October 2004

Fig. 3

Fig.4

Laboratory Evaluation: A complete blood count showed mild thrombocytopenia with platelet count of 60,000. Peripheral blood smear showed mild monocytosis and rare immature myeloid cells.
Diagnosis: Multiple xanthogranulomas, associated with chronic myelomonocytic leukemia.
Histopathology: Six papules from the face, arms and trunk were biopsied and sent for histopathological evaluation. Sections from all the biopsies show similar histology. A confluent dermal infiltrate abuts the epidermis. This infiltrate is composed of histiocytes, with both eosinophilic and foamy cytoplasm, and several giant cells, resembling Touton giant cells. Special stains were negative for fungi and AFB. Immunohistochemical stains were positive for CD3, CD68 (KP-1), and CD31. S-100 was positive in some cells, including occasional giant cells. CD1a stained rare, scattered Langerhans cells.
Course: Due to the unusual diagnosis of adult xanthogranuloma, as well as the abnormal CBC, a full work-up was initiated to evaluate the possibility of an underlying systemic malignancy. The patient was referred to an oncologist, who performed a bone marrow biopsy. This revealed a hypercellular marrow with atypical megakaryocytes and a slightly increased number of blasts, composing less than 5% of the total cellularity. These findings as well as immunohistochemical stains were consistent with the diagnosis of chronic myelomonocytic leukemia (CMML).
The patient's cutaneous lesions responded to intralesional corticosteroids. He continues to be followed by his oncologist for CMML.

Discussion: Xanthogranuloma is classified as a non-Langerhans cell histiocytosis. Xanthogranulomas occurring in adults are quite rare. They are histologically indistinguishable from the more common juvenile xanthogranuloma (JXG), which occurs in infants and young children. Clinically, the majority of xanthogranulomas are solitary, benign, self-limited nodules with no systemic associations. Adult cases may be more persistent. JXGs are most often cutaneous, but ocular xanthogranulomas have been reported in less than 1% of cases; pulmonary, and hepatic xanthogranulomas are even more rare. Some cases of juvenile xanthogranuloma have been associated with hematologic malignancies, especially juvenile chronic myelogenous leukemia. The diagnosis of leukemia often follows the appearance of cutaneous lesions.

References:
Cortes J. CMML: a biologically distinct myeloproliferative disease. Curr Hematol Rep 2003;2(3)202.
Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003;27(5):579.
Gonzalez Ruiz A, Bernal Ruiz AI, Aragoneses Fraile H, Peral Martinez I, Garcia Munoz M. Progressive nodular histiocytosis accompanied by systemic distorders. Br J Dermatol 2000; 143(3)628.
Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB. Juvenile xanthogranuloma. J Am Acad Dermatol. 1997;36(3 pt 1):355.
Zelger B, Cerio R, Orchard G, Wilson-Jones E. Juvenile and adult xanthogranuloma: a histological and immunohistochemical comparison. Am J Surg Pathol. 1994;18(2):126.

This case was presented by Drs. Alison Klenk, Fred Bauschard, and Daniel Santa Cruz.