Splenomegaly, Eosinophilia, and Pruritis: Hodgkin's Disease, or ...?

To the Editor:

A previously well 20-year-old male carpet-layer presented complaining of 3 weeks of severe pruritis unresponsive to treatment with oral promethazine. He denied using any prescription medications, other drugs, or herbal products and reported minimal alcohol consumption. He denied fevers, sweats, or recent weight loss and had no risk factors for human immunodeficiency virus infection. On examination, the patient had marked excoriations, predominantly over the flexor surfaces of both forearms and anterior aspects of his lower legs without an underlying rash evident. The spleen was palpable 2 cm below the left costal margin, without palpable lymphadenopathy.

On examination of a peripheral blood smear, there was moderate eosinophilia of 0.56 × 10⁹/L (normal range, 0.04 to 0.44 × 10⁹/L) without anemia, neutrophilia, or other morphologic abnormalities. Bilirubin, liver enzymes, electrolytes, erythrocyte sedimentation rate (ESR), and serum lactate dehydrogenase were all within normal limits, and serology for acute infectious mononucleosis and lyme disease were negative. The presence of splenomegaly was initially confirmed by ultrasonography, which showed an enlarged organ of 14.0 cm in its long axis with a normal echo texture, no focal lesions, and no evidence of portal hypertension. A plain chest radiograph was normal.

On review 2 weeks later without intervention, the patient's symptoms and physical findings were unchanged, and computerized tomographic scanning of the abdomen and pelvis showed progressive splenic enlargement, now measuring 12 × 5 × 16 cm, with no adenopathy seen. The liver was of normal size without parenchymal abnormality. At this time, infectious diseases review elicited the fact that the patient's girlfriend was similarly afflicted by pruritis. Although there was still no skin rash, burrows, or mites visible, the patient and his girlfriend were treated empirically for presumed scabies infestation with topical permethrin 5% cream (Lyclear; Parke-Davis Wellcome, Caringbah, Australia).

Within 2 days of initiating treatment, the patient's pruritis had resolved completely. When assessed 2 months later, the patient's eosinophilia had also resolved (absolute eosinophil count, 0.36 × 10⁹/L) and repeat ultrasonography showed regression of the splenomegaly (maximum length now 12.4 cm). One year after this episode, the patient remains well and asymptomatic.

The clinical syndrome of severe pruritis, splenomegaly, and peripheral blood esosinophilia in a previously well young man is suggestive of underlying Hodgkin's disease. Pruritis severe enough to result in excoriations has been reported to occur in a mean of 4.0% (43/1,085) of patients with Hodgkin's disease in three series.1-3 In many of these cases, this symptom is the presenting feature of the disease. Peripheral blood eosinophilia was first described as a manifestation of Hodgkin's disease in 1892⁴ and, from four published series, occurs in between 15% and 28% of patients at diagnosis (mean, 15.7% [208/1,327]).5-8 The spleen is pathologically involved in 30% to 45% of patients with Hodgkin's disease at diagnosis,9-11 although, conversely, approximately 35% to 50% of patients with Hodgkin's disease and clinical splenomegaly do not have pathologic involvement documented at staging laparotomy.11 12 

With the presence of this symptom complex and no superficial adenopathy available for biopsy, a diagnostic splenectomy may have been the next step in this patient's work-up. However, consideration of the possibility of infestation with Sarcoptes scabiei (scabies) avoided such procedures.

As judged by their disappearance after empiric specific therapy for ectoparasites, both the eosiniophilia and splenomegaly were attributable to scabies infestation in this case. Although not commonly recognized, a number of older reference texts cite scabies as a potential cause of eosinophilia, without providing actual incidence figures.13 14 Despite a thorough Medline search and scanning of the bibliographies of recent reviews, no previous instances of splenomegaly associated with scabies were identified.

This case illustrates the possibility of scabies presenting with features suggestive of Hodgkin's disease. Consideration of this infestation in the differential diagnosis of patients with itch, eosinophilia, and splenomegaly can obviate the need for invasive and potentially morbid diagnostic procedures. A prompt and durable symptomatic response to empiric topical treatment appears to be a specific diagnostic test for this condition.