Skin rash in meningitis and meningoencephalitis

Abstract

Skin rash and depigmentation are common in patients with meningitis and meningoencephalitis. Skin changes must always be evaluated in conjunction with the clinical symptoms, signs, brain imaging, and laboratory abnormalities, particularly the features of the CSF pleocytosis. The purpose of this montage is to help the clinician identify a specific etiologic agent as early as possible.

Skin rash and depigmentation are common in patients with meningitis and meningoencephalitis. Skin changes must always be evaluated in conjunction with the clinical symptoms, signs, brain imaging, and laboratory abnormalities, particularly the features of the CSF pleocytosis. The purpose of this montage is to help the clinician identify a specific etiologic agent as early as possible.

The distribution of rash can provide useful information. Rash in the extremities is associated with 3 meningitides that should not be missed. The classic rash of Rocky Mountain spotted fever usually begins as erythematous macules on the wrists, hands, and feet (figure 1, A and B), which quickly spread to the trunk and face. It is petechial at times. Meningococcal rash is similar, an erythematous macular and petechial rash on the legs that quickly disseminates, becoming purpuric and even necrotic if meningococcemia remains untreated (figure 1C). Gangrene leading to limb loss can follow purpura fulminans. Morbidity and mortality are significant when a patient presents with meningitis and a predominantly peripheral rash. The maculopapular rash of secondary or tertiary syphilis is seen mostly on the palms of the hands and soles of the feet and is scaly without much erythema (figure 1D).

Figure 1. Rash on the extremities.

A macular or petechial rash with or without erythema is seen predominantly on the extremities and can become widespread in Rocky Mountain spotted fever (A and B), meningococcal meningitis (C), or syphilis (D).

Important differential diagnoses for recurrent meningitis and meningoencephalitis include herpes simplex virus (HSV) and varicella-zoster virus (VZV) reactivation, Behçet disease, and the Vogt-Koyanagi-Harada syndrome (uveomeningoencephalitis). The skin lesions in both HSV and VZV reactivation are erythematous and vesicular, but HSV lesions cluster in patches (figure 2A), whereas VZV produces lesions in a dermatomal distribution (figure 2B). HSV lesions occur primarily on mucosal surfaces of the mouth (mostly HSV-1) or genitalia (mostly HSV-2) and frequently occur on the trunk (mostly HSV-2) in patients with a history of meningitis (often recurrent) or intermittent radiculopathy. Herpetic mucosal and skin lesions are not usually seen with HSV-1 encephalitis. The lesions in Behçet disease can be confused with those produced by HSV and VZV, but are usually aphthous and less vesicular (figure 2, C and D). In the Vogt-Koyanagi-Harada syndrome (uveomeningoencephalitis), lesions are typically characterized by loss of pigment (vitiligo) of skin, hair, and mucous membranes (figure 3).

Figure 2. Vesicular and ulcerative rash on the skin and mucosa.

The vesicular rash of herpes simplex virus reactivation (A) is patchy and that of varicella-zoster virus reactivation (B) is dermatomal. Aphthous ulcers are characteristic of Behçet disease (C and D).

Figure 3. Depigmentation.

Note lightening of eyebrows and eyelashes in a Hispanic man with Vogt-Koyanagi-Harada syndrome.

A number of aseptic meningitides present with similar rashes. The erythematous macular or maculopapular rashes produced by West Nile virus (figure 4A) and enteroviruses such as Coxsackie or ECHO virus (figure 4B) are similar to the lacy-appearing rash seen with Epstein-Barr virus (figure 4C) infection. Identifying the infectious agent in these 3 aseptic meningitides is helpful but not crucial because treatment is supportive.

Figure 4. Maculopapular rash, primarily on the trunk.

Erythematous macular or maculopapular rashes on the trunk and extremities are seen in infections with West Nile virus (A), enteroviruses (B), and Epstein-Barr virus (C).

The skin rash in Lyme disease has a characteristic “target” appearance (figure 5). Chronic rather than acute meningitis is the usual presentation. Peripheral facial palsy, often bilateral, is common.

Figure 5. Erythema migrans.

Lyme disease causes “target” lesions.

The table provides additional information to help the clinical neurologist identify the causative agent in the various meningoencephalitides.

Table.

Skin rash in meningitis and meningoencephalitis

AUTHOR CONTRIBUTIONS

All authors contributed to the study concept and design, data acquisition, analysis and interpretation of data, drafting of the manuscript, critical revision of the manuscript for important intellectual content, and study supervision.

STUDY FUNDING

No targeted funding reported.

DISCLOSURE

The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.