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Gumma

Bone Gumma (etiology)

Gumma is a form of granuloma that occurs in most cases of tertiary syphilis. This soft benign growth develops mainly in the skin, bones, and liver but may appear in any other organ or tissue in the body.


Presentation

Gumma may develop in the skin, bones, liver, lungs, genitals, heart, blood vessels, brain, spinal cord, and eyes. Hence, the clinical manifestations will arise from affected sites. For example, patients with bone involvement and connective tissue inflammation may suffer from arthritis whereas those with hepatic gumma can develop liver cirrhosis and abdominal pain. Additionally, patients with gumma in the lungs feature wheezing and trouble with breathing.

With regards to skin gumma, these lesions are usually painless. However, since these soft tumors are destructive to deep bones, those with bone involvement are prone to pain that usually worsens at night. Also, sites with prior trauma are susceptible to the development of gummas.

As for the physical appearance, skin gummas may be solitary or in groups. These lesions eventually break down, transform into ulcers and heal with scar formation.

Other

Since patients may have all forms of tertiary syphilis, the clinician should determine the presence of neurologic and cardiovascular signs.

Intravenous Drugs
  • Additionally, high rates of syphilis are observed in the MSM group, intravenous drug users, and the HIV population.[symptoma.com]
Prostitute
  • China and Russia have seen a rise in the number of infections in heterosexuals since the 1990s, which is secondary to prostitution, decreased use of condoms, and promiscuity. With regards to gender, syphilis does not have a preference.[symptoma.com]
Lymphedema
  • Sometimes, however, they can also mimic common pathological processes such as cellulite and lymphedema in the mammary region [ 2 , 3 ]. A major cause of morbidity and mortality in patients with breast cancer is the delay in seeking medical help.[acanceresearch.com]
Pathologist
  • Some pathologists hold that a lesion of tertiary syphilis manifesting itself in the lung usually cannot be considered syphilitic in origin unless it shows definite regression under anti-syphilitic therapy.[pubs.rsna.org]
Sputum
  • On admission, the patient had the following complaints: dyspnea and productive cough with rusty sputum of eight months' duration, and a loss of 40 pounds in weight during that time.[pubs.rsna.org]
Abdominal Distension
  • A week-old baby presented with abdominal distension, small-bowel obstruction, bilious vomiting and a large left upper quadrant mass. Histopathological examination of a specimen of the mass confirmed the clinical diagnosis of gumma formation.[ncbi.nlm.nih.gov]
Bilious Vomiting
  • A week-old baby presented with abdominal distension, small-bowel obstruction, bilious vomiting and a large left upper quadrant mass. Histopathological examination of a specimen of the mass confirmed the clinical diagnosis of gumma formation.[ncbi.nlm.nih.gov]
Tachycardia
  • The features of this reaction are fever, headache, myalgia, and tachycardia, which occur in response to the cytokine cascade triggered by dying treponemes. Management of this reaction is usually done by symptomatic therapy.[symptoma.com]
Hyperkeratosis
  • Examination revealed a punched out ulcer of size 5 X 3 cms with surrounding irregular hyperkeratosis on the left shin [Figure - 1] . Multiple ill defined 'tissue paper' scars were seen around the ulcer.[ijdvl.com]
Anterior Uveitis
  • The left eye showed anterior uveitis but a normal optic disc. He was found to be violently seroreactive for Treponema pallidum infection and was also human immunodeficiency virus (HIV) seropositive.[ncbi.nlm.nih.gov]
Photophobia
  • Two weeks later he noted floaters, photophobia, and blurred vision in the left eye. He presented with unilateral optic disc edema in the right eye. The left eye showed anterior uveitis but a normal optic disc.[ncbi.nlm.nih.gov]
Bone Pain
  • The symptoms will depend on which organs or tissue is affected: Skin gumma causes scarring and disfiguration Bone gumma causes bone pain, especially at night Liver gumma causes cirrhosis and abdominal pain Lung gumma causes wheezing and respiratory trouble[symptoma.com]
Bone Pain
  • The symptoms will depend on which organs or tissue is affected: Skin gumma causes scarring and disfiguration Bone gumma causes bone pain, especially at night Liver gumma causes cirrhosis and abdominal pain Lung gumma causes wheezing and respiratory trouble[symptoma.com]
Neglect
  • In our case we were unable to ascertain the immunological status of the patient and understand if the rapidly fatal progression was related to immunosuppression or just the result of a neglected disease.[acanceresearch.com]
Tremor
  • A 26-year-old man presented with a 4-month history of fever, weight loss, dizziness, diarrhea, tremors, and paresthesia.[ajnr.org]
Extrapyramidal Symptoms
  • Clinical examination revealed disorientation, pyramidal and extrapyramidal symptoms, and multiple cutaneous nonpruriginous cicatricial lesions affecting the chest and inferior limbs, suggestive of syphilitic roseola.[ajnr.org]

Workup

A patient with suspicious lesions and a history of untreated syphilis warrants a full workup that includes medical and sexual history, physical examination, and the appropriate studies.

Laboratory tests

Since syphilis can be diagnosed at any stage, the principal screening tools are the Venereal Disease Research Laboratory (VDRL) and rapid plasma reagin (RPR) tests. The sensitivity of these studies for tertiary syphilis ranges from 95% to 98% while the specificity falls between 85% to 99% [7].

If either of the above yields a positive result, confirmation should be attained by one of the following: fluorescent treponemal antibody-absorption (FTA-ABS) or the micro hemagglutination assay T pallidum (MHA-TP). FTA-ABS has a near 100% sensitivity for detecting later stage syphilis and a 96% specificity [7].

Other

The overall clinical picture and the diagnostic guidelines will further aid the clinician's comprehensive workup. Some cases may be quite confusing and complex as more than 1 subtype of tertiary syphilis can manifest. In order to determine what diseases are present, the clinician may need to perform multiple studies including assessment of the cerebrospinal fluid, computed tomography (CT) scan of the cranium, magnetic resonance imaging (MRI) of the brain and spinal cord, and a cerebral angiogram.

Toxoplasma Gondii
  • However, infectious disease diagnostics were performed on the cerebrospinal fluid that included investigations for syphilis and other microbiological agents such as Toxoplasma gondii.[ncbi.nlm.nih.gov]

Treatment

The CDC treatment guidelines recommend penicillin for all stages of all syphilis. Note that the specific stage dictates the treatment course as later phases will require a longer duration of therapy [8] as determined by expert opinion and the results of uncontrolled trials.

The precise regimen for gumma is benzathine penicillin G, which is divided into 3 weekly doses of 2.4 million units intramuscularly for a cumulative amount of 7.2 million units.

Note that neurosyphilis and cardiovascular syphilis have different management guidelines.

Special considerations

The CDC guidelines advocate for pregnant women with penicillin allergies to undergo desensitization and then treatment with penicillin [9].

There are specific guidelines regarding the management of sexual partners. The criteria determining the treatment regimen for partners include the timing of the exposure, serology titers, and other factors as well [9].

Surveillance

The efficacy of the treatment is monitored by the clinical picture and serologic values. The clinician will obtain VDRL levels at 3, 6, 12, and 24 months post-therapy to ensure cure of the disease.

Other

As gumma may lead to complications such as liver cirrhosis, respiratory trouble, etc., these sequelae require treatment.

Side effects

The Jarisch-Herxheimer reaction may occur within a few hours of initiating treatment for syphilis. While it is more common in those with early syphilis [9], it is still worth mentioning as a potential side effect in patients with gumma. The features of this reaction are fever, headache, myalgia, and tachycardia, which occur in response to the cytokine cascade triggered by dying treponemes. Management of this reaction is usually done by symptomatic therapy.

Prognosis

Gumma and the other types of tertiary syphilis are associated with high risks of morbidity and mortality. The particular sequelae of gumma depend on the affected sites and the overall clinical presentation of the patient which may be complicated by arthritis, liver cirrhosis, and breathing problems. If left untreated, the patients may experience poor outcomes such as liver or respiratory failure.

Generally, untreated syphilis has a fatality rate of 8% to 58% (with more deaths occurring in males). Hence, early treatment is judicious.

Of important note, syphilis elevates the risk for transmission of HIV by 2 to 5 times as evidenced by the high number of patients with co-infections. These individuals tend to have significant risks for complications and death.

Also, syphilis has serious implications perinatally as it affects up to 1.6 million pregnancies annually. This infection leads to miscarriages, stillbirths, and congenital syphilis [6]. Moreover, it accounts for 20% of perinatal deaths in sub-Saharan Africa [6].

Etiology

The organism responsible for syphilis is the spirochete Treponema pallidum, which is exclusively a human pathogen that penetrates mucosal membranes and the skin. Its primary mode of transmission is sexual contact although it can be transferred from mother to fetus and through exposure to infected blood. Note that this organism does not survive disinfectants or drying and therefore cannot be spread through dry surfaces.

Risk factors of syphilis

The group with the highest infection rate is men who have sexual intercourse with men (MSM) [3]. Other risk factors include engaging in unprotected sex, having multiple partners, and intravenous drug use. Additionally, occupational hazards place health care workers at risk.

Epidemiology

While gumma is rare in developed countries, it is common in regions that do not have access to sufficient medical care. Since these populations are unlikely to receive treatment for early syphilis, a significant number (one-third of untreated individuals) progress to develop tertiary syphilis. Furthermore, it estimated that a majority of these late stage cases exhibit gummas.

Epidemiology of syphilis

When describing the epidemiology of syphilis, it is pertinent to understand the demographics of this infection as a whole. For example, this disease is more common in regions with lower socioeconomic status and those with inadequate medical care. In fact, more than 90% of new cases in 1999 occurred in developing areas [4].

Additionally, high rates of syphilis are observed in the MSM group, intravenous drug users, and the HIV population. Furthermore, the MSM group has contributed heavily to the increased number of cases in the US, Canada, Europe (including the United Kingdom), and Australia over the past 15 years. China and Russia have seen a rise in the number of infections in heterosexuals since the 1990s, which is secondary to prostitution, decreased use of condoms, and promiscuity [5].

With regards to gender, syphilis does not have a preference. However, there is a discrepancy in the incidence of this infection in the United States as men exceeded women by 6 times as of 2007 whereas the rates in both genders were equal a decade earlier.

Sex distribution
Age distribution

Pathophysiology

Approximately one-third of untreated patients will develop tertiary syphilis, which can be classified into 3 different classes: gummatous syphilis, late neurosyphilis, and cardiovascular syphilis [1] [2]. Gumma, or late benign phase, can develop 1 to 46 years post initial infection (the mean is 15 years).

Gummas emerge as a response to the spirochetes that have entered the tissue.

A gumma is a type of granuloma that is characterized as soft, inflamed tissue that can form in the liver, skin, bone, heart, testes and/or any other tissue or organ [1]. These tumor-like balls vary in size and typically contain a necrotic center. Moreover, histopathological analysis of these lesions reveals macrophages and fibroblasts, with plasma cells at the margins. Treponemes are not usually observed in gummas. Eventually, these gummas ulcerate and become fibrotic.

Prevention

Preventative measures include educating and counseling patients on safe sex practices such as the use of latex condoms and limiting number of sexual partners. Also, infected individuals are advised to disclose their status to all partners. It is crucial that all infected individuals and their exposed partners receive the appropriate treatment.

Patients who engage in intravenous drug use should not share needles.

Additionally, healthcare workers should exercise universal precautions to avoid exposure to infected blood.

Note that screening is essential in high-risk communities like MSM. Additionally, pregnant women should be screened as well.

There is speculation about the role of circumcision in the prevention of syphilis. Two studies have not proven so although this procedure may prevent the spread of viral sexually transmitted infections [10] [11].

Summary

Gumma is a soft, benign growth that emerges in tertiary syphilis. This granulomatous lesion predominantly forms in the skin, long bones, and liver although it may arise in any location of the body [1], including the lungs, heart, brain, spinal cord, testes, and eyes. The clinical signs and symptoms of patients with this disease reflect the affected organs or tissues. There are potentially severe complications such as liver cirrhosis, respiratory failure, arthritis, etc.

While the noninfectious third stage of syphilis is quite rare, it occurs in one-third of untreated patients [1] [2]. Tertiary syphilis also has two other forms, which are cardiovascular syphilis and neurosyphilis. Patients may have features of all 3 types while gumma appears in almost all individuals with tertiary syphilis.

The diagnosis is achieved by a thorough assessment of the patient's history, physical examination, and the relevant diagnostic studies such as serology and other tools. Once gumma is diagnosed, the patient is treated with a preferred regimen of penicillin.

Successful treatment of primary syphilis prevents its progression to gumma and other late manifestations. But the best preventative strategy overall is to avoid contracting syphilis altogether. Hence, it is essential to implement the practice of safe sex and to follow other preventive methods.

Patient Information

What is tertiary syphilis?

Syphilis is a sexually transmitted disease caused by the bacteria known as Treponema pallidum. If the early stages of syphilis are not treated, the patient can go on to develop tertiary syphilis, which comes in 3 forms: Gumma, neurosyphilis, and cardiovascular syphilis.

What is gumma?

This is a soft, noncancerous growth that may form in the skin, bones, liver, heart, brain, spinal cord, testes, eyes, and any other location as well.

Who develops this disease?

Those in developing and underdeveloped areas may lack access to medical care. Hence, if their early syphilis infections are not treated, they are at risk to develop gumma.

What are the signs and symptoms?

The symptoms will depend on which organs or tissue is affected:

How is gumma diagnosed?

There are 2 screening tools used initially: VDRL and RPR tests. If they are positive, further testing is needed to confirm the diagnosis. The confirmatory studies are the FTA-ABS and the MHA-TP.

The clinician may need to perform other tests to determine if neurosyphilis and cardiovascular syphilis are present as well.

How is it treated?

Penicillin is the recommended treatment for syphilis. The different stages of syphilis have different regimens. In the case of gumma, the regimen consists of 3 weekly intramuscular injections each consisting of 2.4 million units of benzathine penicillin G.

Pregnant women with penicillin allergies should undergo penicillin desensitization and then penicillin treatment.

In order to ensure that the treatment is successful for all patients, follow-up blood titers should be obtained at 3, 6, 12, and 24 months after therapy.

How is it prevented?

To prevent gumma and the other types of tertiary syphilis, patients with early stages of this infection should be treated as soon as possible. However, the main goal is to prevent contracting and transmitting syphilis altogether. Education about safe sex practice is very important. All sexually active individuals should use latex condoms every time they engage in sexual intercourse. Also, they should limit the number of sexual partners and disclose their infection status to partners.

Furthermore, all patients at risk for syphilis and those who have been exposed to the disease must see a healthcare professional for testing and of course treatment if needed. Note that pregnant woman should undergo screening for syphilis.

Since syphilis can be contracted through infected blood, intravenous drug users should never share needles.

Finally, healthcare workers should practice universal precautions to avoid exposure to infected blood.

References

Article

  1. Kent ME, Romanelli F. Reexamining syphilis: an update on epidemiology, clinical manifestations, and management. Annals of Pharmacotherapy. 2008;42(2):226-36.
  2. Bhatti MT. Optic neuropathy from viruses and spirochetes. International Ophthalmology Clinics. 2007;47(4):37-66, ix.
  3. Patton ME, Su JR, Nelson R, Weinstock H, Centers for Disease Control and Prevention (CDC). Primary and secondary syphilis--United States, 2005-2013. MMWR Morbidity and Mortality Weekly Report. 2014; 63(18):402-6.
  4. Stamm LV. Global challenge of antibiotic-resistant Treponema pallidum. Antimicrobial Agents and Chemotherapy. 2010;54(2):583-9.
  5. Ficarra G, Carlos R. Syphilis: the renaissance of an old disease with oral implications. Head and Neck Pathology. 2009; 3(3):195-206.
  6. Woods CR. Congenital syphilis-persisting pestilence. Pediatric Infectious Disease Journal. 2009;28(6):536-7.
  7. U.S. Preventive Services Task Force. Screening for Syphilis Infection. Recommendation Statement. 2004.
  8. Ghanem KG, Workowski KA. Management of adult syphilis. Clinical Infectious Diseases. 2011; 53 Suppl 3:S110-28.
  9. Workowski KA, Berman S. Sexually transmitted diseases treatment guidelines, 2010. MMWR Recommendations and Reports. 2010; 59:1-110.
  10. Golden MR, Wasserheit JN. Prevention of viral sexually transmitted infections--foreskin at the forefront. New England Journal of Medicine. 2009; 360(13):1349-51.
  11. Tobian AA, Serwadda D, Quinn TC, et al. Male circumcision for the prevention of HSV-2 and HPV infections and syphilis. New England Journal of Medicine. 2009; 360(13):1298-309.

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Last updated: 2019-07-11 22:55