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Cutaneous Lesions in Graft versus Host Disease  

     Dr Sampurna Roy MD

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Dermatopathology Case 85

   
 

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Graft versus host disease occurs in any situation in which  immunologically competent cells or their precursors are transplanted into immunologically compromised recipients (Eg-  In leukemia, aplastic anaemia and certain immunodeficiency states). GVHD is one of the most important causes of morbidity and mortality following bone marrow transplantation.

Early  acute phase:

Acute phase begins between 7 and 21 days after transplantation.
Besides the cutaneous lesions, the patient also complains of vomiting, diarrhoea and hepatic dysfuncton.

Cutaneous lesions include:
- Erythematous macular rash.
- Occasionally follicular papules resembling folliculitis.
- In severe cases, erythematous to violaceous scaling lesions and even blisters.
-Very rarely toxic epidermal necrolysis may occur.

Late chronic phase:

Chronic phase develops between several months to a year after the transplantation.
1. Early lichenoid phase (both oral and cutaneous lesions clinically resemble
lichen planus.click here.
2. Late sclerodermoid phase may be localized or generalized.
Other lesions noted in late phase include esophagitis, liver disease, sicca syndrome,lupus erythematosus-like eruptions, pyogenic granuloma & other angiomatous lesions & alopecia

MICROSCOPIC FEATURES: Dermatopathology Case 85

There is poor correlation between the biopsy finding following transplantation and clinical severity of the disease and biopsy is of limited value in predicting the progression of the lesion to higher grade GVHD.

Grading:   

Grade 0 - normal ;  
Grade 1 - basal vacuolar change ;
Grade 2 - dyskeratotic cell in epidermis and lymphocytic infiltrate ;
Grade 3 - formation of clefts & microvescicles;
Grade 4 - separation of epidermis from dermis ;       
                        

Acute Phase:

Sparse, diffuse lymphocytic infiltrate in the upper dermis.
Extensive exocytosis
Basal vacuolation
Scattered shrunken, degenerate keratinocytes throughout the epidermis.
Keratocytes have pyknotic nucleus , eosinophilic and hyalinized cytoplasm and are accompanied by one or more lymphocytes known as "satellite cell  necrosis".
In severe cases the appearances are those of
toxic epidermal necrolysis.
Differential diagnosis:  Subacute radiation dermatitis,
drug eruption.

Chronic phase:
Early lichenoid phase greatly resemble lichen planus -
        - acanthosis,
        - satellite cell necrosis in the epidermis,
        - degeneration of basal cell layer,
        - mononuclear cell infiltrate immediately below the epidermis,
        - prominent pigment incontinence
        - In case of follicular papules the features resemble those of lichen planopilaris. 
         - Rarely 'columnar epidermal necrosis' is noted.
Pubmed

Late sclerodermoid phase

         - Atrophy of epidermis.
         - Basal layer vacuolar degeneration.
         - Eosinophilic body formation rare or absent.
         - Thickened or hyalinized collagen bundles which extends into the subcutis.
         - Atrophy of adnexal structures due to dermal fibrosis.

Immunofluorescence:  IgM and C3 in colloid bodies. Granular or linear deposition of IgM in the basement membrane zone is often present.
 

              

Abstract:

Clinical significance of skin biopsies in the diagnosis and management of graft-vs-host disease in early postallogeneic bone marrow transplantation.  Arch Dermatol 2000 ; 136: 717-721  

A reappraisal of the histopathologic criteria for the diagnosis of cutaneous allogeneic acute graft-vs-host disease. Am J Clin Pathol 1999; 112: 791-800  

Value of skin biopsies in assessing prognosis and progression of acute graft-versus-host disease. Am J Surg Pathol 1997; 21: 988-996. 

Lichen planus-like histopathological characteristics in the cutaneous graft-vs host reaction.   Arch Dermatol 1997; 133: 961-965.

LN-3: A diagnostic adjunct in cutaneous graft vs host disease.
Mod Pathol 1990; 3: 643-647.

The histological diagnosis of cutaneous graft versus host disease. Relationship of skin changes to marrow purging and clinical variables. Histopathology 1987; 11: 145-155

Cutaneous graft-versus host reaction . Prognostic features seen by light microscopy. J. Am Acad Dermatol  1985; 12: 468-474.

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