Granulomatous Diseases - Derma

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GRANULOMATOUS TUBERCULOSIS

SKIN DISEASES & SARCOIDOSIS


OUTLINE
Introduction
Classification
Tuberculosis
Sarcoidosis
Conclusion
References
INTRODUCTION
“Granulomatous diseases” refers to
diseases or inflammatory reactions that are
characterized by the formation of a
granuloma.
Granuloma is a distinct type of chronic
inflammation characterized by microscopic
aggregation of activated macrophages
(epithelioid cells) surrounded by a rim of
lymphocytes and central necrosis with or
without giant cells.
INFECTIOUS
GRANULOMATOUS DISEASES
Tuberculosis
Leprosy
Histoplasmosis
Cryptococcosis
Coccidioidomycosis
Blastomycosis
Cat Scratch disease
NON-INFECTIOUS
GRANULOMATOUS DISEASES
Sarcoidosis
Crohn’s disease
Berylliosis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
TUBERCULOSIS
It is caused by Mycobacterium tuberculosis
RISK FACTORS
Contact with infectious HIV positive
Diabetes mellitus
patient
Prolonged corticosteroid/
Healthcare worker immunosuppressive therapy.
Homeless or unstably
housed
From high prevalence
country
TRANSMISSION
It is transmitted via droplet,when an infected
person coughs, sneezes, talks or sings.
Droplet infection are 0.5- 5 micrometers and
contain 1-400 Mycobacterium tuberculosis
bacilli.
PATHOPHYSIOLOGY OF
PRIMARY TB
Initial infection by tubercle bacillus
Symptomatic in immunosuppressed host;
often seen in children, elderly, HIV infected
individuals.
It is usually subpleural, often in the mid to
upper zones
After inhalation, the droplet nucleus is
carried down the bronchial tree to a
respiratory bronchiole or alveolus, where it is
PATHOPHYSIOLOGY
A calcified tubercle in the middle or lower
lung zone is called Ghon focus. A Ghon focus
accompanied by perihilar lymph node is called
Ghon complex.
Ghon focus(primary infection site in lungs)
Bacilli are ingested by macrophages and
may often lead to the clearance of the lesion
PATHOPHYSIOLOGY
The development of cellular immunity
(delayed-type hypersensitivity) during the
next 4 weeks(3–8 weeks)
It results in a positive reaction in the skin to
an intradermal injection of protein from
tubercle bacilli (tuberculin/PPD).
SYMPTOMS
Asymptomatic
When active, 75% are pulmonary
Chronic Cough (>3wks)
Weight loss
Night sweats
Chest pain
Fever
CUTANEOUS TB
Tuberculosis of the skin constitutes about
10% of all extra-pulmonary tuberculosis which
in turn constitutes only a fraction of all cases
of tuberculosis.
It is an uncommon form of extrapulmonary
TB
CUTANEOUS TB
CLASSIFICATION
Inoculation tuberculosis Tuberculous chancre
(exogenous source) Tuberculosis verrucosa cutis
Lupus vulgaris (some).
Secondary tuberculosis
(endogenous source) Scrofuloderma
a) Contiguous spread Orificial tuberculosis.
b) Auto-inoculation

Hematogenous tuberculosis Acute miliary tuberculosis


Lupus vulgaris (some)
Tuberculous gumma
Eruptive tuberculosis (tuberculids)
a) Micropapular Lichen scrofulosorum
b) Papular Papulonecrotic tuberculids
c) Nodular Erythema induratum of Bazin,
nodular tuberculid.
TUBERCULOUS CHANCRE
It occurs due to inoculation of M.
tuberculosis into the skin of an individual
without natural or acquired immunity to
tubercle bacilli.
It is usually seen in face or limbs of children
Presents as a brownish red papule or nodule
that ulcerates to form a ragged ulcer with
undermined edge
It is associated with regional
WARTY TUBERCULOSIS
It is also called Tuberculous verrucosa
cutis or Prosector’s wart
It affects people with moderate or high level
of immunity.
Lesions occur in exposed areas such as foot,
hand, ankle and rarely buttock.
The lesion begins as a small papule, which
becomes hyperkeratotic resembling a wart.
The warty firm plaque is reddish brown and
SCROFULODERMA
Skin lesions result from the direct extension
of a tuberculous process into the skin from an
underlying lymph node, bone or joints, or
lacrimal gland or duct.
It is most found in the neck from cervical
adenitis.
It manifests as bluish red nodules overlying
lymph nodes or joints that break down to form
undermined ulcers with a bluish edge
Clinically it is a deep, cutaneous swelling,
firmly attached to the skin with multiple
discharging sinuses, interspersed with cord-
like scars.
Scrofuloderma
—ruptured
bluish nodules
with
undermined
edges in the
axilla
TUBERCULOSIS CUTIS
ORIFICIALIS
It is a TB infection of the mucosa or the skin
adjoining orifices in a patient with advanced
internal TB.
Any of the orifice- mouth, genitalia and anus
could be involved.
TUBERCULOSIS CUTIS
ORIFICIALIS
The affected patient is usually an adult with
poor general health and impaired cell mediated
immunity, who has long-standing TB of one or
more internal organ

The multiple crusted small ulcers usually


commence as edematous red nodules which
break down. The ulcer are classically chronic with
no tendency to heal spontaneously.
MILIARY TUBERCULOSIS
Miliary TB occurs due to hematogenous
dissemination of tuberculosis in infants and
children or immunosuppressed
The skin lesions are varied- may manifest as
crops of bluish papules, vesicles, pustules or
hemorrhagic lesions
Diagnosis established by skin biopsy which
shows acid fast bacilli
The primary source of TB should be
LUPUS VULGARIS
It is most common type of progressive
cutaneous TB which occurs in a person with a
moderate or high degree of immunity.
The initial lesion is a reddish-brown, soft,
gelatinous plaque which increases in size and
extends peripherally which occurs with
resultant central scarring.
Face and buttocks are the most common
site.
LUPUS VULGARIS
Diascopy reveals ‘apple jelly’ nodules in the
periphery
The various morphological forms are plaque
type, ulcerative and mutilating form,
vegetative form and tumor like.
Nasal mucosa can be involved leading to
resultant destruction of nasal septum.
Rarely squamous cell carcinoma can occur.
TUBERCULIDS
Tuberculids are recurrent, disseminated and
symmetrical skin eruptions which have a
tendency for spontaneous involution.
They are regarded as hypersensitivity
sequelae of tuberculosis
The etiology is poorly understood however it
has been suggested that they might be due to
soluble bacterial toxins.
TUBERCULIDS
They do not contain acid fast organisms and
histology may show varying degrees of
epithelioid cell granulomas
Examples are
i. Lichen scofulosorum,
ii. Papulonecrotic tuberculid and
iii. Erythema induratum
LICHEN SCROFULOSORUM
Found in children
with systemic TB
Appear as
symptomless
papules(0.5-3mm)
Follicular in
distribution & often
occur in groups.
Occur majorly on the
PAPULONECROTIC
TUBERCULID
It occurs as a chronic and recurrent symmetric
eruption of necrotizing skin papules appearing in
clusters and may affect the face, trunk and
extremities.
Later, a central black discoloration occurs due to
necrosis.
Usually affects adolescents and young adults
Tuberculin test is usually strongly positive
Skin lesions resolve after anti-TB therapy.
ERYTHEMA INDURATUM
A persistent or recurring condition
associated a focus of secondary TB elsewhere
in the body. They are nodular lesions that may
ulcerate and occur in the calf.
More common in women (<10% of affected
patients are men).
ERYTHEMA INDURATUM
Lesions arise in small numbers as tender
nodules that may progress to ulceration and
scarring.
Tubercle bacilli are not seen, and
mycobacterial cultures usually are negative.
Erythema induratum
—erythematous
indurated tender
noduloplaque lesion
over the leg
INVESTIGATIONS
Skin Biopsy
Microscopy (tuberculoid granulomas)
Bacteriological culture (Lowenstein Jensen
medium)
Detection of mycobacterial DNA by PCR.
Tuberculin skin tests.
Diascopy
40
TUBERCULIN SKIN TEST
It is also called Mantoux test or Mendel-
Mantoux test.
It is a screening tool for tuberculosis and for
tuberculosis diagnosis.
PROCEDURE
The test is done injecting 0.1ml of a liquid
containing 5 tuberculin unit(TU) purified protein
derivative(PPD) into the top layers of the skin .
The skin test should be read 2-3 days after the
injection .The diameter of hard skin thickening is
recorded in mm.
It is injected intradermally usually in the
forearm region
RESULTS
Induration measuring 5mm or more is
considered positive;
i. for HIV-infected patient ,
ii. in those with risk of developing TB,
iii. in recent close contact or
iv. in those with chest x-ray finding with
fibrotic changes.
PPD measuring > 10mm considered positive
in;
i. Intravenous drug user
ii. HIV negative
iii. Those born in countries of high prevalence.
Induration greater than 15mm is positive for
all others.
0-4mm is negative
TREATMENT
Anti tubercular therapy with four drugs is
given for a period of 6 to 9 months based on
the type of tuberculosis.
Isoniazid 300mg/day
Rifampicin 600mg/day
Pyrazinamide 25mg/kg with max dose of 2g daily
Ethambutol 15-25mg/kg
Directly observed therapy short course
Then isoniazid and rifampicin for a further 4-7
months
ANTI TB DRUGS
i. Aminoglycosides: e.g., amikacin, kanamycin
ii. polypeptides: e.g., capreomycin, viomycin,
enviomycin
iii. Fluoroquinolones: e.g., ciprofloxacin,
ofloxacin, levofloxacin, moxifloxacin
iv. Thioamides: e.g. ethionamide,
prothionamide
v. cycloserine
SURGICAL TREATMENT
The role of surgery in cutaneous TB is
limited.
Hypertrophic and verrucous lesion of Lupus
vulgaris and TB verrucosa have been treated
with electrosurgery, cryosurgery and
curettage with electrodissection as an adjunct
measure with pharmacologic therapy as the
primary method of treatment.
PREVENTION
BCG vaccination: single intradermal injection
at insertion of deltoid. Given to babies
immediately after birth. Should not be given
to immunocompromised individuals and
people with positive tuberculin test. Dose is
0.1mg in 1ml.
Improved social conditions: housing,
nutrition, pasteurization of milk.
Case detection and treatment, contact
ATYPICAL MYCOBACTERIA
Atypical mycobacteria can present with
varied cutaneous features in normal as well as
immunosuppressed patients.
Atypical mycobacteria may cause septic
arthritis, abscesses and skin and bone
infection. They may also affect the lungs,
gastrointestinal tract, lymphatic system and
other parts of the body.
M. MARINUM
It causes swimming pool granuloma or fish
tank granuloma
Infects previously abraded or damaged skin
Usually elbows, knees of swimmers and
hands of aquarists
Incubation period is about 3wks
Rifampin and ethambutol or tetracycline
53
M. ULCERANS
 It causes Buruli ulcers which is a chronic,
indolent, necrotizing disease of the skin and
soft tissue
 It is usually a solitary lesion
 The initial lesion is a closed, diffuse &
painless swelling, painless dermal papule or
subcutaneous nodule (pre-ulcerative phase)
 This then breaks down to form a necrotic
ulcer with extensively undermined edges
M. ULCERANS
 Area of tissue destruction extending further
under the skin far beyond the visible edge of
the ulcer. The ulcer lacks exudate
 Skin and soft tissue necrosis can be
extensive involving as much as 15% of skin
surface and may extend deep, exposing
fascia, muscle and bone
B. ULCERS MAY DESTROY NERVES AND
BLOOD VESSELS.
TREATMENT
 Excision and closure in the pre-ulcerative
phase
 Rifampicin or cotrimoxazole in the anergy
phase
 Thermostatically controlled heating of the
affected limb
 This conserves viable tissue & promotes
repair

SARCOIDOSIS
It is also known as Besnier-Boeck-
Schaumann disease
Multisystemic granulomatous inflammatory
diseases.
It runs an acute or persistent course
interrupted by remissions and relapses.
In addition to the skin, which is involved in
about 25% of cases, other sites of
involvement are the lungs, mediastinal and
peripheral lymph nodes, eyes phalangeal
bones, myocardium, CNS, kidney, spleen, liver
and parotid gland.
SARCOIDOSIS
Cutaneous involvement in sarcoidosis may
be classified as specific, which reveals
granuloma on biopsy, or non specific, which
is mainly reactive, such as erythema
nodosum.
EPIDEMIOLOGY
Affects all age, race and gender although
incidence varies
Commoner in those of black ancestry;
African Americans
worldwide incidence of 16.5/100,000 in men
& 19/100,000 in women
CLINICAL FEATURES
May be asymptomatic
Constitutional symptoms; fever, fatigue, weight loss
Pulmonary symptoms; Dry hacking cough, dyspnea,
chest pain, chest tightness
Ocular symptoms; Anterior uveitis
Neurosarcoidosis
Lymphadenopathy
Skin lesions(20-30%)
SARCOIDOSIS MORPHOLOGY
They may include papules, nodules,
plaques, subcutaneous nodules, scar
sarcoidosis, erythroderma and ulcerations.
The skin lesion may appear before the
systemic disease or may occur simultaneously
or several years after the systemic disease.
SKIN LESIONS IN
SARCOIDOSIS
Erythema nodosum (characteristic of acute
benign sarcoidosis)
Lupus pernio and plaques are characteristic
of chronic severe systemic disease
Papules, nodules, plaques.
Scar sarcoid (secondary lesion)
ERYTHEMA NODOSUM
Usually an acute, self limiting process
Occurs in early stages especially in young
women
Painful red nodules(1-5cm)
Found on the shins
LUPUS PERNIO
It is pathognomonic of sarcoidosis
Presents as red to purple or violaceous,
infiltrated plaques and nodules
Usually over the nose, ears, lips, cheeks but
it can appear on the dorsa of the hands,
fingers, toes and forehead
LUPUS PERNIO
Often associated with sarcoidosis of the
upper respiratory tract.
Course is usually chronic, and may result in
severe cosmetic disfigurement
LOFGREN SYNDROME
An acute clinical presentation of systemic
sarcoidosis consisting of a triad of fever,
Erythema nodosum and bilateral hilar
lymphadenopathy.
Other features include anterior uveitis, fever,
ankle periarthritis, arthralgia and pulmonary
involvement
It is an acute disease with an excellent
prognosis, typically resolving spontaneously
PAPULES,NODULES,PLAQUES
They appear as hypopigmented, itchy
solitary or multiple brownish red in color
They are usually asymptomatic
They are of varying sizes, numbers
&distribution
Seen on face, shoulder, arms
Resolves with hyperpigmentation
PLAQUES ON CHEEK
LABORATORY FINDINGS
 Histology; naked non-caseating granulomas
 Islands of epithelioid cells may contain a few
langerhans giant cells which may contain
asteroid or schaumann bodies
 Kveim test is positive; most specific.
INVESTIGATIONS
Diagnosed by RFTs
exclusion Serum calcium
CXR 24-hr urine calcium
ECG Tissue biopsy of LNs
Ocular exam Chest CT scan
LFTs
TREATMENT
About 30-70% of patients do not require
therapy
NSAIDs e.g. Ibuprofen, Aspirin.
Corticosteroids
Immunosuppressants; Methotrexate (SE-
hepatotoxicity), Azathioprine(SE- bone marrow
suppression, vomiting), Hydroxychloroquine
(SE- hepatotoxicity, alopecia, vomiting)
CONCLUSION
Granulomatous skin diseases are not usually
common but due to the rising incidence of
HIV/AIDS and other immunosuppressed
states, there is increased incidence of these
diseases.
Early treatment is usually preferred to
reduce associated disabilities.
REFERENCES
DM Thappa Essentials in Dermatology
Textbook of Skin Diseases and Sexually
Transmitted Infections by Yetunde Mercy
Olumide
Dermnet
Tuberculosis and non Tuberculosis
Mycobacterial infections by Davide
Schlossberg.

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