Papulonecrotic Tuberculids
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The term tuberculid was first described by Darier in 1896. [1] Subsequently, in 1936, Pautrier established the diagnosis of papulonecrotic tuberculid as a distinct tuberculosis-associated disorder.
Although many types of tuberculids have been described, most are now understood to not be uniquely caused by tuberculosis. However, papulonecrotic tuberculids (PNTs) and lichen scrofulosorum are still widely accepted as true tuberculids.
Papulonecrotic tuberculid is a chronic, recurrent, and symmetric eruption of necrotizing skin papules arising in crops, involving primarily the buttocks and extensor surfaces of the arms and legs. [2] A hallmark of this condition is that lesions heal with varioliform and pitting scarring. The eruption is believed to represent a hypersensitivity reaction to tuberculosis antigens released from a distant focus of infection. Most patients react markedly to the Mantoux skin test (purified protein derivative [PPD]), may exhibit other evidence of current or past tuberculosis infection, and react quickly to antituberculous therapy. [3, 4]
The pathophysiology of papulonecrotic tuberculid is controversial. Most authors believe this disease entity is triggered by an initial Arthus reaction to mycobacterial antigens. This is then followed by a hypersensitivity reaction in which antigens undergo opsonization by antibodies, followed by immune complex deposition in small cutaneous blood vessels. The ensuing complement cascade triggers a leukocytoclastic vasculitis, leading to destruction of vessel walls with ensuing tissue necrosis. Over time, this type III hypersensitivity reaction may be replaced by a type IV reaction. Other authors dispute this mechanism, citing the lack of leukocytoclastic vasculitis in some cases. Instead, they propose that the primary lesion is the result of subacute lymphohistiocytic vasculitis that leads to thrombosis and subsequent tissue necrosis.
A consensus has been reached that papulonecrotic tuberculid represents a true hypersensitivity reaction rather than the result of a local cutaneous tuberculosis infection. This is based on the observation that papulonecrotic tuberculid lesions have consistently failed to either stain positive for, or culture out, mycobacterial organisms. Although the organisms are absent, mycobacterial DNA has been detected in approximately half of the biopsy specimens subjected to polymerase chain reaction (PCR). [3, 5] These observations support the idea that lesions of papulonecrotic tuberculid are the result of released mycobacterial antigens in the setting of a concurrent but distant infection.
The eruption is a form of an exaggerated host immunologic response to a mycobacterial infection involving the cutaneous vessels. Active tuberculosis is reported in as many as 40-75% of patients, [6] most commonly in lymph nodes. Polymerase chain reaction (PCR) amplification for mycobacteria varies. In a series of 12 cases in India, PCR positivity was found in 25% of the cases. [5]
United States
A decreasing incidence of papulonecrotic tuberculid began in the second half of the 1900s and the decline continues to this day. This phenomenon is attributed to aggressive tuberculosis control now found in wide practice. Currently, almost all cases come from areas outside of North America with high endemic rates of tuberculosis. [7] Historically, reports indicate that young women and children are especially susceptible to this disorder. [2]
Papulonecrotic tuberculid–like lesions have also been associated with other mycobacterial infections, including Mycobacterium bovis and Mycobacterium kansasii, and from BCG vaccination. [8]
International
Papulonecrotic tuberculid is an uncommon disorder even in populations with a high prevalence of tuberculosis, occurring in less than 5% of active tuberculosis cases. In the literature, 91 cases were reported during a 17-year period in South Africa in 1974. In addition, 12 cases from a period of longer than 30 years in England were reported in 1986. In the latter study, most patients were immigrants and had presumably acquired the infection outside of England. The incidence of tuberculosis is expected to rise as the influx of refugees from endemic areas like Africa and the Middle East continues to grow. [9] Freiman et al reported a case in a 25-year-old woman from the Philippines who had immigrated to Canada 8 years previously. [10] In a 9-year period, 4 cases were reported in Hong Kong, which was the least common of the cutaneous tuberculosis presenting during that time. [11] In a 5-year period, 12 cases were reported in India, a country that has the highest burden of tuberculosis in the world. [5, 12]
Females seem to be at a slightly increased risk for developing this disorder.
Children and young adults are more susceptible to this condition than other people. Papulonecrotic tuberculids represent 4% of pediatric skin tuberculosis. [5] In a 1974 study from South Africa, two thirds of the patients were younger than 30 years.
Without treatment, patients typically follow a waxing and waning course lasting many years, although spontaneous resolution has been reported.
Papulonecrotic tuberculids respond promptly to appropriate antituberculosis therapy. New lesions cease forming within days to weeks after therapy is initiated, and existing lesions heal rapidly, usually within weeks. Recurrences are rare after appropriate therapy.
Significant varioliform or pitted scarring is a sequela, and progression to lupus vulgaris has been observed. [13, 14] An association with Takayasu arteritis of the aortic arch has also been documented. [15]
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Wong S, Rizvi H, Cerio R, O’Toole EA. An unusual case of vulval papulonecrotic tuberculid. Clin Exp Dermatol. 2011 Apr. 36 (3):277-80. [Medline].
Braun-Falco O, Thomas P. [The tuberculid concept from the current viewpoint]. Hautarzt. 1995 Jun. 46 (6):383-7. [Medline].
Saffari M, Jolandimi HA, Sehat M, Nejad NV, Hedayati M, Zamani M, et al. Smear grading and the Mantoux skin test can be used to predict sputum smear conversion in patients suffering from tuberculosis. GMS Hyg Infect Control. 2017. 12:Doc12. [Medline].
Tirumalae R, Yeliur IK, Antony M, George G, Kenneth J. Papulonecrotic tuberculid-clinicopathologic and molecular features of 12 Indian patients. Dermatol Pract Concept. 2014 Apr. 4(2):17-22. [Medline]. [Full Text].
Asiniwasis R, Dutil MT, Walsh S. Molluscum-Like Papules as a Presentation of Early Papulonecrotic Tuberculid in Association with Nodular Tuberculid in a Male with Asymptomatic Active Pulmonary Tuberculosis. J Cutan Med Surg. 2014 Aug 1. 18(0):1-4. [Medline].
Sloan JB, Medenica M. Papulonecrotic tuberculid in a 9-year-old American girl: case report and review of the literature. Pediatr Dermatol. 1990 Sep. 7(3):191-5. [Medline].
Iden DL, Rogers RS 3rd, Schroeter AL. Papulonecrotic tuberculid secondary to Mycobacterium bovis. Arch Dermatol. 1978 Apr. 114(4):564-6. [Medline].
Lillebaek T, Andersen AB, Dirksen A, Smith E, Skovgaard LT, Kok-Jensen A. Persistent high incidence of tuberculosis in immigrants in a low-incidence country. Emerg Infect Dis. 2002 Jul. 8 (7):679-84. [Medline].
Freiman A, Ting P, Miller M, Greenaway C. Papulonecrotic tuberculid: a rare form of cutaneous tuberculosis. Cutis. 2005 Jun. 75(6):341-6. [Medline].
Vashisht P, Sahoo B, Khurana N, Reddy BS. Cutaneous tuberculosis in children and adolescents: a clinicohistological study. J Eur Acad Dermatol Venereol. 2007 Jan. 21 (1):40-7. [Medline].
World Health Organization. Global tuberculosis report 2016. Available at http://apps.who.int/iris/bitstream/10665/250441/1/9789241565394-eng.pdf?ua=1. 2016; Accessed: January 29, 2017.
Morrison JG, Fourie ED. The papulonecrotic tuberculide. From Arthus reaction to lupus vulgaris. Br J Dermatol. 1974 Sep. 91(3):263-70. [Medline].
Senol M, Ozcan A, Aydin A, Karincaoglu Y, Sasmaz S, Sener S. Disseminated lupus vulgaris and papulonecrotic tuberculid: case report. Pediatr Dermatol. 2000 Mar-Apr. 17(2):133-5. [Medline].
Lim AY, Lee GY, Jang SY, Gwag HB, Choi SH, Jeon ES, et al. Comparison of clinical characteristics in patients with Takayasu arteritis with and without concomitant tuberculosis. Heart Vessels. 2016 Aug. 31 (8):1277-84. [Medline].
Milligan A, Chen K, Graham-Brown RA. Two tuberculides in one patient–a case report of papulonecrotic tuberculide and erythema induratum occurring together. Clin Exp Dermatol. 1990 Jan. 15(1):21-3. [Medline].
Thappa DM, Karthikeyan K, Jayanthi S. Tuberculid in a child: transformation from papulonecrotic to lichen scrofulosorum. Pediatr Dermatol. 2003 Jan-Feb. 20(1):91-3. [Medline].
Chalermdamrichai P, Puavilai S, Jerasutus S, Boonsarngsuk V, Kiattboonsri S, Suwatanapongched T. Sarcoidosis presenting as papulonecrotic tuberculid-like lesions: report of a case. J Med Assoc Thai. 2004 Jul. 87(7):839-44. [Medline].
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Çavuşoğlu C, Durusoy R, Yaşar M, Kayın M. [Evaluation of QuantiFERON®-TB Gold in Tube Test and Tuberculin Skin Test in the diagnosis of Mycobacterium tuberculosis infection]. Mikrobiyol Bul. 2017 Apr. 51 (2):115-126. [Medline].
Puri N. A clinical and histopathological profile of patients with cutaneous tuberculosis. Indian J Dermatol. 2011 Sep-Oct. 56(5):550-2. [Medline]. [Full Text].
[Guideline] World Health Organization. Recommendations for Investigating Contacts of Persons with Infectious Tuberculosis in Low- and Middle-Income Countries. Available at http://apps.who.int/iris/bitstream/10665/77741/1/9789241504492_eng.pdf. Accessed: October 23, 2014.
Manuel Valdebran, MD Junior Specialist Physician, Visiting Scholar in Dermatology/Dermatopathology, Department of Dermatology, University of California, Irvine, School of Medicine
Manuel Valdebran, MD is a member of the following medical societies: International Dermoscopy Society, Medical Dermatology Society, Society for Pediatric Dermatology
Disclosure: Nothing to disclose.
Logan William Thomas University of California, Irvine, School of Medicine
Disclosure: Nothing to disclose.
Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology
Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association
Disclosure: Nothing to disclose.
Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory
Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association
Disclosure: Nothing to disclose.
William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine
William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology
Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.
David Barnette Jr, MD Voluntary Associate Clinical Professor, University of California San Diego School of Medicine
David Barnette Jr, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology
Disclosure: Nothing to disclose.
Robert L Chen, MD, PhD Instructor, Department of Medicine, Section of Dermatology, University of Chicago Medical Center
Robert L Chen, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Dermatology Foundation, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.
Papulonecrotic Tuberculids
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