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LETTER TO THE EDITOR
Year : 2012  |  Volume : 3  |  Issue : 3  |  Page : 222-224  

Systemic sclerosis presenting as lobular panniculitis


1 Department of Dermatology, Government Medical College, Thrissur, Kerala, India
2 Department of Internal Medicine, Government Medical College, Thrissur, Kerala, India

Date of Web Publication29-Sep-2012

Correspondence Address:
V G Binesh
Department of Dermatology, Government Medical College, Thrissur, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.101830

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How to cite this article:
Binesh V G, Krishnan J, Asokan N, Ajithkumar K. Systemic sclerosis presenting as lobular panniculitis. Indian Dermatol Online J 2012;3:222-4

How to cite this URL:
Binesh V G, Krishnan J, Asokan N, Ajithkumar K. Systemic sclerosis presenting as lobular panniculitis. Indian Dermatol Online J [serial online] 2012 [cited 2019 Sep 22];3:222-4. Available from: http://www.idoj.in/text.asp?2012/3/3/222/101830

Sir,

Panniculitis includes diseases with major focus of inflammation in the subcutaneous layer. Systemic sclerosis (SS) is a rare cause for panniculitis. [1] The commonest type of panniculitis described in SS is septal without vasculitis. [2] We report a case of SS in which lobular panniculitis was one of the major presenting features.

A 42-year-old female presented with an indurated mildly erythematous plaque of 13 × 10 cm 2 size over the medial aspect of the left arm just above the elbow, of four months duration. [Figure 1]. There were depressed scars and mild dry scaling on the surface of the plaque. She gave history of recurrent fever and polyarthralgia with early morning stiffness mainly affecting the knee and elbow joints for the previous 4 months. She also had difficulty in breathing on routine activities (grade III dyspnea). There was no history of diabetes mellitus, jaundice, rheumatic fever, alopecia, oral ulcer, or photosensitivity. There was generalized edema and mild diffuse sclerosis of the skin, predominantly affecting the extremities extending proximal to metacarpophalangeal joints. A provisional diagnosis of panniculitis associated with connective tissue disease, possibly SS or systemic lupus erythematosus (SLE) was made.
Figure 1: Presenting skin lesion

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The results of laboratory tests revealed raised ESR (110 mm/h), proteinuria (0.5 g/day), and mildly elevated serum LDH (620 U/l; N < 480). Blood sugar, liver function tests, thyroid function tests, peripheral blood smear, and serum creatine phosphokinase were normal. ANA ELISA, dSDNA, and the Mantoux test were negative. Fluorescent ANA assay revealed Scl-70 positivity.

Chest X-ray showed cardiomegaly and bilateral patchy shadows on mid and lower zones. A computerized tomography (CT) scan of chest showed patchy areas of consolidation in the left upper and middle lobes and both lower lobes suggesting nonspecific interstitial pneumonitis. An echocardiogram revealed mild pericardial effusion. An ultrasound scan of abdomen and pelvis was normal. Pulmonary function tests (PFT) showed a mild restrictive pattern. Skin biopsy showed severe lymphocytic infiltration and hyalinization of collagen in the dermis suggestive of lymphocytic type of lobular panniculitis without prominent vasculitis [Figure 2] and [Figure 3].Direct immunofluorescence showed deposition of fibrinogen on blood vessel walls and a negative lupus band.
Figure 2: Lobular panniculitis with severe lymphocytic infiltration, hyalinization of collagen in the dermis (H and E, ×100)

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Figure 3: Lobular panniculitis with severe lymphocytic infiltration (H and E, ×400)

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Based on the findings of proximal sclerosis, exertional dyspnea, interstitial pneumonitis, restrictive pattern of PFT and Scl-70 positivity, a diagnosis of SS was made. There were no clinical or immunological evidence of any other autoimmune disease such as SLE or dermatomyositis.

There are only a few reports of panniculitis occurring in SS. The commonest type described is septal panniculitis without vasculitis. [2] There is a recent report of lipomembranouspanniculitis in SS. [3] To the best of our knowledge, the present one is the first reported case of lobular panniculitis in SS. It was also notable that panniculitis was one of the major presenting manifestations of this patient. It would be of interest to know whether the prognosis of SS patients with lobular panniculitis is different from others.

 
   References Top

1.Mcnutt NS, Moreno A, Contreras F. Panniculitis. In: Elder D, Elenitsas R, Jaworsky C, Johnson B Jr, editors. Lever's Histopathology of the Skin. 8 th ed. NewYork: Lippincott-Raven; 1997.  Back to cited text no. 1
    
2.Requena L, Yus ES. Panniculitis. Part I. Mostly septalpanniculitis. J Am Acad Dermatol 2001;45:163-83;quiz 184-6.  Back to cited text no. 2
    
3.Almeida MS, Lima SC, Carvalho LL, Almeida JV, Santos LG, Rolim JR, et al. Panniculitis-An unusual cutaneous manifestation of systemic sclerosis. J Cutan Pathol 2010;37:1170-3.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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