Infantile pyramidal protrusion as a manifestation of lichen sclerosus et atrophicus.

BACKGROUND: A perineal infantile lesion previously described as "skin tag/fold" had recently been named infantile perianal pyramidal protrusion. It appears on the perineal median raphe of girls as a pyramidal soft tissue swelling, covered by smooth, red or rose-colored skin. Its pathogenesis is unknown. As in the case of other perianal lesions, knowledge about it is important, as concern about signs of child abuse grows. OBSERVATIONS: Four girls, 2 of them sisters, with infantile perianal pyramidal protrusion were studied. Three of these girls showed subtle clinical evidence of classic lichen sclerosus et atrophicus on first examination. The other girl developed vulvar lesions of lichen sclerosus et atrophicus months after the diagnosis of infantile perianal pyramidal protrusion. All 4 protrusions disclosed histopathological findings diagnostic of lichen sclerosus et atrophicus. CONCLUSIONS: Infantile perianal pyramidal protrusion is, at least in some patients, a peculiar form of lichen sclerosus et atrophicus that can precede other, more characteristic manifestations. We suggest changing the name to the more precise infantile perineal protrusion. Knowledge of this hitherto unrecognized clinical form of lichen sclerosus et atrophicus can help to explain anogenital symptoms and avoid its misinterpretation as a sign of sexual abuse.

A quarter of a century with AIDS.

In Northwestern Spain (NWS), the annual incidence of AIDS diagnoses increased from 1984 (when the first case was diagnosed) until 1996. However, since 1996, this incidence has reduced considerably, including a notable 40% reduction between 1997 and 1998. The Galician Register of AIDS supplies information on the evolution of AIDS pathology in NWS. This report compiles data on patients who were diagnosed with AIDS in NWS between 1984 and 2008. From 1981, when the first case of AIDS was described, until December 31, 2008, a total of 3,766 AIDS cases were registered in NWS. Of these, 2,085 cases (55.4%) resulted in death. Examining data from individual provinces revealed that the highest number of cases was in A Coruña (1,548 cases) followed by Pontevedra (1,485 cases).For almost half of the new cases of AIDS diagnosed between 2003 and 2008 (44%), less than six months passed between the diagnosis of infection and manifestations of the disease. Thus, the number of patients that do not receive early diagnosis of HIV infection has remained high.With regard to the transmission mechanism, 64% of the cases occurring during these years resulted from needle-sharing among injected drug users (IDUs). Unprotected heterosexual and homosexual practices were responsible for 20% and 17% of the cases, respectively.

Bullous pemphigoid triggered by influenza vaccination? Ecological study in Galicia, Spain.

BACKGROUND: Influenza vaccination has been suggested to be a trigger for bullous pemphigoid (BP). In our area most new patients with BP are admitted to hospital. OBJECTIVES: To detect a difference in the rate of admissions to hospital for BP during population influenza vaccination programmes, which are mainly targeted at individuals aged 65 years or older, start in the last week of September and last for 2 months. METHODS: An ecological study in Galicia, north-west Spain (1997-2005), restricted to people aged 65 years or older. Using Poisson regression, results were controlled for mean age of admission and percentage of the population vaccinated each year. RESULTS: There were 29 admissions registered during 'vaccination periods' (i.e. approximately 10 weeks per year, including the period of vaccination and two further weeks), and 158 during nonvaccination periods (the rest of the year). The incidence rate ratio of admissions for BP in vaccination vs. nonvaccination periods was 0.85 (95% confidence interval 0.57-1.27, P = 0.43). CONCLUSIONS: Our data do not support the hypothesis of influenza vaccination being an important trigger for severe BP on a population level.

Cowden's disease and Down syndrome. An exceptional association.

Cowden's disease with typical mucocutaneous features developed in a 24-year-old woman who had Down syndrome. To our knowledge this association has not been reported previously. The interest lies in the overlap of some characteristic features of both diseases.

Necrobiosis lipoidica: a case with prominent cholesterol clefting and transepithelial elimination.

Transepithelial elimination of degenerated collagen through the hair follicle in necrobiosis lipoidica is rare, clinically manifesting as comedo-like plugs. Also unusual in necrobiosis lipoidica is the finding of cholesterol cleft formation. We report a case of necrobiosis lipoidica with transepithelial elimination of cholesterol crystals through hair follicles. This has been described in necrobiotic xanthogranuloma, demonstrating some overlap in the histopathologic findings in both necrobiotic granulomas. Additional criteria should be used to establish the diagnosis.

Multiple eruptive verrucous hemangiomas: a variant of multiple hemangiomatosis.

We present a case of an 8-year-old girl, who has, since birth, developed multiple angiomatous lesions of the skin. The evolution has been progressive and eruptive, with the lesions, beginning as reddish papulae, acquiring later a verrucous appearance. The case has revealed no associated systemic symptoms nor relevant family history of any interest. The clinical picture suggests multiple hemangiomatosis. The histopathology seems to relate this disorder to verrucous hemangioma, and therefore needs to be differentiated from angiokeratotic nevi. To our knowledge, reports of a similar clinicopathological picture are not mentioned in the literature.

A cutaneous multilobated B-cell lymphoma.

A patient with multilobated B-cell malignant lymphoma with lesions limited to the skin is described. The light, electron microscopic, and immunohistochemical features of this unusual morphologic variant of non-Hodgkin's lymphoma are described. The nosologic position of this histologic subtype is discussed. The clinical course, with lesions confined to the skin, and the response to treatment suggest a favorable prognosis.

Abdominal nodules as a presentation of obstruction of the inferior vena cava and factor V Leiden mutation.

Abdominal nodular lesions are not usually considered a sign of deep venous thrombosis. However, we have seen a patient with abdominal nodular lesions due to venous thrombosis, that led to the diagnosis of factor V Leiden mutation, a recently described cause of hypercoagulability.

Acrochordons are not a component of the Birt-Hogg-Dubé syndrome: does this syndrome exist? Case reports and review of the literature.

Multiple fibrofolliculomas, trichodiscomas, and acrochordons compose the triad of cutaneous lesions characterizing the Birt-Hogg-Dubé syndrome, inherited in an autosomal dominant fashion. We report a case of a family who had the triad of tumors of the Birt-Hogg-Dubé syndrome. Two members were observed clinically and histologically. Biopsies of the facial papules disclosed features of the fibrofolliculoma/trichodiscoma spectrum. Lesions that were clinically acrochordon-like proved to correspond to the same histopathologic spectrum. The characterization of the nature of the acrochordon-like lesions in our patients and the review of the literature allow us to question if acrochordons (skin tags) should be maintained as a component of this association. In light of our conclusion that fibrofolliculoma, trichodiscoma, and the acrochordon-like lesions are histologic variations of a single lesion, we further question whether the term "syndrome" is valid.

[Enzymatic and molecular studies in a case of hepato-erythropoietic porphyria. Homozygote form of type familial cutaneous porphyria]. / Etudes enzymatique et moléculaire d'un cas de porphyrie hépato-érythrocytaire. Forme homozygote de la porphyrie cutanée de type familial.

BACKGROUND: Porphyrias are either hepatic or erythroid, depending on the principal site of the specific enzymatic defect. Homozygous uroporphyrinogen decarboxylase deficiency, known as hepato-erythropoietic porphyria (HEP), can involve several mutations. CASE REPORT: A young man, aged 20 years, had gradually developed photosensitivity since the age of 1 year, leading to hypertrichosis and sclerodermoid changes in sun-exposed areas of skin. He displayed high urinary uroporphyrin and 7-carboxylic porphyrins, and elevated fecal and red blood cell iso-coproporphyrin and coproporphyrin. Erythrocyte uroporphyrinogen decarboxylase activity of the patient was reduced to 18% of normal control values, while those of his grandmother and his half-brother were 62-65% of normal. MOLECULAR BIOLOGY: Amplification of the genomic DNA by PCR and hybridization with allele-specific oligonucleotides (ASOs) demonstrated the presence of a Gly 281-->Glu mutation in the patient and in his grandmother and half-brother. CONCLUSION: Enzymatic studies and details of the familial lineage are important for precisely classifying this type of porphyria. Molecular biology studies are necessary before considering any future gene therapy.

Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites.

BACKGROUND: Lymphangiectasia results from acquired dilation of lymphatic vessels. Areas of skin affected by obstruction or destruction of lymphatic drainage are said to be prone to the development of lymphangiectasia. Cirrhosis is a cause of alterations of lymph flow. METHODS: Case report. RESULTS: We report a case of acquired, late-onset, lymphangiectasia associated with alcoholic hepatic cirrhosis. Lesions were scattered over the right, lower, anterior abdominal wall, a region that is drained by a common group of lymphatic vessels that were probably disordered. CONCLUSION: We think that this is the first reported case of lymphangiectasia associated with altered lymph flow in cirrhosis and ascites.

Inpatient dermatology: characteristics of patients and admissions in a Spanish hospital.

BACKGROUND: Inpatient dermatology has not been properly described in many countries. National differences might be important in the evaluation of its usefulness and the applicability of politics of health expenditure restrictions. OBJECTIVE: To describe inpatient activity and readmission rates in a dermatology department in Spain. STUDY DESIGN: Cross-sectional prospective study in a single hospital. SETTING: Secondary care hospital of the National Health Service in Pontevedra (Spain). METHODS: From May 1997 to December 2000, all discharge sheets (1048) were included in the study, codified and described. RESULTS: Surgery was the reason for admission in 37% of the inpatients. The most frequent diagnosis were: neoplasm (36%), infection (15%), psoriasis (10%), other (10%), dermatitis (6%) and drug reaction (5%). Readmission rates were 1.8% within 30 days, and 12.5% within 1 year. CONCLUSIONS: Inpatient dermatology is different in different countries. Compared with what has been described in the USA or UK, our data suggest an important surgical content of inpatient dermatology in Spain, not reported in those countries. Medical diagnoses also differ, consisting of more infections, and less psoriasis and dermatitis in our setting. Readmission rates are low when compared with previously published ones, a finding that supports a long-term benefit of hospitalization.