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Indian Journal of Medical Sciences, Vol. 58, No. 6, June, 2004, pp. 250-252 Case Report Long-term pruritus as the initial and sole clinical manifestation of occult Hodgkin's disease Omidvari Shapour H, Khojasteh Habib Noorani , Mohammadianpanah Mohammad , Monabati Ahmad , Mosalaei Ahmad , Ahmadloo Niloofar Department of Radiation Oncology, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz Code Number: ms04045 ABSTRACT Pruritus or itch is a frequent symptom of patients with Hodgkin's disease. It often occurs during the clinical course of the disease and rarely may precede the diagnosis of underlying disease. In this report, we present a 16-year-old patient who had history of generalized pruritus without any skin rash for 4 years before the diagnosis of Hodgkin's disease. Within that period, she had received symptom-oriented medications, with no significant effect. After the first cycle of chemotherapy, her pruritus resolved completely. This case suggests that long-term generalized pruritus may be indicative of a significant underlying problem like Hodgkin's disease. INTRODUCTION Itch has been defined as a sensation, which provokes the desire to scratch. When it has no skin manifestation, the diagnostic evaluations should be directed at a systemic underlying cause.[1] In most cases successful treatment of the underlying disease relieves itch. Itching occurs in about 30% of patients with Hodgkin′s disease.[2] It is often worse at night and tends to occur in the area drained by involved lymph nodes in localized disease or become generalized especially in nodular sclerosis type with mediastinal involvement.[2] Although pruritus is frequently seen in Hodgkin′s disease, its presence several years before diagnosis of the disease is rarely reported.. Here we present such a case. CASE REPORT A 16 year-old girl with four years history of generalized pruritus while she had had no any rash and or other skin manifestation was referred to radiation oncology department. She had received various topical and systemic symptom-oriented medications without significant benefit. She had no history of fever, weight loss or night sweats. She had experienced cervical lymphadenopathy and dry cough for two months when biopsy from an enlarged lymph node in the neck was reported as Hodgkin′s disease, nodular sclerosis type. [Figure - 1]. In referring, she had multiple enlarged and matted lymph nodes in right side of neck but no hepatosplenomegaly. There were excoriations over the anterior aspect of forearms and legs. Complete blood count, liver enzymes, bilirubin, serum lactate dehydrogenase were normal but erythrocyte sedimentation rate (ESR) was high (50mm/hr). Chest X-ray showed huge mediastinal mass. In ultrasonography, liver and spleen had normal size and echotexture and no para-aortic or pelvic lymphadenopathy was seen. Chemotherapy with ABVD regimen was started. Two weeks after first injection all enlarged cervical nodes disappeared and pruritus resolved completely. After completing the fourth course of chemotherapy, radiotherapy with Mantle technique was started and the patient received 3600 cGY in 20 fractions (one fraction per day, five fractions per week). One year after conclusion of treatment, now she has no evidence of tumor recurrence or pruritus. DISCUSSION Pruritus, a common manifestation of dermatologic diseases, may be associated with a broad variety of systemic disorders. It may be the initial presentation of an occult malignancy or the clinical features of a previously diagnosed tumor.[1],[2],[3] Systemic disease is detected in approximately 10-50% of all patients seeking medical attention for pruritus. Among patients with malignancy, lymphoproliferative disorders, particularly H.D are the most important and well-known causes of unexplained pruritus. It was considered as prognostic factor and incorporated in the definition of constitutional B symptom of H.D from 1965 until 1971.[1],[2],[3] Although it is not currently considered as a B symptom, some recent reports suggested more aggressive therapy because of their poor prognosis, for H.D patients with severe generalized pruritus.[1] Such a poor clinical course is not the rule, since most H.D patients in this situation can achieve a favorable response and good outcome.[4] Generalized pruritus often occurs months or even a year before H.D is first diagnosed. It may be rarely preceded the clinical onset of the disease by as long as 5 years.[2],[5] Although long-term pruritus as the isolated initial manifestation of H.D, such as that seen in our patient, is rare; it may be an important clue to the detection of occult H.D in previously healthy patient.[3] Stadie and Marsch reported a 35-year-old patient who had suffered from paroxysmal itching followed by immediate profuse hyperhydrosis for 6 months duration before the diagnosis of Hodgkin′s disease. They concluded that a malignant underlying disease like H.D should be considered in the differential diagnosis of a patient who presents with unclear paroxysmal itching and hyperhydrosis.[6] The initial systemic evaluation includes detailed medical history, comprehensive physical examination, and the routine screening laboratory tests. Further studies are done according to abnormal findings discovered during the preliminary evaluation. Successful treatment of the underlying H.D usually leads to elimination of the itch and other dermatologic symptoms.[4] Because pruritus may be the isolated presenting symptom of H.D in patients with unexplained persistent pruritus, the possibility of a significant underlying systemic disease such as H.D should be kept in mind. REFERENCES
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