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LT Kenneth J. Meehan, PA-C, MPAS, DEPARTMENT
EDITOR
Widespread rash in a man with leukemia
Shylashree Chikkamuniyappa, MD; Jeffrey Christal, MD
Dr. Chikkamuniyappa and Dr. Christal work in the Department of Pathology, University of Texas Health Science Center at San Antonio. The authors have indicated no relationships to disclose relating to the content of this article. Lieutenant
Meehan practices dermatology at Tripler Army Medical Center, Honolulu, Hawaii.
A 75-year-old man from
the Texas Gulf Coast was referred to the dermatology clinic at our institution for
evaluation. He had developed a generalized skin eruption a couple of weeks after
being started on dapsone (Avlosulfon) for a skin condition. He had a history of
chronic lymphocytic leukemia but had no other medical problems.
The physical examination showed an
afebrile man with a widespread rash, which was erythematous and indurated. The plaques
were distributed on the lateral surfaces of the upper extremities. There was loss
of touch and pinprick sensation on the lesions. No vesicles or bullae were seen.
Two 4-mm punch biopsy samples of skin from the lesions were obtained.
What is your diagnosis?
- Leukemia cutis
- Hansen’s disease
- Granulomatous dermatitis
- Nonspecific dermatitis
Discussion
This patient had the form of Hansen’s disease
known as tuberculoid (paucibacillary) leprosy. Leprosy is caused by infection with Mycobacterium leprae, an obligate, intracellular, acid-fast, gram-positive
bacillus with a unique ability to enter nerves and grow in cooler parts of the body. In the United States, Hansen’s
disease is endemic in Texas, Hawaii, Louisiana, and California, where animal reservoirs
like the nine-banded armadillo are common. The disease is typically seen in immigrants
from developing countries, especially Southeast Asia, India, Central Africa, and
South and Central America. The clinical manifestations of Hansen’s
disease are largely a result of the immunologic response of the host to the antigens
of M leprae. The disease has subtypes such as borderline, borderline tuberculoid,
borderline lepromatous, indeterminate, lepromatous, and tuberculoid leprosy. Common
to all forms of leprosy is nerve infection, which is manifested as local areas of
cutaneous anesthesia and enlarged peripheral nerves — especially the ulnar,
superficial peroneal, radial, and median nerves. Indeterminate leprosy typically
causes only a few flat, ill-defined skin lesions on the face or trunk, with minimal
sensory changes. In tuberculoid leprosy, an erythematous plaque is seen with sharp
outer margins fading centrally to a flattened clear zone of healing that is rough,
anhidrotic, hairless, hypopigmented, and anesthetic. Lepromatous leprosy causes
shiny macules, plaques, or nodules, and advanced cases can involve the eye, face,
ears, and liver, among other sites.
A granulomatous reaction pattern was
seen in the biopsy results from the patient in this case. This is consistent with
a number of conditions, including various subtypes of leprosy, tuberculosis, subcutaneous
sarcoidosis, leishmaniasis, rosacea, and fully formed lichen nitidus. Numerous fusiform
granulomas were associated with histiocytic cells (see Figure 1). Fite’s staining
demonstrated a single acid-fast bacillus (see figure inset).
The patient was treated with the multidrug
regimen for paucibacillary leprosy recommended by World Health Organization, which
is rifampin (Rifadin, Rimactane), 600 mg once a month, and dapsone, 100 mg daily,
both given for a period of 6 months.
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