Browsing by Author "Lencastre, A"
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- Argyria. Case ReportPublication . Lencastre, A; João, A; Lobo, MA 70-year-old male rural worker was referred to our clinic with widespread grey pigmentation of the skin and nails. The condition had been asymptomatic for its entire duration (5 years). He reported past intranasal application of 10% Silver Vitellinate. A skin biopsy was performed and histology corroborated the clinical diagnosis of Argyria. This case represents a currently rare dermatological curiosity. Although silver colloids and salts have been withdrawn and/or banned by some drug surveillance agencies, they continue to be freely sold and unregulated as food supplements and as ingredients in alternative medicines, thereby risking the emergence of new cases of silver poisoning.
- Birt-Hogg-Dubé SyndromePublication . Lencastre, A; Ponte, P; Apetato, M; Nunes, L; Lestre, SA 45-year-old woman with a history of renal carcinoma was observed for facial, cervical and truncal flesh-colored papules. Relatives had similar skin findings and a brother had repeated episodes of pneumothorax. The computerized tomography scan revealed multiple cysts on both lungs. A skin biopsy revealed a perifollicular fibroma. The clinical diagnosis of Birt-Hogg-Dubé syndrome (BHDS) was corroborated by identification of a novel frameshift c.573delGAinsT (p.G191fsX31) mutation in heterozygosity on exon 6 of the folliculin gene. The presence of multiple and typical benign hair follicle tumors highlights the role of the dermatologist in the diagnosis of this rare genodermatosis that is associated with an increased risk of renal cell cancer and pulmonary cysts, warranting personal and familial follow-up and counseling.
- Blastic Plasmacytoid Dendritic Cell NeoplasmPublication . Lencastre, A; Farinha, P; Cabete, J; Ferreira, G; João, A; Lestre, SBlastic plasmacytoid dendritic cell neoplasm is a rare and aggressive hematodermic neoplasia with frequent cutaneous involvement and leukemic dissemination. We report the case of a 76-year-old man with a 2 month history of violaceous nodules and a tumor with stony consistency, located on the head, and mandibular, cervical and supraclavicular lymphadenopathies. Multiple thoracic and abdominal adenopathies were identified on computerized tomography. Flow cytometry analysis of the skin, lymph node and bone marrow biopsies demonstrated the presence of plasmocytoid dendritic cell neoplastic precursor cells (CD4+, CD45+, CD56+ and CD123+ phenotype). After initial clinical and laboratorial complete remission with chemotherapy, the patient died due to relapse of the disease associated with the appearance of a cervical mass with medullary compromise.
- Case for DiagnosisPublication . Lencastre, A; Cabete, J; Apetato, MAn 83 year-old man was observed for an 8 year-long pruritic, erythematous plaque of the left axilla. He had been continuously medicated with topical antifungals and steroids with occasional symptomatic relief, but with disease progression. After a clinical and histological diagnosis of Extramammary Paget's Disease, a total excision with wide margins was performed. He remains disease-free 12 months after surgery. We report this rare presentation of Extramammary Paget's Disease on a male patient.
- Case for DiagnosisPublication . Lencastre, A; Paiva Lopes, MJA 27 year-old-man, with no known personal or familial history of disease, mentioned a 10-year history of asymptomatic groups of telangiectasias, with a Blaschko lines distribution on the right lateral aspect of the neck and asymptomatic. He denied any episodes of disease or drug intake that could be associated with the disease. Blood work had no changes, namely of liver enzymes or infectious serologies. The clinical diagnosis of Idiopathic Acquired Unilateral Nevoid Telangiectasia was made, an uncommon, benign vascular malformation. The patient declined doing a cutaneous biopsy or treatment with a cosmetic intent.
- A Comparative Study of Onychomycosis and Traumatic Toenail Onychodystrophy Dermoscopic PatternsPublication . Ramos Pinheiro, R; Dias Domingues, T; Sousa, V; Galhardas, C; Apetato, M; Lencastre, ABackground: Onychomycosis (OM) and traumatic onychodystrophy (OD) are common causes of toenail changes. A clinical diagnosis is often impossible without mycology. Dermoscopy is helpful in this setting but yet underexplored. Prospective comparative studies between OM and OD onychoscopic findings have not been previously performed. Objectives: We sought to determine distinguishing dermoscopic presentations of OM and traumatic OD. Methods: We performed a prospective, observational study including patients presenting with ≥1 toenail onychodystrophy. All underwent onychoscopy, clinical and mycological examination. Based on these results, patients received a final diagnosis of OM or OD. Dermoscopic presentations of OM and OD patients were classified in patterns and compared. Results: In all, 110 cases of OM and 82 of traumatic OD were compared. Statistical analyses revealed that the distal pulverized and the irregular spiked macular dermoscopic patterns were predictors of an OM diagnosis. The regular macular, the non-classifiable, the total and partial homogeneous background dermoscopic patterns correlated with traumatic OD diagnosis. Conclusions: We demonstrated that OM and traumatic OD have distinctive onychoscopic presentations. Dermoscopy may be an important ancillary tool to guide their differential.
- Conservative Treatment of Chondrodermatitis Nodularis Chronica Helicis with Topical NitroglycerinPublication . Cabete, J; Lencastre, A; Paiva Lopes, MJ; Lestre, S; Serrão, VBackground: Chondrodermatitis nodularis chronica helicis (CNCH) is a benign, painful, indurated inflammatory nodule arising on the pinna. Different treatment modalities have been used with varying efficacy rates. Despite lower efficacy, conservative methods are most desirable. Case reports: Three patients diagnosed with CNCH were treated with 0.4% topical nitroglycerin ointment, twice daily. These included two women and one man, aged 66, 86, and 60-years-old, respectively. Two painful nodules were located on the antihelix, and one on the helix. One patient had been previously treated with intralesional corticosteroids, and another with intralesional corticosteroids, CO2 laser and photodynamic therapy. All three patients experienced significant clinical and symptomatic improvement with topical nitroglycerin. No side effects were reported. Discussion: The etiology of CNCH is uncertain. Dermal and chondral ischemia arising from perichondrial arterial narrowing has been recently described as a possible cause for this benign condition, hence the potential of nitroglycerin as a smooth muscle relaxant and vasodilator. A previous report described significant improvement in twelve patients treated with 2% topical nitroglycerin. Conclusion: Topical nitroglycerin ointment appears to be a noninvasive alternative for the treatment of CNCH. A lower concentration (0.4%) may be as effective and with fewer side effects, but more studies are warranted.
- Cutaneous Leiomyomatosis in a Mother and DaughterPublication . Lencastre, A; Cabete, J; Gonçalves, R; João, A; Fidalgo, AA 34-year-old woman with no known medical history was evaluated for multiple painful brown nodules and papules on the anterior aspect of the trunk. She mentioned a history of similar cutaneous findings on her mother. Biopsies of three lesions revealed piloleiomyomata. Renal and adrenal ultrasound revealed an isolated simple cortical cyst, and pelvic and endovaginal ultrasound revealed two uterine myomata. The clinical diagnosis of hereditary leiomyomatosis and renal cell cancer was corroborated by the identification of a heterozygous variant on exon 5 of the fumarate hydratase gene (c.578C>T p.T193I). Identification of the tumor piloleiomyoma should alert the dermatologist to this rare genodermatosis, which is associated with an increased risk of renal cell tumors, demanding multidisciplinary follow-up, and personal and family counseling.
- Cutaneous Leukemic Infiltration Following Varicella - a Case of Wolf's Isotopic ResponsePublication . Brasileiro, A; Lencastre, A; João, A; Fidalgo, AWolf's isotopic response designates the appearance of two subsequent unrelated dermatoses in the same anatomic location. We report the case of a 51-year-old man with a medical history of chronic lymphocytic leukemia without known extra-hematopoietic involvement. The patient developed a disseminated papulo-vesiculous eruption, diagnosed as varicella. Few days after recovering, an erythematous and violaceous papular dermatosis with histopathological examination compatible with leukemic infiltration appeared on the scars of previous herpetic lesions. Complete remission was obtained under systemic corticotherapy, without cutaneous recurrence or blastic transformation. Wolf's isotopic response is attributed to a localized immunologic imbalance following a certain stimulus. In this patient, herpetic infection acted as a local spur for inaugural cutaneous leukemic infiltration, with no impact on the prognosis for the underlying disease.
- Granuloma Glutaeale Infantum: A Re-emerging Complication of Diaper DermatitisPublication . Ramos Pinheiro, R; Matos-Pires, E; Baptista, J; Lencastre, AGranuloma gluteale infantum is a rare nodular complication of irritant diaper dermatitis. The association of this condition with the widespread use of nondisposable cloth diapers has been increasingly recognized. We present the case of an 18-month-old girl with granuloma gluteale infantum. Our aims are to emphasize the importance of clinical recognition of this re-emerging complication of diaper dermatitis and to point out the potential role of topical calcineurin inhibitors as a treatment option.
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