Flávia Regina Ferreira

Porokeratosis of Mibelli - literature review and a case report

Porokeratosis is a disorder of epidermal keratinization characterized by annular plaques with an atrophic center and hyperkeratotic edges, and includes a heterogeneous group of disorders that are mostly inherited in an autosomal dominant form. This report describes a 5 year-old female patient, with porokeratosis of Mibelli confirmed histopathologically. The rarity of this disorder, its clinical exuberance and the destructive character of the lesions, as well as the facial and mucosal involvements, unusual in this form of porokeratosis, and also its onset in early childhood motivated this report.

Prevalência de dermatoses pediátricas em um hospital universitário na região sudeste do Brasil

BACKGROUND Numerous dermatoses affects children, depending on age, region and socioeconomic status. OBJECTIVE To determine the prevalence of pediatric dermatoses at the Dermatology Department of a University Hospital, involving the diagnosis, age and sex. METHODS Epidemiologic cross-sectional study carried out in the period between July 2006 and December 2007. There were reviewed the handbooks of 264 patients from the Pediatric Dermatology Department of a University Hospital in this period. The dependent variable was the existence or not of dermatoses in children of age up to 19 years. Independent variables were: clinical diagnosis, sex and age. RESULTS Of the 264 handbooks reviewed, there was a higher prevalence of allergic dermatoses in 74 cases (28,0%), followed by inflammatory dermatoses in 49 cases (18,6%), pigmentary dermatoses in 42 cases (15,9%), infectious dermatoses with 38 cases (14,4%), benign tumors in 25 cases (9,5%), miscellaneous in 14 cases (5,3%), genodermatosis with 12 cases (4,5%) and skin annexes disorders with 10 cases (3,8%). The infants represented 11,3 % of the total, the pre-school 15,9 %, the schoolboys 48,8 %, and the adolescents 23,8 %. There was greater prevalence of allergic dermatoses in pre-school with 15 cases (35,7%), infants with 10 cases (33,3%) and schoolboys with 39 cases (30,2 %). Among adolescents are highlighted inflammatory dermatoses. The study showed no statistical differences between sex and age. CONCLUSIONS The study of the epidemiological profile makes easy the diagnosis of pediatric dermatoses encouraging good history and search for prevention.

Epidemiological profile of nonmelanoma skin cancer in renal transplant recipients: experience of a referral center

BACKGROUND Nonmelanoma skin cancer is the most common form of cancer in humans and also the malignant disease that is increasingly common among kidney transplant recipients. OBJECTIVE To determine the epidemiological characteristics of renal transplant recipients with nonmelanoma skin cancer seen at a referral transplantation center. METHODS Cross-sectional descriptive study with renal transplant recipients presenting nonmelanoma skin cancer, treated at a transplantation referral center between 08/01/2004 and 08/31/2009. Analyzed variables were: gender, age, skin phototype, occupational and recreational sun exposure, use of photoprotection, personal and family history of non-melanoma skin cancer, clinical type and location, time between transplantation and the appearance of the first nonmelanoma skin cancer, occurrence of viral warts, timing of transplantation, type of donor, cause of kidney failure, previous transplants, comorbidities, pre-transplant dialysis, type and duration of dialysis. RESULTS 64 subjects were included. Males - 71.9%; low skin phototypes (up to Fitzpatrick III) - 89%; mean age - 57.0 years - and mean age at transplant - 47.3 years; sun exposure - 67.2% occupational - and 64.1% recreational; photoprotection - 78.2% (although only 34.4% in a regular manner); squamous cell carcinoma - 67.2%; squamous cell carcinoma/basal cell carcinoma ratio - 2:1; personal history of nonmelanoma skin cancer - 25% - and family history - 10.9%; location at photoexposed area - 98.4%; average latency time between transplantation and first nonmelanoma skin cancer appearance - 78.3 months; viral warts (HPV) after transplant - 53.1%; average timing of transplantation - 115.5 months; living donor - 64.1%; triple regimen (antirejection) - 73.2%; comorbidities - 92.2%; pre-transplant dialysis - 98.4%; hemodialysis - 71.7%; average duration of dialysis - 39.1 months; previous transplants - 3.1%; hypertension as cause of renal failure - 46.9%. CONCLUSION This study allowed the epidemiological characterization of a population of kidney transplant recipients with nonmelanoma skin cancer.

Association of giant congenital melanocytic nevus, halo nevus and vitiligo in a 75-year-old patient

A giant congenital melanocytic nevus represents a rare condition. The halo phenomenon may be seen in congenital or acquired melanocytic nevi. In the literature, association of halo nevus and giant congenital melanocytic nevus is rare and the association of both with vitiligo even more rare. A 75-yearold woman at first consultation complained of a hyperchromic bluish-brown hairy macula on the lower back, buttocks and thighs present since birth and an achromic halo of onset three years ago. The histological features were consistent with congenital melanocytic nevus and halo nevus, respectively. After two years the patient developed achromic areas in normal skin, histologically consistent with vitiligo. The authors emphasize the rarity of this triple combination, the patients age and the absence of malignant degeneration to date.

Involucrin in the differential diagnosis between linear psoriasis and inflammatory linear verrucous epidermal nevus: a report of one case

Inflammatory linear verrucous epidermal nevus is a variant of verrucous epidermal nevus, characterized by recurrent inflammatory phenomena. Despite well-established clinical manifestations, the differential diagnosis between inflammatory linear verrucous epidermal nevus and linear psoriasis remains difficult. Clinical history, physical examination and histopathology analysis may not be sufficient to confirm the diagnosis. We report the case of a 4-year-old girl in which the involucrin immunostaining was helpful in the diagnosis of inflammatory linear verrucous epidermal nevus. Our findings confirm that involucrin immunohistochemistry is a useful tool in such cases.

Differences in age and topographic distribution of the different histological subtypes of basal cell carcinoma, Taubaté (SP), Brazil

BACKGROUND Basal cell carcinoma is the most common form of cancer in humans. OBJECTIVES To identify the epidemiology of basal cell carcinoma in Taubaté-SP and verify a possible association between topography and the different histological subtypes of this tumor. METHODS This was a cross-sectional study conducted at The University Hospital of Taubaté between 01/01/08 and 12/31/09. The study included patients with a confirmed diagnosis of basal cell carcinoma, of both genders, without age restrictions. The variables studied were incidence of basal cell carcinoma, topography, histological subtype, skin color, age and gender. We employed the chi-square test to identify the association between histological subtype and topography, and the students t test to compare the mean age of onset for the different histological subtypes. RESULTS The study included 239 individuals. The mean age of the sample was 68.0 years. Male subjects (57.7%) and whites (87.1%) predominated in the study. The predominant histological subtype was nodular (34.7%), followed by the superficial subtype. The most frequent sites of involvement were the head and neck (areas exposed to light), with predominance of the nasal region. The superficial subtype was an exception, as it showed a strong association with unexposed areas like the trunk. The mean age of onset of superficial basal cell carcinoma also differed from that of the other histological subtypes, 63.0 and 69.0 years, respectively. CONCLUSION The results of this study suggest an association of the superficial histological subtype with younger patients and unexposed areas of the body, linking this type of tumor with a pattern of intermittent sun exposure, more similar to the standard photocarcinogenesis of melanoma.

Nail trichrome vitiligo: case report and literature review.

MADAM, A 10-year-old boy, phototype V, presented with a 6-month history of a dark macula on his second right toenail. The patient had been under treatment for vitiligo for the previous 3 years with topical and systemic corticosteroids and phototherapy (ultraviolet B narrowband radiation). The lesions on the skin fluctuated with periods of improvement and deterioration. During the last 4 months only topical steroids had been used. One month ago the pigmentation changed and light brown patches appeared, intermixed with areas of a deeper brown colour and associated with a deterioration in the skin lesions. On examination there was a light brown macula surrounding a darker brown macula on the second right toenail. The proximal nail fold presented achromic areas alternating with a dark-brown pigmentation. Dermoscopic examination (DermLite FOTO; 3Gen, San Juan Capistrano, CA, U.S.A.), showed an achromatic macula surrounded by normal skin on the medial part of the proximal nail fold (Fig. 1). The lateral nail folds and periungual tissues did not show any abnormal pigmentation. The distal portion of the medial side of the nail plate showed a pigmentation that was subdivided into two different bands: a light-brown band and a more usual darker-brown band. The remaining part of the nail plate had no abnormal pigmentation. Onycholysis was observed in the distal medial part of the nail plate. Histopathological examination by haematoxylin and eosin and Periodic Acid Schiff stains did not show blood or fungi. In the Ziehl–Neelsen stain melamine was highlighted. After a thorough clinical history and dermatological, dermoscopic and histopathological examination, our diagnosis was trichrome vitiligo. Three months later, a regression of the light-brown and darker-brown band beneath the nail plate was observed, and an achromic area appeared in its place (Fig. 2). Vitiligo is an acquired, idiopathic disorder characterized by circumscribed depigmented maculae and patches. Functional melanocytes disappear from the involved skin by a mechanism that has not been identified. It is a multifactorial disorder with both genetic and nongenetic factors, and many hypotheses are quoted in the literature. The most common form of vitiligo is a totally amelanotic macula surrounded by normal skin. Vitiligo maculae characteristically have fairly discrete margins, round, oval or linear in shape. The borders are usually convex, as if the depigmenting process was ‘invading’ the normally pigmented skin. Lesions enlarge centrifugally over time, but the rate may be slow or rapid. In acrofacial vitiligo, periungual involvement of one or more digits may be associated with lip depigmentation; however, the latter can be an isolated finding. The clinical classification of vitiligo can be presented as localized (focal, segmental, mucosal), generalized (vulgaris, acrofacial, mixed) or universal. Clinical variants are known in vitiligo, such as vitiligo ponctué, trichrome vitiligo, quadrichrome vitiligo, pentachrome vitiligo and the isomorphic Koebner phenomenon. The term trichrome vitiligo was first suggested in 1964 by Fitzpatrick. The lesions have an intermediate zone of hypochromia located between the achromic centre and the peripheral unaffected skin. This results in

Genitogluteal porokeratosis - Case report

We report the case of a patient diagnosed with genitogluteal porokeratosis, a disorder of epidermal keratinization. The location described is extremely rare and very often late diagnosed or even misdiagnosed. Histopathology showed a typical cornoid lamella of great value to support this diagnosis. The importance of awareness of this entity by the specialist is emphasized as a differential diagnosis among genital diseases of chronic evolution and difficult treatment.

Most frequent dermatoses at a vulvar pathology outpatient clinic

The vulva corresponds to the external female genitalia. Special features of this region favor a wide range of diseases, whose knowledge allows for better clinical management, impacting on the quality of life. This is a cross-sectional and descriptive study carried out at a vulvar pathology outpatient clinic, between May and December/ 2015. Data obtained from a standard form included demographic parameters, habits, and vulvar dermatosis and allowed to identify the epidemiological profile of patients with vulvar dermatosis treated in this outpatient clinic and to determine the most prevalent dermatoses. Our results, partially concordant with the literature, provide original data that should stimulate further studiesThe vulva corresponds to the external female genitalia. Special features of this region favor a wide range of diseases, whose knowledge allows for better clinical management, impacting on the quality of life. This is a cross-sectional and descriptive study carried out at a vulvar pathology outpatient clinic, between May and December/ 2015. Data obtained from a standard form included demographic parameters, habits, and vulvar dermatosis and allowed to identify the epidemiological profile of patients with vulvar dermatosis treated in this outpatient clinic and to determine the most prevalent dermatoses. Our results, partially concordant with the literature, provide original data that should stimulate further studies.

Neutrophilic dermatosis of the dorsal hands: response to dapsone monotherapy

Neutrophilic dermatosis of the dorsal hands is considered a rare and localized variant of Sweets syndrome. Although the etiology is unknown, there are reports of association with infections, neoplasias, autoimmune diseases and medications. Histopathology shows a dense neutrophilic inflammatory infiltrate in the dermis. Treatment is based on the administration of systemic corticosteroids; however, a combination of medications is useful, given the frequency of relapses. The authors report a classic and clinically exuberant case of neutrophilic dermatosis of the dorsal hands, with excellent response to oral dapsone treatment, and offer a brief literature review.Neutrophilic dermatosis of the dorsal hands is considered a rare and localized variant of Sweets syndrome. Although the etiology is unknown, there are reports of association with infections, neoplasias, autoimmune diseases and medications. Histopathology shows a dense neutrophilic inflammatory infiltrate in the dermis. Treatment is based on the administration of systemic corticosteroids; however, a combination of medications is useful, given the frequency of relapses. The authors report a classic and clinically exuberant case of neutrophilic dermatosis of the dorsal hands, with excellent response to oral dapsone treatment, and offer a brief literature review.