Friday, July 19, 2019

Gluteal Lupus Vulgaris

Case Presentation
by Dr. Henry Foong
Ipoh, Malaysia
A 50-yr-old man presented with painful fissures at the right perianal area for one month.  It started as a small lesion and subsequently increased in size.  it was occasionally painful. 

He had seen a general surgeon previously and was treated with recurrent courses of antibiotics but had not improved. There was no bleeding per rectal.  He had no history of contact with TB.

Examination showed an ulcerated indurated plaque 8 x 22 cm on the right perianal area extending to the right gluteal area.  A similar plaque 3 x 7 cm was noted on the left perianal area. The edge of the lesion was irregular, slightly raised and nodular.  His regional nodes were not enlarged. 




A skin biopsy was performed. Section shows a fragment of skin composed of epidermis and dermis. A granulomatous inflammation is seen in the dermis. The granulomas are composed of epithelioid cells, lymphocytes and plasma cells. Infiltrates of neutrophils and eosinophils are also seen. Multi-nucleated giant cells and Langhan's giant cells are seen. In one granuloma caseation necrosis is seen. The overlying epidermis is unremarkable. There is no evidence of malignancy. Ziehl-Neelsen stain for acid fast bacilli is negative.
Periodic acid Schiff stain for fungi is negative.

Diagnosis: Cutaneous tuberculosis (lupus vulgaris)

Discussion
Base on the clinical and histopathological examination features this patient most likely has cutaneous tuberculosis (lupus vulgaris).  The word "lupus" means wolf and indeed the appearance of the face chewed by a wolf. Apart from the face, it can also affect the buttocks and the legs. The plaque type as seen in this patient is the commonest, though there are several variants eg ulcerative, hypertrophic, vegetating and nodular type. 0.5 to 10% develop complications of malignancy eg SCC/BCC.The diagnosis of cutaneous TB is often delayed when the index of suspicion is low.  Hence, it is often missed when it should not be missed because of its sequelae.

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Within an hour of uploading this, Professor Sharquie alerted us to a similar case he published this year.  See Reference 1.

Reference: 
1. Granulomatous Reaction at the Site of Positive Tuberculin Skin Test is a Marker of Active TB (Clinical and Histopathological Study) American Journal of Dermatology and Venereology 2019;  8(4): 55-60  Khalifa E. Sharquie, Adil A. Noaimi, Fatima A. Khalaf Department of Dermatology, College of Medicine, University of Baghdad, Iraq FREE FULL TEXT [Courtesy of Professor Sharquir)


 Mathur M, Pandey SN. Kathmandu Univ Med J (KUMJ). 2014 Oct-Dec;12(48):238-41.

6 comments:

  1. From Professor Sharquie: I have similar case but the biopsy was nonspecific.When I did tuberculin was positive and granuloma was seen at the site of tuberculin reaction and this supported the diagnosis of TB
    Hence a study was arranged which is sent to you. [Note: Prof. Sharquies reference added to page'

    ReplyDelete
  2. From Robert Shapiro, Hilo, Hawaii: I think I would search for more proof of Tb. Perhaps PCR, culture is pending? I don't know if “caseation necrosis” can be over-read by the pathologist. I would add granuloma gluteale and majocci’s to the differential diagnosis clinically.

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  3. From Dato Cheng Leng Ong: May I congratulate Dr Henry for his expert management of this patient. Even the caseation necrosis is consistent.
    Interestingly I still remember being taught when I was still in medical school in Singapore, that fistula in ano can be caused by tuberculosis. This present case is pretty close!

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  4. From Henry Fong: "Many thanks for the comments. In Malaysia, we do CXR and Mantoux test in all patients suspected of tuberculosis. The presence of caseating granuloma and granulomas with Langerhans giant cells are highly suspicious of tuberculosis but they are not specific. TB culture or PCR studies are occasionally done to confirm diagnosis. The use of skin biopsy at the TST site is a novel one in predicting active TB and we may consider doing this too if patient agrees. The management of all forms of TB is carried out in a government general hospital under the national TB control program.”

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  5. More from Professor Sharquie: The classical lupus vulgaris of the old days is no more seen at the present practice.Nowadays TB might presents as skin cold abscess,plaques,ulcerative lesions that resemble cutaneous leishmaniasis or multiply sinus in the anogenital region. So the name lupus vulgaris is no more used and could be considered obsolete name.

    ReplyDelete
  6. Great teaching case!
    The most probable diagnosis is lupus vulgaris. Is IGRA available in Malaysia?How common is TB in your country?Is TB vaccination mandatory there?
    Have you started therapy?
    I'd suggest rectoscopy and taking a mucosal biopsy, just to make sure nothing serious is missed.
    Best,

    ReplyDelete

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