Eosinophilic cellulitis as a cutaneous manifestation of idiopathic hypereosinophilic syndrome

Three patients with eosinophilic cellulitis associated with sustained peripheral blood eosinophilia of unidentified cause are reported. They also presented with diversities of extracutaneous symptoms such as bronchospasm, sensory polyneuropathies, epigastralgia, and gangrenous eosinophilic enteritis. These cases suggest that eosinophilic cellulitis can develop as a cutaneous manifestation of idiopathic hypereosinophilic syndrome.     

Skin lesions as the first manifestation of the hypereosinophilic syndrome

An unusual case of the hypereosinophilic syndrome (HES) is described in which lesions have been confined to the skin after a 2-year period of follow-up. The pathogenesis of the HES remains unsolved. A hypersensitivity phenomenon might be involved, although in this case no immunological abnormalities could be detected. The role of the eosinophilic granulocyte, however, seems to be a secondary one.     

以消化道症状为首发症状的嗜酸粒细胞增多综合征一例

患者女,59岁,因躯干、双下肢红斑、丘疹伴痒1个半月于2009年1月31日入院.患者于1个半月前无明显诱因躯干出现数个大小不等红斑,其上覆米粒大小丘疹,瘙痒明显,在当地诊所给予维生素C、维生素B1、维生素B12、病毒灵口服,曾自服中药(具体不详),并拔火罐治疗,部分皮疹可消退,留有色素沉着.     

Case of hypereosinophilic syndrome with cutaneous necrotizing vasculitis

A 25-year-old male presented with pruritic papules over his trunk and extremities. The pruritic eruption had appeared on the arms 5 months before and gradually spread over the trunk. Blood tests showed a white blood cell count of 13 760/microL with 42.2% eosinophils. A skin biopsy from the leg revealed necrotizing vasculitis in the upper dermis with prominent eosinophil infiltration in the dermis and subcutis. Liver dysfunction in accordance with eosinophilia was also seen. The underlying disorder causing the eosinophilia was not detected. We diagnosed this case as hypereosinophilic syndrome. Although systemic corticosteroid therapy using prednisolone was effective, the symptoms recurred during a reduction of prednisolone. The addition of cyclosporine resulted in improvement of his symptoms. Both drugs were successfully tapered without relapsing symptoms. Hypereosinophilic syndrome with cutaneous necrotizing vasculitis is a rare condition, and this case suggests that cyclosporine may be a useful therapeutic adjunct in facilitating steroid tapering.     

Erythroderma as the initial presentation of the acquired immunodeficiency syndrome.

A 32-year-old black homosexual man, seronegative for human immunodeficiency virus antibody, presented with erythroderma. His peripheral blood was significant for circulating Sézary-like cells bearing the CD8(+) phenotype. Eighty-eight percent of his lymphocytes were CD8(+) as well. He seroconverted 3 months after the initial presentation. We conclude that erythroderma was the presenting sign of the acquired immunodeficiency syndrome.     

Superficial venous thrombophlebitis as the initial manifestation of hypereosinophilic syndrome: study of the first 3 cases

BACKGROUND: Superficial venous thrombophlebitis (SVT), often perceived as benign, can coexist with hypercoagulable states. Predisposing risk factors for SVT are similar to those observed for deep venous thrombosis. Association of eosinophilia with SVT is a rare situation that can reveal neoplasia, malignant blood disorders, or vasculitis, but it has never been described in hypereosinophilic syndrome (HES). We herein describe the clinical and biological features, outcome, and response to therapy of 3 patients with SVT associated with eosinophilia that revealed HES. OBSERVATIONS: Superficial venous thrombophlebitis was the initial manifestation of HES in all 3 patients. The mean eosinophil count at diagnosis was 2.4 x 10(3)/muL. All patients received corticosteroids and anticoagulants as the initial treatment, with marked improvement of SVT and return of the eosinophil count to reference limits. All patients experienced relapse and remained dependent on corticosteroid therapy. Two patients received interferon alfa with dramatic regression of SVT, allowing a decrease in the dose of corticosteroids. CONCLUSIONS: We report, to our knowledge, the first 3 cases of SVT related to HES. Superficial venous thrombophlebitis was difficult to treat, with dependence on corticosteroid therapy and partial efficacy of anticoagulant and antiplatelet therapy. Interferon alfa was effective in preventing relapse of SVT related to HES. Mechanisms implied in this thrombogenesis are multiple and remain speculative.     

Erythroderma as first manifestation of squamous cell lung cancer: rare case report

Erythroderma as the first manifestation of a solid organ malignancy is rare. The underlying cancer is a challenging condition to diagnose. There are a few cases of erythroderma in cancer patients reported in the literature. We here describe the case of a 70-year-old man who presented with asthenia, weight loss, dry cough and total body erythema with desquamation over the past month. A chest computed tomography scan showed a nodular lesion, which was finally diagnosed as a squamous cell lung carcinoma. To our knowledge, as an erythroderma presentation, only 13 cases have been reported in the literature. This case report demonstrates the need to search for a neoplasm in patients presenting with erythroderma, particularly in the presence of accompanying debilitating symptoms.     

X-linked Ichthyosis Presenting as Erythroderma: A Rare Case

X-linked ichthyosis is a rare form of dermatological disease and when it presents as erythroderma it is even rarer. History of consanguineous marriage and prolonged labor during birth of patient, generalized scaling which gets better in summer months, flexural involvement, cryptorchidism made a diagnosis of X-linked ichthyosis. We report this case because of its rarity as erythroderma.     

Rare manifestation of hypereosinophilic syndrome: Diffuse-type hair loss with massive perifollicular eosinophils

A 46-year-old woman consulted our hospital with diffuse alopecia and blood eosinophilia. Histological examination of the scalp revealed dense eosinophilic infiltration around the hair follicles and in the surrounding subcutis. Oral corticosteroid was effective to reduce hair loss and blood eosinophilia, but these conditions immediately relapsed after ending treatment. In addition to alopecia, she had diarrhea and colitis showing histological findings of dense eosinophilic infiltrations in the submucosa. We diagnosed hypereosinophilic syndrome based on hypereosinophilia of blood and tissue with clinical symptoms of alopecia and diarrhea. We suppose diffuse alopecia showing massive eosinophilic infiltration around the hair follicle is a rare symptom of hypereosinophilic syndrome.     

Erythroderma, an unusual manifestation of B cell lymphoma

A case is described in which the initial manifestation of a malignant lymphoma was an erythematous skin rash over the face, scalp and trunk. Immunological surface marker analysis showed the malignant cells to be of B lymphocyte origin with a homogeneous secretion of IgM-k. Repeated skin and lymph node biopsies and finally post mortem documented the progression of the lymphoma from that of a mixed lymphocytic-histiocytic type with a nodular pattern of growth to a diffuse histiocytic lymphoma with frequently bizarre cells. The theory of ecotaxis or homing of lymphocytes to the skin and other non-lymphoid organs of the body is discussed in an attempt to explain the occurrence of cutaneous involvement as a primary manifestation of lymphcma.     

红皮病性大疱性类天疱疮报告1例

报告1例红皮病性大疱性类天疱疮。患者男,66岁,躯干、四肢皮肤水疱、红斑脱屑2月,加重2周就诊。全身皮肤红斑、脱屑伴浸润,组织病理和免疫病理符合大疱性类天疱疮诊断。