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Challenging Manifestations of Malignancies

CASE 2. Langerhans Cell Histiocytosis Presenting With a Skin Rash

Dana-Farber Cancer Institute and Brigham and Women's Hospital, Boston, MA

A 73-year-old white woman presented with a skin rash for 2.5 years. The rash initially began on her forehead as erythematous papules with crusting (Fig 1A). It progressed to involve her entire scalp, resulting in seborrhea and mild alopecia. She also noted confluent erythematous patches involving skin folds of the trunk (beneath breasts and pannus), axilla, groin, and gluteal region. The rash worsened 6 months before admission and was described as pruritic and burning. She was initially treated with medicated shampoos and a variety of topical creams, including antifungal agents, without significant improvement. The patient also complained of increased thirst and noted consumption of excessive amounts of fluid. Physical examination revealed a vesicular eruption on the forehead with widespread scaling of the scalp. Skin examination was significant for intensely erythematous patches involving intertrigonal regions and diffuse erythema, with scaling involving more than 90% of her back (Fig 1B). The liver edge was palpable 4 to 5 cm below the right costal margin, spleen was nonpalpable, and cranial nerves were intact. Initial laboratory evaluation was significant for leukocytosis (WBC, 25,000/mg³) with 30% monocytes, anemia, and thrombocytopenia. Serum sodium was 152 mEq/L with urine osmolarity of 90 mOsm/kg, characteristic of central diabetes insipidus. Liver function tests were elevated: AST, 76 U/L; ALT, 7 U/L; gamma-glutamyltranferase, 474 U/L; alkaline phosphatase, 1,829 U/L; and total bilirubin, 2.4 mg/dL. Mild hypercalcemia was present (corrected Ca, 10.7 mg/dL). Amylase and lipase were within normal limits. Computed tomography (CT) of the head showed no evidence of mass or bony lesions, the bone scan was negative, and the chest CT was negative for mediastinal masses and lymphadenopathy. Abdominal CT revealed a pancreatic cyst versus tumor and cholelithiasis, but no evidence of lymphadenopathy. A skin biopsy of the rash revealed an atypical mononuclear cell infiltrate consistent with Langerhans cell histiocytosis (Fig 2). In the skin biopsy (Fig 2), the appearance under low power (Fig 2A) reveals a focal superficial dermal and epithelial infiltrate with edema. At higher power (Fig 2B), hyperkeratosis and acanthosis are evident as well as dermal vessel involvement and abscess formation. The Langerhans mononuclear cells (Fig 2C) are elongated, folded, and have nuclei with fine chromatin, longitudinalgrooves, small nucleoli, and abundant cytoplasm. Immunohistochemistry reveals the histiocytic mononuclear cells to be strongly positive for S-100 (Fig 2D) and CD1a (Fig 2E).

View larger version (58K): [in this window] [in a new window]   Fig 1.

View larger version (108K): [in this window] [in a new window]   Fig 2.

Langerhans cell histiocytosis is a rare clonal disorder of monocytes that affects mainly children. Cases have been reported in adults.¹ Bone involvement occurs in 80%, skin involvement occurs in 60%, liver and spleen involvement occurs in 33%, lung involvement occurs in 25%, and diabetes insipidus involvement occurs in 15% of patients.² Skin biopsy is diagnostic when a mononuclear cell infiltrate is present that stains positively for CD1a by immunohistochemistry or contains Birbeck granules (shaped like a tennis racket) by electron microscopy. Cells also stain positive for S-100 protein. Treatment with 2-CDA has been reported to have efficacy in adults, with major toxicities reported as infection and transient myelosuppression.³

Authors' Disclosures of Potential Conflicts of Interest

The authors indicated no potential conflicts of interest.

REFERENCES

1. Scolozzi P, Lombardi T, et al: Multisystem Langerhans' cell histiocytosis (Hand-Schuller-Christian disease) in an adult: A case report and review of the literature. Eur Arch Otorhinolaryngol 261:326-330, 2004[Medline]

2. Usmani GN, Westra SJ, Younes S: A 14 month-old boy with hepatomegaly, perianal lesions, and a bony lump on the forehead. N Engl J Med 348:1692-1701, 2003[Free Full Text]

3. Pardanani A, Phyliky RL, et al: 2-Chlorodeoxyadenosine therapy for disseminated Langerhans cell histiocytosis. Mayo Clin Proc 78:301-306, 2003[Medline]

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