A 61-year-old male presented with a 2-year history of a tender, erythematous macular skin lesion on his back. Biopsy of the lesion revealed acid-fast bacilli and spindle-shaped cells forming a storiform pattern, consistent with leprosy. Leprosy, also known as Hansen's disease, is caused by Mycobacterium leprae and presents along a clinical spectrum from tuberculoid to lepromatous forms. While treatable with multidrug therapy, leprosy continues to cause disability through inflammatory episodes known as reactions.
2. •CC: 2 years history of an
erythematous macular skin lesion with
well define boarders noted at the Back
area (4x5 cm).
•Nonpruritic, painful (PS 6-7/10)
•No history of fever.
•No lymphadenopathies.
•Tissue Biopsy was done.
• 61 years old
• Male
12. RULE IN RULE OUT
Age: 61 years old
Gender: Male
Area of Lesion: Trunk (Back)
Microscopy: (+) spindle-shaped cells
forming storiform pattern under
microscopy
Signs & Symptoms: (+) tender lesion
(+) erythematous macular rash
(4x5 cm)
(-) tan to brown papules
(+) acid fast bacilli in microscope
Benign Fibrous histiocytoma(Dermatofibroma)
• refers to a heterogeneous family of
morphologically and histogenetically
related benign dermal neoplasms of
uncertain lineage
• Lesions are asymptomatic and
tender
• Usually adult males > 25 years
(range 6 - 84 years)
• Usually extremities, head and neck,
trunk; rarely in deep soft tissue of
retroperitoneum, mediastinum or
pelvis
• These neoplasms appear as firm, tan
to brown papules most are less than 1
cm in diameter
• Microscopy: Prominent storiform
pattern of uniform spindle cells with
ill defined eosinophilic cytoplasm
13. RULE IN RULE OUT
(+) macular lesion on the upper
trunk (back)
(-) short hyphae and spores on
microscopy
Lesion is nonpruritic and
painful
Tinea Vesicolor
• usually occurs on the upper trunk and is
highly distinctive in appearance, may appear
lighter or darker than usual, with mild itching
• Caused by Malassezia furfur
• the lesions consist of groups of macules of
varied size and color with a fine peripheral
scale, not painful
• Microscopy:
Short hyphae and spores (spaghetti and
meatballs) with GMS or PAS stains
Variably pigmented macules of all sizes,
with orthokeratotic hyperkeratosis, yeast
spores and pseudohyphae within stratum
corneum
14. RULE IN RULE OUT
(+) chronic erythematous
macular lesion on the back
which is nonpruritic and
painful
Microscopy:
(+) acid fast bacilli under
microscopy
(+) Inclusion body (Virchow
cells)
(+) spindle cell proliferation
with storiform pattern
(+) dense infiltration of
macrophages
Leprosy
• Chronic cutaneous infection caused
by Mycobacterium leprae and Mycobacterium
lepromatosis
• Also known as Hansen’s disease
• Microscopy:
(+) acid fast bacilli under microscopy
(+) Inclusion body (Virchow cells)
(+) spindle cell proliferation with storiform
pattern
(+) dense infiltration of macrophages
18. Pathogenesi
s
Mycobacterium leprae is an obligate
intracellular gram positive and weakly acid
fast organism
The complexity of presentation
is related to the varied
immunologic responses
The incubation period is
usually 3 - 5 years
19. Pathogenesi
s
Source of Infection and Route of
Transmission: human respiratory
secretions and soil are likely origins.
M. Leprae is taken up by
macrophages and disseminates
in the blood
Replication occurs primarily in
relatively cool tissues of the skin
and extremities
proliferates best at
32° to 34°C
20. Pathogenesi
s
Does not secrete toxins,
Virulence is based on the properties of its
cell wall
Has two different patterns of
disease
a.) tuberculoid
b.) lepromatous
People can also have
intermediate forms of disease,
called borderline leprosy.
21. Clinical
Features
TUBERCULOID LEPROSY LEPROMATOUS LEPROSY BORDERLINE LEPROSY
- begins with localized flat, red skin
lesions that enlarge and develop irregular
shapes with indurated, elevated,
hyperpigmented margins and depressed
pale centers (central healing)
-occurs in individuals with good cell
mediated immunity
-patients develop granulomatous
response
-Nerves become enclosed within
granulomatous inflammatory
Reactions
- On microscopic examination, all sites of
involvement have granulomatous lesions
closely resembling those found in
tuberculosis.
- Has macules, papules and plaques,
but firm nodules may also be seen in
the face
-involves the skin, peripheral nerves,
anterior eye chamber, upper airways
(down to the larynx), testes, hands, and
feet
- Occurs in individuals with poor cell
mediated immunity
- Patients do not develop a
granulomatous response
- Lepromatous lesions contain large
aggregates of lipid-laden macrophages
(lepra cells), often filled with masses
(“globi”) of acid-fast bacilli
- Intermediate form between
tuberculoid and lepromatous leprosy
- Lesions have hypopigmented macules
24. 2 3
Immunohistochemical
markers
TUBERCULOID LEPROMATOUS
- predominance of helper
CD4+ over CD8+
- Rich in interleukin 2 (IL-
2), interferon γ (IFN-γ),
and IL-12
- Scarce in , IL-4, IL-5, and
IL-10
- 2:1 ratio of CD8+ to CD4
- rich in mRNAs for IL-4,
IL-5, and IL-10 and poor
in those for IL-2, IFN-γ,
and IL-12
27. Journal Presentation
ABSTRACT
Despite significant improvements in leprosy (Hansen's
disease) treatment and outlook for patients since the introduction
of multidrug therapy (MDT) 3 decades ago, the global incidence
remains high, and patients often have long-term complications
associated with the disease. In this article, we discuss recent
findings related to genetics, susceptibility, and disease reservoirs
and the implications of these findings for Hansen's disease control
and health outcomes for patients.
28. Journal Presentation
ABSTRACT
We describe the continued difficulties associated with
treatment of inflammatory episodes known as "leprosy reactions,"
which cause much of the disability associated with the disease
and can affect people for many years after MDT is complete. We
also discuss some of the contemporary challenges for physicians
and patients, including international and internal migration of
people affected by the disease. We suggest some important areas
of focus for future Hansen's disease research.
29. Journal Presentation
DISCUSSION
Leprosy, or Hansen’s disease (HD), is an ancient bacterial disease that,
although curable, continues to be a significant health problem in many
parts of the world. HD results from infection with the Mycobacterium
leprae bacillus, which produces a chronic infection in humans that
affects mainly peripheral nerves and skin but may also affect sites such
as the eyes, mucous membranes, bones, and testes and produces a
spectrum of clinical phenotypes.
30. Journal Presentation
DISCUSSION
In the last few decades, particularly with the advent of
multidrug therapy (MDT) and the use of anti-inflammatory
therapies, there have been substantial improvements in long-
term health outcomes for individuals diagnosed with HD.
31. Journal Presentation
DISCUSSION
Although the worldwide prevalence of this disease has
significantly decreased, HD is still a poorly understood illness,
and often, the statistics do not capture the disability and
dysfunction that remain after MDT is complete.
32. Journal Presentation
DISCUSSION
The classification of leprosy should be determined by clinical
prognosis and to distinguish which cases may be potentially
infectious. TheWorld Health Organization suggests a simple
scheme for distinguishing different types of HD, which is used as
the basis of the current treatment model;
33. Journal Presentation
DISCUSSION
in this model, HD is classified based on visible symptoms and
(ideally) the presence or absence of bacilli in slit-skin smears
from cooler regions of the body (generally from earlobes,
elbows, and/or knees) where bacilli proliferate:
34. Journal Presentation
DISCUSSION
those patients with just 1 to 5 diagnostic skin patches and no
apparent bacilli in slit-skin smears are classified as having
“paucibacillary” disease, and those with 5 skin patches and
bacilli visible by microscopic analyses of skin smears are
classified as having “multibacillary” disease. In areas without
access to slit-skin smears, the criterion for diagnosis is the
number of visible lesions.
35. Journal Presentation
DISCUSSION
Gupta et al. found that this fairly arbitrary model based on the
number of lesions that are identifiable can result in both over-
and underdiagnosis of HD; they suggested adding additional
criteria that take into account the size of the lesions and
accompanying nerve enlargement.
36. Journal Presentation
CONCLUSION
HD continues to represent a significant global health problem
and one for which we are still lacking answers for many aspects
of the natural history of the disease. Generating more interest
in and funding for research on HD is an important challenge for
the future.
37. Journal Presentation
CONCLUSION
Another potentially positive step in HD control is the recent
development of a new diagnostic tool, a lateral flow test that
requires a drop of blood to measure the presence of antibodies
to HD bacilli before symptoms appear, specifically through the
use of the M. leprae-specific phenolic glycolipid I (PGL-I)
antigen.
38. Journal Presentation
CONCLUSION
The Mycobacterium bovis BCG (bacillus Calmette-Guérin)
vaccine used to prevent disseminated forms of tuberculosis has
been reported to impart protection against HD in different
populations.
39. Journal Presentation
CONCLUSION
However, we believe that a key public health outcome, along
with decreasing further transmission, is to focus control efforts
on preventing HD-associated disability and to foster
improvements in the quality of life for those affected by HD.
40. References
Robbins and Cotran Pathologic Basis of Diseases 9th edition
Rosai and Ackerman’s Surgical Pathology 11th edition
Harrison’s Principle of Medicine 20th edition
www.pathologyoutline.com
https://pubmed.ncbi.nlm.nih.gov/25567223/
The bright red stains (yellow arrow) depicts acid fast bacilli under Ziehl-Neelsen stain.
Microscopy:
Prominent storiform pattern of uniform spindle cells with ill defined eosinophilic cytoplasm and bland, elongated or plump vesicular nuclei with no atypia
Often hemangiopericytoma-like vasculature
Scattered lymphocytes, either multinucleated giant cells, osteoclastic giant cells or foam cells in 59%
Usually less than 5 mitotic figures / 10 HPF
Stroma is myxoid or hyaline
Borders are non-infiltrative, with no trapping of fat cells
Necrosis or angiolymphatic invasion are rare
Tinea versicolor usually occurs on the upper trunk and is
highly distinctive in appearance. Caused by Malassezia
furfur (a yeast, not a dermatophyte
the lesions consist of groups of macules of varied size and color with a fine
peripheral scale.
The histologic features of dermatophytoses are variable,
depending on the properties of the organism, the host response,
and the degree of bacterial superinfection. There may be
mild eczematous dermatitis associated with intraepidermal
neutrophils (Fig. 25-40B). Due to cell walls rich in mucopolysaccharides,
fungi stain bright pink to red with periodic acid–
Schiff stain. They are found in the anucleate cornified layer of
lesional skin, hair, or nails (Fig. 25-40B, inset). Culture of material
scraped from these areas usually permits the identification
of the offending species.
Hansen disease, is a slowly progressive infection
caused by M. leprae that mainly affects the skin and
peripheral nerves
Microscopy:
Tuberculoid leprosy: epithelioid histiocytes surround small cutaneous nerves; Langhans giant cells may be seen but without necrosis; the infiltrate may involve the papillary dermis up to the epidermis; may destroy arrectores pilorum muscle; bacilli are usually scarce
Lepromatous leprosy: macrophages (Virchow cells, lepra) are found in poorly circumscribed masses in the dermis, with few / no lymphocytes; macrophages may be distended with large groups of leprosy bacilli (globi); bacteria are present in large numbers in cutaneous nerves and in endothelium and media of small and large vessels; may invade arrectores pilorum muscle; may have subcutaneous nodules (erythema nodosum leprorum)
Borderline leprosy: perineural fibrosis with lamellar or onion skin pattern; more circumscription of the granulomatous response, more lymphocytes and closer relationship to nerves
Indeterminate leprosy: scanty superficial and deep lymphohistiocytic infiltrate in the dermis with some tendency to localize around appendages; increased mast cells
Histiocytoid leprosy: spindle cell proliferation with storiform pattern suggestive of fibrous histiocytoma
Lucio phenomenon: leukocytoclastic vasculitis and epidermal infarction
Mycobacterium leprae is an obligate intracellular gram positive and weakly acid fast organism
The complexity of presentation is related to the varied immunologic responses
The incubation period is usually 3 - 5 years
Mycobacterium leprae is an obligate intracellular gram positive and weakly acid fast organism
The complexity of presentation is related to the varied immunologic responses
The incubation period is usually 3 - 5 years
