Extreme hypereosinophilia in a young male patient presenting with palindromic morphoea

S. A. W. Fadilah, S. K. Cheong and S. Shahdan1

Division of Haematology and
1 Division of Rheumatology, Faculty of Medicine, University of Kebangsaan Malaysia (UKM), Kuala Lumpur, Malaysia

Correspondence to: S. A. W. Fadilah, Department of Medicine, Hospital University of Kebangsaan Malaysia (HUKM), Jalan Tenteram, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia.

SIR, Apart from allergic diseases and helminthic parasites, a raised eosinophil count, especially a moderate or high count, is unusual [1]. Similarly, localized scleroderma lesions developing cyclically, named `palindromic morphoea', are extremely rare [2]. We describe an unusual variant of localized scleroderma associated with unexplained peripheral extreme hypereosinophila that responded well to steroids. The differential diagnosis of localized scleroderma and marked eosinophilia is presented.

A 19-yr-old male patient presented with a 2 yr history of recurrent episodes of painless and non-itchy swellings over both forearms and the right cheek. The swelling seemed to follow a migratory pattern as it often started at the right cheek, then the left cheek, left forearm and right forearm. These episodes usually lasted for a week and resolved spontaneously, only to recur 2 weeks later. There was no history of personal or family history of atopy or asthma. He had been in good health in the past. He denied any cardiac, chest, gastrointestinal or neurological symptoms. There was no oral ulcer, dry eye or mouth, vasculitic rash, arthritis or Raynaud's phenomenon. His appetite and weight had remained unchanged. He had worked as a car cleaner for many years. There was no recent exposure to drugs, chemicals or parasites.

Physical examination was normal apart from firm, well-defined, non-tender swellings over the extensor aspect of the forearms and right cheek with induration of the overlying skin. The leucocyte count was markedly elevated at 86.8 x 109/l with 88.9% eosinophils (77.17 x 109/l), with normal haemoglobin level and platelet count. Levels of complement, immunoglobulins and muscle enzymes were normal. Rheumatoid factor and antinuclear antibodies were negative. The erythrocyte sedimentation rate (ESR) was 6 mm/h. Bone marrow smears showed an excess of mature eosinophils (40% of the total nucleated cells) with no blast cells (see Fig. 1Go), and biopsy of the right forearm swelling confirmed localized scleroderma (see Fig. 2Go). There was no evidence of parasitic infestation on stool examination. The electrocardiogram, chest radiograph and lung function test were normal. A diagnosis of palindromic morphoea was made because of the unique clinical course of the skin lesions. Treatment with oral prednisolone at 1 mg/kg/day was commenced. Within 1 week, his eosinophil count had returned to 0.9% (0.1 x 109/l) and the swellings rapidly resolved.



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FIG. 1.  Aspirate of the bone marrow showing increased numbers of eosinophils (40% of the total nucleated cells), with a shift to the left in eosinophil maturation (MGG, x400).

 


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FIG. 2.  Histology of the skin lesion showing increased collagen deposition in the dermis extending into the s.c. fatty tissue. Lymphocytic infiltrates are evident in the collagen bundles and perivascular region (H&E, x400).

 
Morphoea, or localized scleroderma, refers to a collection of cutaneous disorders characterized by thickening and induration of the skin and s.c. tissue without significant organ involvement. The histological appearance of morphoea reveals homogenization of collagen bundles, reduction in the number or size of dermal appendages, and perivascular lymphocytic infiltrates. In a study conducted over 33 yr, the average incidence rate was 3.6%/year [3]. The morphoea subtypes are classified according to the clinical presentation as well as the depth of tissue involvement. They are categorized into five distinct groups including plaque, generalized, bullous, linear and deep [4], with plaque being the commonest form (56%). The shortest active disease duration was found in the plaque group (50% resolution or skin softening by 2.7 yr compared to 5.5 yr in the deep group). Arthralgias, synovitis, uveitis and joint contractures were more frequent in the linear and deep categories. Several case reports [5, 6] have reported a progression of morphoea to systemic sclerosis. In a large population-based study of morphoea and all its subtypes [3] diagnosed between 1960 and 1993, none developed severe internal organ involvement or progressed to systemic sclerosis. There was no significant change in location or laterality over time among the groups. Hence, new morphoea lesions developing cyclically is indeed an unusual phenomenon. Apart from this case, our research revealed another case of palindromic morphoea reported in the literature [2]. With the exception of the deep group, the majority of the patients had no specific laboratory studies. Eosinophilia was observed in 16% of the deep group, and the mean eosinophil count at diagnosis and at last follow-up was 5.4 and 2.8% of the total leucocyte count, respectively, and marked eosinophilia was not observed [3]. The association between palindromic morphoea and extreme hypereosinophilia had not been previously described. Numerous therapeutic agents, including corticosteroids, chloroquine, vitamin B12 and triamcinolone acetonide injections, have been reported to be effective in localized scleroderma, but controlled studies are rare [7].

Soft-tissue swelling in the presence of peripheral eosinophilia should alert us to the possibility of eosinophilic fasciitis. Although suggested by some as a variant of systemic sclerosis, it has now been recognized as a distinct clinical entity [8]. The presence of prodromal symptoms, muscle ache following extreme physical exertion, painful induration, elevated ESR, hypergammaglobulinaemia and extensive infiltrate of eosinophils within the fascial plane and extending into skeletal muscle are useful features to distinguish eosinophilic fasciitis from other conditions presenting as scloroderma- like skin changes. In our patient, the absence of eosinophilic infiltrates and inflammation in the affected lesion excludes this diagnosis.

The causes of eosinophilia can be classified according to the degree and frequency of occurrence. In the majority of cases, eosinophilia is reactive rather than neoplastic. Marked eosinophilia is >5x109/l, and typically seen in drug hypersensitivity, parasitic infections, Churg–Strauss syndrome, eosinophilic leukaemia, lymphoid malignancy and idiopathic hypereosinophilic syndrome (HES). Apart from in allergic or parasitic diseases, an eosinophil count of >1.5x109/l is unusual [1]. In uncomplicated asthma, the eosinophil count is rarely in excess of 2x109/l and higher levels may indicate either allergic aspergillosis or the Churg–Strauss syndrome. Eosinophilia of >=1.5x109/l is an important diagnostic criterion in making the diagnosis of Churg–Strauss syndrome [1]. The distinction from eosinophilic leukaemia, a very rare disorder, is made principally by determining the presence of blast cells in the marrow (and blood). The presence of a clonal cytogenetic abnormality and progressive anaemia and thrombocytopenia also indicates leukaemia. Eosinophilia is a rare manifestation of non-haemopoietic malignancy [1]. It is usually associated with widespread malignant disease, but rarely may provide a clue to a localized tumour. Eosinophilia may also occur as a reaction to lymphoid malignancy, particularly Hodgkin's disease, T-lineage non-Hodgkin's lymphoma and T-acute lymphoblastic leukaemia. There remains a group of patients with persistent, moderate or marked eosinophilia for which no cause can be found despite detailed investigation. Chusid et al. [9] described the three defining features of HES: a sustained blood eosinophilia of >1.5 x 109/l for >6 months; the absence of parasitic or other known causes of eosinophilia; and presumptive signs and symptoms of organ involvement. Our patient did not satisfy the diagnostic criteria for HES, as there was no detectable internal organ involvement or eosinophilic infiltrates in the skin biopsy specimens.

This patient presented with an unusual form of localized scleroderma in which he had recurrent morphoea over 30 times in 2 yr and, in addition, the lesions were associated with extremely high eosinophil counts. Extensive investigations failed to disclose known causes of marked eosinophilia, which led us to conclude that the skin lesion may be responsible for the hypereosinophilia. To our knowledge, this is the first description of extreme hypereosinophilia in a patient with palindromic morphoea, a unique form of localized scleroderma.

References

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  2.  Mizutani H, Tanaka H, Okada H, Mizutani T, Shimizu M. Palindromic morphea: multiple recurrences of morphea lesions in a case of systemic sclerosis. J Dermatol 1992;19:298–301.[Medline]
  3.  Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960–1993. J Rheumatol 1997;24:73–80.[ISI][Medline]
  4.  Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). Mayo Clin Proc 1995;70:1068–76.[ISI][Medline]
  5.  Mayorquin FJ, McCurley TL, Levernier LE, Myers LK, Becker JA, Graham TP. Progression of childhood linear scleroderma to fatal systemic sclerosis. J Rheumatol 1994;21:1955–7.[ISI][Medline]
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  8.  Varga J, Kahari VM. Eosinophilia-myalgia syndrome, eosinophilic fasciitis, and related fibrosing disorders. Curr Opin Rheumatol 1997;9:562–70.[Medline]
  9.  Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine 1975;54:1–27.[ISI][Medline]
Accepted 19 April 1999





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