Loknath Ghoshal, MD

A 42-year-old businessman presented with recurrent redness, swelling, warmth, and burning pain on both hands and both feet for the preceding year (Figure 1). His symptoms were worse during heat exposure or physical effort and were relieved by immersing his hands and feet in cold water and by elevating his limbs. He had no other local or systemic symptoms, and he had not been on any medications. His medical history was noncontributory, and there was no family history of similar illness.

The erythema blanched when pressure was applied (Figure 2). The affected areas were slightly tender. Other areas of his skin and mucosae were normal. Neurologic examination and examination of other systems were normal. Results of laboratory testing (complete blood cell count, biochemistry panel, antinuclear antibody test) and gastrointestinal endoscopy were normal.

Q: What is the diagnosis?

• Fabry disease
• Peripheral neuropathy
• Polycythemia
• Primary idiopathic erythromelalgia
• Erythrodysesthesia syndrome

A: The correct diagnosis is primary idiopathic erythromelalgia.

Erythromelalgia is a relatively rare clinical condition of uncertain etiology, characterized by the triad of episodic redness, warmth, and burning pain in the extremities.^1,2

The condition has primary (ie, no underlying cause is found) and secondary forms.^1,3 The primary form may be inherited in an autosomal dominant manner (in which case the symptoms begin in childhood), or it may be idiopathic.⁴ On the other hand, erythromelalgia can also be secondary to polycythemia vera and other myeloproliferative disorders, connective tissue disorders, neuropathies, spinal cord diseases, carcinoma of the colon and thyroid, and astrocytomas.^1–6

The common pathologic mechanism of erythromelalgia is thought to be microvascular arteriovenous shunting.⁵ A mutation in the voltage-gated sodium channel alpha subunit Na_V1.7 may result in primary erythromelalgia. Small-fiber neuropathy² can manifest as erythromelalgia and may respond to steroids.

The symptoms can be intermittent or, in rare cases, constant.³ The lower limbs are more commonly involved than the upper ones.³ Involvement is often bilateral and symmetric.³ The symptoms may worsen at night, after alcohol consumption, with higher environmental temperature, and with moderate exercise.³ In rare cases, ulceration and gangrene may occur. Patients may get relief by cooling the affected areas.²

DIAGNOSIS BY EXCLUSION OR BASED ON THE PRESENTATION

The diagnosis of erythromelalgia is based on a detailed history and physical examination during a painful episode.^2,3 Because the condition is intermittent, only about two-thirds of patients have abnormal findings on physical examination at the time of presentation; in such cases, the diagnosis is based on the history alone.²

Testing is needed to exclude other diagnoses and to determine the cause of secondary erythromelalgia. Histopathologic study of lesions is not helpful, as the features are nonspecific and hence nondiagnostic.²

In our patient, the typical clinical features and the lack of an obvious cause on diagnostic testing confirmed the diagnosis.

THE DIFFERENTIAL DIAGNOSIS

Other conditions can be ruled out by clinical features and laboratory testing.

Fabry disease causes paresthesia and burning pain in the extremities but not erythema. Characteristic dark red keratotic papules are seen all over the body (angiokeratoma corporis diffusum). It is often associated with progressive renal insufficiency.

Peripheral neuropathy of varying causes may also cause pain in the extremities but not erythema. Neurologic examination and a nerve conduction velocity study can resolve the diagnostic problem. Clinical features and laboratory testing often help to pinpoint the cause of neuropathy.

Polycythemia may cause erythema in the hands, feet, and face and mucosal engorgement. Telangiectasia, petechiae, and cyanosis may also occur. Our patient’s normal complete blood cell count excluded this condition.

Erythrodysesthesia syndrome, typically caused by chemotherapeutic drugs, was not included in the differential diagnosis since our patient had taken no medications.

Other causes of palmar erythema to rule out, depending on the patient’s presentation, may include thyrotoxicosis, chronic febrile illness, leukemia, hepatic insufficiency, chronic alcoholism, and rheumatoid arthritis.

TREATMENT AND PROGNOSIS

There is no definitive therapy for erythromelalgia.⁴ Treatment is often difficult and needs a multidisciplinary approach. Simple measures such as cooling² (eg, applying cold towels, immersion in cool water, walking on cold floors) or elevating the affected extremity often relieve symptoms. Patients should avoid precipitating factors such as warmth, dependency of extremities, exercise, tight footwear, and alcohol intake.

If there is an underlying disease, treating the disease may also alleviate the symptoms.⁷ Aspirin^2,7 is the therapy of choice for erythromelalgia in patients with an underlying myeloproliferative disorder, and some authors have advocated it for all patients with erythromelalgia unless there is a contraindication.⁷

Other possible first-line treatments include the synthetic prostaglandin E₁ analogue misoprostol (Cytotec) and prostacyclins. Gabapentin (Neurontin), serotonin reuptake inhibitors such as sertaline (Zoloft) and venlafaxine (Effexor), and intravenous nitroprusside (Nitro-press) are considered second-line drugs.⁷

Surgical sympathectomy⁸ has also been tried, with variable results.

Outcomes in patients with erythromelalgia

In a case series from Mayo Clinic,² approximately equal numbers of patients with erythromelalgia became worse, stayed the same, or got better, and the disease resolved in 10% over a mean of 8.7 years.

THE OUTCOME IN OUR PATIENT

We advised our patient to avoid strenuous activity in a warm environment and to work in cooler areas as much as possible. We told him to wrap his affected extremities with cold towels during attacks, and we prescribed aspirin (650 mg/day) for 3 months. The treatment did not cure his condition, but his symptoms lessened within 2 months. We later referred him to a pain clinic.

A 42-year-old businessman presented with recurrent redness, swelling, warmth, and burning pain on both hands and both feet for the preceding year (Figure 1). His symptoms were worse during heat exposure or physical effort and were relieved by immersing his hands and feet in cold water and by elevating his limbs. He had no other local or systemic symptoms, and he had not been on any medications. His medical history was noncontributory, and there was no family history of similar illness.

The erythema blanched when pressure was applied (Figure 2). The affected areas were slightly tender. Other areas of his skin and mucosae were normal. Neurologic examination and examination of other systems were normal. Results of laboratory testing (complete blood cell count, biochemistry panel, antinuclear antibody test) and gastrointestinal endoscopy were normal.

Q: What is the diagnosis?

• Fabry disease
• Peripheral neuropathy
• Polycythemia
• Primary idiopathic erythromelalgia
• Erythrodysesthesia syndrome

A: The correct diagnosis is primary idiopathic erythromelalgia.

Erythromelalgia is a relatively rare clinical condition of uncertain etiology, characterized by the triad of episodic redness, warmth, and burning pain in the extremities.^1,2

The condition has primary (ie, no underlying cause is found) and secondary forms.^1,3 The primary form may be inherited in an autosomal dominant manner (in which case the symptoms begin in childhood), or it may be idiopathic.⁴ On the other hand, erythromelalgia can also be secondary to polycythemia vera and other myeloproliferative disorders, connective tissue disorders, neuropathies, spinal cord diseases, carcinoma of the colon and thyroid, and astrocytomas.^1–6

The common pathologic mechanism of erythromelalgia is thought to be microvascular arteriovenous shunting.⁵ A mutation in the voltage-gated sodium channel alpha subunit Na_V1.7 may result in primary erythromelalgia. Small-fiber neuropathy² can manifest as erythromelalgia and may respond to steroids.

The symptoms can be intermittent or, in rare cases, constant.³ The lower limbs are more commonly involved than the upper ones.³ Involvement is often bilateral and symmetric.³ The symptoms may worsen at night, after alcohol consumption, with higher environmental temperature, and with moderate exercise.³ In rare cases, ulceration and gangrene may occur. Patients may get relief by cooling the affected areas.²

DIAGNOSIS BY EXCLUSION OR BASED ON THE PRESENTATION

The diagnosis of erythromelalgia is based on a detailed history and physical examination during a painful episode.^2,3 Because the condition is intermittent, only about two-thirds of patients have abnormal findings on physical examination at the time of presentation; in such cases, the diagnosis is based on the history alone.²

Testing is needed to exclude other diagnoses and to determine the cause of secondary erythromelalgia. Histopathologic study of lesions is not helpful, as the features are nonspecific and hence nondiagnostic.²

In our patient, the typical clinical features and the lack of an obvious cause on diagnostic testing confirmed the diagnosis.

THE DIFFERENTIAL DIAGNOSIS

Other conditions can be ruled out by clinical features and laboratory testing.

Fabry disease causes paresthesia and burning pain in the extremities but not erythema. Characteristic dark red keratotic papules are seen all over the body (angiokeratoma corporis diffusum). It is often associated with progressive renal insufficiency.

Peripheral neuropathy of varying causes may also cause pain in the extremities but not erythema. Neurologic examination and a nerve conduction velocity study can resolve the diagnostic problem. Clinical features and laboratory testing often help to pinpoint the cause of neuropathy.

Polycythemia may cause erythema in the hands, feet, and face and mucosal engorgement. Telangiectasia, petechiae, and cyanosis may also occur. Our patient’s normal complete blood cell count excluded this condition.

Erythrodysesthesia syndrome, typically caused by chemotherapeutic drugs, was not included in the differential diagnosis since our patient had taken no medications.

Other causes of palmar erythema to rule out, depending on the patient’s presentation, may include thyrotoxicosis, chronic febrile illness, leukemia, hepatic insufficiency, chronic alcoholism, and rheumatoid arthritis.

TREATMENT AND PROGNOSIS

There is no definitive therapy for erythromelalgia.⁴ Treatment is often difficult and needs a multidisciplinary approach. Simple measures such as cooling² (eg, applying cold towels, immersion in cool water, walking on cold floors) or elevating the affected extremity often relieve symptoms. Patients should avoid precipitating factors such as warmth, dependency of extremities, exercise, tight footwear, and alcohol intake.

If there is an underlying disease, treating the disease may also alleviate the symptoms.⁷ Aspirin^2,7 is the therapy of choice for erythromelalgia in patients with an underlying myeloproliferative disorder, and some authors have advocated it for all patients with erythromelalgia unless there is a contraindication.⁷

Other possible first-line treatments include the synthetic prostaglandin E₁ analogue misoprostol (Cytotec) and prostacyclins. Gabapentin (Neurontin), serotonin reuptake inhibitors such as sertaline (Zoloft) and venlafaxine (Effexor), and intravenous nitroprusside (Nitro-press) are considered second-line drugs.⁷

Surgical sympathectomy⁸ has also been tried, with variable results.

Outcomes in patients with erythromelalgia

In a case series from Mayo Clinic,² approximately equal numbers of patients with erythromelalgia became worse, stayed the same, or got better, and the disease resolved in 10% over a mean of 8.7 years.

THE OUTCOME IN OUR PATIENT

We advised our patient to avoid strenuous activity in a warm environment and to work in cooler areas as much as possible. We told him to wrap his affected extremities with cold towels during attacks, and we prescribed aspirin (650 mg/day) for 3 months. The treatment did not cure his condition, but his symptoms lessened within 2 months. We later referred him to a pain clinic.

A 42-year-old businessman presented with recurrent redness, swelling, warmth, and burning pain on both hands and both feet for the preceding year (Figure 1). His symptoms were worse during heat exposure or physical effort and were relieved by immersing his hands and feet in cold water and by elevating his limbs. He had no other local or systemic symptoms, and he had not been on any medications. His medical history was noncontributory, and there was no family history of similar illness.

The erythema blanched when pressure was applied (Figure 2). The affected areas were slightly tender. Other areas of his skin and mucosae were normal. Neurologic examination and examination of other systems were normal. Results of laboratory testing (complete blood cell count, biochemistry panel, antinuclear antibody test) and gastrointestinal endoscopy were normal.

Q: What is the diagnosis?

• Fabry disease
• Peripheral neuropathy
• Polycythemia
• Primary idiopathic erythromelalgia
• Erythrodysesthesia syndrome

A: The correct diagnosis is primary idiopathic erythromelalgia.

Erythromelalgia is a relatively rare clinical condition of uncertain etiology, characterized by the triad of episodic redness, warmth, and burning pain in the extremities.^1,2

The condition has primary (ie, no underlying cause is found) and secondary forms.^1,3 The primary form may be inherited in an autosomal dominant manner (in which case the symptoms begin in childhood), or it may be idiopathic.⁴ On the other hand, erythromelalgia can also be secondary to polycythemia vera and other myeloproliferative disorders, connective tissue disorders, neuropathies, spinal cord diseases, carcinoma of the colon and thyroid, and astrocytomas.^1–6

The common pathologic mechanism of erythromelalgia is thought to be microvascular arteriovenous shunting.⁵ A mutation in the voltage-gated sodium channel alpha subunit Na_V1.7 may result in primary erythromelalgia. Small-fiber neuropathy² can manifest as erythromelalgia and may respond to steroids.

The symptoms can be intermittent or, in rare cases, constant.³ The lower limbs are more commonly involved than the upper ones.³ Involvement is often bilateral and symmetric.³ The symptoms may worsen at night, after alcohol consumption, with higher environmental temperature, and with moderate exercise.³ In rare cases, ulceration and gangrene may occur. Patients may get relief by cooling the affected areas.²

DIAGNOSIS BY EXCLUSION OR BASED ON THE PRESENTATION

The diagnosis of erythromelalgia is based on a detailed history and physical examination during a painful episode.^2,3 Because the condition is intermittent, only about two-thirds of patients have abnormal findings on physical examination at the time of presentation; in such cases, the diagnosis is based on the history alone.²

Testing is needed to exclude other diagnoses and to determine the cause of secondary erythromelalgia. Histopathologic study of lesions is not helpful, as the features are nonspecific and hence nondiagnostic.²

In our patient, the typical clinical features and the lack of an obvious cause on diagnostic testing confirmed the diagnosis.

THE DIFFERENTIAL DIAGNOSIS

Other conditions can be ruled out by clinical features and laboratory testing.

Fabry disease causes paresthesia and burning pain in the extremities but not erythema. Characteristic dark red keratotic papules are seen all over the body (angiokeratoma corporis diffusum). It is often associated with progressive renal insufficiency.

Peripheral neuropathy of varying causes may also cause pain in the extremities but not erythema. Neurologic examination and a nerve conduction velocity study can resolve the diagnostic problem. Clinical features and laboratory testing often help to pinpoint the cause of neuropathy.

Polycythemia may cause erythema in the hands, feet, and face and mucosal engorgement. Telangiectasia, petechiae, and cyanosis may also occur. Our patient’s normal complete blood cell count excluded this condition.

Erythrodysesthesia syndrome, typically caused by chemotherapeutic drugs, was not included in the differential diagnosis since our patient had taken no medications.

Other causes of palmar erythema to rule out, depending on the patient’s presentation, may include thyrotoxicosis, chronic febrile illness, leukemia, hepatic insufficiency, chronic alcoholism, and rheumatoid arthritis.

TREATMENT AND PROGNOSIS

There is no definitive therapy for erythromelalgia.⁴ Treatment is often difficult and needs a multidisciplinary approach. Simple measures such as cooling² (eg, applying cold towels, immersion in cool water, walking on cold floors) or elevating the affected extremity often relieve symptoms. Patients should avoid precipitating factors such as warmth, dependency of extremities, exercise, tight footwear, and alcohol intake.

If there is an underlying disease, treating the disease may also alleviate the symptoms.⁷ Aspirin^2,7 is the therapy of choice for erythromelalgia in patients with an underlying myeloproliferative disorder, and some authors have advocated it for all patients with erythromelalgia unless there is a contraindication.⁷

Other possible first-line treatments include the synthetic prostaglandin E₁ analogue misoprostol (Cytotec) and prostacyclins. Gabapentin (Neurontin), serotonin reuptake inhibitors such as sertaline (Zoloft) and venlafaxine (Effexor), and intravenous nitroprusside (Nitro-press) are considered second-line drugs.⁷

Surgical sympathectomy⁸ has also been tried, with variable results.

Outcomes in patients with erythromelalgia

In a case series from Mayo Clinic,² approximately equal numbers of patients with erythromelalgia became worse, stayed the same, or got better, and the disease resolved in 10% over a mean of 8.7 years.

THE OUTCOME IN OUR PATIENT

We advised our patient to avoid strenuous activity in a warm environment and to work in cooler areas as much as possible. We told him to wrap his affected extremities with cold towels during attacks, and we prescribed aspirin (650 mg/day) for 3 months. The treatment did not cure his condition, but his symptoms lessened within 2 months. We later referred him to a pain clinic.

A 12-year-old boy presents with painless swelling and ulceration on and around his nose that has progressed gradually over the last 6 months. The lesion has increased in size despite treatment with topical neomycin and oral erythromycin. He has no systemic symptoms.

On examination (Figure 1), we note an indurated, nontender plaque with scarring at places on his right cheek, nose, and the vermilion border of the lip. In addition, there are two purulent ulcerations on the nose partly destroying the right nasal wing. The upper lip is also infiltrated, studded with a solitary ulceration. There is no regional lymphadenopathy. An examination of systems is normal.

Q: What is the diagnosis?

• Lupus vulgaris (tuberculosis of the skin)
• Wegener granulomatosis
• Midline lethal granuloma (natural killer T-cell lymphoma)
• Hansen disease (leprosy)
• Lupoid form of cutaneous leishmaniasis

A: Lupus vulgaris is the correct diagnosis.

Cutaneous tuberculosis occurs in many forms, and lupus vulgaris is one of the most common.¹ Lupus vulgaris usually arises as a result of hematogenous spread from an endogenous source. It may also arise from exogenous inoculation or as a complication of vaccination with bacille Calmette-Guérin.²

Several morphologic variants have been described.^1,2 One form is characterized by plaques, often studded with psoriasiform scales. Large plaques may show irregular areas of scarring with islands of active lupus tissue and a thickened and hyperkeratotic margin. Ulcerative and mutilating variants of lupus vulgaris are characterized by scarring, ulceration, crusts over areas of necrosis, and destruction of the deep tissues and cartilage, resulting in deformities. The vegetative form produces marked infiltration, ulceration, and necrosis, with minimal scarring. Mucous membranes and cartilages are often destroyed. Tumor-like hypertrophic lesions and multiple papular and nodular lesions may also be seen. Nasal lesions may start as nodules, which may bleed and then ulcerate, sometimes resulting in cartilage destruction.

CLINICAL FEATURES AND LABORATORY WORKUP CLINCHED THE DIAGNOSIS

A number of factors helped to confirm the diagnosis in this patient:

• A strongly positive Mantoux test (22-mm induration at 48 hours)
• Acid-fast bacilli on Ziehl-Neelsen staining of the smear taken from the purulent ulceration
• Isolation of Mycobacterium tuberculosis from the purulent exudates via culture in Lowenstein-Jensen medium

• 

  A suggestive histopathologic picture (Figure 2)
• The features on presentation
• A significant clinical improvement within 2 months of starting antituberculosis therapy.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis includes all the conditions in the question above. However, the absence of respiratory and renal involvement helps rule out Wegener granulomatosis; the absence of impaired sensation and nerve thickening helps rule out Hansen disease; and the absence of a nasal septal defect helps rule out Wegener granulomatosis, midline lethal granuloma, and Hansen disease.

On the other hand, the lupoid form of cutaneous leishmaniasis usually presents as an erythematous, infiltrated plaque that often closely resembles lupus vulgaris, but these lesions are usually less destructive than lupus vulgaris. However, the laboratory workup including the microbiological and histopathologic examination clearly excluded the other potential diagnoses in this patient.

TREATMENT

Lupus vulgaris is treated with standard antituberculosis therapy.³ The first phase of a fourdrug regimen is given for 2 months—isoniazid, rifampin (Rifadin), pyrazinamide, and ethambutol (Myambutol). The second phase consists of isoniazid and rifampin for 4 months.³

Early recognition and confirmation of the diagnosis followed by treatment are of immense importance for preventing permanent disfigurement.

A 12-year-old boy presents with painless swelling and ulceration on and around his nose that has progressed gradually over the last 6 months. The lesion has increased in size despite treatment with topical neomycin and oral erythromycin. He has no systemic symptoms.

On examination (Figure 1), we note an indurated, nontender plaque with scarring at places on his right cheek, nose, and the vermilion border of the lip. In addition, there are two purulent ulcerations on the nose partly destroying the right nasal wing. The upper lip is also infiltrated, studded with a solitary ulceration. There is no regional lymphadenopathy. An examination of systems is normal.

Q: What is the diagnosis?

• Lupus vulgaris (tuberculosis of the skin)
• Wegener granulomatosis
• Midline lethal granuloma (natural killer T-cell lymphoma)
• Hansen disease (leprosy)
• Lupoid form of cutaneous leishmaniasis

A: Lupus vulgaris is the correct diagnosis.

Cutaneous tuberculosis occurs in many forms, and lupus vulgaris is one of the most common.¹ Lupus vulgaris usually arises as a result of hematogenous spread from an endogenous source. It may also arise from exogenous inoculation or as a complication of vaccination with bacille Calmette-Guérin.²

Several morphologic variants have been described.^1,2 One form is characterized by plaques, often studded with psoriasiform scales. Large plaques may show irregular areas of scarring with islands of active lupus tissue and a thickened and hyperkeratotic margin. Ulcerative and mutilating variants of lupus vulgaris are characterized by scarring, ulceration, crusts over areas of necrosis, and destruction of the deep tissues and cartilage, resulting in deformities. The vegetative form produces marked infiltration, ulceration, and necrosis, with minimal scarring. Mucous membranes and cartilages are often destroyed. Tumor-like hypertrophic lesions and multiple papular and nodular lesions may also be seen. Nasal lesions may start as nodules, which may bleed and then ulcerate, sometimes resulting in cartilage destruction.

CLINICAL FEATURES AND LABORATORY WORKUP CLINCHED THE DIAGNOSIS

A number of factors helped to confirm the diagnosis in this patient:

• A strongly positive Mantoux test (22-mm induration at 48 hours)
• Acid-fast bacilli on Ziehl-Neelsen staining of the smear taken from the purulent ulceration
• Isolation of Mycobacterium tuberculosis from the purulent exudates via culture in Lowenstein-Jensen medium

• 

  A suggestive histopathologic picture (Figure 2)
• The features on presentation
• A significant clinical improvement within 2 months of starting antituberculosis therapy.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis includes all the conditions in the question above. However, the absence of respiratory and renal involvement helps rule out Wegener granulomatosis; the absence of impaired sensation and nerve thickening helps rule out Hansen disease; and the absence of a nasal septal defect helps rule out Wegener granulomatosis, midline lethal granuloma, and Hansen disease.

On the other hand, the lupoid form of cutaneous leishmaniasis usually presents as an erythematous, infiltrated plaque that often closely resembles lupus vulgaris, but these lesions are usually less destructive than lupus vulgaris. However, the laboratory workup including the microbiological and histopathologic examination clearly excluded the other potential diagnoses in this patient.

TREATMENT

Lupus vulgaris is treated with standard antituberculosis therapy.³ The first phase of a fourdrug regimen is given for 2 months—isoniazid, rifampin (Rifadin), pyrazinamide, and ethambutol (Myambutol). The second phase consists of isoniazid and rifampin for 4 months.³

Early recognition and confirmation of the diagnosis followed by treatment are of immense importance for preventing permanent disfigurement.

A 12-year-old boy presents with painless swelling and ulceration on and around his nose that has progressed gradually over the last 6 months. The lesion has increased in size despite treatment with topical neomycin and oral erythromycin. He has no systemic symptoms.

On examination (Figure 1), we note an indurated, nontender plaque with scarring at places on his right cheek, nose, and the vermilion border of the lip. In addition, there are two purulent ulcerations on the nose partly destroying the right nasal wing. The upper lip is also infiltrated, studded with a solitary ulceration. There is no regional lymphadenopathy. An examination of systems is normal.

Q: What is the diagnosis?

• Lupus vulgaris (tuberculosis of the skin)
• Wegener granulomatosis
• Midline lethal granuloma (natural killer T-cell lymphoma)
• Hansen disease (leprosy)
• Lupoid form of cutaneous leishmaniasis

A: Lupus vulgaris is the correct diagnosis.

Cutaneous tuberculosis occurs in many forms, and lupus vulgaris is one of the most common.¹ Lupus vulgaris usually arises as a result of hematogenous spread from an endogenous source. It may also arise from exogenous inoculation or as a complication of vaccination with bacille Calmette-Guérin.²

Several morphologic variants have been described.^1,2 One form is characterized by plaques, often studded with psoriasiform scales. Large plaques may show irregular areas of scarring with islands of active lupus tissue and a thickened and hyperkeratotic margin. Ulcerative and mutilating variants of lupus vulgaris are characterized by scarring, ulceration, crusts over areas of necrosis, and destruction of the deep tissues and cartilage, resulting in deformities. The vegetative form produces marked infiltration, ulceration, and necrosis, with minimal scarring. Mucous membranes and cartilages are often destroyed. Tumor-like hypertrophic lesions and multiple papular and nodular lesions may also be seen. Nasal lesions may start as nodules, which may bleed and then ulcerate, sometimes resulting in cartilage destruction.

CLINICAL FEATURES AND LABORATORY WORKUP CLINCHED THE DIAGNOSIS

A number of factors helped to confirm the diagnosis in this patient:

• A strongly positive Mantoux test (22-mm induration at 48 hours)
• Acid-fast bacilli on Ziehl-Neelsen staining of the smear taken from the purulent ulceration
• Isolation of Mycobacterium tuberculosis from the purulent exudates via culture in Lowenstein-Jensen medium

• 

  A suggestive histopathologic picture (Figure 2)
• The features on presentation
• A significant clinical improvement within 2 months of starting antituberculosis therapy.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis includes all the conditions in the question above. However, the absence of respiratory and renal involvement helps rule out Wegener granulomatosis; the absence of impaired sensation and nerve thickening helps rule out Hansen disease; and the absence of a nasal septal defect helps rule out Wegener granulomatosis, midline lethal granuloma, and Hansen disease.

On the other hand, the lupoid form of cutaneous leishmaniasis usually presents as an erythematous, infiltrated plaque that often closely resembles lupus vulgaris, but these lesions are usually less destructive than lupus vulgaris. However, the laboratory workup including the microbiological and histopathologic examination clearly excluded the other potential diagnoses in this patient.

TREATMENT

Lupus vulgaris is treated with standard antituberculosis therapy.³ The first phase of a fourdrug regimen is given for 2 months—isoniazid, rifampin (Rifadin), pyrazinamide, and ethambutol (Myambutol). The second phase consists of isoniazid and rifampin for 4 months.³

Early recognition and confirmation of the diagnosis followed by treatment are of immense importance for preventing permanent disfigurement.