Emiliano Grillo, MD

A 47-year-old man had had a chronic itch on his back for 2 years. He had no history of trauma to the site, nor did he recall applying topical products to that area.

He was otherwise healthy. He worked as an electrician and said he occasionally experienced cervical and back pain while working.

An examination revealed two grayish-brown ovoid patches on the upper back, each 5 cm to 7 cm in diameter (Figure 1).

DIAGNOSIS: NOTALGIA PARESTHETICA

Chronic, brown-gray, itching patches on the back in an adult patient are characteristic of notalgia paresthetica.

Conditions that may be included in the differential diagnosis but that do not match the presentation in this patient include the following:

• Cutaneous sarcoidosis, which may exhibit several morphologies, but itching would be unusual
• Chronic discoid lupus erythematosus, characterized by scarring and atrophic plaques, but mainly on the face and scalp
• Contact dermatitis, an itchy eczematous condition, characterized by scaly erythematous plaques
• Lichen amyloidosis, a variant of cutaneous amyloidosis characterized by the deposition of amyloid or amyloid-like proteins in the dermis, resulting in red-brown hyperkeratotic lichenoid papules, usually on the pretibial surfaces.

CAUSES AND MANAGEMENT

Notalgia paresthetica is a neuropathic syndrome of the skin of the middle of the back characterized by localized pruritus.^1–3 Although common, it often goes undiagnosed.^1,3,4 It tends to be chronic, with periodic remissions and exacerbations.

Notalgia paresthetica is thought to be a sensory neuropathy and may result from compression of the posterior rami of spinal nerve segments T2 to T6. Slight degenerative changes are often but not always observed, and their clinical significance is uncertain.^1,2,4 The condition affects people of all races and both sexes, usually adults ages 40 to 80.

Clinically, it presents as localized pruritus on the back, usually within the dermatomes T2 to T6.⁵ Examination reveals a hyperpigmented patch, sometimes with excoriations.⁵

Diagnosis is based on clinical findings. Laboratory tests are not useful. Imaging is not needed, but magnetic resonance imaging and evaluation by an orthopedic surgeon are appropriate when there is chronic focal pain. Skin biopsy is usually not necessary, although it may be useful in some patients to exclude other conditions. When biopsy is done, macular amyloidosis or postinflammatory hyperpigmentation is seen.

Treatment is difficult. Topical steroids and oral antihistamines are usually ineffective,⁵ but topical capsaicin may provide temporary relief.³ The most recommended treatment in patients with notalgia paresthetica and underlying spinal disease is evaluation and conservative management of the spinal disease, including progressive exercise and rehabilitation.² Other therapies include oxcarbazepine, gabapentin, transcutaneous electrical nerve stimulation, phototherapy,⁶ and botulinum toxin injection.

TREATMENT OF OUR PATIENT

In our patient, an orthopedic evaluation revealed cervicothoracic scoliosis. He underwent 6 months of conservative treatment under the care of his family physician and a dermatologist. Treatment consisted of exercise and rehabilitation for his scoliosis, and daily application of topical mometasone. The pain and itch gradual improved.

A 47-year-old man had had a chronic itch on his back for 2 years. He had no history of trauma to the site, nor did he recall applying topical products to that area.

He was otherwise healthy. He worked as an electrician and said he occasionally experienced cervical and back pain while working.

An examination revealed two grayish-brown ovoid patches on the upper back, each 5 cm to 7 cm in diameter (Figure 1).

DIAGNOSIS: NOTALGIA PARESTHETICA

Chronic, brown-gray, itching patches on the back in an adult patient are characteristic of notalgia paresthetica.

Conditions that may be included in the differential diagnosis but that do not match the presentation in this patient include the following:

• Cutaneous sarcoidosis, which may exhibit several morphologies, but itching would be unusual
• Chronic discoid lupus erythematosus, characterized by scarring and atrophic plaques, but mainly on the face and scalp
• Contact dermatitis, an itchy eczematous condition, characterized by scaly erythematous plaques
• Lichen amyloidosis, a variant of cutaneous amyloidosis characterized by the deposition of amyloid or amyloid-like proteins in the dermis, resulting in red-brown hyperkeratotic lichenoid papules, usually on the pretibial surfaces.

CAUSES AND MANAGEMENT

Notalgia paresthetica is a neuropathic syndrome of the skin of the middle of the back characterized by localized pruritus.^1–3 Although common, it often goes undiagnosed.^1,3,4 It tends to be chronic, with periodic remissions and exacerbations.

Notalgia paresthetica is thought to be a sensory neuropathy and may result from compression of the posterior rami of spinal nerve segments T2 to T6. Slight degenerative changes are often but not always observed, and their clinical significance is uncertain.^1,2,4 The condition affects people of all races and both sexes, usually adults ages 40 to 80.

Clinically, it presents as localized pruritus on the back, usually within the dermatomes T2 to T6.⁵ Examination reveals a hyperpigmented patch, sometimes with excoriations.⁵

Diagnosis is based on clinical findings. Laboratory tests are not useful. Imaging is not needed, but magnetic resonance imaging and evaluation by an orthopedic surgeon are appropriate when there is chronic focal pain. Skin biopsy is usually not necessary, although it may be useful in some patients to exclude other conditions. When biopsy is done, macular amyloidosis or postinflammatory hyperpigmentation is seen.

Treatment is difficult. Topical steroids and oral antihistamines are usually ineffective,⁵ but topical capsaicin may provide temporary relief.³ The most recommended treatment in patients with notalgia paresthetica and underlying spinal disease is evaluation and conservative management of the spinal disease, including progressive exercise and rehabilitation.² Other therapies include oxcarbazepine, gabapentin, transcutaneous electrical nerve stimulation, phototherapy,⁶ and botulinum toxin injection.

TREATMENT OF OUR PATIENT

In our patient, an orthopedic evaluation revealed cervicothoracic scoliosis. He underwent 6 months of conservative treatment under the care of his family physician and a dermatologist. Treatment consisted of exercise and rehabilitation for his scoliosis, and daily application of topical mometasone. The pain and itch gradual improved.

A 47-year-old man had had a chronic itch on his back for 2 years. He had no history of trauma to the site, nor did he recall applying topical products to that area.

He was otherwise healthy. He worked as an electrician and said he occasionally experienced cervical and back pain while working.

An examination revealed two grayish-brown ovoid patches on the upper back, each 5 cm to 7 cm in diameter (Figure 1).

DIAGNOSIS: NOTALGIA PARESTHETICA

Chronic, brown-gray, itching patches on the back in an adult patient are characteristic of notalgia paresthetica.

Conditions that may be included in the differential diagnosis but that do not match the presentation in this patient include the following:

• Cutaneous sarcoidosis, which may exhibit several morphologies, but itching would be unusual
• Chronic discoid lupus erythematosus, characterized by scarring and atrophic plaques, but mainly on the face and scalp
• Contact dermatitis, an itchy eczematous condition, characterized by scaly erythematous plaques
• Lichen amyloidosis, a variant of cutaneous amyloidosis characterized by the deposition of amyloid or amyloid-like proteins in the dermis, resulting in red-brown hyperkeratotic lichenoid papules, usually on the pretibial surfaces.

CAUSES AND MANAGEMENT

Notalgia paresthetica is a neuropathic syndrome of the skin of the middle of the back characterized by localized pruritus.^1–3 Although common, it often goes undiagnosed.^1,3,4 It tends to be chronic, with periodic remissions and exacerbations.

Notalgia paresthetica is thought to be a sensory neuropathy and may result from compression of the posterior rami of spinal nerve segments T2 to T6. Slight degenerative changes are often but not always observed, and their clinical significance is uncertain.^1,2,4 The condition affects people of all races and both sexes, usually adults ages 40 to 80.

Clinically, it presents as localized pruritus on the back, usually within the dermatomes T2 to T6.⁵ Examination reveals a hyperpigmented patch, sometimes with excoriations.⁵

Diagnosis is based on clinical findings. Laboratory tests are not useful. Imaging is not needed, but magnetic resonance imaging and evaluation by an orthopedic surgeon are appropriate when there is chronic focal pain. Skin biopsy is usually not necessary, although it may be useful in some patients to exclude other conditions. When biopsy is done, macular amyloidosis or postinflammatory hyperpigmentation is seen.

Treatment is difficult. Topical steroids and oral antihistamines are usually ineffective,⁵ but topical capsaicin may provide temporary relief.³ The most recommended treatment in patients with notalgia paresthetica and underlying spinal disease is evaluation and conservative management of the spinal disease, including progressive exercise and rehabilitation.² Other therapies include oxcarbazepine, gabapentin, transcutaneous electrical nerve stimulation, phototherapy,⁶ and botulinum toxin injection.

TREATMENT OF OUR PATIENT

In our patient, an orthopedic evaluation revealed cervicothoracic scoliosis. He underwent 6 months of conservative treatment under the care of his family physician and a dermatologist. Treatment consisted of exercise and rehabilitation for his scoliosis, and daily application of topical mometasone. The pain and itch gradual improved.

A 54-year-old woman presented with a 7-day history of odynophagia, pharyngeal swelling, and painful skin lesions on her left ear. She had been on antiretroviral therapy for human immunodeficiency virus infection but had not been fully compliant with the treatment.

See editorial

On examination, she had painful erythematous vesicles and pustules on the left auricle and in the external auditory canal (Figure 1), as well as small vesicles and circumscribed erosions on the left anterior twothirds of her tongue (Figure 2) and left palate. Facial sensory function was normal; however, she had lagophthalmos, a flattened nasolabial fold, ptosis of the oral commissure, and a loss of the forehead wrinkles on the left side of her face—all signs of peripheral facial nerve paralysis.

Q: Which is the most likely diagnosis?

• Ramsay Hunt syndrome
• Herpes simplex
• Contact dermatitis
• Malignant external otitis
• Erysipelas

A: This patient had Ramsay Hunt syndrome, also known as herpes zoster oticus. It is a rare complication of herpes zoster in which the reactivation of latent varicella-zoster virus infection in the geniculate ganglion causes the triad of ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle. Taste perception, hearing (eg, tinnitus, hyperacusis), and lacrimation can be affected.¹

Ramsay Hunt syndrome is generally considered a polycranial neuropathy of cranial nerves VII (facial) and VIII (acoustic). In some cases other cranial neuropathies may be present and may involve cranial nerves V (trigeminal), IX (glossopharyngeal), and X (vagus). Vestibular disturbances such as vertigo are also often reported. It is more severe in patients with immune deficiency. Because the classic symptoms are not always present at the onset, the syndrome can be misdiagnosed.

DIAGNOSIS

Once the vesicular rash caused by herpes zoster has appeared, the diagnosis is usually readily apparent. The other main disease to consider in the differential diagnosis is herpes simplex. Herpes zoster infection is characterized by a painful sensory prodrome, dermatomal distribution, and lack of a history of a similar rash. However, if the patient has had a similar vesicular rash in the same location, then recurrent zosteriform herpes simplex should be considered. A noninfectious cause to consider is contact dermatitis. However, contact dermatitis usually produces intense itch rather than pain.

If the clinical presentation is uncertain, then viral culture, direct immunofluorescence testing, and a polymerase chain reaction assay is indicated to confirm the diagnosis. Polymerase chain reaction testing is the most sensitive test.³

TREATMENT

The rapid start of antiviral therapy is particularly critical in immunocompromised patients,⁴ even if the vesicles have been present for 72 hours. Immunocompromised patients with Ramsay Hunt syndrome and other forms of complicated herpes zoster infection should be hospitalized for intravenous acyclovir therapy.

Corticosteroids and oral acyclovir (10 mg/kg three times daily for 7 days) are commonly used in Ramsay Hunt syndrome. In a recent review,⁵ combination therapy with a corticosteroid and intravenous acyclovir did not show a benefit over corticosteroids alone in promoting resolution of facial neuropathy after 6 months.⁵ However, randomized clinical trials are needed to evaluate both therapies.

Although antiviral therapy reduces pain associated with acute neuritis, pain syndromes associated with herpes zoster can still be severe. Nonsteroidal antiinflammatory drugs and acetaminophen are useful for mild pain, either alone or in combination with a weak opioid analgesic (eg, tramadol, codeine). For moderate to severe pain that disturbs sleep, a stronger opioid analgesic (eg, oxycodone, morphine) may be necessary.⁶

Vestibular suppressants may be helpful if vestibular symptoms are severe. Temporary relief of otalgia may be achieved by applying a local anesthetic to the trigger point, if in the external auditory canal. Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.⁷

OTHER CONSIDERATIONS

Once drug therapy is started, the patient should be seen at 2 weeks, 6 weeks, and 3 months to monitor the evolution of nerve paralysis.⁸

A 54-year-old woman presented with a 7-day history of odynophagia, pharyngeal swelling, and painful skin lesions on her left ear. She had been on antiretroviral therapy for human immunodeficiency virus infection but had not been fully compliant with the treatment.

See editorial

On examination, she had painful erythematous vesicles and pustules on the left auricle and in the external auditory canal (Figure 1), as well as small vesicles and circumscribed erosions on the left anterior twothirds of her tongue (Figure 2) and left palate. Facial sensory function was normal; however, she had lagophthalmos, a flattened nasolabial fold, ptosis of the oral commissure, and a loss of the forehead wrinkles on the left side of her face—all signs of peripheral facial nerve paralysis.

Q: Which is the most likely diagnosis?

• Ramsay Hunt syndrome
• Herpes simplex
• Contact dermatitis
• Malignant external otitis
• Erysipelas

A: This patient had Ramsay Hunt syndrome, also known as herpes zoster oticus. It is a rare complication of herpes zoster in which the reactivation of latent varicella-zoster virus infection in the geniculate ganglion causes the triad of ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle. Taste perception, hearing (eg, tinnitus, hyperacusis), and lacrimation can be affected.¹

Ramsay Hunt syndrome is generally considered a polycranial neuropathy of cranial nerves VII (facial) and VIII (acoustic). In some cases other cranial neuropathies may be present and may involve cranial nerves V (trigeminal), IX (glossopharyngeal), and X (vagus). Vestibular disturbances such as vertigo are also often reported. It is more severe in patients with immune deficiency. Because the classic symptoms are not always present at the onset, the syndrome can be misdiagnosed.

DIAGNOSIS

Once the vesicular rash caused by herpes zoster has appeared, the diagnosis is usually readily apparent. The other main disease to consider in the differential diagnosis is herpes simplex. Herpes zoster infection is characterized by a painful sensory prodrome, dermatomal distribution, and lack of a history of a similar rash. However, if the patient has had a similar vesicular rash in the same location, then recurrent zosteriform herpes simplex should be considered. A noninfectious cause to consider is contact dermatitis. However, contact dermatitis usually produces intense itch rather than pain.

If the clinical presentation is uncertain, then viral culture, direct immunofluorescence testing, and a polymerase chain reaction assay is indicated to confirm the diagnosis. Polymerase chain reaction testing is the most sensitive test.³

TREATMENT

The rapid start of antiviral therapy is particularly critical in immunocompromised patients,⁴ even if the vesicles have been present for 72 hours. Immunocompromised patients with Ramsay Hunt syndrome and other forms of complicated herpes zoster infection should be hospitalized for intravenous acyclovir therapy.

Corticosteroids and oral acyclovir (10 mg/kg three times daily for 7 days) are commonly used in Ramsay Hunt syndrome. In a recent review,⁵ combination therapy with a corticosteroid and intravenous acyclovir did not show a benefit over corticosteroids alone in promoting resolution of facial neuropathy after 6 months.⁵ However, randomized clinical trials are needed to evaluate both therapies.

Although antiviral therapy reduces pain associated with acute neuritis, pain syndromes associated with herpes zoster can still be severe. Nonsteroidal antiinflammatory drugs and acetaminophen are useful for mild pain, either alone or in combination with a weak opioid analgesic (eg, tramadol, codeine). For moderate to severe pain that disturbs sleep, a stronger opioid analgesic (eg, oxycodone, morphine) may be necessary.⁶

Vestibular suppressants may be helpful if vestibular symptoms are severe. Temporary relief of otalgia may be achieved by applying a local anesthetic to the trigger point, if in the external auditory canal. Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.⁷

OTHER CONSIDERATIONS

Once drug therapy is started, the patient should be seen at 2 weeks, 6 weeks, and 3 months to monitor the evolution of nerve paralysis.⁸

A 54-year-old woman presented with a 7-day history of odynophagia, pharyngeal swelling, and painful skin lesions on her left ear. She had been on antiretroviral therapy for human immunodeficiency virus infection but had not been fully compliant with the treatment.

See editorial

On examination, she had painful erythematous vesicles and pustules on the left auricle and in the external auditory canal (Figure 1), as well as small vesicles and circumscribed erosions on the left anterior twothirds of her tongue (Figure 2) and left palate. Facial sensory function was normal; however, she had lagophthalmos, a flattened nasolabial fold, ptosis of the oral commissure, and a loss of the forehead wrinkles on the left side of her face—all signs of peripheral facial nerve paralysis.

Q: Which is the most likely diagnosis?

• Ramsay Hunt syndrome
• Herpes simplex
• Contact dermatitis
• Malignant external otitis
• Erysipelas

A: This patient had Ramsay Hunt syndrome, also known as herpes zoster oticus. It is a rare complication of herpes zoster in which the reactivation of latent varicella-zoster virus infection in the geniculate ganglion causes the triad of ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle. Taste perception, hearing (eg, tinnitus, hyperacusis), and lacrimation can be affected.¹

Ramsay Hunt syndrome is generally considered a polycranial neuropathy of cranial nerves VII (facial) and VIII (acoustic). In some cases other cranial neuropathies may be present and may involve cranial nerves V (trigeminal), IX (glossopharyngeal), and X (vagus). Vestibular disturbances such as vertigo are also often reported. It is more severe in patients with immune deficiency. Because the classic symptoms are not always present at the onset, the syndrome can be misdiagnosed.

DIAGNOSIS

Once the vesicular rash caused by herpes zoster has appeared, the diagnosis is usually readily apparent. The other main disease to consider in the differential diagnosis is herpes simplex. Herpes zoster infection is characterized by a painful sensory prodrome, dermatomal distribution, and lack of a history of a similar rash. However, if the patient has had a similar vesicular rash in the same location, then recurrent zosteriform herpes simplex should be considered. A noninfectious cause to consider is contact dermatitis. However, contact dermatitis usually produces intense itch rather than pain.

If the clinical presentation is uncertain, then viral culture, direct immunofluorescence testing, and a polymerase chain reaction assay is indicated to confirm the diagnosis. Polymerase chain reaction testing is the most sensitive test.³

TREATMENT

The rapid start of antiviral therapy is particularly critical in immunocompromised patients,⁴ even if the vesicles have been present for 72 hours. Immunocompromised patients with Ramsay Hunt syndrome and other forms of complicated herpes zoster infection should be hospitalized for intravenous acyclovir therapy.

Corticosteroids and oral acyclovir (10 mg/kg three times daily for 7 days) are commonly used in Ramsay Hunt syndrome. In a recent review,⁵ combination therapy with a corticosteroid and intravenous acyclovir did not show a benefit over corticosteroids alone in promoting resolution of facial neuropathy after 6 months.⁵ However, randomized clinical trials are needed to evaluate both therapies.

Although antiviral therapy reduces pain associated with acute neuritis, pain syndromes associated with herpes zoster can still be severe. Nonsteroidal antiinflammatory drugs and acetaminophen are useful for mild pain, either alone or in combination with a weak opioid analgesic (eg, tramadol, codeine). For moderate to severe pain that disturbs sleep, a stronger opioid analgesic (eg, oxycodone, morphine) may be necessary.⁶

Vestibular suppressants may be helpful if vestibular symptoms are severe. Temporary relief of otalgia may be achieved by applying a local anesthetic to the trigger point, if in the external auditory canal. Carbamazepine may be helpful, especially in cases of idiopathic geniculate neuralgia.⁷

OTHER CONSIDERATIONS

Once drug therapy is started, the patient should be seen at 2 weeks, 6 weeks, and 3 months to monitor the evolution of nerve paralysis.⁸