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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
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.