甲扁平苔藓研究进展

甲扁平苔藓临床少见,可伴有皮肤扁平苔藓或独立存在.甲扁平苔藓的流行病学研究较少,其流行病学特点尚未完全阐明.甲扁平苔藓分5型:Ⅰ型为典型皮损伴有甲损害,Ⅱ型为不典型皮损伴有甲损害,Ⅲ型为头皮损害伴有甲损害,Ⅳ型为黏膜损害伴有甲损害,Ⅴ型为单纯甲损害.甲扁平苔藓有许多特征性临床表现,如翼状胬肉.临床上需与甲真菌病及其他可引起甲营养不良的疾病鉴别.甲扁平苔藓的组织病理特征与其他部位的扁平苔藓类似,皮肤镜有助于辅助诊断本病.本病治疗困难,缺少特效治疗方法.系统应用激素是唯一有循证研究支持的疗法.阿维A治疗甲扁平苔藓有效.甲氨蝶呤、环孢素等免疫抑制剂也可有效治疗甲扁平苔藓.甲扁平苔藓预后差,复发率高.     

Eosinophilic panniculitis: a clinicopathologic study

Study of 18 patients with biopsy diagnoses of eosinophilic panniculitis revealed diverse patterns of systemic disease, including Wells' syndrome, vasculitis, atopy, and erythema nodosum as well as localized panniculitis. Significant associated diseases included psychiatric illness, 6 (drug dependency, 4); atopy, 5 (asthma, 3); malignancies, 5; immune complex vasculitis, 4; thyroid disease, 2; Wells' eosinophilic cellulitis, 2; glomerulonephritis and sarcoidosis, 1 each. The skin lesions varied from urticarial papules and plaques to purpura, pustules, and ulcerative lesions but always included a nodular subcutaneous component, frequently as a presenting complaint. Eosinophilic panniculitis is a non-specific finding that can signify localized disease, such as an insect bite or injection lipophagic granuloma in a drug-dependent patient, or systemic lymphoma or immune reactive disease. Eosinophilic panniculitis in erythema nodosum is perhaps its most confusing presentation.     

Case of keratosis lichenoides chronica with atypical sarcoidal granulomatous inflammation

Keratosis lichenoides chronica (KLC) is a rare chronic disease characterized by violaceous, papular and nodular lesions typically arranged in a linear and reticulate pattern. The etiology of KLC is unknown, but it may be associated with internal diseases such as hypothyroidism, glomerulonephritis and lymphoproliferative disorders. Herein, we describe the case of 44-year-old male patient with characteristic lesions of KLC on the trunk and extremities, present for 12 years. The clinical diagnosis was proven by histopathological examination on several occasions. In the years following the diagnosis of KLC, he developed bilateral hilar and multiple mediastinal, cervical and inguinal lymphadenopathies and hepatosplenomegaly. In 2000, diffuse interstitial and then reticulonodular pulmonary infiltrates associated with fever, weight loss, malaise and subcutaneous nodules developed. Biopsies taken from peripheral and mediastinal lymph nodes, pulmonary parenchyma, pleural tissue, bone marrow and skin showed non-necrotizing granulomas, indicating a sarcoidal granulomatous reaction. Characteristic histopathological findings and the absence of atypical cells, clonality and a high proliferative index excluded lymphomas. Furthermore, detailed tests showed no evidence of an infectious granulomatous disease. As far as we know, this is the first reported case of KLC associated with a sarcoidal granulomatous reaction.     

Vascular variant of keratosis lichenoides chronica associated with hypothyroidism and response to tacalcitol and acitretin

Keratosis lichenoides chronica (KLC) is a rare chronic progressive cutaneous disease that is part of the heterogeneous group of lichenoid dermatoses. The typical clinical presentation is characterized by lichenoid hyperkeratotic papules and nodules arranged in a linear and reticular pattern on the trunk and extremities. Our case confirms the existence of a vascular variant of KLC. There is no consensus about its treatment, since it is refractory to many different treatment modalities. We report the effectiveness of acitretin in KLC in combination with tacalcitol. KLC is of unknown aetiology, but is perhaps associated with systemic diseases, most importantly glomerulonephritis and lymphoma. This is the second case associated with hypothyroidism.     

Panniculitis. Part II. Mostly lobular panniculitis

The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and alpha(1)-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) Learning objective: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.     

Role of slit skin smear examination in cutaneous T-cell lymphomas and other chronic dermatoses

The purpose of this study was to evaluate the utility of slit-skin smear examination in the diagnosis of various chronic dermatoses. The study included 24 patients with chronic dermatoses who presented to the skin outpatient department. Ten patients had idiopathic erythroderma, seven were diagnosed with airborne contact dermatitis, four had cutaneous T-cell lymphoma, two had chronic actinic dermatoses, and a single patient had panniculitis. Slit skin smears were obtained from all patients, stained with Leishman stain, and examined under microscope. Out of 24 patients, all four cases of cutaneous T-cell lymphoma showed abnormal cells suggestive of lymphomatous infiltration, two patients with airborne contact dermatitis showed reactive lymphocytes, and two idiopathic erythroderma cases showed numerous eosinophils in the smear. Slit skin smear examination is a simple rapid, decisive test in the diagnosis of cutaneous T-cell lymphoma. It is a useful screening test, especially in Sezary syndrome and diseases with specific skin infiltrate.     

From circumscribed scleroderma (morphea) to subcutaneous panniculitis-like T-cell lymphoma: case report

Subcutaneous panniculitis-like T-cell lymphoma is a rare form of non-Hodgkin lymphoma. It presents clinically as panniculitis, with erythematous, firm subcutaneous infiltrates and recurrent papulonodules. A 45-year-old male Croat presented with a 6-month history of tender erythematous subcutaneous skin lesions without systemic symptoms. Analysis of a lesion biopsy specimen showed circumscribed scleroderma (morphea). During hospitalization, skin biopsies from two subcutaneous lesions on the stomach were repeated, and pathohistologic analysis corresponded primarily to panniculitis in the context of morphea. Pathohistologic analysis and immunohistochemistry of a specimen obtained by tumor excision from the back indicated subcutaneous panniculitis-like T-cell lymphoma. Histologic analysis revealed a dense infiltrate of atypical T-lymphoid cells expressing CD8(+) phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. Specific hematologic analysis and medical treatment were continued. This case report illustrates the importance of continuous follow-up with repeat biopsy and use of immunohistologic techniques for early diagnosis.     

Non‐infectious inflammatory disorders of the nail apparatus

Inflammatory non‐infectious diseases of the nails are not uncommon. The nail changes may look different in the same diseases, but also very much alike in various different nail disorders depending on which particular structure of the nail apparatus is involved. Of all skin diseases, psoriasis is the one with the most frequent nail involvement. The presence of many pits allows the diagnosis to be made, but salmon spots and onycholysis are also characteristic features. Lichen planus may occur isolated on the nails or in association with lesions of the skin and adjacent mucosal membranes. Longitudinal striations and loss of the nail plate shine are the most frequent features of nail lichen planus. The more severe the alopecia areata is and the younger the patient is, the more often it affects the nail, usually with a rough surface and loss of shine. Eczemas and various autoimmune dermatoses may also affect the nails but the changes are not clinically specific.     

Fatal cytophagic histiocytic panniculitis after a short response to cyclosporine

Cytophagic histiocytic panniculitis (CHP) is a disorder characterized by a histiocytic infiltrate in the subcutaneous adipose tissue which often extends to involve systemic organs. Fever, pancytopenia, hepatosplenomegaly, mucosal ulcers and serositis are common systemic symptoms. Although remissions have been reported, the disease tends to follow a chronic course often complicated by terminal hemorrhagic diathesis and death. Several reports in the recent literature have suggested that cyclosporine is the treatment of choice in CHP. We report the first case of cyclosporine treatment failure in CPH. Despite an initial response to treatment and persistent resolution of cutaneous lesions, the internal disease progressed resulting in the patient's death. Therefore the evolution of skin lesions and signs of systemic involvement may not be an accurate assessment of disease activity in patients with CHP being treated with cyclosporine.     

Sclerosing panniculitis

Sclerosing panniculitis is a chronic panniculitis characterized by skin induration and hyperpigmentation of one or both legs that often occurs in patients who have venous insufficiency. The indurated plaques are often painful and the legs frequently have a characteristic "inverted wine bottle" appearance. Histopathology is characterized by mostly lobular panniculitis with necrosis of fat and without vasculitis, and in fully developed lesions by septal sclerosis and membranocystic changes. Treatment of sclerosing panniculitis includes compression therapy with graded stockings and anabolic steroids.     

Pediatric onset keratosis lichenoides chronica: a case report

Keratosis lichenoides chronica (KLC) is an acquired keratinization disorder that is rare in childhood. We report a case of sporadic pediatric-onset KLC with seborrheic dermatitis-like lesions on the forehead, papules in a retiform pattern in the axillae and mons pubis, and eye and oral mucosal involvement,with additional features of premature canities and a transient photosensitive eruption.     

Sclerosing postirradiation panniculitis

Sclerosing postirradiation panniculitis is an unusual variant of panniculitis that appears as a complication of megavoltage radiotherapy. Most patient are women with a history of breast cancer who develop nodular lesions on the anterior chest skin several months after receiving megavoltage therapy for breast carcinoma. Sclerosing postirradiation panniculitis is an unusual cutaneous complication of megavoltage radiotherapy that should be distinguished from subcutaneous metastatic disease, cellulitis, and connective tissue diseases involving the subcutaneous fat. The differential diagnosis can be established on the basis of the characteristic histopathologic features.     

Keratosis lichenoides chronica: proposal of a concept

It has been a subject of controversy whether keratosis lichenoides chronica (KLC) is a distinctive inflammatory disease of the skin or whether it represents a manifestation of another well-known disease, such as lichen planus, lupus erythematosus, or lichen simplex chronicus. In search of clear criteria for diagnosis of KLC the entire literature pertinent to the subject was studied and findings clinical and histopathologic as they were telegraphed in them were compared with a patient of my own experience. Review of the literature reveals more than 60 patients in whom the diagnosis of KLC was made. Three categories emerge based on whether the findings presented in a particular article (1) do not permit any diagnosis to be rendered; (2) do allow a diagnosis specific to be made, such as of lichen simplex, lichen planus, or lupus erythematosus; or (3) do not correspond to any disease well defined, such as lichen simplex, lichen planus, lupus erythematosus, but seem to show attributes morphologic, clinically and histopathologically, that are repeatable. Patients diagnosed as having KLC obviously represent a potpourri of different diseases, the most common of them being lichen simplex chronicus, lichen planus, and lupus erythematosus. Fewer than 25 patients reported on, however, presented themselves with lesions very similar to one another clinically, namely, an eruption that involved the face in a manner reminiscent of seborrheic dermatitis and with tiny papules on the trunk and extremities, which assumed linear and reticulate shapes by way of confluence of lesions. Individual papules were infundibulocentric and acrosyringocentric. Findings histopathologic were those of a lichenoid interface dermatitis affiliated with numerous necrotic keratocytes and covered by parakeratosis housing neutrophils in staggered fashion. These patients seem to have an authentic and distinctive condition that is exceedingly rare. In conclusion, the diagnosis of KLC should be made only for patients who present themselves with features clinical and findings histopathologic that resemble closely those of what is summarized in this article under category 3.     

Nasal-type natural killer cell lymphoma preceded by benign panniculitis arising in an asymptomatic HTLV-1 carrier

We report a case of an Epstein-Barr virus (EBV)-associated nasal-type natural killer cell lymphoma (NKCL) preceded by benign panniculitis, which arose in a 48-year-old woman with an asymptomatic human T-cell leukemia/lymphoma virus type-1 (HTLV-1) infection. A biopsy of the initial panniculitis lesion demonstrated lobular panniculitis with a germinal center composed of benign mononuclear cells with a phenotype of CD4+CD45RO+CD5sCD3+ cCD3 epsilon + T-cell intracellular antigen-1 (TIA-1)- and granzyme B-. One year after oral prednisolone therapy, the patient developed subcutaneous nodules composed of atypical lymphoid cells with a phenotype of CD4-CD45RO+CD56+sCD3-cCD3 epsilon + (TIA-1)+ and granzyme B+. In the initial panniculitis lesion, neither EBV-encoded RNA (EBER-1) nor clonal proliferation of EBV-infected cells was identified. In later lesions, however, a large number of atypical cells were positive for EBER-1, and a clonal expansion of EBV-infected cells was detected. No clonal rearrangement of T-cell receptor-alpha, -beta, or -gamma genes was found in either specimen. This patient was an asymptomatic carrier of human T-cell leukemia/lymphoma virus type-1 (HTLV-1) without clonal integration of proviral HTLV-1 in neither the peripheral blood nor the skin lesions. These observations suggest that EBV-associated NKCL occurred subsequently in the clinical course of benign panniculitis under the influence of immunosuppression caused by prednisolone treatment and HTLV-1 infection.     

Subcutaneous panniculitic T-cell lymphoma and cytophagic histiocytic panniculitis

A 43-year-old Maori man presented with a 1 month history of malaise, weight loss, anorexia, arthralgia, recurrent fever and tender erythematous subcutaneous skin lesions. Histological examination of an incisional biopsy of a lesion revealed a lobular panniculitis with an inflammatory infiltrate of atypical lymphocytes and evidence of cytophagocytosis consistent with a diagnosis of subcutaneous T-cell lymphoma. The systemic symptoms and skin lesions resolved spontaneously within 3 weeks, only to recur 2 months later, requiring treatment with oral prednisolone. T-cell gene rearrangement studies demonstrated a monoclonal T-cell receptor (gamma-chain) gene rearrangement, further supporting the diagnosis of subcutaneous panniculitic T-cell lymphoma. Treatment with chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) led to remission of symptoms. Four months after completing chemotherapy, the patient remained asymptomatic with a few indurated subcutaneous plaques on the chest. Biopsy of these areas revealed lobular panniculitis, lymphocytic infiltrate without cytological atypia, abundant lipophages and fibrosis and sclerosis consistent with a healing response. He remains well 24 months following chemotherapy.     

Subcutaneous panniculitis-like T-cell cutaneous lymphoma

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma classified in the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification as a unique extranodal lymphoma with characteristic by T cell receptor (TCR) gene rearrangement. We report here a case of SPTCL in a 22 year-old woman who had presented with variably sized multiple nodules on both her legs. Initial differential diagnoses considered were panniculitis and lupus panniculitis. The histopathology showed a predominantly subcutaneous lobular infiltrate with atypical lymphocytes, karyorrhexis and rimming of adipocytes by lymphoid cells. Immunohistochemistry showed CD4-, CD8+, CD56- T-cell phenotype. Although TCR rearrangement studies were not done, the above T-cell phenotype and sparing of epidermis and dermis suggested the possibility of an SPTCL alpha/beta type. The patient received five cycles of a cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) regimen which resulted in the regression in her skin lesions and constitutional symptoms.     

Lupus erythematodes

The typical clinical forms of cutaneous lupus erythematosus (LE) are the butterfly rash, acute, subacute and chronic cutaneous lupus, intermediate lupus (lupus tumidus), chilblain- and bullous lupus, lupus profundus, and ulcerating lesions on the mucous membrane. Besides the typical lupus forms, nonspecific skin lesions are also observed such as dermal mucinosis, acneiform skin lesions, different variants of livedo, necrotizing vasculitis with ulcers, purpura, urticaria vasculitis, neutrophilic dermatosis, hyperpigmentation, hair and nail changes as well as overlap syndromes with erythema multiforme, scleroderma, Sjögren syndrome, Raynaud phenomenon, lichen planus, bullous pemphigoid und psoriasis. There are lupus imitators which create differential diagnostic challenges, such as infections with atypical mycobacteria or subcutaneous T-cell lymphoma both of which are similar to lupus profundus. All these skin lesions can present as maximal pathological findings seen in lupus or be caused by a variety of pathological laboratory findings such as the anti-phospholipid antibodies or a deficiency of complement factors. In the latter situation severe lupus often with complications can be expected.     

Lupus erythematosus panniculitis in a patient with autoimmune hepatitis

Lupus erythematosus panniculitis (profundus), a rare variant of chronic panniculitis, sometimes develops during the course of discoid lupus erythematosus or systemic lupus erythematosus. A 61-year-old woman had suffered from autoimmune hepatitis type I for 5 years. Prednisolone had been administered as maintenance therapy and her hepatitis had been well controlled. However, asymptomatic erythematous indurated nodules developed symmetrically in both pre-auricular regions, and skin biopsy revealed lupus erythematosus panniculitis (profundus). Increase in dosage of prednisolone resolved the skin lesion, leaving depressed atrophic scars. This is the first report of lupus erythematosus panniculitis complicating autoimmune hepatitis.     

伴有皮肤表现的慢性B淋巴细胞白血病

报告1例伴有特异性和非特异性皮损的慢性B淋巴细胞白血病.患者男,66岁.临床表现为全身广泛淋巴结增大;躯干及四肢出现红斑、丘疹、坏死;左耳郭有浸润性肿块.皮损组织病理检查示左耳郭皮损为淋巴细胞白血病皮肤浸润,其余皮损为血管周围炎及脂膜炎改变.经淋巴结组织病理、骨髓细胞学、骨髓组织病理及流式细胞仪检测诊断为慢性B淋巴细胞白血病.     

Pseudomembranous changes in the dermis: A novel observation and potential clue for evolving lipodermatosclerosis?

Lipodermatosclerosis (LDS) is a well‐recognized form of fibrosing panniculitis. Although chronic cases are readily diagnosed, early and evolving lesions may be clinically mistaken for cellulitis and other forms of panniculitis. Most pathologists are familiar with a pseudomembranous type of fat necrosis as a useful feature of chronic LDS. Although nonspecific, this distinctive pattern of fat necrosis helps in supporting a diagnosis of LDS in the appropriate clinical context. The histopathologic features of early and evolving LDS and those involving the dermis are less well documented. We report a case of early LDS showing extensive pseudomembranous changes in the dermis on a superficial skin biopsy where progression to a classic established lesion was documented clinically. We suspect that this previously unreported and unusual finding may be a histopathologic clue for evolving lesions of LDS.