary cutaneous nocardiosis of the scalp with intracranial and intraorbital involvement was made.

Figure 7
Figure 7. Complete healing of sinuses with scarring after six months of therapy

Because of the dangerous ocular extension, the patient began intensive therapy with two drugs. First an intensive phase was administered consisting of daily injections of Streptomycin (0.75gm) IM and oral trimethoprim-sulphamethoxazole (DS) 800 mg - 1600 mg twice daily for three months. On follow-up the patient responded with a decrease in discharge and a cessation of new lesion formation. The patient was then maintained with oral rifampicin 600 mg and trimethoprim-sulphamethoxazole (DS) 800 mg - 1600 mg twice daily for the next five months. Complete resolution of the nodules and sinuses with scarring was noted at the end of this therapy. In more usual cutaneous cases, trimethoprim-sulphamethoxazole alone is recommended.

Discussion

Nocardiosis is an acute to chronic suppurative disease caused by the aerobic gram positive filamentous bacteria, Nocardia. Primary infection is usually pulmonary, but hematogenous spread to other organs may occur. The incidence of Nocardial mycetoma in Indian reports varies from 5.2 percent to 35 percent [4, 5].

The infective process is initiated by local trauma, such as a puncture wound by a splinter or a thorn [2, 6, 7, 8, 9]. The saprophytic nocardia species present on these materials are implanted into the puncture wound and initiate the infection [8, 10, 11]. The usual sites of involvement are hands, feet, back, shoulder, and scalp, which correspond to the usual sites contacted in carrying loads during agricultural work [12]. Our patient had a history of repeated trauma to the scalp by wooden twigs, which he would regularly carry on his head.

Cutaneous nocardiosis presents as a primary cutaneous infection or as a secondary disease. The clinical presentations described are mycetoma, lymphocutaneous, superficial acute, and secondary cutaneous infection with disseminated disease [8, 13]. Mycetoma is the most common mode of presentation. Among the several species of nocardia causing cutaneous infections N. brasilienses is the most common species isolated, as was noted in our case [8, 13, 14, 15, 16].

Primary cutaneous infection caused by N. brasilienses is more inflammatory, locally invasive, and progressive than the self-limited course of the lesions caused by N. asteroides [17]. Mycetoma caused by N. brasilienses has a propensity to involve the bone and osteolytic changes are frequently observed radiologically. Compressive myelopathy has been reported as a sequel to the bone involvement underlying a mycetoma caused by N. brasilienses [18]. Our patient showed osteolytic changes on X-ray of the skull. CT scan and MRI showed a moth-eaten appearance of the bone with dural enhancement and a space-occupying lesion in the left eyelid area that had caused proptosis of the same eye. In our detailed survey we have been unable to find other reports of intra-orbital involvement by Nocardia species.

Primary cutaneous nocardiosis remains a diagnostic challenge. Demonstration of the organism from clinical specimens such as granules, pus, or aspirated fluid from a nodule using Gram, Ziehl-Neelsen, and modified Kinyoun stains is the main way of making the diagnosis. Gram positive and acid fast, thin branching filaments are the characteristic appearance of the organism. Identification by culture is a tedious process because the organism is slow-growing and can take up to 2-3 weeks for isolation from a clinical specimen [19]. Western blot assay, using monoclonal antibodies against 54-kDa circulating antigens of nocardia, species-specific DNA probes, and ELISA help in the rapid and definitive diagnosis of nocardiosis [17]. Radiological examination of the underlying bone or joint should be done in all cases of mycetoma to rule out bony involvement.

Patients with primary cutaneous nocardiosis respond well to medical therapy. Trimethoprim-sulphamethoxazole is the mainstay of the therapy [20]. Other effective drugs reported include dapsone, amikacin, amoxycillin, clindamycin, ciprofloxacin, and imepenem [17]. The drugs should be continued for an additional three months after clearance to prevent recurrence. The treatment given to this patient is a modified variant of the Welsh regimen, which ensures good compliance and completion of therapy. Our patient responded to this therapy in the form of complete clearance with scarring within 5 months of initiation of therapy. The treatment was continued for 3 more months to ensure no recurrence.

This case is being reported for the rare manifestation of primary cutaneous nocardiosis with intraorbital and intracranial involvement. The incidence of primary cutaneous nocardiosis was thought to be relatively uncommon, with case reports appearing sporadically. This case re-emphasizes the need to consider the actinomycetes group as well as tuberculosis when a patient presents with an intracranial mass with infection in different planes and an overlying discharging sinus. Appropriate cultures and stains are required to reveal the true nature of the organism and ensure that appropriate therapy is instituted.

Acknowlegments: The authors would like to acknowledge Dr. Manish Gautam and Dr. Miskeen for their assistance.

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