Cutaneous Lesions in Graft versus Host Disease

Microcystic Adnexal Carcinoma

Dr Sampurna Roy MD

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Dr Sampurna Roy MD

http://www.histopathology-india.net/dermpath.htm

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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:Image link (webpath);Image Link(JHU)

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.