Page 119 - The Netter Collection of Medical Illustrations - Integumentary System_ Volume 4 ( PDFDrive )

Plate 4-34 Rashes

GRAFT-VERSUS-HOST DISEASE
Acute GVHD. Mild-
moderate petechial
With the ever-increasing number of bone marrow rash that becomes
transplantations and increasing survival rates of patients confluent
undergoing these procedures, graft-versus-host disease
(GVHD) is becoming more prevalent. Two distinct
clinical cutaneous forms exist, acute and chronic, each
with its own manifestations and treatment options.
Acute GVHD is often manifested by mucocutaneous
eruptions that can range from a mild macular rash to
life-threatening blistering of the skin. Chronic cutane-
ous GVHD is entirely different in clinical manifestation
than its acute counterpart. The two forms are also seen
during specific time frames: Acute GVHD is most likely
to occur within the first 3 months after transplantation,
whereas chronic GVHD occurs later, typically 4 months
or longer after transplantation.
GVHD can be seen not only after bone marrow
transplantation but in any immunosuppressed patient
who has receives antigenically and immunologically
viable cells from a donor. This may occur during organ
transplantation or, rarely, during blood transfusion.
The use of leuko-poor blood has helped decrease the
chance of GVHD after blood transfusions.
Clinical Findings: Acute GVHD is a common com-
plication after bone marrow transplantation. The inci-
dence has been reported to be as high as 90%. The
degree of involvement is variable. GVHD affects males
and females equally, and there is no racial preference.
Patients who develop acute GVHD typically begin
having symptoms soon after their cell counts recover,
usually 1 to 2 weeks after transplantation. Skin rashes
that develop within the first week after transplantation
are usually not from GVHD. The skin, upper and lower
digestive tract, and liver are frequently involved, and
these organ systems are evaluated to help make the
diagnosis of GVHD. The rash of acute GVHD can
range from a fine maculopapular rash to severe
blistering of the skin that can resemble toxic epidermal
necrolysis and can be life-threatening. It is difficult, if Sclerodermatous GVHD. Unlike acute
not impossible, to predict the development and course GVHD, the chronic sclerodermatous form
of acute GVHD. These patients are always taking mul- shows thickening of the collagen within
tiple medications, and the differential diagnosis includes the dermis. A thinned atrophic epidermis
a drug rash. Histological evaluation of a skin biopsy and a decrease in the number of adnexal
cannot differentiate the two. The coexistence of muco- structures are also noted.
sitis, diarrhea, and elevated liver enzymes makes the
diagnosis of acute GVHD more plausible. The constel-
lation of all these symptoms leads one to make the
diagnosis.
Chronic GVHD has entirely different clinical mani-
festations. This form of GVHD typically begins 3 to 6
months after transplantation. The skin is the organ
system most commonly involved. Two distinct forms
of chronic cutaneous GVHD occur, lichenoid and Severe acute GVHD.
sclerodermatous. The lichenoid variant manifests as The skin peels off in large
red papules, patches, and plaques. They can occur sheets due to necrosis of
anywhere on the surface of the skin. There is a slight the skin and subsequent blistering.
resemblance to lichen planus. The sclerodermatous
variant is less common and manifests as thickened, firm
skin with poikilodermatous changes. The surface of the vacuolar and interface changes; grade 2 shows signs of Corticosteroids are commonly used in cases of GVHD,
skin is shiny, and the loss of adnexal structures is vari- keratinocyte death; grade 3 shows clefting within the both acute and chronic. The acute form has also been
able. This variant of chronic GVHD can be localized subepidermal space; and grade 4 is full bulla formation treated with FK506 and cyclosporine. Many other
to a small area, or it can be generalized and may include with epidermal parting. immunosuppressants have been used.
the entire surface area of the skin. The amount of Lichenoid chronic GVHD shows a lichenoid derma- Chronic GVHD is difficult to manage. There is
surface area involved is directly related to the morbidity titis with a predominantly lymphocytic infiltrate. The no cure for GVHD, and treatment is directed at sta-
the patient experiences. sclerodermatous form of chronic GVHD shows abnor- bilizing and improving skin function and increasing
Histology: Histological evaluation of skin biopsy mally thick dermal collagen, much like that seen in the patient’s functional capabilities. Phototherapy
specimens cannot differentiate acute GVHD from drug scleroderma. has been used successfully, as has extracorporeal
exanthems. Acute GVHD has been graded on a histo- Treatment: The treatment of acute GVHD is based photopheresis.
logical scale of 1 to 4. Grade 1 shows basal layer on the clinical symptoms and the type of skin lesions.

THE NETTER COLLECTION OF MEDICAL ILLUSTRATIONS 105