A Rare Case of Lesion in the Vulva: Pigmented Apocrine Hamartoma.

ABSTRACT: Apocrine hamartoma is a rare benign neoplasm. The histology is characterized by an excess of apocrine glands located predominantly in the reticular dermis. Pigmented apocrine hamartoma represents a histopathological variation of apocrine hamartoma containing tubules and linear cysts covered by apocrine cells on the inside with melanin and on the outside with myoepithelial cells. At this time, 4 cases of this pathology have been described. This case report aims to present a case of pigmented apocrine hamartoma of the vulva in a young patient, emphasizing that while occurrence is rare, it must be considered when diagnosing a pigmented lesion of the vulva in young patients.

Lichenoid folliculitis of the scalp in four patients with ichthyosiform skin disorders and cicatricial alopecia.

Ichthyosis is a heterogeneous group of inherited skin disorders characterized by a defect of keratinization. Patients diagnosed with lamellar ichthyosis (LI) and some ichthyosiform syndromes, such as the Conradi-Hünermann-Happle syndrome (CHHS), usually present with hair loss. Even though only few dermatologic complaints carry as many emotional overtones as hair loss, there are very few data available in the literature regarding scalp histopathological features in ichthyosis. A better understanding of scalp changes in such context may result in new therapeutic strategies that in turn would enhance patients' self-esteem and quality of life. The aim of this paper is to describe the scalp histopathological findings of four young patients with cicatricial alopecia: three diagnosed as having LI and the fourth with CHHS.

Presence of Merkel cell polyomavirus DNA and large-T antigen in keratinocyte carcinomas and its correlation with immunohistochemical markers p16, p53 and ki67.

BACKGROUND: Merkel cell polyomavirus (MCPyV), a human polyomavirus that is unequivocally linked to merkel cell carcinoma (MCC), has been found in association with keratinocytes carcinomas (KC), especially basal cell carcinoma (BCC) and cutaneous squamous cell carcinoma (cSCC). Nevertheless, there is scarce information about the possible involvement of MCPyV in the development of KC. OBJECTIVES: To assess the presence of MCPyV DNA and Large-T Antigen (LT-Ag) via Polymerase Chain Reaction (PCR) and Immunohistochemistry (IHC) in cases of KC, and to correlate its presence with immunohistochemical markers p16, p53, and ki67, tumor type and subtype, sun-exposed location, and epidemiological data. METHODS: The prevalence of MCPyV DNA, LT-Ag, and immunohistochemical markers p16, p53, and ki67 was assessed by PCR and Immunohistochemistry (IHC) in 127 cases of KC, these results were correlated with tumor type and subtype, sun-exposed location, and epidemiological data. RESULTS: The MCPyV DNA was detected in 42.57% (43 of 101) cases by PCR, the LT-Ag was detected in 16.4% (20 of 122) of cases, p16 in 81.5% (97 of 119), p53 in 66.4% (83 of 125), ki67 in 89% (73 of 82). No correlation between MCPyV LT-Ag and DNA confronted with tumor type, subtype, location site, and immunohistochemical markers was found. A single correlation between the MCPyV LT-Ag and cSCC tumors and peri-tumoral lymphocyte cells was noted. STUDY LIMITATIONS: Further steps need to be taken to better evaluate the MCPyV influence and its possible role in KC carcinogenesis, as the evaluation of the virus genome state, the gene sequence that encodes LT-Ag in the KC tumor cells, and in situ hybridization for viral DNA or RNA in these cells. CONCLUSIONS: Despite the frequent detection of MCPyV in KC, the data available so far does not support the hypothesis of a causal relationship between them.

Transepidermal retinoic acid delivery using ablative fractional radiofrequency associated with acoustic pressure ultrasound for stretch marks treatment.

BACKGROUND: Striae distensae (SD) treatment still remains a therapeutic challenge to dermatologists. Ablative fractional laser and radiofrequency (RF) enhance skin-drug permeability for SD treatment. OBJECTIVE: To clinically evaluate the efficacy and safety as well as patient's satisfaction in relation to a method using ablative fractional RF associated with retinoic acid 0.05% cream and an acoustic pressure wave ultrasound (US) in patients with alba-type SD on the breast. METHODS AND MATERIALS: Eight patients with alba-type SD on the breast were treated with three step procedure: (1) fractional ablative RF for skin perforation; (2) topical application of retinoic acid 0.05% on the perforated skin; and (3) US was applied to enhance the retinoic acid penetration into the skin. Other eight patients with alba-type SD on the abdominal area were submitted to RF treatment isolated without retinoic acid or US. Three of them were submitted to skin biopsies. RESULTS: Three patients with SD on the breast area improved from "severe" to "moderate;" two patients improved from "severe" to "mild;" two patients from "moderate" to "mild;" one patient from "marked" to "mild." Clinical assessment demonstrated significant improvement in the appearance of SD in all patients treated with RF associated with retinoic acid 0.05% cream and US (P = 0.008), with low incidence of side effects and high level of patient's satisfaction. Among the patients treated only with RF, two patients improved from "severe" to "marked;" one patient from "marked" to "moderate;" and one patient improved from "marked" to "mild." Four patients did not show any sort of improvement. Clinical assessment demonstrated no significant improvement in the appearance of SD treated with RF isolated with low incidence of side effects, but low-level of patient's satisfaction. CONCLUSION: Ablative fractional RF and acoustic pressure US associated with retinoic acid 0.05% cream is safe and effective for alba-type SD treatment.

Merkel cell polyomavirus and its etiological relationship with skin tumors.

Viruses have been frequently identified in several human neoplasms, but the etiological role of these viruses in some tumors is still a matter of controversy. Polyomaviruses stand out among the main viruses with oncogenic capacity, specifically the Merkel cell polyomavirus (MCPyV). Recent revisions in the taxonomy of polyomaviruses have divided the Polyomaviridae family into six genera, including 117 species, with a total of 14 currently known human-infecting species. Although the oncogenicity of polyomaviruses has been widely reported in the literature since 1950, the first description of a polyomavirus as an etiological agent of a neoplasm in humans was made only in 2008 with the description of MCPyV, present in approximately 80% of cases of Merkel cell carcinoma (MCC), with the integration of its genome to that of the tumor cells and tumor-specific mutations, and it is considered the etiological agent of this neoplasm since then. MCPyV has also been detected in keratinocyte carcinomas, such as basal cell carcinoma and squamous cell carcinoma of the skin in individuals with and without immunosuppression. Data on the occurrence of oncogenic viruses potentially involved in oncogenesis, which cause persistence and tissue injury, related to the Merkel cell polyomavirus are still scarce, and the hypothesis that the Merkel cell polyomavirus may play a relevant role in the genesis of other cutaneous carcinomas in addition to MCC remains debatable. Therefore, the present study proposes to explore the current knowledge about the presence of MCPyV in keratinocyte carcinomas.

Clinical, dermoscopic, and histopathologic findings of hidroacanthoma simplex: a case report and literature review.

Hidroacanthoma simplex (HS) is a rare benign intraepidermal tumor that originates in the acrosyringial portion of the eccrine duct. Clinically, they are well-defined lesions, flat or verrucous brownish plaque, and can be misdiagnosed with other types of benign or malignant tumors. Dermoscopy reveals small black globules and fine scales. The histopathology of HS is characterized by typical intraepidermal nests, well-circumscribed, composed of uniform basaloid and poroid cells within the acanthotic epidermis with cystic or ductal structures in the nests. We report a case of HS that changes its clinical appearance, dermoscopy, and histopathologic findings over time. Differential diagnoses included seborrheic keratosis, Bowen disease, melanoma, and malignant HS.

[Mycosis fungoides in children and adolescents: a report of six cases with predominantly hypopigmentation, along with a literature review]. / Micose fungóide em crianças e adolescentes: Relato de seis casos com predomínio da forma hipocromiante e revisão da literatura.

UNLABELLED: Mycosis fungoides (MF), the most common form of cutaneous T cell lymphoma (CTCL), is mainly manifested in the elderly. However, it has been described in children and teenagers. OBJECTIVES: To report six patients with mycosis fungoides diagnosed in patients under 20 years of age. Our focus is on epidemiologic data, clinical features, histopathological aspects, and immunophenotypical findings. METHODS: The files of all patients diagnosed with CTCL at Hospital Universitário Antônio Pedro (HUAP) / Universidade Federal Fluminense (UFF), Niterói, Brazil, from 2007 to 2010 were searched to identify patients under 20 years of age. Slides were reviewed with routine methods (H&E) and immunohistochemical stains by two dermatopathologists and one surgical pathologist. RESULTS: Among a total of 66 patients with MF, six were children and adolescents between five and nineteen years of age. Most of them had dark skin and presented with the hypopigmented variant of MF; some expressed the T cell CD8+ phenotype, although the prognosis remains the same as for classical CTCL. The main histopathological findings were basilar lymphocytes, Pautrier microabscesses, eccrine infiltration, and dermal fibrosis. One patient had shown pityriasis lichenoides chronica for 10 years before the diagnosis of MF. CONCLUSIONS: The incidence of juvenile mycosis fungoides has increased, corresponding to 9.1 percent of patients diagnosed with MF in our institution in four years. In this sample, 83.3 percent of the patients had the hypopigmented variant and 50 percent of them showed the CD3+/CD8+ T cells phenotype. We emphasize the occurrence of pityriasis lichenoides chronica before the onset of MF in one of our cases. This association, although rare, must be considered in cases of atypical evolution of PLC. The diagnosis of hypopigmented MF should also be considered in patients when hypochromic patches are persistent. We would like to highlight the importance of Pautrier microabscesses, basilar distribution, and eccrine involvement by neoplastic lymphocytes as histopathological diagnostic criteria for this variant of MF.

Frequency of lichen sclerosus in children presenting with phimosis: A systematic histological study.

BACKGROUND: The incidence of preputial lichen sclerosus (PLS) among children presenting with phimosis varies from 10 to 95%, depending on the age, the protocol for the treatment of pediatric phimosis, the method of diagnosis (clinical versus histological), and case mix (congenital versus acquired phimosis). OBJECTIVE: PLS may not be clinically obvious. Our aim is to show that a systematic histological examination of the prepuce may diagnose PLS in clinically unsuspected cases. METHODS: Prospective observational study of the histology of all prepuces resected from boys undergoing circumcision for phimosis but not clinically suspected to have PLS. RESULTS: PLS was diagnosed histologically in 22 boys (32%). Boys with PLS were significantly older (mean 8.4 versus 4.7 years old). Diagnosis of PLS was not related to the degree of phimosis (summary figure). In three patients (grade 4 phimosis) glans discoloration was observed during surgery, and all had PLS. Follow up for boys found to have PLS ranged from 1 to 10 years. One patient developed recurrent phimosis, attributed to inappropriate conservative resection, and required further surgery. There were no cases of meatal stenosis. DISCUSSION: Subtle cases of PLS may be difficult to detect clinically. Children are frequently asymptomatic, except for being unable to retract the prepuce. Physical examination has a low negative predictive value for the diagnosis of PLS. Complete removal of the prepuce with permanent glans exposure is regarded as essential to cure PLS and to avoid recurrent phimosis, but our patients were treated with partial circumcisions for cultural reasons. Only one needed reoperation for recurrent phimosis. CONCLUSION: Histological PLS was present in approximately 1/3 of boys with phimosis, frequently without typical manifestations. Those patients may be cured with partial circumcisions.

[Oleoma: A case report with good response to tetracycline]. / Oleoma: Relato de caso com boa resposta à tetraciclina.

Oleoma or paraffinoma is defined as a foreign body granuloma that results from the injection of oily substances into the skin or subcutaneous tissue. We present a young patient with ulcerated lesions secondary to infiltration of oily material. He had undergone the injections with the aim of increasing muscle mass and definition. The treatment of these cases is quite complex, often with unsatisfactory results. The use of long-term oral tetracycline proved to be helpful.

A man with erythematous nodules: what is the diagnosis? Lepromatous leprosy.

A 65-year-old man presented with an approximately 5-year history of cutaneous nodules of the arms and legs. In addition, physical examination revealed bilateral thickening of ulnar and tibial nerves, distal weakness with hallux extension and finger abduction, and distal hypoesthesia of the left hallux. Histopathological analysis of the skin biopsy specimen showed a dense inflammatory infiltrate in the hypodermis, characterized by vacuolated macrophages containing multiple organisms. The Fite stain was positive confirming the diagnosis of multibacillary leprosy.

Pseudoxanthoma elasticum.

A 55-year-old man with progressive loss of vision was referred for dermatology consultation for the evaluation of his skin lesions. The cutaneous examination of the patient revealed multiple small yellow papules, coalescing into plaques, on his neck, axillae and periumbilical regions. He also had redundant skin folds on the axillae. His peripheral pulses and blood pressure were normal. Angioid streaks were found in the ocular fundi. A skin biopsy specimen of the papule showed fragmentation of elastic fibers as well as calcification in the dermis. Given the clinical manifestations and the histopathologic findings, the patient's illness was diagnosed as pseudoxanthoma elasticum. The patient was then sent to undergo a thorough cardiovascular evaluation.

White fibrous papulosis of the neck: the first South American case.

A 69-year-old woman presented with white papules on her neck. These papules were multiple, white, discrete, asymptomatic, non-follicular, 2 to 3 mm in diameter, and located on both sides of the neck. On histopathological examination, there was a thickened and abnormal organization of collagen bundles in the papillary to mid-dermis and a decrease in the number of elastic fibers. This clinical and histological picture suggested the diagnosis of white fibrous papulosis of the neck. No treatment was given because of the benign nature of the disease. This is the first case report of this condition in South America.

Syringoid Eccrine Carcinoma in the Abdominal Wall: A Rare Case Report and Literature Review.

BACKGROUND Syringoid eccrine carcinoma (SEC) is an extremely rare malignant adnexal neoplasm derived from eccrine sweat glands, of unknown pathogenesis. We report a case of this rare entity presenting in the abdomen, which is the only one reported in this area and the only case of SEC in a patient with so many comorbidities. CASE REPORT A 58-year-old black male from Brazil reported a nodular lesion in the abdomen with a progressive increase in size and pain and local burning sensation. The histopathological examination showed a syringoid eccrine carcinoma. CONCLUSIONS We present a rare case of SEC and did an extensive literature review in order to describe the clinical characteristics, histopathological findings, immunohistochemical profile, treatments, and difficulties found in the diagnosis of this tumor. To avoid misdiagnosis, we gave special attention to biopsy quality.

Fibrosing Alopecia in a Pattern Distribution (FAPD) in 16 African-Descent and Hispanic Female Patients: A Challenging Diagnosis.

BACKGROUND: Fibrosing alopecia in a pattern distribution (FAPD) has only been described in Caucasian patients, and it is not clear whether it can develop in dark-skin ethnicities. MATERIALS AND METHODS: Sixteen Brazilian female patients, 12 of African descent and 4 Hispanic, with progressive scarring alopecia in a pattern distribution were analyzed. RESULTS: Dermatoscopic features showed perifollicular erythema and scaling (14/16), hair fiber diameter diversity (16/16), loss of follicular ostia (16/16), and follicular keratosis (3/16). Late stages showed a honeycomb pigmented network (12/16), a hyperpigmented perifollicular halo (12/16), and small white patches (12/16). Histopathological features showed lichenoid perifollicular infiltrate (14/16), follicular miniaturization (16/16), concentric fibrosis (16/16), perifollicular lymphocytic infiltrate (16/16), and vellus hair involvement (10/16). Premature desquamation of the inner root sheath was found in 11 patients. CONCLUSIONS: The concomitant findings of cicatricial pattern hair loss (with or without the recess of the front hair line), hair fiber diversity, perifollicular erythema and scaling, a whitish perifollicular halo, and histological findings of androgenetic alopecia, with vacuolar interface alteration of the upper portion of the follicular epithelium, are the main key features to suggest the diagnosis of FAPD. FAPD is a possible diagnosis in patients of color with cicatricial pattern hair loss. Clinical, dermatoscopic, and histopathological examination allow a proper final differential diagnosis.

Acquired fibrokeratoma presenting as a giant pedunculated lesion on the heel.

Acquired digital fibrokeratomas (ADF) are benign and uncommon lesions consisting of collagenous papules and nodules covered by hyperkeratotic epidermis. These tumors occur mainly on the fingers and toes and infrequently on the palms and soles. They may possibly be triggered by a reaction to a trauma, ADF usually present as small and solitary dome-shaped lesions with a collarete of slightly raised skin at the base. We report a rare case of fibrokeratoma of the heel, presenting as a large and pedunculated nodule.

Sycosiform tinea barbae caused by Trichophyton rubrum.

Tinea barbae is an uncommon superficial dermatophyte infection of the beard and moustache areas. It was more frequently observed in the past, before single-use razors became available. In most cases, the zoophilic ectothrix Trichophyton mentagrophytes and Trichophyton verrucosum are responsible for this type of infection. Its clinical presentation is variable; it can mimic many other skin disorders such as sycosis, iododerma, contact dermatitis, perioral dermatitis, and actinomycosis. We report a case of tinea barbae caused by an uncommon agent Trichophyton rubrum, misdiagnosed as sycosis, and review the approach and management of the disease.