High prevalence of esophageal involvement in lichen planus: a study using magnification chromoendoscopy.

BACKGROUND AND STUDY AIMS: The first cases of squamous cell carcinoma in esophageal lichen planus were recently described. We performed a study to establish the prevalence of endoscopic and histopathologic abnormalities consistent with lichen planus and (pre-) malignancy in a cohort of patients with lichen planus. PATIENTS AND METHODS: A total of 24 patients with lichen planus were prospectively studied using high-magnification chromoendoscopy. Focal esophageal abnormalities were mapped, classified, and biopsied. Biopsies were also taken from normal-appearing esophageal mucosa at three levels (proximal, middle, and distal). The presence of a lymphohistiocytic interface inflammatory infiltrate and Civatte bodies (i. e. apoptotic basal keratinocytes) at histopathologic examination was considered diagnostic for esophageal lichen planus. Symptoms were assessed using validated questionnaires. RESULTS: A total of 38 focal abnormalities were biopsied. These consisted of: layers of mucosa peeling off, hyperemic lesions, papular lesions, submucosal plaques/papules, a flat polypoid lesion, and segments of cylindrical epithelium. No endoscopic signs of dysplasia were present. Esophagitis consistent with gastroesophageal reflux disease was noted in 12 / 24 patients. Histopathology showed chronic inflammation of the esophageal mucosa in the majority (18 / 24) of patients. In 50 % (12 / 24), the diagnosis of esophageal lichen planus was made. Dysplasia was not present. There were no differences in symptoms between patients with and without esophageal lichen planus. CONCLUSIONS: At screening endoscopy a high prevalence (50 %) of esophageal lichen planus was found in patients with orocutaneous lichen planus. No dysplasia was found.

[Mucocutaneous manifestations of amyloidosis]. / Mucocutane manifestaties van amyloyïdose.

Amyloidosis is the collective term for a group ofuncommon metabolic disorders in which insoluble amyloid protein-fibres are deposited in tissues and organs. Mucocutaneous manifestations are frequently found in this disease. The different types ofamyloidosis are divided into a systemic and a non-systemic group. Systemic amyloidosis is characterised by amyloid deposits in several organs. In the most frequent type, amyloid light chain (AL) systemic amyloidosis, the skin is involved in 29-40% of the cases. These mucocutaneous manifestations are sometimes the first clue to the discovery of systemic involvement. The non-systemic group comprises primarily localised amyloid deposits in skin and mucosa. The treatment of localised mucocutaneous amyloidosis is aimed at the local changes themselves. The mucocutaneous manifestations due to systemic amyloidosis may improve when it is possible to treat the underlying disease successfully.

A leg ulcer as manifestation of metastatic squamous cell vaginal carcinoma.

Cutaneous metastasis of vaginal carcinoma is extremely rare. So far, the total number of reported skin metastasis of vaginal carcinoma is only one. We present another case with an unusual manifestation of vagina carcinoma metastasis: skin metastasis presenting as a leg ulcer on the lower leg.

Pulmonary complications after T-cell-depleted allogeneic stem cell transplantation: low incidence and strong association with acute graft-versus-host disease.

Lung injury limits the success of allogeneic stem cell transplantation (SCT). The overall incidence varies from 30 to 50% and non-infectious causes occur in one-third to one-half of these. We reviewed pulmonary complications in 369 consecutive patients who received a partially T-cell-depleted myeloablative allogeneic hematopoietic SCT at our institution between 1993 and 2003. All patients were treated uniformly with cyclophosphamide followed by total body irradiation. Control subjects were matched on sex, underlying diagnosis, age, type of transplantation and cytomegalovirus (CMV)-serostatus. Sixty-one patients (16.5%) developed pulmonary complications. Twenty-one patients (5.7%) developed infectious pneumonia. Forty patients developed non-infectious complications which were further subclassified as bronchiolitis obliterans (3.5%), bronchiolitis obliterans-organizing pneumonia (0.5%), diffuse alveolar hemorrhage (0.8%), idiopathic pneumonia syndrome (5.5%) or mixed etiology (0.5%). Acute graft-versus-host disease (GVHD) > or =grade II was significantly more common in pulmonary patients than in the controls (36/61 versus 22/61 patients, P=0.02). There was no significant difference in the incidence of chronic GVHD (P=0.09). CMV reactivation was significantly more frequent in patients with lung injury (P=0.02). Median survival was 41 weeks for the pulmonary patients and 350 weeks for the controls (P=0.001). Altogether, the incidence of pulmonary complications is low after T-cell-depleted SCT and is associated with acute GVHD and CMV reactivation.

[Nephrogenic fibrosing dermopathy]. / Nefrogene fibroserende dermopathie.

A 58-year-old man with renal insufficiency, who was being treated by haemodialysis, developed progressive skin lesions. He had thickening and hardening of the skin at the extremities and swelling of the toes and fingers with flexion contractures. His face was not affected. Laboratory evaluation was unremarkable and a skin biopsy [table: see text] showed an increase of collagen and mucin, without an inflammatory infiltrate. These clinical features resemble a recently reported new disorder: nephrogenic fibrosing dermopathy. This disorder manifests as scleromyxedema-like cutaneous skin lesions without associated paraproteinemia, occurring in the setting of renal disease. The histopathologic features of nephrogenic fibrosing dermopathy, i.e. thickened collagen and mucin deposition, are unique. The incidence, prevalence and cause of the disease are unknown and there is currently no effective treatment. The Centers for Disease Control and Prevention (CDC) in the USA are calling on physicians who have encountered patients suffering from this type of lesions to contact the CDC for an intended control study.

[Five patients with metastasized basal-cell carcinoma]. / Vijf patiënten met een gemetastaseerd basaalcelcarcinoom.

During the period 1986-2001, a metastasised basal-cell carcinoma of the head was diagnosed in five patients (a 35-year-old woman and four men aged 40, 44, 54 and 54 years) at the Utrecht University Medical Centre, the Netherlands. Metastases were found in the cervical lymph nodes, the skeleton, the parotid region and the lungs. The tumours were all of the morphoeic or 'wispy' type. The treatment consisted of excision and sometimes radiotherapy. Two patients died, one of whom of a cause unrelated to the tumour, two patients were free of symptoms 24 months after the last treatment and one patient was still being treated with radiotherapy. It is often assumed that basal-cell carcinomas do not metastasised, but a frequency of 0.0028-0.55% is reported in the literature. An important risk factor is the size of the tumour. Surgical excision or Mohs' micrographic surgery is the preferred method of treatment because this allows histological inspection of the excised margins. Due to the low incidence, there are no clear therapeutic guidelines for the treatment of patients with metastasised basal-cell carcinoma.

Differential diagnosis of skin lesions after allogeneic haematopoietic stem cell transplantation.

Allogeneic haematopoietic stem cell transplantation (i.e. bone marrow or peripheral blood stem cell transplantation) is a common procedure in the treatment of various haematological disorders such as aplastic anaemia, (pre)leukaemias, some malignant lymphomas, multiple myeloma and immunodeficiency states. Many of these patients develop erythematous skin lesions following transplantation. Although graft- versus-host disease is the major differential diagnosis in these situations, many other causes of erythema are encountered. The large number of transplant patients means that more and more pathologists are confronted with the challenging problem of making a correct diagnosis in these situations. In this review article we therefore describe the different causes of erythema and their differential diagnoses. In most cases the clinical presentation is related to the microscopical features. Besides acute and chronic graft-versus-host disease, we discuss the (common) drug reactions and non-specific features such as Sweet's syndrome, erythema nodosum and eosinophilic folliculitis. In addition, we deal with the recurrence of original diseases and infections. With this knowledge every pathologist should feel comfortable when looking at skin biopsies of patients after haematological stem cell transplantation.

[Netherton's syndrome in two sisters]. / Het syndroom van Netherton bij twee zusjes.

Two girls, sisters aged 4.5 years and 6 months, had experienced serious erythrodermia since birth, with scarcely any hair growth and they exhibited poor growth despite a hypoallergenic diet. On the basis of the dermatological condition ichthyosis linearis circumflexa and microscopic examination of a hair shaft in which trichorrhexis invaginata (bamboo hair) was observed, the diagnosis of Netherton's syndrome was established. In this autosomal recessive hereditary condition there is a defective production or maintenance of the stratum corneum. Apart from the skin and the hair abnormalities there is often an atopic constitution as well. The treatment consists of skin ointments and a high-energy diet due to the loss of protein via the skin.

Towards quality assurance of the sentinel node procedure in malignant melanoma patients: a single institution evaluation and a European survey.

The status of the regional lymph node (LN) is a critical component in staging patients with malignant melanoma. Biopsy of the first tumour-draining LN (sentinel node, SN) may replace routine elective LN dissection. However, until now, the applied methods have differed widely. Therefore, the aim of this study was to formulate recommendations for the pre-operative identification and intra-operative retrieval of the SN. We present the results of an independent survey of the clinical practice of the SN procedure via a postal questionnaire among 136 nuclear physicians in different institutes throughout 16 European countries. Moreover, the results of the SN procedure in our institution in an open prospective intervention trial in 80 patients with malignant melanoma without palpable LNs are also presented. In our protocol, on average, 6 h prior to surgery, 80 MBq technetium-99m nanocolloid was injected intracutaneously around the circumference of the diagnostic excision scar of the primary melanoma. No additional blue dye procedure was used to judge the accuracy of the radioguided SN procedure on its own. For successful identification of the radiolabelled SN, dynamic and static images were performed and the skin projection of the detected SN was marked with a cobalt-57 source. For intra-operative mapping a hand-held gamma probe was used. Forty of the 83 respondents of the European-wide questionnaire (48%) performed the SN procedure. Although many different regimens are used, the following recommendations could be deduced for the SN procedure in patients with malignant melanoma and non-palpable LNs: (1) local, intradermal injection of 40 MBq 99mTc-nanocolloid around the diagnostic excision scar of the primary melanoma; (2) two-phase LS: dynamic imaging (20 frames of 60 s, 128x128 matrix, LEAP collimator) followed by static images 1-2 h later (180 s per record); (3) intra-operative retrieval of the SN with a gamma probe; (4) histopathological examination of the SN on serial sections. In our trial, surgical retrieval of the SN was successful in 95% of the cases. Dynamic lymphoscintigraphy (LS) contributed to the SN procedure by showing anatomically unpredictable lymph flow to extra-regional SNs (10% of the patients in this study) and multiple SNs. Of the 77 retrieved SNs, 13 contained metastatic disease (17%). Consequently, these patients underwent a formal LN dissection of the affected basin. In conclusion, the SN concept is a rational approach to select patients who could, theoretically, benefit from early LN dissection of the affected basin. Standardisation of the SN procedure will improve the results of this approach, and could be useful for quality control and for making comparisons with other countries in coming years.