Luís Soares de Almeida

Desmoplastic Malignant Melanoma : A Clinicopathologic Analysis of 113 Cases

Desmoplastic melanoma (DM) is a rare variant of spindle cell melanoma, which usually develops in sun-damaged skin of elderly patients. Often the lesion is nonpigmented and frequently mistaken for a nonmelanocytic proliferation, which delays diagnosis and treatment and therefore worsens the prognosis.The spindle shape of neoplastic melanocytes, the prominent desmoplasia, and the frequent neurotropism of neoplastic melanocytes are its most characteristic histopathological features.We have studied the clinicopathologic features of 113 cases of DM. The mean age of the patients was 71.1 years; 48% of the patients were males and 52% were females. The neoplasm was located on the head in 72% of the cases. Malignant melanoma was the initial clinical diagnosis in only 27% of the cases.Histopathologically, all lesions appeared as poorly demarcated neoplasms that involved the entire dermis and often extended into the subcutaneous tissue. The neoplasms were composed of ill-defined fascicles of spindle cells. Desmoplasia was defined as the presence of spindle cells associated with a fibrotic stroma. Fifty-one cases (45%) were classified as “pure DM” when the lesion was entirely desmoplastic, and 62 cases (55%) were considered as “combined DM” when a recognizable desmoplastic component was seen in an otherwise conventional malignant melanoma. In 81% of the cases, an atypical intraepidermal melanocytic component (in situ malignant melanoma) was identified, whereas in the remaining 19% of the cases the intraepidermal component was lacking. Seventy-one percent of the cases were histologically amelanotic, 23% showed a small amount of pigment, and only 6% were heavily pigmented. Neural involvement was identified in 40/113 cases (35%), predominantly in the thickest tumors. Lymphoid nodules, found in 42/113 cases (37%), were significantly more frequent in pure DM than in combined DM (53% vs 24%). The null hypothesis of homogeneity of the “pure” and “combined” subgroups should be rejected (P < 0.002). Solar elastosis, with variable intensity, was seen in 82% of the cases. Mean Breslow thickness was 4.1 mm (4.6/3.7 mm, in the pure/combined subgroups, respectively), median was 4.0 mm (4.0/3.0 mm); Breslow thickness ranged from 0.3 to 11.0 mm, with half of the cases thicker than 4 mm. Only 4% of the cases showed Clark level below IV. The predominant neoplastic cells consisted of spindle-shaped melanocytes in 85% of the cases, whereas the remaining 15% of the cases demonstrated round neoplastic cells forming the main mass of the neoplasm. The mitotic rate of the neoplastic cells was low in 72% of the cases, 23% had an intermediate mitotic rate, and 5% showed a high mitotic rate.On follow-up, 55/113 patients (49%) (with an average of 55 months) demonstrated persistence of the disease. About 4% had local recurrences, 2% of lymph node invasion, 9% systemic metastases, and 12% died from the disease (2 cases of pure DM and 5 cases of combined DM).Although a better prognosis has been postulated for DM when compared with conventional cutaneous malignant melanomas of the same thickness, in most cases, a DM is diagnosed only in established long-standing and thick melanomas. Therefore, dermatologists and dermatopathologists should be more aware of this clinicopathologic variant of cutaneous malignant melanoma.

Treponema pallidum distribution patterns in mucocutaneous lesions of primary and secondary syphilis: an immunohistochemical and ultrastructural study

To study the different patterns of Treponema pallidum distribution in primary and secondary syphilis, 34 biopsy specimens of 8 patients with primary and 26 with secondary syphilis were assessed. Histopathological features, silver stain, and immunohistochemical T pallidum polyclonal antibody expression were investigated. The number and distribution of spirochetes were evaluated, and ultrastructural studies were performed. Spirochetes were identified with Warthin-Starry stain in 17 specimens (4/8 primary and 13/26 secondary syphilis), whereas immunohistochemical analysis disclosed spirochetes in 29 (8/8 primary and 21/26 secondary syphilis). In secondary syphilis, an epitheliotropic pattern characterized by abundant spirochetes in the lower mucosa/epidermis in an intercellular distribution was observed. In contrast, primary syphilis exhibited a mixed epitheliotropic and vasculotropic pattern further manifested by treponemes surrounding the vascular walls. These differences were statistically significant. Ultrastructural examination confirmed these results. Immunohistochemistry shows greater sensitivity when compared with Warthin-Starry staining. The immunohistochemical pattern of T pallidum distribution may permit the diagnostic differentiation of primary from secondary syphilis.

Clinical and Laboratory Features in Acute Generalized Pustular Psoriasis A Retrospective Study of 34 Patients

AbstractBackground: Acute generalized pustular psoriasis (AGPP) is a rare variant of psoriasis that can be lethal without proper treatment. It can be caused by the withdrawal of corticosteroids and, among its extracutaneous manifestations, liver abnormalities are frequently under-reported or attributed to drugs. Objective: The aim of this study was to assess the clinical and laboratory data, treatment options, and disease outcome in patients with AGPP and to search for significant differences between subgroups of these patients. Study Design: This was a retrospective analysis of the clinical files from inpatients with AGPP observed in our department between 1973 and 2008. Statistical tests were performed at a significance level of 5%. Setting: This was an inpatient, single-center study. Main Outcome Measures: Outcome measures were a previous history of psoriasis, corticosteroid use before admittance, mortality rate, white blood cell count, absolute neutrophil count, and abnormalities in liver enzymes. Results: Atotal of 34 patients fulfilled the inclusion criteria, of whom 61%were men and 65%had a previous history of psoriasis vulgaris. Topical corticosteroids were applied by 50%of patients before admittance. Skin lesions remitted with methotrexate, etretinate, or acitretin treatment in all but two patients who died of sepsis. Abnormalities in liver enzymes were present in 47%of patients. Patients without a previous history of psoriasis had a significantly younger age at the first episode of AGPP. In the comparison between the groups of patients with and without liver abnormalities, a male preponderance and higher leukocyte counts were found in the former, with a positive correlation between the absolute neutrophil count and total bilirubin also being observed. Previous use of retinoids or methotrexate was not associated with these hepatic alterations. Limitations: Limitations of the data were that this was a single-center, retrospective study with a small sample size. Conclusions: Withdrawal of systemic or topical corticosteroids can precipitate or worsen AGPP and these agents should not be used in these patients. Liver abnormalities can be considered an extra-cutaneous manifestation of AGPP. As in other series, no association between the use of drugs and changes in liver tests was found and therefore the deleterious withdrawal of efficient drugs, namely acitretin and methotrexate, should be avoided.

Sweet's syndrome: a retrospective study of 42 admitted patients in a Portuguese hospital

Background  Sweets syndrome is the most common neutrophilic dermatosis, but its pathophysiology is still largely unknown and different subgroups can be defined.

Squamous cell carcinoma arising in ulcerated necrobiosis lipoidica diabeticorum.

Dear Editors, Ulceration constitutes the main complication of necrobiosis lipoidica (NL) and usually occurs after trauma. Ulcerated NL predisposes patients to infections and very rarely is associated with malignant transformation, mainly to squamous cell carcinoma (SCC). We describe the case of a 68-year-old Caucasian male diagnosed with disseminated NL associated with type 2 diabetes mellitus (DM), presenting a SCC on an ulcerated plaque of NL located on the left forearm and chronic osteomyelitis of the radius.

The importance of patch tests in the differential diagnosis of adverse drug reactions

Exudative erythema multiforme is an acute self-limited skin disease often associated with infections (usually viral), and also with systemic diseases and drugs. We report the case of a 39-year-old woman diagnosed with systemic lupus erythematosus, who presented at the emergency clinic with exudative erythema multiforme which started 10 days after taking amoxicillin and clavulanic acid for tonsillitis together (almost simultaneously) with the pneumococcal vaccine. Rowells syndrome was also considered to be a possibility. Skin patch tests were carried with the standard battery of patches (GPEDC) and the active ingredients of the suspected drugs (Chemotechnique ®), with readings at D2 and D3. The tests were positive for amoxicillin 10% pet (++), ampicillin 10% pet (+ +) and penicillin G potassium 10% pet (+). We accepted the diagnosis of erythema multiforme due to amoxicillin, confirmed by patch testing.

Erythema multiforme on exposed and non‐exposed skin following polymorphic light eruption: a case report

We report a 35‐year‐old caucasian female with a history of polymorphic light eruption (PLE) who, after the outbreak of pruritic papules following the first sun exposure of the year, developed target lesions in two different bouts, the first on sun‐exposed skin and the second on sun‐protected sites. A diagnosis of erythema multiforme (EM) developing as a result of PLE was made. As far as we were able to search in the literature, EM occurring in two bouts has not been described previously. We discuss the differential diagnosis.

Recurrence of peripheral T-cell lymphoma as granulomas in the lower limbs

The presence of granulomas in the skin of T-cell lymphoma patients is a rare but well-known phenomenon. The authors present the case of a 44-year-old Caucasian male, with a previously treated peripheral T-cell lymphoma, with cutaneous infiltration and extensive dyschromia on his lower limbs. Skin biopsies revealed the presence of sarcoid granulomas associated with the recurrence of the lymphoma, confirmed by immunostaining and molecular analysis. Although a new scheme of chemotherapy was started, he died 18 months later. There are two different patterns of skin granulomas associated with lymphoma: granulomatous infiltrates admixed with neoplastic cells and cutaneous granulomas constituting a nonspecific manifestation of the underlying lymphoma, but presently there is no evidence supporting their relationship with lymphoma prognosis.

Extended remission of metastatic epithelioid angiosarcoma of the heart with liposomal doxorubicin

Angiosarcoma is the most common primary malignant tumour of the heart. It is a rare and aggressive neoplasm that almost always has a short and fatal evolution. By the time it produces symptoms it has usually progressed to a mass causing haemodynamic compromise. Initial presentation with metastatic disease is unusual. We report the case of a 72-year-old man who presented with painful skin lesions on both hands. The skin biopsy was diagnosed as intravascular metastasis of epithelioid angiosarcoma. Body computed tomography scan disclosed a solid mass in the left atrium. The tumour was judged unresectable and the patient was treated with systemic chemotherapy, consisting of liposomal doxorubicin, which resulted in a complete clinical response. The patient remains free of disease after 48 months of follow-up. The excellent clinical evolution of our patient verifies that liposomal doxorubicin may be effective in the treatment of these tumours and significantly prolong patients’ lifespan.

Infiltrados Linfocitários Cutâneos Simuladores de Linfoma

Os pseudolinfomas cutâneos englobam um grupo heterogeneo de entidades clinico-patologicas que tem em comum o facto de simularem clinica ou histologicamente linfomas cutâneos primarios. O seu diagnostico e dificil pois nao existem criterios precisos que diferenciem os infiltrados linfocitarios reactivos dos infiltrados linfocitarios neoplasicos. Os pseudolinfomas podem ser classificados de acordo com a predominância de linfocitos do tipo B versus T, ou agrupados segundo o tipo de linfoma cutâneo que mimetizam. O objectivo deste trabalho e discutir as pistas diagnosticas (histologicas e/ou clinicas) mais importantes na separacao entre as varias entidades simuladoras de linfoma e os linfomas cutâneos que estas mimetizam.