Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients

BACKGROUND: Mastocytosis is a rare, heterogeneous group of disorder with abnormal increase of mast cells in one or more organ systems. OBJECTIVE: To evaluate the demographic and clinical features of cutaneous mastocytosis (CM). METHODS: Records of 55 patients with cutaneous mastocytosis were retrospectively analysed. RESULTS: Of the 22 females and 33 males, 80% had urticaria pigmentosa/maculopapular CM and 20% had mastocytoma. Of all cases, 81.8% had first lesions in childhood. The most common presentation was involvement of trunk together with extremities. Thirteen (23.6%) patients had history of bulla; Darier's sign was positive in 34 of 38 patients. Itching was the most common complaint, provocated by hot weather/bath. CONCLUSION: Clinical presentations of urticaria pigmentosa/maculopapular CM and mastocytoma are similar regarding gender, age of onset, age of diagnosis, and presence of Darier's sign and history of bulla. In contrast to mastocytoma, urticaria pigmentosa/maculopapular CM lesions were frequently located on trunk together with extremities.     

Cutaneous solar ultraviolet exposure and clinical aspects of photodamage

Solar ultraviolet (UV) radiation reaching the earth is a combination of UVB (290-320 nm) and UVA (320-400 nm) wavelengths. Since UVA is less energetic than UVB, UVB has long been thought to be the factor responsible for the damaging effects of solar radiation. But with modern tools such as in vitro models, it has been proven that UVA plays a major role. The objective of this review is to show how skin may be exposed to UV light and to highlight the clinical aspects of UV-induced skin damages with the respective contribution of UVB or UVA. Even if UVA is less energetic than UVB, it is more abundant and penetrates deeper into the skin, reaching as far as the dermis. Various factors also influence skin exposure to UV light: the latitude, season, and time of the day. Acute as well as chronic sun exposure induces short- and long-term clinical damages. Erythema and pigmentation are immediate responses of normal human skin exposed to UV radiation. The long-term effects are photoaging and photocarcinogenesis. In particular, UVA appears to play a major role in the deterioration of dermal structure leading to the photoaged appearance of the skin.     

Cutaneous leishmania in HIV patient in Ouagadougou: clinical and therapeutic aspects

BACKGROUND: Immune suppression cause by HIV infection is a risk factor in the progression of leishmania diseases. In Burkina Faso atypical clinical presentations of leishmaniases have been observed among people living with HIV. The goal of this study was to describe clinical and evolutionary aspects of cutaneous leishmania and HIV co-infection among patients followed at Ouagadougou University Hospital. PATIENTS AND METHODS: This 16-month prospective study was carried out from January 2003 to April 2004 among HIV-seropositive patients with a diagnosed cutaneous leishmania infection. At baseline, infection and lesions were classified. Clinical diagnosis of cutaneous leishmania depended on finding parasites by microscopy in smears or tissue biopsies. Histological examinations were done if clinical and parasitological diagnosis were not concordant. Treatment consisted of three 21-day rounds of pentavalent antimonial, (Glucantime(R)). Clinical evolution was monitored at the end of each treatment round. RESULTS: Thirty-two HIV-1 positive patients (16 women and 16 men) were included. Mean age was 35.5 (10-67 years old). Leishmania lesions had been evolving, on average, for 12 weeks. Eleven patients were taking HAART and 21 patients were taking cotrimoxazole prophylaxis against opportunistic infections. Cutaneous lesions were found: in the face (15 cases), torso (18 cases), upperlimbs (26 cases) and lower-limbs (28 cases). Observed clinical forms were: papulo-nodular (9 cases), ulcerative (14 cases), infiltrative (12 cases), lepromatous and diffuse (15 cases), psoriasis-like (5 cases), cheloid, histioid or kaposi-like (1 case each). Some patients presented more than one clinical form. Prognosis was satisfactory in 24 patients after the first treatment. Twelve patients relapsed after the first treatment, among those 10 were only taking cotrimoxazole. At the end of the third treatment, 24 patients were cured, 3 died and 5 were lost to follow-up. CONCLUSION: Clinical polymorphism of cutaneous leishmania has been observed in HIV-patients, thereby increasing the risk of differential diagnosis.     

Cutaneous clear cell squamous cell carcinoma in situ : clinical, histological and immunohistochemical characterization

BACKGROUND: Clear cell squamous cell carcinoma in situ (SCCIS) has not been defined in the literature with respect to its clinical, histological, and immunohistochemical features. METHODS: Nearly 1500 consecutive cases of SCCIS were assessed for percent clear cell change. The clinical features of all SCCIS with >or=10% clear cell change, including age, sex and site distribution, were compared with classical SCCIS using chi-square analysis. PAS special staining and immunohistochemical analysis with 11 cell markers were performed to characterize the clear cell of origin. RESULTS: Eighty SCCIS cases with a spectrum of clear cell change of >or=10% were identified. Six cases with >or=80% clear cells were defined as clear cell SCCIS. The clinical features of the cases did not vary significantly from classical SCCIS. Antibodies labeling outer root sheath (ORS) cells also labeled clear cells in the cases and included K8.12 (labeling CK13 and CK16), cellular retinoic acid binding protein II, CAM 5.2 and CK15. Antibodies that did not label ORS cells, but did label eccrine glands (CK7 and CK18) or sebocytes (EMA), also did not label the cases. CONCLUSION: Clear cell change in SCCIS is part of a spectrum which displays ORS differentiation.     

Cutaneous field cancerization: clinical,histopathological and therapeutic aspects

The concept of "field cancerization" was first introduced by Slaughter in 1953 when studying the presence of histologically abnormal tissue surrounding oral squamous cell carcinoma. It was proposed to explain the development of multiple primary tumors and locally recurrent cancer. Organ systems in which field cancerization has been described since then are: head and neck (oral cavity, oropharynx, and larynx), lung, vulva, esophagus, cervix, breast, skin, colon, and bladder. Recent molecular studies support the carcinogenesis model in which the development of a field with genetically altered cells plays a central role. An important clinical implication is that fields often remain after the surgery for the primary tumor and may lead to new cancers, designated presently as "a second primary tumor" or "local recurrence," depending on the exact site and time interval. In conclusion, the development of an expanding pre-neoplastic field appears to be a critical step in epithelial carcinogenesis with important clinical consequences. Diagnosis and treatment of epithelial cancers should not only be focused on the tumor but also on the field from which it developed. The most important etiopathogenetic, clinical, histopathological and therapeutic aspects of field cancerization are reviewed in this article.     

Cutaneous pseudosarcomatous polyp: a histological and immunohistochemical study

Two unusual acquired polypoid skin lesions exhibited prominent histological atypia, but were biologically benign. Both patients were elderly females. The lesions clinically mimicked fibroepithelial polyp or nevus lipomatosus. Both had been present for about 20 years. One lesion was located on the back, the other on the posterior thigh. Each lesion exhibited dilated, hyalinized vessels in the dermis with focal fibrin deposits, myxoid stroma, and a population of bizarre, pleomorphic spindle to stellate cells, some of which were multinucleated. Occasional atypical mitoses were present. One lesion had abundant admixed fat. Immunohistochemical staining was strongly positive only for vimentin. The lesions share features with degenerating angiofibroma and vaginal pseudosarcomatous polyp. As in these lesions, the atypia is most probably reactive and degenerative.     

Cutaneous lupus erythematosus: a review of etiopathogenic,clinical, diagnostic and therapeutic aspects

Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.     

Cutaneous melioidosis with unusual histological features

Melioidosis is caused by the saprophytic gram‐negative bacillus Burkholderia pseudomallei, and has varied presentations, with cutaneous manifestations occurring in about 13% of cases. The usual histopathological features of melioidosis are suppurative to chronic granulomatous inflammation. Recommended treatment of melioidosis is sequential use of intravenous followed by oral antibiotics for a few months, although oral antibiotics alone can be used in primary cutaneous melioidosis. We report a case of cutaneous melioidosis in a healthy young man, with the unusual histopathological feature of lymphoplasmacytic inflammation, and failing an initial trial of oral antibiotics alone.     

Eosinophilic cellulitis--clinical and histological aspects

Eosinophilic cellulitis (Wells' syndrome) is a rare skin disease whose aetiology is still unknown. The onset is acute, and different disease stages may exist simultaneously during the course. Histological examination shows flame figures as a characteristic, but not pathognomonic, feature. In later stages the infiltrate adopts granulomatous characteristics. Malignant haematological disorders, which frequently occur with this disease, should be excluded.     

Chronic verrucous varicella zoster virus skin lesions: clinical, histological, molecular and therapeutic aspects

The outbreak of HIV infection introduced a new phenomenon in varicella zoster virus (VZV) pathology, namely the long-standing wart-like skin lesions that are frequently associated with resistance to thymidine kinase (TK)-dependent antiviral agents. This paper reviews the clinical, histological, and molecular aspects and the therapeutic management of these verrucous lesions. The majority of lesions are characterized by chronically evolving, unique or multiple wart-like cutaneous lesions. The main histopathological features include hyperkeratosis, verruciform acanthosis and VZV-induced cytopathic changes with scant or absent cytolysis of infected keratinocytes. The mechanism that establishes the chronic nature of the lesions appears to be associated with a particular pattern of VZV gene expression exhibiting reduced or nondetectable gE and gB synthesis. Drug resistance to TK-dependent antiviral agents is a result of nonfunctional or deficient viral TK. This necessitates alternative therapeutic management using antiviral agents that target the viral DNA polymerase.     

Cutaneous aspects of Down syndrome

Down syndrome is associated with an increased incidence of cutaneous manifestations such as atopic dermatitis, alopecia areata, elastosis perforans serpiginosa, syringomas, and skin infections. We describe 3 patients with Down syndrome: an 8-year-old boy with alopecia areata, a 17-year-old boy with crusted scabies, and a 39-year-old woman with a cutaneous collagen vascular disease-like disorder. We also review cutaneous signs of this syndrome.     

皮肤链格孢病2例临床分析并文献复习

目的:探讨皮肤链格孢病的临床特征、诊断、治疗及预后。方法:对我院近两年收治的2例皮肤链格孢病进行总结,并检索国内万方数据库和中国知网数据库和近10年国外Pubmed数据库报告的皮肤链格孢病,共60例,进行文献分析。结果:共2例患者,男、女各1例,均有肾病综合症口服免疫制剂病史,通过组织病理检查和真菌培养确诊,并通过分子生物学方法确诊为链互隔孢(Alternaria alternate)引起的皮肤链格孢病。1例给予手术切除联合伊曲康唑治疗后失访,1例给予口服伊曲康唑及他克莫司减量治疗,随访1年,未复发。结论:免疫功能低下是皮肤链格孢病的危险因素,组织病理检查和真菌培养为主要确诊手段,治疗主要为系统应用伊曲康唑或联合其他治疗。