José Pedro Reis

Trichilemmal carcinoma : review of 8 cases

Clinical and hislological data from 8 trichilemmal carcinomas are reviewed. The tumors occurred mainly on sun‐exposed skin of the face of elderly people as small solitary, frequently ulcerated nodules. Histologically, they consisted of multilobulate, infiltrative growths, connected to the epidermis and piloscbaceous structures and showing features reminiscent of the outer root sheath of the hair follicle. The presence of lobules of clear, glycogen‐rich cells with peripheral palisading, hyaline basement membranes, trichilemmal keratinization, and abortive follicular sheaths and the absence of ductal or acinar differentiation allow distinction from other clear cell tumors of the skin. Although the histological picture suggests a high‐grade malignant neoplasm, trichilemmal carcinoma has an indolent course. No recurrence or metastases have been observed.

Epidemiology of basal cell carcinomas and squamous cell carcinomas in a Department of Dermatology: a 5 year review

BACKGROUND Non-melanoma skin cancer, a common designation for both basal cell carcinomas and squamous cell carcinomas, is the most frequent malignant skin neoplasm. OBJECTIVE Epidemiologic characterization of the population with Non-melanoma skin cancer. METHODS Retrospective analysis of all patients diagnosed with Non-melanoma skin cancer based on histopathologic analysis of all incisional or excisional skin biopsies performed between 2004 and 2008 in a Department of Dermatology. RESULTS A total of 3075 Non-melanoma skin cancers were identified, representing 88% of all malignant skin neoplasms (n=3493) diagnosed in the same period. Of those, 68,3% were basal cell carcinomas. Most Non-melanoma skin cancer patients were female and over 60 years old. Of all Non-melanoma skin cancer, 81,7% (n=1443) were located in sun-exposed skin, and represented 95,1% of malignant skin neoplasms in sun-exposed skin. Non-melanoma skin cancer was the most frequent malignant skin neoplasm in most topographic locations, except for abdomen and pelvis - over 95% of all malignant skin neoplasms in the face, neck and scalp were Non-melanoma skin cancer. Basal cell carcinomas were clearly predominant in all locations, except in upper and lower limbs, lower lip and genitals, where squamous cell carcinomas represented respectively 77,7%, 77,4%, 94,7% and 95,3% of the Non-melanoma skin cancers. CONCLUSION Being the most common skin cancer, Non-melanoma skin cancer should be under constant surveillance, in order to monitor its epidemiologic dynamics, the efficiency of preventive measures and the adaptation of the healthcare resources.

Vancomycin‐associated linear IgA bullous dermatosis mimicking toxic epidermal necrolysis

4 Simonart T, Noel JC, Van Vooren JP, et al. Role of viral agents in the pathogenesis of Kaposi’s sarcoma. Dermatology 1998; 196: 447–449. 5 Guttman-Yassky E, Cohen A, Kra-Oz Z, et al. Familial clustering of classic Kaposi’s sarcoma. J Infect Dis 2004; 189: 2023–2026. 6 Monti M, Mancini LL, Ceriani R, et al. Iatrogenic Kaposi’s sarcoma and HCV infection. J Eur Acad Dermatol Venereol 2004; 18: 191–193. 7 Montella M, Serraino D, Crispo A, et al. Infection with human herpes virus type 8 in an area at high prevalence for hepatitis C virus infection in southern Italy. J Viral Hepat 2004; 11: 268–270.

Paraneoplastic pemphigus with clinical features of lichen planus associated with low-grade B cell lymphoma

Background  Neoplasia‐induced lichen planus is described as a cell‐mediated reaction to unknown epithelial antigens. Paraneoplastic pemphigus (PNP), characterized by the presence of a specific array of autoantibodies, probably represents a different form of presentation of the same autoimmune syndrome where the mucocutaneous expression depends on the dominant pathologic mechanism.

Benign follicular tumors

Benign follicular tumors comprise a large and heterogeneous group of neoplasms that share a common histogenesis and display morphological features resembling one or several portions of the normal hair follicle, or recapitulate part of its embryological development. Most cases present it as clinically nondescript single lesions and essentially of dermatological relevance. Occasionally, however, these lesions be multiple and represent a cutaneous marker of complex syndromes associated with an increased risk of visceral neoplasms. In this article, the authors present the microscopic structure of the normal hair follicle as a basis to understand the type and level of differentiation of the various follicular tumors. The main clinicopathological features and differential diagnosis of benign follicular tumors are then discussed, including dilated pore of Winer, pilar sheath acanthoma, trichoadenoma, trichilemmoma, infundibuloma, proliferating trichilemmal cyst/tumor, trichoblastoma and its variants, pilomatricoma, trichodiscoma/fibrofolliculoma, neurofollicular hamartoma and trichofolliculoma. In addition, the main syndromes presenting with multiple follicular tumors are also discussed, namely Cowden, Birt-Hogg-Dubé, Rombo and Bazex-Dupré-Christol syndromes, as well as multiple tumors of follicular infundibulum (infundibulomatosis) and multiple trichoepitheliomas. Although the diagnosis of follicular tumors relies on histological examination, we highlight the importance of their knowledge for the clinician, especially when in presence of patients with multiple lesions that may be the cutaneous marker of a cancer-prone syndrome. The dermatologist is therefore in a privileged position to recognize these lesions, which is extremely important to provide further propedeutic, appropriate referral and genetic counseling for these patients.

Lipomatous neurofibroma associated with segmental neurofibromatosis

To the Editor, Intralesional fat in a neurofibroma has rarely been observed.1– 5 Focal fatty changes in neurofibroma have been ascribed to senescent, metaplasia and chronic injury such as shaving, combing or exposure to ultraviolet radiation.1– 3 Lipomatous neurofibroma is more frequently located on the head and neck of older patients and in long-standing lesions.1– 3 A 71-year-old man presented to our dermatology department with asymptomatic skin lesions distributed on the right forearm with 50 years of duration. He was in good health and no other members of his family exhibited a similar condition. Physical examination showed multiple, soft, dome-shaped, skin-coloured papules and nodules, ranging from 5 to 17 mm in diameter, which were distributed in a linear pattern over the ulnar aspect of the right forearm (Fig. 1). A diagnosis of segmental neurofibromatosis (NF) was made and a skin biopsy disclosed a well-circumscribed, nonencapsulated dermal lesion with normal overlying epidermis composed of loosely spaced spindle cells and wavy collagenous strands in a clear matrix. Focal areas of the lesion were replaced by adipose cells, which appeared with clear round cytoplasmic vacuoles and compressed thin nuclei at the periphery (Fig. 2). The present case of segmental NF showed an unusual histopathological feature in the form of lipomatous neurofibroma, characterized by the presence of well-differentiated adipose tissue embedded in the tumor. Although cutaneous neurofibroma is a common tumor, some pathological variants are rare.1– 4,6 These variants include cellular, myxoid, collagenous, hyalinized, epithelioid, pigmented, granular cell, pacinian, atypical, xanthomatized and lipomatous neurofibroma.2– 4,6 Lipomatous neurofibroma is the latest described variant.1– 3 A recent study showed that the intraneoplastic fat was divided into two groups: focal and diffuse (regularly interspersed).3 Focal fatty change can be a consequence of metaplasia of tumor cells or resident fibroblasts,1– 4 or fatty infiltration from adjacent tissue.2,4 Senescent change or chronic injury in older patients and in long-standing lesions can account for fat deposition (tissue metaplasia).1– 3 However, the diffuse lipomatous neurofibroma represents a distinctive tumor of the peripheral nerve sheath, owing to the differentiation of pluripotential neural crest cells after emigration.2– 5 In our case, the focal presence of

Nucleolar Organizer Regions Argyrophilic Associated Proteins in Cutaneous Melanocytic Lesions

We applied a simple silver staining technique to visualize nucleolar organizer regions associated proteins (AgNORs) for the study of 47 melanocytic lesions (20 malignant melanomas, 5 dysplastic nevi, 4 Spitz nevi, 2 Reed and Gartmans fusiform nevi and 16 melanocytic nevi). A statistically significant difference existed between the numbers of AgNORs per cell in benign and malignant lesions as a group. However, some overlapping counts were found, limiting the usefulness of the technique in differentiating benign from malignant lesions in individual cases.

Multiple adult-onset xanthogranuloma, an uncommon diagnosis

©2017 by Anais Brasileiros de Dermatologia Dear Editor, A 77-year-old Caucasian male patient was referred to our Service for presenting brownish and erythematous-violet papules (4 to 8 mm), located bilaterally in arms, armpits, lower abdominal quadrants and thighs (Figure 1). There was no involvement of the face, dorsal surface of the joints or mucous membranes. The lesions, which were always asymptomatic, had evolved progressively for ten months. At the general objective examination, there were no significant alterations, namely ophthalmologic, cardiopulmonary and neurological. No systemic semiology or relevant family history coexisted. Among the patient’s personal antecedents were major depression and anxiety disorder. Diagnoses of histiocytosis, particularly generalized eruptive histiocytoma, multiple adult xanthogranuloma (XGA), Erdheim-Chester disease and cutaneous metastases, were considered. The histopathological study of a lesion showed infiltration of the dermis and, in particular, of the hypodermis by histiocytes with foamy cytoplasm, accompanied by Touton cells, occasional lymphocytes and rare eosinophils (Figure 2). Infiltrate cells were CD68 positive and protein S100 and CD1a negative (Figure 3). No alterations were observed in the complementary evaluation, which included: hemogram with leukogram; blood biochemistry; electrophoretic proteinogram and serum immunofixation; urinalysis, diuresis monitoring and pituitary hormone study; skeletal radiography; electrocardiogram; respiratory function tests; cranioenchephalic and thoraco-abdominopelvic tomodensitometry studies.

Secondary Syphilis with Persisting Hard Chancre on the Forearm

© 2013 The Authors. doi: 10.2340/00015555-1355 Journal Compilation

Psoríase, Psicofármacos e Comorbilidades Psiquiátricas: Um Estudo de Caso-Controlo numa População de Doentes Hospitalizados

Introduction: Psoriasis is a chronic inflammatory dermatosis with significant impact on quality of life and multiple comorbidities, with recognition especially given to cardiovascular risk factors. Recently it has been highlighted the high prevalence of psychiatric comorbidities.Methods: The primary outcome was to assess the prevalence of psychotropic drugs in the chronic medication of patients with diagnosis of psoriasis. We performed a case-control study at the Department of Dermatology of Coimbra Hospital and University Centre, between 2010 and 2015. The results were compared with age- and gender-matched controls. The medication was grouped according to the INFARMED. The secondary outcome was to compare the prevalence of treatments for cardiovascular risk factors in both groups and to compile the information on psychiatric comorbidities available in the medical records of the psoriasis patients included in this study. The data were processed by STATA 14 and statistical significance was considered when p<0.05. Results: The sample consisted of 79 patients, 67% male and 33% female, mean age of 59.30±17.17 years. There was association (p<0.01) between psoriasis and psychotropic drugs, especially anxiolytics and antipsychotics, with 63% of patients with at least one psychotropic drug. It was found an association between psoriasis and antihypertensive drugs (p<0.05). Frequently, psychiatric diagnoses were not mentioned or were inaccurately described in the medical records.Conclusion: The results highlight the high prevalence of psychotropic drugs and reinforce previous studies about the lack of assessment of psychopathology in dermatology, a topic of high relevance in patients with psychodermatologic diseases, such as psoriasis.