Pott’s Disease and Scrofuloderma as Presentation of Clinically

Extrapulmonary Tuberculosis in 15 Years Old Girl

1
Resident of Child Health Department, Faculty of Medicine, Udayana University,
Denpasar, Bali, Indonesia
2
Department Of Child Health, Faculty of Medicine, Udayana University,
Denpasar, Bali, Indonesia
3
Department Of Child Health, Faculty of Medicine, Udayana University,
Denpasar, Bali, Indonesia
4
Department Of Child Health, Faculty of Medicine, Udayana University,
Denpasar, Bali, Indonesia
5
Department Of Child Health, Faculty of Medicine, Udayana University,
Denpasar, Bali, Indonesia
6
Department Of Pathological Anatomy, Faculty of Medicine, Udayana University,
Denpasar, Bali, Indonesia

*Corresponding author: Davit Soesanto; Resident of Child Health Department,

ABSTRACT
Backgrounds: Extrapulmonary tuberculosis (TB) accounts 15–20% of all
tuberculosis cases with lymph node being the most common affected organ.
Spinal tuberculosis and scrofuloderma are rare manifestation of extrapulmonary
TB, constitutes less than 5% of the cases. Establishing diagnosis is a challenge.
Eradication and prevention of sequelae complicate further management of the
disease.
Objective: To describe the clinical manifestation and the management of
spondylitis tuberculosis and scrofuloderma in a child.
Case presentation: A 15-years-old girl came with chief complaint of lower
extremities weakness for a week following monthly chronic low back pain. She
also reported painless lesion on her back with no sign of acute inflammation. She
also complained intermittent fever with night sweats and lost 3 kilos of body

1
weight in last two months. From physical examination we found a kyphotic
posture and severe malnutrition. Skin examination revealed open wound on her
right lower back, size 4x3 cm, no sign of inflammation. The tuberculin skin test
was positive with 15 mm induration. The spinal magnetic resonance imaging
revealed destruction of the vertebral corpus thoracal segment, hyperintense
sacrum and right ileum bone with cold abscess suggested spondylitis TB. A
wound biopsy with hematoxylin-eosin stain concluded chronic granulomatous
inflammation and showed multinucleated giant cell Langhans-type, suggestive for
cutaneous tuberculosis. Microbiology examination (PCR GeneXpert) from wound
biopsy and ziehl neelsen stain failed to detect Mycobacterium tuberculosis. The
patient was diagnosis with Pott’s disease and scrofuloderma. Anti-tuberculous
therapy was commenced, and patient showed good responses after several months
of treatment.
Conclusions: The diagnosis of extrapulmonary TB manifests as spinal
tuberculosis and scrofuloderma could be made from clinical manifestation and
imaging.

Keywords: Pott’s disease, Scrofuloderma, Tuberculosis, Children

INTRODUCTION

Tuberculosis can infect any places in the body beside lungs such as
bones, brain, skin, and other inner organs. This condition is called
extrapulmonary tuberculosis. Extrapulmonary tuberculosis constitutes 15–20%
of all cases of tuberculosis with the commonest site is lymph node. Spinal
tuberculosis affected approximately only 1 to 5% in all cases of extrapulmonary
TB. It causes bone destruction, spinal deformity, and neural complications.
Scrofuloderma is a rare condition which accounts for between 0.5 - 2 % of
extrapulmonary tuberculosis. It occurs due to direct involvement and breakdown
of skin from contiguous tubercular foci–like-infected lymph node or bone. The
diagnosis is based on clinical, microbiological confirmation, and radiological
evidence, particularly in the endemic areas of the world. The treatment targets
are to confirm the diagnosis, eradicate the infection, achieve decompression of
the spinal canal material, and correct or prevent spinal deformity and a possible
sequel.

THE CASE
A 15-years-old girl came to our emergency room with the chief complaint of
weakness over her both legs. Her leg became paresthesia at first in the following

2
7 days. She also present with low back pain since 2 months ago. At that time, she
still can walked normally, but, in the last 3 days, her legs became weak and she
cannot walked anymore. This condition affected her routine because she had
limited walking ability. The patient still had a good movement and strength on
her both arms.
The patient was also complaining about painless lesion on her back for
about 2 months. At the beginning, the patient reported that she had a small mass
on the back before the installation of the ulceration. There was no acute
inflammation on the wound lesion. She reported also an intermittent fever with
night sweats and a weight loss of 3 kg over 2 months. Further anamnesis revealed
that her grandmother is confirmed case pulmonary tuberculosis patient.
From physical examination, we found a kyphotic posture and severe
malnutrition patient with a BMI of 10 kg/m2. Bacillus Calmette-Guerin (BCG)
scar on the arm (+). Dermatological examination found open wound on her right
lower back as high as lumbosacral level, size 4x3 cm, there is no sign of
inflammation (figure 1). There were no palpable lymph nodes. Her lower
extremity strength were 4/5 for right leg and 3/5 for left leg. Laboratory
examination showed leucocyte 10.61 x103 uL; neutrophil 89.49%; lymphocyte
9.7%; hemoglobin 13.68 g/dL; hematocrit 46.99%; platelet 336 x 10 3 uL; sodium
140 mmol/L; potassium 4.44 mmol/L; chloride 101.1 mmol/L; and calcium 9.7
mg/dL. Serologic testing for human immunodeficiency virus (HIV) was negative.
The tuberculin skin test was positive (15 mm induration). Chest X-ray AP/ Lateral
showed fibrotic line on basal zone left lung suspected old inflammation (figure 2).
The spinal magnetic resonance imaging (MRI) revealed destruction of the
vertebral corpus thoracic segment with cold abscess, hyperintense on T2W1 at
right ileum bone and S1 with abscess para lumbar and para sacral region
suggested spondylitis TB (figure 3). Sputum geneXpert was not available,
meanwhile wound geneXpert MTB was not detect Mycobacterium tuberculosis,
but wound biopsy with hematoxylin-eosin stain concluded chronic granulomatous
inflammation and showed multinucleated giant cell langhans (figure 4), The
histopathological features were suggestive of cutaneous tuberculosis.
Unfortunately, the ziehl neelsen stain failed to showed acid fast bacilli.

3
 Figure 1. Painless Wound lesion

Figure 2. Chest X-ray showed fibrotic area on basal zone left lung

4
 Figure 3. (A) Magnetic Resonance Imaging (MRI) showed destruction of the
adjacent thoracic vertebrae (white arrow) and (B) hyperintense of right ileum
bone to sacral (white arrow)

Figure 4. (A) Chronic Granulomatous Infection and (B) Multinucleated Giant Cell
Langhans (red arrow)

From these findings, we established diagnosis of Pott’s disease and

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DISCUSSION
This case report is a 15 years old girl with Pott’s disease and
scrofuloderma. The accuracy of diagnosis and treatment are the most important
prognostic factor of the patient. Tuberculosis is still one of the main health
problems and one of the top ten common causes of death in the world. Based on
the World Health Organization (WHO) global TB report in 2020, TB morbidity
rate in the world is around 10.0 million people and 1.2 million of them died.
Moreover, WHO also stated that 70% of patients were new cases and more than
85% of deaths were reported in Southeast Asia and Africa. The Sustainable
Development Goals (SDGs) issued in 2015 has the End TB program with the
goal of ending the TB epidemic in the world. This program has three success
main indicators, there are a reduction in the incidence of TB in the world by 80%
in 2030 compared to 2015, a reduction in mortality rate by 90% compared to
2015, and 0 (zero) costs that need to be incurred by TB patients in treating their
disease. In Indonesia, TB is one of the national priorities for disease control
programs because it has a wide impact on the quality of life and the economy
burden. World Health Organization noted that Indonesia ranks second for the
most TB incidences in the world after India 1,2
Tuberculosis in children cannot be separated from the TB control
program because the number of children less than 15 years is 40-50% of the
population and there are approximately 500,000 children in the world who suffer
from TB every year. Based on WHO 2020, tuberculosis in children less than 15
years accounted for 12% of all cases of TB. Moreover, The Centers for Disease
Control and Prevention (CDC) stated the highest prevalence TB in children at
ages 1- 4 years.1,3,4 In our case, patient was 15 years old girl. Although the
highest prevalence of tuberculosis is range from 1 to 4 years old, older children
who live in endemic areas such as in our country are susceptible to infection.
Extrapulmonary tuberculosis is tuberculosis outside of the lungs that can
be presented as tuberculosis meningitis, abdominal tuberculosis, skeletal
tuberculosis included Pott's disease (spine), scrofuloderma (skin), and

6
genitourinary tuberculosis. It is estimated accounts for 15–25% of all cases of
TB. In general, extrapulmonary tuberculosis is more difficult to diagnose than
pulmonary TB and often requires invasive procedures to obtain tissue or fluid
samples. Besides possible fever and weight loss, the symptoms and signs
generally relate specifically to the affected organ system. Approximately 10% to
20% of extrapulmonary TB involves the bone structures. The common location
of which are the spine (spondylitis TB) followed by hip joint (coxitis) and knee
joint (gonitis). 5,6,7
Mycobacterium Tuberculosis enter the human body through the
respiratory or gastrointestinal system. Pott’s disease is usually secondary to
hematogenous spread from a primary site of infection or lymphatics spread. It
usually occur at thoracal or lumbar vertebrae. Spinal TB predilection might be due
to good vascularization at vertebral region. The spread of infection to vertebral
through arteries, venous, or percontinuatum. An arterial arcade, in the subchondral
region of each vertebra, is derived from anterior and posterior spinal arteries; this
arcade form a rich vascular plexus. This vascular plexus facilitates hematogenous
spread of the infection in the paradiskal regions..7,8,9 In our cases, spinal TB was
suspected from the hematogenous and lymphatic spread of primary site infection
that might be from the lung which showed fibrotic line from chest x-ray
examination.
Leg weakness, limping, and difficulty standing are the most complication
of spinal tuberculosis. This is caused by formation of debris, pus, and granulation
tissue due to destruction of bone and intervertebral disk. In the most severe form
of Pott's disease, spinal deformation and collapse can compress the spinal cord,
thus causing neurological disorders such as paresis and paralyses. Incidence of
neurological deficit in spinal tuberculosis varies from 23 to 76%. The other
clinical features that can be found is back pain. Back pain is the most frequent
symptom of spinal tuberculosis. The intensity of pain varies from constant mild
dull aching to severe disabling. The pain may be worsened by spinal motion,
coughing, and weight bearing, because of advanced disk disruption and spinal
instability, nerve root compression, or pathological fracture. In longstanding
cases, there may be multiple levels of vertebral body collapse, resulting in a

7
gibbous deformity. The other symptoms of spinal TB is classical constitutional
features of tuberculosis such as malaise, loss of weight and appetite, night sweats,
evening rise in temperature, generalized body aches, and fatigue. Spondylitis TB
in pediatric usually presents with an insidious onset, slow course, and mild initial
symptoms.6,7,10,11
In our case, the patient has the weakness of the limb and low back pain as
her complaint. She also caught with intermittent fever, night sweats, and weight
losses for several months that indicate the classical features of tuberculosis
infection.
The Investigation on patients with spinal tuberculosis include
hematological examination, tuberculin test, microbiological examination, and
radiological examination including computed tomography (CT) scan and
magnetic resonance imaging. Tuberculin test being positive in 63% to 90% of
the patients with TB. It does not differentiate the active infections from the latent
ones or induced reaction due to the vaccine BCG (Bacillus Calmette-Guérin). A
positive Mantoux test will require detailed investigation and a negative test
cannot rule out the diagnosis of TB. 12,13
The cytological and microbiology confirmation analysis of a vertebral
lesion via neuroimaging guided-needle biopsy from the affected site is the gold
standard technique for the early histopathological diagnosis of spinal tuberculosis.
Open biopsy of the spine is usually performed when either closed techniques have
proved insufficient or other procedures, such as decompression and possibly
arthrodesis, are planned. Histologic studies confirm the diagnosis of spinal
tuberculosis in approximately 60% of patients. The most common cytological
findings observed are epithelioid cell granulomas (90%), granular necrotic
background (83%), and lymphocytic infiltration (76%). Scattered multinucleated
and Langhans giant cells may be seen in up to 56% of cases. False-negative
results of biopsy are common and, therefore, diagnosis of spinal tuberculosis must
be made based on clinical manifestations and radiology when bacteriology proves
negative.7,12
Chest x-ray should be done in patient with spinal tuberculosis to search the
primary infection that most common happened in lungs. The conventional radio

8
imaging give enough information to diagnosis spinal TB, especially in less
sources area, but, it should be noticed that radiolucent lesion only appears on the
X-ray where 30% of the mineral density of the bone has been lost. The normal
finding of plain imaging can causes by the initial phase of the disease and does
not exclude TB infection. Spinal Tuberculosis usually presents with osteopenia of
the vertebrae, narrowing of the articulation segment and loss of definition of the
paradiscal margins of the vertebral bodies. The central type lesions generally
present with destruction, ballooning and concentric collapsing of the vertebral
bodies. In the infection of the posterior elements, the destruction of the pedicules
and lamina, the erosion of adjacent ribs and the posterior cortical of the vertebral
body can occur. The cold paravertebral abscesses are observed on simple X-rays
as shades on the soft tissues adjacent to the column. In the case of longer duration
abscess, the imaging showing concave erosions in the anterior margin of the
vertebral bodies.12
The computed tomography (CT) scan modal could showing vertebral
alterations earlier in time than X-ray, giving the characteristics in a more detailed
way regarding the osseous lesions, from the involvement of the posterior
elements, the involvement of the craniovertebral junction and the cervicothoracic
junction and the sacroiliac articulations. Four patterns of destruction can be
observed in a CT: are fragmentary, osteolytic, sclerotic, and subperiosteally. CT
also allows to evaluate the involvement of the soft tissues and paravertebral
abscesses, being an excellent method to detect abscess calcifications and is also
useful in the evaluation of the medullar compression by the inflammatory tissue or
sequestrum.12
Magnetic resonance imaging (MRI) is a better sensitivity tool than plain
radio imaging and more specificity than CT. The sensitivity and the specificity
described for this method of image are 96% and 93%. MRI also allows for the
rapid determination of the mechanism for neurologic involvement. It useful for an
earlier diagnosis than any other method of image. The use of contrast increases
the precision of the MRI, particularly at early stages. Characteristic findings of
spinal tuberculosis include, destruction of two adjacent vertebral bodies in
opposing end-plates with the vertebral disc relatively well preserved or the

9
reaching of multiple vertebral bodies, oedema of the vertebral body seen as
hyperintense signal on T2 and STIR (short tau inversion recovery) images,
extension of the infection beneath the longitudinal anterior ligament and the
presence of pre-vertebral, para -vertebral, intraosseous or epidural abscesses that
are generally smooth with thin walls. The subligamentous spread of a paraspinal
mass and the involvement of multiple contiguous bones and intramedullary spinal
changes can be very well demonstrated by MRI. MRI also presents high precision
to differentiate granular tissues from cold abscesses and allows assessing in a
detailed way the involved tissues, the anatomical localization of the abscesses, the
involvement of the neurological structures and the vertebral non-continuous
disease.7,12,14
According to the 2013 revised World Health Organization (WHO) case
definitions, spinal TB were classified as bacteriologically confirmed case and or
clinically diagnosed case. Confirmed cases were those patients for whom the
presence of M. tuberculosis in a spine biopsy was demonstrated by a positive
Xpert MTB/RIF test, positive culture for M. tuberculosis or positive smear
microscopy for acid-fast bacilli. Clinically diagnosed cases were those patients
for whom spine imaging, clinical presentation and, in some instances, confirmed
TB from other sites indicated spinal TB.15
In our case, we did not do the spinal biopsy for the spinal lesion. The
MRI showed destruction of the adjacent thoracic vertebrae, narrowing the
intervertebral space, sacrum and ileum bone marrow edema, and cold abscess.
Based on these findings, we diagnosed patient as clinically case of spinal
tuberculosis.
The other extrapulmonary TB form is Scrofuloderma. It is the most
common form of cutaneous TB among children. Scrofuloderma arises due to
contiguous spread of an underlying tuberculous focus to the overlying skin.
Lymph nodes are the most common underlying foci out of which cervical lymph
nodes being the most common group being involved. Systemic TB foci are seen
in up to 66% of cutaneous TB cases. Bones, joints, testes, breast, and lacrimal
glands are the other underlying foci of infections leading to scrofuloderma.
Clinically, scrofuloderma is characterized by asymptomatic subcutaneous

10
swellings which persist for several months before softening and ulcerating to
form discharging sinuses and ulcers. Typically, the ulcers are shallow with
undermined and bluish edges.16 In our case, the patient had a painless lesion at
the skin overlays on right ileum bones as the underlying focus of infection. The
lesion was beginning with small subcutaneous mass before then became a
shallow ulcer.
Scrofuloderma histopathological include chronic granulomatous
inflammatory infiltrate associated with caseous necrosis, langhans giant cells and
the detection of acid-fast bacilli. Tubercular epithelioid cell granulomas with
lymphocytes and Langhans type giant cells are the hallmark of cutaneous TB. In
response to the tuberculosis infection, the activated macrophages, cytokine
interferon (IFN), and T cell activity produces a type IV reaction. This reaction
combined with ischemia results in central caseation necrosis in the tuberculous
granuloma. Demonstration of the mycobacterium tuberculosis can be done with
Ziehl-Neelsen staining, but has low sensitivity (14%). This is due to tuberculosis
infection in children is often in the form of paucibacillary so that examination
smears of acid-fast bacilli often give negative results, moreover, skin tissue is
slightly favorable to the reproduction of the bacilli unlike pulmonary tissue
growth. Other modality for microbiological confirmation is culture and real time
polymerase chain reaction (RT PCR).16,17,18,19
In our case, the geneXpert MTB/RIF of soft tissue specimen showed
negative results. Histopathological examination from a wound biopsy using
hematoxylin-eosin stain concluded chronic granulomatous inflammation and
showed multinucleated giant cell langhans, but the the ziehl neelsen stain was
failed to found acid fast bacilli.
Based on the WHO guidelines, tuberculosis should treat with multidrug
regimen. The following dosages of anti-TB medicines should be used daily for
the treatment of TB in children:20
1. Isoniazid (H) 10 mg/kg (range 7–15 mg/kg); maximum dose 300 mg/day
2. Rifampicin (R) 15 mg/kg (range 10–20 mg/kg); maximum dose 600 mg/day
3. Pyrazinamide (Z) 35 mg/kg (range 30–40 mg/kg)
4. Ethambutol (E) 20 mg/kg (range 15–25 mg/kg)

11
Children with suspected or confirmed extrapulmonary TB except meningitis TB
and osteoarticular TB should be treat with four drug regimen (HRZE) for 2
months, followed by a two-drug regimen (HR) for 4 months, meanwhile,
osteoarticular TB should be treated with a four drug regimen (HRZE) for 2
months, followed by a two-drug regimen (HR) for 10 months, the total duration
of treatment being 12 months. The wound treatment of scrofuloderma focus on
hygiene and topical ointment to prevent the secondary infection. Other topical
treatment include compress with normal saline solution on the wound surface
area.15
In our case, patient was taking 12 months duration of treatment divided to
four drug regimen (HRZE) for 2 months, followed by a two-drug regimen (HR)
for 10 months. There is no major adverse event developed during the observation.
The patient showed good responses after several months of antituberculosis
treatment. The skin lesion was treat by plastic and reconstructive surgeon with
antimicrobial dressing and hygiene around the wound area was also applied.

CONCLUSION
We reported one case of spondylitis concomitant with scrofuloderma. Establishing
the diagnosis is challenging due to the diversity of the symptoms, clinician
experience, and difficulty in obtaining an adequate sample for confirmation. It
could be made from clinical manifestations such as paresthesia, limping, painless
chronic wound, investigation of radiology such as destruction vertebral body, and
appearance of multinucleated giant cells langhans of wound biopsy when the
proof of bacteriologic confirmation was not found.

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