Rosário Alves

Cutaneous polyarteritis nodosa treated with intravenous immunoglobulins

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A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, highly aggressive hematopoietic malignancy that is characterized by cutaneous and bone marrow involvement and leukemic spread, representing 0.7% of primary cutaneous lymphomas1. Formerly known as CD4+/CD56+ hematodermic neoplasm or blastic NK-cell lymphoma2, BPDCN is derived from precursors of plasmacytoid dendritic cells and was only recently established as a distinct entity3. A 74-year-old man presented with a 4-month history of asymptomatic cutaneous lesions that initially appeared on his face but progressively spread to his thorax and arms. He had no systemic symptoms. His medical history included peripheral arterial disease and dyslipidemia, for which he was on acenocumarol and rosuvastatin. Physical examination revealed infiltrated violaceous plaques and nodules on his face and thorax and firm subcutaneous nodules on his arms along with reticulated purpuric macules (Fig. 1). Axillary and submandibular lymph nodes were bilaterally palpable. Laboratory data disclosed severe normochromic normocytic anemia (hemoglobin, 4.4 g/dl), thrombocytopenia (53×109/L), leukocytosis (16.48×109/L) with 44% of morphologically immature atypical cells and elevated β2-microglobulin (3.8 mg/L). Flow cytometry (FCM) of the skin cells and the peripheral blood showed 35% and 37%, respectively, of cells with the following immunophenotype (Fig. 2A, B): positive CD45 (dim), CD4, CD56, CD123, CD7 (dim), HLA-DR, and CD38. Other T/NK (CD1a, CD2, CD3, TCR, CD5, CD8, CD16, CD94, CD161), B (CD19, CD20, CD79a), myeloid (CD11b, CD13, CD14, CD15, CD33, CD64, myeloperoxidase) and immature cell (CD34) markers tested negative. Histopathology of a skin lesion showed a monomorphous diffuse infiltrate of large blastic cells with irregular nuclei occupying the entire dermis and infiltrating the subcutaneous tissue (Fig. 2C, D). Immunohistochemical analysis confirmed the presence of cells with the aforementioned immunophenotypic features. No chromosomal alterations were detected by cytogenetic analysis of peripheral blood cells. Computed tomography scans disclosed basal lung consolidation suggestive of malignant infiltration, mediastinal, hilar, axillary and retroperitoneal lymphadenopathy, hepatomegaly, and splenomegaly, findings that were compatible with widespread disease. Bone marrow analysis was not performed once its involvement was confirmed by the altered peripheral blood profile. Together these findings fulfilled the requirements for the diagnosis of BPDCN. The patient started palliative chemotherapy with cytarabine and mitoxantrone but died 3 weeks later of multi-organ failure. Fig. 1 (A, B) Features of the cutaneous lesions on the face and thorax

Atopic Dermatitis-Like Non-Erythrodermic Leukemic Variant of CD3−/+dim CD4+ Cutaneous T-Cell Lymphoma Preceded by Cutaneous Papular Xanthomatosis

We report a patient with cutaneous papular xanthomatosis who 4 years later developed a CD3−/+dim/CD4+ T-cell lymphoma. Pruritic xerotic non-erythrodermic skin, eosinophilia and hyper-IgE were present and erroneously classified as atopic dermatitis. Flow cytometry and DNA ploidy analysis of both blood and skin lymphocytes, skin histology and blood T-cell receptor gene rearrangement studies confirmed diagnosis of T-cell lymphoma. Monoclonal CD3−/+dim/CD4+ T-cells were especially prone to the synthesis of IL-13, a cytokine that is involved in IgE-secretion, and comprised both a medium (diploid) and large (hyperploid) sized T-cell populations with a similar immunophenotype. The majority of the normal residual T-cells were large granular lymphocytes, expressed activation-related and natural-killer -associated markers and secreted high levels of interferon gamma, suggesting that they might correspond to active cytotoxic cells directed against the neoplastic T-lymphocytes.

Granuloma annulare of the penis ― subcutaneous presentation

ejd.2011.1353 Auteur(s) : Teresa Pinto-Almeida teresap.almeida@hotmail.com, Tiago Torres, Madalena Sanches, Rosario Alves, Manuela Selores Department of Dermatology, Hospital de Santo Antonio, Rua D. Manuel II, s/n 4100 Porto, Portugal Granuloma annulare (GA) is a benign skin inflammatory disorder of unknown etiology. Clinical variants include localized, generalized, subcutaneous, perforating and patch types. The subcutaneous type is more frequent in children and typically affects the extremities [...]

Brachioradial pruritus in a patient with cervical disc herniation and Parsonage-Turner syndrome

Brachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-yearold woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome. The patient started gabapentin 900mg/day with good control of itching. Corticosteroids and antihistamines are often ineffective in the treatment of BP. Gabapentin has been used with encouraging results. All patients with Brachioradial pruritus should be evaluated for cervical spine injuries.

Adult mastocytosis: a review of the Santo António Hospital 's experience and an evaluation of World Health Organization criteria for the diagnosis of systemic disease

BACKGROUND Mastocytosis is a clonal disorder characterized by the accumulation of abnormal mast cells in the skin and/or in extracutaneous organs. OBJECTIVES To present all cases of mastocytosis seen in the Porto Hospital Center and evaluate the performance of World Health Organization diagnostic criteria for systemic disease. METHODS The cases of twenty-four adult patients with mastocytosis were reviewed. Their clinical and laboratorial characteristics were assessed, and the properties of the criteria used to diagnose systemic mastocytosis were evaluated. RESULTS The age of disease onset ranged from 2 to 75 years. Twenty-three patients had cutaneous involvement and 75% were referred by dermatologists. Urticaria pigmentosa was the most common manifestation of the disease. One patient with severe systemic mast cell mediator-related symptoms showed the activating V560G KIT mutation. The bone marrow was examined in 79% of patients, and mast cell immunophenotyping was performed in 67% of the participants. Systemic disease was detected in 84% of cases, and 81% of the sample had elevated serum tryptase levels. All the diagnostic criteria for systemic mastocytosis had high specificity and positive predictive value. Bone marrow biopsy had the lowest sensitivity, negative predictive value and efficiency, while the highest such values were observed for mast cell immunophenotyping. Patients were treated with regimens including antihistamines, sodium cromoglycate, alpha-interferon, hydroxyurea and phototherapy. CONCLUSIONS Cutaneous involvement is often seen in adult mastocytosis patients, with most individuals presenting with indolent systemic disease. Although serum tryptase levels are a good indicator of mast cell burden, bone marrow biopsy should also be performed in patients with normal serum tryptase, with flow cytometry being the most adequate method to diagnose systemic disease.

Can the level of CD52 expression on Sézary cells be used to predict the response of Sézary syndrome to alemtuzumab

In this population, a predisposition toward autoimmunity is seen as 38.5% of patients had a personal history of a third concomitant autoimmune disease, in addition to psoriasis and fibrosing disorder, whereas 42.3% reported a history of a first-degree relative with an autoimmune disease (Table II). A positive antinuclear antibody titer was observed in 70.1% of those tested. No pattern emerges for these diseases in regard to order of diagnosis, time between diagnoses, or disease course; there were patients who experienced a severe course of both diseases and patients for whom the courses were mild. Time between diagnoses ranged from 1 to 54 years. LSA represented the largest number of cases and the presence of a third concomitant autoimmune disease was highest in this disease subtype. In patients with morphea and psoriasis, we found an increased prevalence of generalized morphea, consistent with the findings of Leitenberger et al, who demonstrated that generalized morphea occurs at a higher rate in populations with concomitant autoimmune disease. Our results also show a prevalence of a third autoimmune disease in 25% of patients with morphea and psoriasis. The number of cases of SS was few and definitive conclusions are difficult. One case was rapidly fatalewithin 1 year of diagnosisesimilar to a case series reported by Harrison et al in which 2 of 3 patients with previous psoriasis went on to develop severe SS. This study was limited by its retrospective nature, as well as the fact that we were not able to ascertain the total number of patients with LSA, morphea, or SS seen during the study period, thus preventing an assessment of the prevalence of concomitance. Further studies of patients with psoriasis and fibrosing disorders are required to understand the relative disease courses and prognoses as well as potential common pathophysiologic mechanisms.

Proliferating trichilemmal tumor of the nose

Proliferating trichilemmal tumor is a rare tumor originating in the external root sheath, that is usually found in the scalp of middle-aged or elderly females. Its histologic appearance may not correlate with its clinical behavior. In addition, there are no guidelines available for the treatment of these tumors, making its management a challenge for physicians. We report the case of a 53 year-old woman with a proliferating trichilemmal tumor on her nose, which is a very uncommon location for these lesions.

Vulvoperineal Crohn's disease responsive to metronidazole

Crohns disease is a multisystem chronic granulomatous inflammatory disease that primarily affects the gastrointestinal tract. In the majority of the cases, the cutaneous manifestations follow the intestinal disease, but occasionally dermatological lesions are the inaugural event and may constitute the only sign of the disease. Vulvoperineal involvement is rare, may precede bowel symptoms by months to years and may go unrecognized. Due to the paucity of reports of Crohns disease at this location and in the absence of randomized trials, there are no standard treatments for the cutaneous disease. We describe the case of a 47 year-old woman with vulvoperineal Crohns disease without digestive involvement, that was successfully managed with metronidazole.

Nodular secondary syphilis

Auteur(s) : Aristoteles ROSMANINHO arisrosmaninho@gmail.com, Madalena SANCHES, Ines LOBO, Rosario ALVES, Manuela SELORES Department of Dermatology, Centro hospitalar do Porto-HSA, Rua D. Manuel II s/n°, 4099-001 Porto, Portugal A 64-year-old man presented with an eighteen-day history of a non-pruritic eythematous eruption localized to his upper limbs, neck and back. He denied recent genital or buccal ulceration or heterosexual extramarital sexual intercourse as well as systemic complaints. His [...]