Multiple cutaneous reticulohistiocytosis

BACKGROUND: Multicentric reticulohistiocytosis is a non Langerhans cell histiocytosis. This rare disease is characterized by cutaneous papules and nodules and a destructive polyarthritis; multisystem involvement may occur. Multiple and diffuse cutaneous reticulohistiocytosis have been more rarely reported. We present a case which was distinctive by the existence of multiple cutaneous plaques. CASE REPORT: A 65-year-old woman presented cutaneous papules and nodules associated with a destructive arthritis affecting the hands. Histological examination of a cutaneous biopsy associated with immunophenotyping and electronic microscopy permitted us to make the diagnosis of multicentric reticulohistiocytosis. The search for visceral involvement or underlying neoplasia was negative. Rapidly, cutaneous aggravation occurred with multiple and diffuse infiltrated plaques on the back, the face, the ears, the thighs and the forearms. The same histological aspect was found for these lesions. Treatment with corticosteroids and cyclophosphamide was successful. DISCUSSION: This case report is the first one with diffuse cutaneous lesions of multicentric reticulohistiocytosis with aspect of infiltrated plaques. Diffuse cutaneous lesions in multicentric reticulohistiocytosis have been rarely reported with diffuse papules or nodules pattern. A visceral involvement seems to be more frequent for diffuse cutaneous involvement. In all cases, the association of multicentric reticulohistiocytosis with neoplasia in up to 25 p. 100 is of interest. Treatment of multicentric reticulohistiocytosis consists in corticosteroids at the initial phase associated with alkylants agents or methotrexate.     

Diffuse cutaneous reticulohistiocytosis

The reticulohistiocytoses make up a heterogeneous group of diseases whose origin lies in an accumulation of cells of histiocytic lineage in different tissues and primarily in the skin. Three main clinical forms have been described (multicentric, solitary, diffuse cutaneous), which present with identical histological, ultrastructural and immunohistochemical characteristics. We present a case of diffuse cutaneous reticulohistiocytosis, which is the least common clinical pattern in the spectrum of this disease.     

Multicentric reticulohistiocytosis

Multicentric reticulohistiocytosis is a rare granulomatous disease of unknown etiology, characterized by cutaneous nodules and destructive arthritis. Skin lesions can cause significant deformity, and approximately half of affected patients develop a severe disabling arthritis. The disease is often associated with malignancy; however, the paraneoplastic nature of multicentric reticulohistiocytosis is not established. The diagnosis is confirmed by the presence of oncocytic (“ground-glass”) histiocytes and multinucleated giant cells on histopathology of the cutaneous nodules and the synovial membrane.     

多中心网状组织细胞增生症1例

患者女,55岁,面颈部皮疹1年,指关节变形6个月.皮肤科情况:面颈部、耳廓弥漫性红斑,以曝光部位为主;鼻周散在黄红色丘疹,无破溃.齿龈见红色丘疹,前胸V字区见水肿性红斑,无水疱.双手X线片示近节及远节指间关节均有不同程度破坏,致使关节畸形,软组织肿胀.鼻翼皮损组织病理示:真皮内大量组织细胞及嗜酸性胞浆的多核巨细胞浸润....     

伴有肺部损害的多中心网状组织细胞增生症1例

报道1例伴有肺部损害的多中心网状组织细胞增生症。患者男、43岁。其临床特征为全身皮肤多发性丘疹和结节,毁损性关节炎和肺部多发性小结节。皮肤及肺结节组织病理示大量浆呈伊红和毛玻璃样外观的组织细胞和多核巨细胞浸润;免疫组化染色：CD68（＋）,S－100（－）,HHF35（－）,AE1／AE3（－）。     

多中心网状组织细胞增生症1例

报告1例多中心网状组织细胞增生症。患者男,50岁。四肢关节疼痛1年,头面部,躯干、四肢起丘疹、结节4个月。皮损组织病理改变符合多中心网状组织细胞增生症。给予雷公藤多苷片,泼尼松治疗后好转。     

Paraneoplastic multicentric reticulohistiocytosis: a clinicopathologic challenge

Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.     

Multicentric reticulohistiocytosis in a child

A case of multicentric reticulohistiocytosis in an 8-year-old girl, which is a diagnosis rarely seen in children, is presented. Multicentric reticulohistiocytosis is a disorder of unknown aetiology, predominantly affecting the joints, skin and mucosa. Joint symptoms, but not cutaneous lesions, have improved with treatment with methotrexate.     

Ultraviolet light-induced Köbner phenomenon contributes to the development of skin eruptions in multicentric reticulohistiocytosis

Multicentric reticulohistiocytosis is a rare systemic disease of unknown aetiology characterized by erosive arthritis and cutaneous lesions consisting of multiple reddish-brown papules and nodules, mainly involving the face and distal upper extremities. It has been suggested that skin eruptions in multicentric reticulohistiocytosis are associated with K?bner phenomenon due to their characteristic distribution, such as on the dorsal aspects of the hands and fingers. We report here a case of a Japanese woman with multicentric reticulohistiocytosis, in whom erythematous macules and papules were widely distributed over the face, ears, neck and the V-area of the chest. Notably, repeated irradiation of ultraviolet (UV) B on the uninvolved back skin resulted in the induction of erythematous macules with infiltration of reticulohistiocytes, indicating the association of UVB-induced K?bner phenomenon with the development of skin lesions, especially on the sun-exposed area. This is the first known report demonstrating the contribution of UV-light-induced K?bner phenomenon for the development of skin eruptions in patients with multicentric reticulo-histiocytosis.     

Multicentric reticulohistiocytosis

A case of multicentric reticulohistiocytosis associated with a pleural mesothelioma is reported. The cutaneous and joint manifestations resolved after treatment with cyclophosphamide, and the remission persisted until the patient's death 3 years later. As corticosteroids have such an unpredictable effect on this condition, cyclophosphamide may be regarded as the treatment of choice until more cases can be assessed.     

Multicentric reticulohistiocytosis associated with Burkitt lymphoma and adenocarcinoma

Multicentric reticulohistiocytosis (MRH) is a rare disease of unknown etiology characterized by cutaneous nodules and destructive, sometimes crippling, polyarthritis. The diagnosis is confirmed by histopathologic features of the cutaneous nodules or synovial tissue, including an infiltrate composed of histiocytes, many of them multinucleate, with a ground glass appearance. Multicentric reticulohistiocytosis has been associated with a number of chronic conditions and various malignancies. We report a case of MRH in a patient with Burkitt lymphoma and metastatic adenocarcinoma of the gastrointestinal tract.     

Multicentric reticulohistiocytosis-mimicking dermatomyositis. Case report and review of the literature

Multicentric reticulohistiocytosis is a rare disorder of unknown etiology, characterized by skin and mucosal papulonodular eruptions and destructive polyarthritis. Histopathological study of these lesions shows a nodular infiltrate composed of histiocytes and multinucleated giant cells, with an eosinophilic, granular, 'ground-glass' cytoplasm. We report a case of multicentric reticulohistiocytosis with skin lesions mimicking dermatomyositis and we also review previously reported cases describing such a clinical situation. Our case further emphasizes that multicentric reticulohistiocytosis can mimic clinical features of dermatomyositis. A macular or patch-like erythema in a photodistributed fashion, in addition to other clinical manifestations, can be mistaken for dermatomyositis. However, skin biopsies from these areas may early differentiate both conditions with different treatment options and morbidity.     

Multicentric reticulohistiocytosis presenting with a rash and arthralgia

A case of multicentric reticulohistiocytosis in a 53-year-old woman with a history of generalized cutaneous eruptions followed by arthralgia is presented. A thorough malignancy screen was performed with negative results. Treatment was commenced with oral prednisone 20 mg daily, which rapidly resulted in marked improvement of joint symptoms and resolution of self-perceived functional disability. As there was little improvement of her rash, oral azathioprine was added at 100 mg daily. Both drugs were well-tolerated and the patient was discharged from hospital on these medications. Two months later the azathioprine was reduced to 50 mg per day orally, following a small rise in hepatic transaminases. Within 4 months there had been dramatic improvement in the rash and cutaneous nodules with complete remission of the arthralgia and pruritus. During this time the oral prednisone had gradually been decreased to 10 mg daily. To date only the periungual nodules persist.     

Extramammary Paget's disease: prognosis and relationship to internal malignancy

Extramammary Paget's disease is a rare cutaneous adenocarcinoma, usually of epidermal origin and glandular differentiation and frequently associated with an underlying adnexal carcinoma and perhaps with underlying internal malignancy. One hundred ninety-seven cases of extramammary Paget's disease (196 cases reported in the English literature from 1962 to 1982 and one case of my own) are reviewed. It remains a rare cutaneous malignancy that occurs primarily in elderly people. It is seen more frequently in women than in men and occurs predominantly in vulvar and perianal locations. Twenty-six percent of patients with this disease will ultimately die of it or an associated internal malignancy. Twenty-four percent of patients with the disease have an associated underlying cutaneous adnexal adenocarcinoma. These patients have a higher mortality rate--46%--than patients with extramammary Paget's disease without underlying cutaneous adnexal adenocarcinoma. Twelve percent of patients with extramammary Paget's disease have an associated concurrent underlying internal malignancy. The location of the underlying internal malignancy appears to be closely related to the location of the extramammary Paget's disease--i.e., a perianal location is associated with adenocarcinoma of the digestive system, a penile-scrotal-groin location with genitourinary malignancy, etc. A directed internal malignancy search may be of benefit in patients who are diagnosed as having extramammary Paget's disease.     

多中心网状组织细胞增生症 1例

患者,男，65岁。手部皮色丘疹结节伴关节痛3月余，组织病理示：真皮可见大量组织细胞和多核巨细胞，胞体大，胞浆丰富，部分胞浆红染，呈“毛玻璃”样。PAS染色阳性，免疫组化Vimentin（+），CD68（+），CD1a（-），S100（-）。诊断：多中心网状组织细胞增生症。经“强的松、甲氨蝶呤、雷公藤”联合口服治疗后皮疹和关节症状明显改善。     

A case of multicentric reticulohistiocytosis with multiple lytic skull lesions

Multicentric reticulohistiocytosis (MR) is a rare multisystemic disorder of unknown aetiology characterized by cutaneous and joint manifestations. It is associated with malignancy in up to 31% of cases. Common radiological findings are peri-articular erosions and osteolytic punched-out areas. We present a case of MR with cutaneous nodules, joint pains, and multiple lytic skull lesions--a combination that has not been described before. Osteolytic activity of proinflammatory cytokines (tumour necrosis factor-alpha and interleukin-1) may explain the peri-articular erosions often seen in MR, and the multiple lytic skull lesions seen in our patient.     

Cardiac and systemic complications in multicentric reticulohistiocytosis

A 75-year-old man with a short history of cutaneous lesions of multicentric reticulohistiocytosis, preceded by a few months of a symmetrical polyarthritis is described. Within 5 months of onset of symptoms, he developed congestive cardiac failure secondary to pericardial involvement by the disease and succumbed despite therapy with cyclophosphamide and methylprednisolone. Postmortem revealed the true extent of the disease, with nodules seen in the epiglottis and aryepiglottic folds, duodena) mesentery, pleura, pericardium and myocardium, Although the hallmarks of the disease are the papulonodular skin lesions, together with a severe, sometimes mutilating polyarthropathy, its widespread systemic nature is not often appreciated. We review five other cases in the literature with pericardial involvement and discuss aids to earlier diagnosis by synovial fluid cytology; gallium scanning is discussed as a potentially useful means of detecting the extent of systemic involvement in multicentric reticulohistiocytosis.     

Spectrum of cutaneous metastases in 1287 cases of internal malignancies: a study from Turkey

Cutaneous metastases may be either the initial manifestation of an internal malignancy or represent recurrent neoplastic disease. The aim of this study was to investigate the incidence and characteristics of cutaneous metastases in cases of internal malignancy. A total of 1287 patients with internal malignancy were included in the study. Dermatological examinations were performed on all of the patients. Skin biopsies were obtained from the suspected lesions. The type of malignancy, the time of diagnosis of the malignancy, the presence of cutaneous metastasis, and the localization sites of the cutaneous malignancy were noted. Metastases of any kind were seen in 27.4% of cases. Cutaneous metastases were seen in 1.2% of cases and were most frequently localized on the anterior chest as nodules. Cutaneous metastasis was the first sign of internal malignancy in one case. It is concluded that cutaneous metastases occur rarely and the presentation of internal malignancy with skin involvement is uncommon.     

Cutaneous histiocytic malignancy. Immunohistochemical re-examination of cases previously diagnosed as cutaneous "histiocytic lymphoma" and "malignant histiocytosis"

True histiocytic lymphoma (THL) and malignant histiocytosis (MH) have been defined by clinical and histologic findings and enzyme histochemistry. We reviewed cases previously diagnosed as cutaneous histiocytic lymphoma (HL) and MH with cutaneous lesions. These cases had been classified as "histiocytic" on the basis of previous enzyme histochemistry profiles of frozen tissue. Cutaneous tumor cells were reevaluated using a panel of immunohistochemical stains in formalin-fixed, paraffin-embedded tissue in correlation with histopathologic examination. The antibodies used in this study were directed against CD45 (leukocyte common antigen [LCA]), CD20 (L26) for B cells, CDS and CD45RO (UCHL-1) for T cells, CD68 (KP-1) and lysozyme for histiocytes, as well as CD30 (BerH2) for Ki-1 positive cells. On re-evaluation, the seven cases originally classified as HL were reclassified as one case of THL with neoplastic cells positive for CD68 (KP-1) and lysozyme, two cases with immunohistochemical features of Ki-1 lymphoma (including one of T-cell lineage), three cases of T-cell lymphoma, and one case of B-cell lymphoma, all associated with variable degrees of reactive histiocytosis. The four cases originally classified as MH were reclassified as two cases of MH and two cases of uncertain lineage. Although rare, histiocytic malignancies do exist. However, the diagnosis of histiocytic malignancy should be made only after careful correlation of atypical tumor cells in histopathologic sections and sections stained immunohistochemically. Erroneous classification of reactive histiocytes as neoplastic histiocytes using only enzyme histochemistry in frozen sections is a pitfall to be avoided.     

Development of Ki-1 lymphoma in a child suffering from multicentric reticulohistiocytosis.

We report a case of Ki-1 lymphoma that developed in a 16-year-old youth who had suffered from multicentric reticulohistiocytosis for 10 years. Over the past 3 years he had had a peculiar sclerosing lesion of the leg for which oral prednisone 5 mg daily was tried for one year, with a moderate effect. He developed a marked swelling of the inguinal lymphadenopathy on the same side as the affected leg lesion, which also developed a prominent swelling of the skin surrounding the sclerosed area. Immunohistochemical analysis of the lymph node biopsy revealed the features of Ki-1 lymphoma. This is the first case of association of multicentric reticulohistiocytosis with Ki-1 lymphoma.