[Eccrine angiomatous hamartoma: a report of 2 cases]. / Hamartoma angiomatoso ecrino: presentación de dos casos.

Eccrine angiomatous hamartoma is a rare, benign tumor characterized by a proliferation of eccrine and vascular structures. We present 2 cases and review the characteristics of this disorder. The first patient was a 33-year-old woman who consulted for a brownish lesion on her back associated with local hyperhidrosis. The second patient was a 25-year-old man with an asymptomatic erythematous lesion on his left palm. In both patients a diagnosis of eccrine angiomatous hamartoma was made based on the histological findings. Eccrine angiomatous hamartoma is usually present at birth or develops during childhood. It is typically a solitary lesion and signs and symptoms can vary; diagnosis is therefore based on histological study. The most common site is on the distal parts of limbs. The lesions tend to be asymptomatic, but there may be associated pain and hyperhidrosis. Treatment is not usually necessary except in cases with persistent symptoms, excessive sweating, or cosmetic concerns.

[Lung involvement in pyoderma gangrenosum: a case report and review of the literature]. / Pioderma gangrenoso con afectación pulmonar: caso clínico y revisión de la literatura.

Pyoderma gangrenosum is a neutrophilic dermatosis that, in addition to its characteristic skin manifestations, can cause visceral alterations. Our patient was a 34-year-old woman with pyoderma gangrenosum that was exacerbated by iodine and that also affected the lungs. Other published cases of lung involvement in pyoderma gangrenosum are reviewed and we discuss the possible exacerbation of this disease by iodine. Lung involvement is the most common extracutaneous manifestation of pyoderma gangrenosum and the main differential diagnosis is then with Wegener granulomatosis. It is important to remember the possible presentation of extracutaneous manifestations of pyoderma gangrenosum, including particularly lung involvement, in order to avoid subjecting a patient to aggressive diagnostic tests, at least in the initial stages.

Observer agreement in toenail disorders: implications for diagnosis and clinical research.

BACKGROUND: Toenail disorders are frequent, especially onychomycosis. The interobserver variability of nail signs needs to be known before these signs can be confidently applied for diagnosis. OBJECTIVES: To describe observer agreement in toenail findings as described by dermatologists in standard clinical practice, focusing on signs that could be useful for diagnosis of onychomycosis. METHODS: Prospective cross-sectional study in five dermatology departments. Eighty-six patients with abnormal toenails that could have onychomycosis as a differential diagnosis were independently examined by a pair of dermatologists using a predefined questionnaire, to describe the presence of 10 findings on previous history and 14 physical signs. RESULTS: Agreement was fine for previous history findings: it was very good (kappa > 0.81) for previous diagnosis of diabetes, smoking and use of public dressing rooms or swimming pools. Agreement was good (kappa 0.61-0.80) for immune suppression (drugs or cancer), previous diagnosis of fungal disease and worsening in the last year. It was moderate (kappa 0.41-0.60) for previous diagnosis of arterial disease, trauma induced by work or sports, and distal vs. proximal or lateral vs. central start of the lesion. Agreement was worse for physical signs: we found good agreement for the presence of the same disease in fingernails, abnormal plantar desquamation, deformity causing nail trauma, and subungual hyperkeratosis. It was moderate for the presence of nail destruction, tinea interdigitalis, onycholysis, and the type of material obtained by subungual curettage (dust vs. hard). Agreement was fair (kappa 0.21-0.40) for the presence of longitudinal or transverse striae, trachyonychia, pachyonychia, and change in colour of the nail plate. Pitting was too infrequent to allow for kappa calculation. Chance expected agreement was between 51% and 84% for all signs except pitting. CONCLUSIONS: Agreement is adequate for most signs. It is low for the presence of longitudinal or transverse striae, trachyonychia, and change in colour of the nail plate. Pitting is rare in toenails.

Staining of neuroendocrine Merkel cells of human epidermis using the uranaffin reaction.

The uranaffin reaction (UR) stains neurosecretory (NS) granules of the neuroendocrine system under certain experimental conditions of staining and rinsing solutions. Human normal neuroendocrine (NE) Merkel cells stained using the UR exhibit a positive reaction in their NS granules, ribosomes, and nuclear chromatin. The average values of maximum granular diameter (GD = 69.1 nm) and area (GA = 3637.8 mm2) of NS granules measured in the adult NE Merkel cells stained with UR are significantly greater than those (GD = 61.4 nm; GA = 2699.8 nm2) seen in the fetal NE Merkel cells also stained with UR. No differences in form factor are found between fetal and adult NS granules. On different samples of human adult and fetal epidermis it is demonstrated that UR is a useful cytochemical marker for the NS granules of normal NE Merkel cells.

Tissue concentrations of prothymosin alpha: a novel proliferation index of primary breast cancer.

In 71 patients with classic invasive ductal carcinomas, levels of prothymosin alpha (PT alpha), as assayed by a radioimmunoassay that detects thymosin alpha 1 (the NH2-terminal fragment of PT alpha), were significantly greater in tumour samples than in normal breast tissue. PT alpha levels were correlated with (a) the number of positive axillary lymph nodes (rs = 0.5384, P < 0.01), and (b) the percentage of tumour cells in the S or G2/M phase as assessed by flow cytometry (rs = 0.5027, P < 0.01). Since the beginning of this study in 1989, 21 patients have presented distant metastases, all of whom were previously shown to have tumour PT alpha levels greater than 124 ng of thymosin alpha 1/mg protein. The present report indicates that PT alpha might be used to identify breast cancer patients at high risk for distant metastases.

Cellular distribution of prothymosin alpha and parathymosin in rat thymus and spleen.

By means of immunohistochemical methods, we have investigated the cellular distribution of prothymosin alpha and parathymosin in rat thymus and spleen, using specific antibodies raised against thymosin alpha-1 and against parathymosin. We observed prothymosin alpha immunoreactivity in lymphoid cells both in thymus and spleen. In the thymus, prothymosin alpha staining was more marked in cortex than in medulla. In the spleen, prothymosin alpha was found in lymphocytes of the periarteriolar lymphatic sheaths and was especially prominent in the germinal centers. Parathymosin immunoreactivity in the thymus was mainly localized in the medulla; positive cells were reticuloepithelial cells from the thymic reticulum and the blood barrier. Thymocytes were negative. In spleen, parathymosin was found in reticular cells arranged in a ring between the periarteriolar lymphatic sheath and the marginal zone. Our results do not support an exclusive role for these peptides as immune system hormones or cytokines.

Treatment of alpha1-antitrypsin-deficiency panniculitis with minocycline.

A small proportion of patients with alpha1-antitrypsin (alpha1AT) deficiency experience recurrent ulcerating panniculitis. Studies suggest that alpha1AT-deficiency panniculitis reflects an uncontrolled inflammatory process because of the serum's incapacity to neutralize proteolytic enzymes released by leukocytes in the skin. Dapsone is considered the treatment of choice for this entity, but it is limited by its side effects, especially hematologic ones. Tetracyclines have anticollagenase and anti-inflammatory activity and have been used successfully to treat this type of panniculitis. We report the case of a 42-year-old woman with recurrent alpha1AT-deficiency panniculitis who did not tolerate the side effects of dapsone or systemic corticosteroid. Minocycline treatment led to disappearance of lesions, and long-term administration prevented recurrences without evident negative side effects. We consider minocycline a safe treatment that allows control of alpha1AT-deficiency panniculitis.

Neuroendocrine cells of the human lung express substance-P-like immunoreactivity.

The occurrence of substance P (SP) in the neuroendocrine population of human lungs was investigated by immunohistochemical methods. All individuals studied (n = 16) had SP-like immunoreactive cells, being more numerous in lungs of fetuses and newborn infants than in adults. These cells, both solitary and forming neuroepithelial bodies, were found at all levels of the respiratory mucosa. Solitary neuroendocrine cells and neuroepithelial bodies were found in the bronchial and bronchiolar mucosa, while at the alveolar level neuroepithelial bodies were also seen. A more intense SP-like immunoreactivity was found in the basal cytoplasm of these cells. Occasionally they show cytoplasmic prolongations which interdigitate with neighboring epithelial cells. These facts suggest that SP-like immunoreactive cells may have a paracrine or local secretion function, acting over surrounding epithelial cells or structures situated in the lamina propria. The evidence of great numbers of SP-like immunoreactive neuroendocrine cells in fetuses and infants might be the expression of a functional role of SP in lung development.

Evolution of prothymosin alpha and proliferating cell nuclear antigen (PCNA) immunoreactivity through the development of rat ovarian follicles.

The cellular distribution of prothymosin alpha (ProT) was studied in ovarian follicles of adult cycling rats. We found positive granulosa and theca cells throughout follicular maturation. When both ProT and proliferating cell nuclear antigen (PCNA) immunoreactivity was studied, we observed that both proteins were expressed in the same granulosa and theca cells, although sometimes ProT immunoreactivity was weak or absent in the mitotic (M) phase. Moreover, both peptides share the nuclear distribution, but ProT immunoreactivity was never seen in nucleoli. Therefore, we conclude that in mitotic cells ProT is expressed only in actively proliferating cells, since all ProT-positive cells were also positive for PCNA. ProT and PCNA immunoreactivities during the meiotic division were studied in oocytes. The presence of PCNA was, unlike ProT, constant throughout follicle development (except atretic oocytes). Oocytes expressed ProT from primordial follicles to the eighth generation, but more developed oocytes and atretic oocytes were not immunoreactive. In hypophysectomized rats, all oocytes were immunoreactive. Interestingly, in hypophysectomized rats treated with follicle stimulating hormone (FSH) that promoted follicle development, the more developed oocytes did not show ProT immunoreactivity. Since hypophysectomized rats were not treated with luteinizing hormone we conclude that ProT expression is not required to complete meiotic division I.

Localization of serotonin-like immunoreactivity in the Merkel cells of pig snout skin.

The presence of serotonin in the Merkel cells of pig snout epidermis was investigated by the peroxidase-antiperoxidase immunohistochemical technique. Serotonin-like immunoreactive Merkel cells were found in groups located at the base of epidermal rete pegs and in the external root sheath of sinus hair follicles (vibrissae). Immunoreactivity was stronger on the basal side of the Merkel cells, where dense-cored granules are most numerous. Neither the nerve terminal associated with the Merkel cell nor the neighbouring epidermal cells were immunostained. These results are the first evidence of serotonin-like immunoreactivity in mammalian Merkel cells. The fact that immunoreactivity is strongest in those parts of the Merkel cells with the highest granule density suggests that in these cells serotonin is probably localized in the dense-cored granules.

Synaptophysinlike immunoreactivity in the Merkel cells of pig-snout skin.

Synaptophysin is a membrane glycoprotein (Mr 38,000) found in small vesicles with clear content. It is present in neurons and in a variety of neuroendocrine cells and neoplasms, including the neuroendocrine carcinoma of the skin (Merkel cell tumor). We report the presence of synaptophysinlike immunoreactivity in normal Merkel cells of the pig-snout epidermis and vibrissae. The immunostaining was primarily localized in the Merkel cell side opposite to the nerve terminal. This fact suggests that in Merkel cells, as well as in other neuroendocrine cells, synaptophysin could occur in small clear vesicles (accumulated in Merkel cells in the side opposite to the neurite) and not in the secretory granules themselves (which are concentrated in the cellular side apposed to the neurite).

Solitary cutaneous metastasis on the buttock: a disclosing sign of pancreatic adenocarcinoma.

A 48-year-old man, previously healthy and asymptomatic, showed a unique skin lesion located on the right buttock. The histopathological study of the lesion disclosed an adenocarcinoma metastatic to the skin. The primary tumour was found at the head of the pancreas, and was confirmed by biopsy. Six months after the beginning of treatment with Gemcitabine the patient is still asymptomatic, and the cutaneous lesion has disappeared. Carcinomas of the pancreas represent less than 5% of human malignant neoplasms, skin involvement is rare, and metastasis generally multiple and situated in the periumbilical area. To our knowledge, very few patients have been reported with a solitary cutaneous lesion disclosing a pancreatic adenocarcinoma. The atypical location, the absence of related symptoms or analytical disorders, and the good progress of the patient make this case especially interesting.

Rubinstein--Taybi syndrome and ulerythema ophryogenes in a 9-year-old boy.

Rubinstein-Taybi syndrome is characterized by the presence of a peculiar facies, mental retardation, and broad thumbs and great toes. Several associated cutaneous abnormalities have been reported with this syndrome. Ulerythema ophryogenes is a form of follicular keratosis associated occasionally with other ectodermal defects and congenital anomalies. We describe a 9-year-old child with Rubinstein-Taybi syndrome and ulerythema ophryogenes. This association has not been described previously to our knowledge.