Atypical cutaneous tuberculosis with an unusual course
Zineb Zeggwagh1, Hasna Azendour1, Kaoutar Znati2, Karima Senouci1
1 Department of Dermatology and Venerology, Ibn Sina Hospital, Mohammed V University in Rabat, Rabat, Morocco
2 Department of Anatomopathology, Ibn Sina Hospital, Mohammed V University in Rabat, Rabat, Morocco
Correspondence Address:
Zineb Zeggwagh
Department of Dermatology and Venerology, Ibn Sina Hospital, Mohammed V University in Rabat, Rabat
Morocco
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/ijmy.ijmy_4_23
Cutaneous tuberculosis represents the fifth extrapulmonary form in Morocco after pleural, lymph node, urogenital, and intestinal tuberculosis. It is recognized to form a continuous immunopathologic spectrum, ranging from a high intensity to a low intensity of cell-mediated immunity, which explains the multiplicity and heterogeneity of anatomo-clinical forms. Association of multiple forms in the same patient is really rare. In this regard, we report a case of scrofuloderma on axillary tuberculosis adenitis associated to a lupus vulgaris in an immunocompetent patient, which was confirmed by histology, QuantiFERON-TB Gold test, and polymerase chain reaction. He received an antituberculous therapy with clinical regression of the lesions. In conclusion, cutaneous tuberculosis is still endemic in developing countries.The diagnosis is difficult because of its clinical polymorphism. That's why it's should be suspected clinically in the presence of any destructive or verrucous skin lesion evolving without healing for a long period and confirmed by bacteriological examinations and histology.
Keywords: Cutaneous tuberculosis, lupus vulgaris, multifocal tuberculosis, scrofuloderma
Tuberculosis is an infectious disease, systemic contagious and nonimmunizing due to Mycobacterium tuberculosis. The pulmonary involvement occupies the first place.[1] Cutaneous tuberculosis is rare, representing only 1%–2% of all extra-pulmonary cases.[2]
The clinical forms are numerous and depend on the mode of inoculation, the quantity of bacterial inoculum as well as the immune status of the patient.
The association of different anatomo-clinical forms of cutaneous tuberculosis in the same patient is exceptional. There are only three cases in the literature describing the association between scrofuloderma and lupus vulgaris.[3]
Herein, we present the fourth case of scrofuloderma associated with lupus vulgaris.
Case ReportA 43-year-old man whose consent form we obtained, with no medical history, was admitted to our hospital for two painless ulcerative tumors, one in his right armpit and the second one in his right wrist, progressively evolving for 30 years and increasing in size. The patient received a lot of antibiotics, without any improvement. Twenty-eight years later, he reported the appearance of new nodules on his right cheek, left arm, and right thigh. He had no previous history of immunosuppressive therapy, weight loss, fever, or cough.
Dermatologic examination revealed in the right armpit, an irregular, firm, purple erythematous ulcerative tumor, measuring 10 cm of diameter with a purple nodule on its upper part [Figure 1]. In the right wrist, he presented an irregular, firm, purple erythematous eroded tumor, measuring 7 cm of diameter [Figure 2]. Three erythematous, firm nodules were seen on his right cheek, left arm, and right thigh [Figure 3], [Figure 4], [Figure 5].
General and systemic examinations findings were insignificant, except for right axillary lymphadenopathy.
Skin biopsy taken from the tumor of the armpit and the wrist showed epithelioid and gigantocellular granulomas dermatitis with onset of necrosis [Figure 6].
Figure 6: Histopathological examination of plaque revealing tuberculoid granulomasQuantiFERON-TB Gold test was positive, the polymerase chain reaction (PCR) on biopsy was positive to tuberculosis, and the culture of material obtained from biopsy of the lesion was also positive for Mycobacterium tuberculosis.
Ultrasound of lymph node areas showed a well-limited right axillary adenopathy measuring 17 mm × 23 mm with regular contours with disappearance of the fatty hilum in favor of a tuberculous origin.
After discussion with radiologists and thoracic surgeons, lymph node biopsy was not done due to the obesity of the patient.
Chest X-ray and X-ray of the right wrist and left arm were normal. A magnetic resonance imaging of his forearm was normal.
Laboratory findings were normal, and serology for antibodies to human immunodeficiency virus was negative.
On the basis of clinical, pathological, bacteriological, and radiological findings, a final diagnosis of scrofuloderma on axillary tuberculosis adenitis was made associated to an ulcerated vegetative lupus vulgaris. The ulcerovegetative aspect is rare, as well as the association of a paucibacillary and multibacillary form, which makes this observation special.
Treatment with isoniazid 5 mg/kg/day, rifampicin 10 mg/kg/day, ethambutol 20 mg/kg/day, and pyrazinamide 25 mg/kg/day was started for 2 months, then followed by isoniazid plus rifampin given for an additional 4 months with a good tolerability. At the end of treatment, the evolution was marked by a complete regression of the lesions leaving hyperpigmented patches [Figure 7] and [Figure 8].
Figure 7: Significant improvement after treatment of the ulcerative tumor of the right armpitFigure 8: Significant improvement after treatment of the ulcerative tumor of the right wrist DiscussionIn Morocco, the incidence of tuberculosis is 94 cases per 100,000 population.[4] It remains a major global health problem. The cutaneous form of tuberculosis has varied clinical presentations, which is determined by both route of infection and status of cellular immunity of the host.[5] It represents the fifth extrapulmonary form in Morocco after pleural, lymph node, urogenital, and intestinal tuberculosis. The diagnostic delay can be very long and is more difficult in atypical forms.
Scrofuloderma and gumma are the most common clinical types encountered in Morocco, in contrast to other series from Europe, USA, and India, where lupus vulgaris has been more commonly seen.[6]
The presence of multiple lesions in extrapulmonary sites, with or without pulmonary involvement, defines multifocal systemic tuberculosis.[7] Tuberculous adenitis is the form most frequently associated to cutaneous involvement. A few observations were reported in immunocompetent individuals in the literature, and skin involvement was even rarer.[8]
The association of different anatomo-clinical forms of cutaneous tuberculosis in the same patient is rarely seen. Only three cases in the literature described association between scrofuloderma and lupus vulgaris.[3] To the best of our knowledge, our patient is the fourth case, and he presented a scrofuloderma on axillary tuberculosis adenitis associated to an ulcerated vegetative lupus vulgaris at different parts of his body. In our opinion, this association is linked to the long course of the untreated lymph node disease.
Scrofuloderma results from contiguous tubercular involvement of the skin from an underlying infected structure; in our case, it was secondary to tuberculous adenitis. While lupus vulgaris is acquired either exogenously by direct inoculation or endogenously by hematogenous or lymphatic spread of acid-fast bacilli.[5],[9]
Scrofuloderma most often affects the supraclavicular, axilla, and both sides of the neck. Our patient presented with it on his right axilla.
Lupus vulgaris presents itself under very varied, sometimes misleading clinical aspects. It's usually located in the face, especially the nose and cheeks. It is characterized by plaque with an apple-jelly nodule that extends irregularly. The ulcerovegetative tuberculous lupus (lupus vorax) carries out sometimes appalling mutilations of the tip of the nose. The lesion is often single; multiple forms are rare. Even if it is a hematogenous spread from an initial deep focus, usually no concomitant progressive tuberculous focus is found.
Without treatment, its course usually extends over many years, leading to a significant impairment of function and disfiguration. The ulcerovegetative aspect is rare, as well as the association of a paucibacillary and multibacillary form, which makes the particularity of this observation.
The diagnosis is based on histology, but especially on the PCR, which will detect the mycobacterial DNA sequences by amplification.
In Morocco, the current national first-line treatment for pulmonary and extrapulmonary tuberculosis consists of isoniazid, rifampin, ethambutol, and pyrazinamide administered for the first 2 months, followed by isoniazid plus rifampin given for an additional 4 months.
ConclusionThe diagnosis of cutaneous tuberculosis must be clinically suspected in the presence of any destructive or verrucous skin lesion evolving without healing over a long period, even in the case of a negative culture.
The diagnosis of paucibacillary forms has been facilitated by the advent and then the generalization of the practice of PCR amplification.
There are only a few cases in the literature which describe scrofuloderma with lupus vulgaris, which makes this case special.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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References
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