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 Dr Sampurna Roy MD

          

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                     Path Quiz Case- 55

           Myxoid Tumours of Soft Tissue

       Dr  Sampurna Roy  MD

         Path Quiz 55: History and images:

 

February 2008
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Infectious Disease Online; INDEX: A-D ; INDEX: E-L ; INDEX: M-P INDEX: Q-Z ; FUNGAL DISEASE ; VIRAL DISEASE.
Cutaneous infection and infestations

Histopathological patterns in cutaneous infections

1: Bacterial, Rickettsial and Chlamydial infection

2 : Spirochetal Infection

3 : Mycoses and algal infections

4 : Protozoal Infections

5 : Helminth Infections

6 : Viral Infections

Cutaneous lesion associated with AIDS

Cutaneous Deposits

Calcinosis Cutis

Cutaneous Ossification (Osteoma Cutis)

Cartilaginous lesions of skin

Lipoid Proteinosis

Gout

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 Pathology of the Eyelid

Inflammatory diseases of the Eyelid

Tumour and tumour-like lesions of the Eyelid

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Epidermal tumours of the Eyelid

Mesenchymal Tumours of the Eyelid

Melanocytic tumours of the Eyelid

Granulomatous Reaction Pattern of the Skin

Granuloma Annulare

Necrobiosis Lipoidica

Necrobiotic Xanthogranuloma   

Rheumatoid Nodule

Lupus Vulgaris

Cutaneous Sarcoidosis

Melkersson Rosenthal Syndrome

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Skin lesion in Crohn's Disease

Blastomycosis-like pyoderma

Foreign body granuloma

Rosacea

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Granulomatous T-cell lymphoma

                       

Necrobiosis lipoidica (NL), originally known as necrobiosis lipoidica diabeticorum,  is a disorder of collagen degeneration with a granulomatous response and thickening of blood vessels.
Diabetes mellitus is present in more than half the patients with necrobiosis lipoidica.

Age & sex: Average age of onset is 30 years (may occur at any age) and females are commonly affected.

Site: Most cases are located on the leg specially above the tibiae, but may also occur on the face, scalp, forearm and trunk.

Clinical presentation: Lesions may be single but multiple lesions are more common. NL may present as red papules which may enlarge to form patches or plaques with an atrophic yellowish-brown and slightly depressed center.
The lesions may resolve spontaneously or become persistent chronic lesions which may ulcerate.


Histopathological features:      
Diagram: click here
In a fully developed case the characteristic features are present at the edge of the lesion.    

Necrobiotic granuloma and inflammatory infiltrate:

Full thickness of the dermis is involved with extension into the subcutis.

The inflammatory cells are composed of histiocytes, lymphocytes, plasma cells and occasional eosinophils are arranged in two or three tiers. These are aligned parallel to the skin surface.

There are several layers of necrobiosis within the reticular dermis. Necrobiotic areas are rimmed by histiocytes and multinucleate Langhans or foreign body giant cells.

The necrobiosis is irregular and less complete than in granuloma annulare.

( Note: Palisaded granuloma in necrobiosis lipoidica- Early lesions show prominent collagen degeneration. Late lesions show crowded and thickened collagen bundles. )

The intervening areas of the dermis are also abnormal.
Lymphoid cell aggregates with germinal centers may be present.
 

Abnormalities present in the reticular dermis are also present in the septa of the subcutaneous tissue (septal panniculitis with granulomatous inflammation).

Vascular changes:

Vascular changes are more prominent in diabetic patients.

Superficial and deep perivascular inflammatory infiltrate.

Plasma cells are conspicuous.

Superficiall vessels are telangiectatic & increased in number.

Deeper vessels may show endothelial swelling.

Lymphocytic vasculitis may be present.

Epithelioid granulomas within or adjacent to the vessel wall.

Other features:
Intradermal nerves are reduced in number.

Old and atrophic lesions show dermal fibrosis and thickened septa of the subcutaneous fat .

Lipid in the upper part of the dermis can be demonstrated by Sudan black and oil red O stain.

Stains for mucin (colloidal iron or alcian blue) are usually negative.

Differential diagnosis:
Granuloma annulare: click
Rheumatoid nodule: click
Necrobiosis Xanthogranuloma: click
Interstitial Granulomatous Dermatitis: click

                    

Abstracts:

Necrobiosis lipoidica resulting in squamous cell carcinoma.: Conn Med. 2005;69(7):401-3.

Epidermoid carcinoma and perforating necrobiosis lipoidica: a rare association. J Eur Acad Dermatol Venereol. 2005;19(6):756-8

Light-chain-restricted plasmacellular infiltrates in necrobiosis lipoidica -- a clue to an underlying monoclonal gammopathy. J Cutan Pathol. 2005;32(4):263-7.

gli-1 Oncogene is highly expressed in granulomatous skin disorders, including sarcoidosis, granuloma annulare, and necrobiosis lipoidica diabeticorum.Arch Dermatol. 2005 Feb;141(2):259-62.

Necrobiosis lipoidica. Dermatol Online J. 2004;10 (3):18.

Necrobiosis lipoidica associated with Kobner's phenomenon in a patient with diabetes. Cutis 2001;67(2):158-60.

Necrobiosis lipoidica. A case with prominent cholesterol clefting and transepithelial  elimination. Am J Dermatopathol 1999; 21:575-577.

Diffuse necrobiosis diabeticorum associated with non-insulin dependent diabetes mellitus. Clin Exp Dermatol 1998; 23:271-273.

Perforating elastosis in necrobiosis lipoidica diabeticorum.Cutis. 1996;57(5):336-8.

Ulceration in necrobiosis lipoidica-a case report and study. Clin Exp Dermatol 1993;18:366-369.

A new histopathologic feature of necrobiosis lipoidica diabeticorum: lymphoid nodules.J Cutan Pathol. 1988; 15(2):75-7.

Necrobiosis lipoidica diabeticorum: a clinicopathologic study. J Am Acad Dermatol 1988;18:530-37.

Necrobiosis lipoidica diabeticorum with cholesterol clefts in the differential diagnosis of necrobiotic xanthogranuloma.
J Cutan Pathol. 1988;15(1):18-21.

Necrobiosis lipoidica and granuloma annulare. Simultaneous occurrence in a patient.Arch Dermatol 1982 ;118: 192-193 

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Normal Histology of Skin

Glossary   

Gross examination of the skin specimen

Reporting of biopsies taken for Inflammatory Skin Diseases

Lichenoid (Interface)Tissue Reaction Pattern

Psoriasiform Reaction Pattern

Vesiculobullous Reaction Pattern

Spongiform Reaction Pattern

Vasculopathic Reaction Pattern

Lichen planus-like lesions

Bullous Pemphigoid

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Hailey-Hailey Disease

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Classification of Malignant Sweat Gland Tumours   

Clear cell carcinoma

Tumours of the Hair Follicle

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Role of immunohistochemistry in Dermatopathology 

Cutaneous Pseudolymphoma

Pathology of the Eyelid

Inflammatory diseases of the Eyelid

Tumour and tumour-like lesions of the Eyelid

Adnexal Tumours of the eyelid

Epidermal tumours of the Eyelid

Mesenchymal Tumours of the Eyelid

Melanocytic tumours of the Eyelid