Monday, November 30, 2015

Sarcoidal Granulomas

Presented by Christine Shanahan, Third Year Student
University of Virginia School of Medicine

Abstract: 36 yo male with diffuse erythematous pruritic plaques

HPI: The patient is an uninsured 36 yo male with a 6 month history of diffuse erythematous pruritic plaques on the chest, back and buttocks. The condition worsened over the past 2 months. He has no history of prior skin diseases, diabetes mellitus or other systemic conditions. He denies shortness of breath, wheezing, chest pain, eye problems, night sweats, unintentional weight loss or fatigue. He usually receives medical care in South America and frequently travels between South America and the United States.


On Examination: Diffuse erythematous pruritic plaques present on the chest, back and buttocks. No evidence of eyelid margin or oral cavity involvement.

Clinical Images:


 

Lab:
Chest radiograph showed no abnormalities.
Serum angiotensin-converting enzyme (ACE) level was found to be normal.
Recommended undergoing pulmonary function tests, ophthalmologic exam and chest CT scan.


Pathology:
Punch biopsy specimen from mid-back revealed numerous well-formed epithelioid cell granulomas in various levels of the dermis, a few patchy lymphocytic infiltrates and no necrosis or suppurative inflammation. No evidence of infectious microorganisms were found with special stains for acid-fast bacilli and fungi. Second pathologist evaluated specimen and confirmed diagnosis. Tuberculoid type of Hansen’s disease was added to histologic differential diagnosis but Fite stain showed no M. leprae.


Diagnosis: Sarcoidal granulomatous inflammation consistent with Sarcoidosis.


Plan: The patient was counseled on the importance of screening tests, including pulmonary function tests and an ophthalmologic examination, due to the insidious onset of systemic sarcoidosis and the frequency of pulmonary and ocular manifestations. The patient was reluctant to have any further workup beyond treatment of the skin condition. He was treated with 1 cc of intramuscular triamcinolone acetonide 40mg/cc (kenalog®-40) and topical steroids at follow-up visit. He was scheduled for follow-up visit in 1 month and referred for further workup of systemic disease.


Discussion:
Early diagnosis of sarcoidosis can be difficult, so cutaneous lesions can be key findings in helping to expedite this diagnosis.  Cutaneous sarcoidosis occurs in 20% to 35% of patients and can present at the early onset of disease. Also, skin involvement has been associated with more rapid progression of systemic sarcoidosis. The skin manifestations are described as specific or nonspecific lesions and are usually asymptomatic with pruritus in about 10% of patients. This patient had pruritic specific lesions with noncaseating granulomas visualized as plaques, one of the various typical morphologies. Although this patient appears to be in the early stages of the disease, the plaque presentation is typically associated with chronic forms of systemic disease. Sarcoidal lesions can mimic many other conditions including Hansen’s disease, leishmaniasis and lupus vulgaris. Specifically, tuberculoid type of Hansen’s disease is histologically similar and “the Fite method” is commonly used to distinguish the diseases. Cutaneous leishmaniasis remains endemic to many countries in South America such as Brazil, Colombia and Peru. The histopathology may be similar to sarcoidosis and some recent recommendations have been made to order PCR for Leishmania-specific DNA for any presentation of sarcoidal type granulomas in patients living in endemic areas


Questions:
  1. Could this still be Hansen’s disease with a negative Fite stain?
  2. Could this be a form of Leishmaniasis?
  3. Any recommendations for further workup of sarcoidosis for a low-income, uninsured patient?  

    References:
    1) Cutaneous sarcoidosis: differential diagnosis
    Clinics in Dermatology, Volume 25, Issue 3, Pages 276-287
    Esteban Fernandez-Faith, Jonelle McDonnell
     
    2) Hansen's Disease: An Imitator of Cutaneous Sarcoidosis
    Katsuya Chinen
    , Kazuhiko Hirano, Yasunori Fujioka
    Pictures in Clinical Medicine. Internal Medicine Vol. 50 (2011) No. 19  P 2257-2258

    3) Comorbidity of Leishmania Major with cutaneous sarcoidosis 
    Moravvej H, Vesal P, Abolhasani E, Nahidi S, Mahboudi F.
    Indian Journal of Dermatology. 2014;59(3):316. Free PMC Article 

    4. The Sarcoid-Lymphoma Syndrome. (MedScape) 

2 comments:

  1. I have a similar case and wonder what the mantoux test and the IGRA test results were in this patient , In our patient the diagnosis is a tuberculid from latent TB .

    ReplyDelete
  2. I don't think this is sarcoidal lymphoma from reviewing the literature and a recent discussion with a pathologist. In the articles reviewed, the patients had evidence of sarcoid in the skin and later developed lymphoma or simultaneously were diagnosed with lymphoma via ct scan or evaluation of a lymph node.
    Is there any followup on the patient after the intramuscular injection of kenalog?

    ReplyDelete

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