[Grouped blisters and mediastinal tumor in a 50-year-old woman].
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Biomedical subjects
Publications and source records attributed to Christian Rose.
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The Georg Klingmüller Clinic is part of Mbarara University Teaching Hospital (MUST) in south-western Uganda. Established in 1999, this clinic cares for about 6,000 dermatological patients per year. We review the disease spectrum, available diagnostic tools, and therapeutic options in this clinic. In addition, the importance of dermatology for the fight against HIV/AIDS is discussed with respect to the socio-economic background of a sub-Saharan country. Co-operation with dermatology departments in Germany may not only help to diagnose skin diseases in Mbarara, but may also be valuable for the visiting dermatologist to manage the increasing numbers of patients with dark skin at home.
The occurrence of a granulomatous inflammation following herpes zoster infection is uncommon and its pathogenesis is unclear. A 74-year-old male patient developed multiple red-brown papules following a secondary generalized infection with varicella zoster virus in the same area. The patient was suffering from a chronic lymphocytic B-cell leukaemia. Histopathologically, granulomatous dermatitis with multinucleate giant cells was found. Varicella zoster virus DNA was identified by polymerase chain reaction in the tissue. After a renewed antiviral therapy, skin lesions disappeared completely.
UNLABELLED: We report a 7-year-old boy with the typical features of encephalocraniocutaneous lipomatosis (ECCL) including unilateral skin, eye, bone, and asymptomatic central nervous system involvement. The presenting sign were numerous ipsilateral odontomas which have been described in only two previous patients so far. We reviewed all 32 cases reported to date and focus on the dermatological hallmark of the condition, namely a fatty tissue naevus of the scalp for which the term naevus psiloliparus was recently coined. Sporadic occurrence, patchy type of organ involvement and roughly balanced sex ratio in ECCL are compatible with the concept of a lethal autosomal mutation only surviving in a mosaic state. CONCLUSION: In children presenting with a congenital smooth hairless lesion on the scalp, the diagnosis of naevus psiloliparus should be considered and other signs of encephalocraniocutaneous lipomatosis should be looked for.
The purpose of this study was to assess the long-term outcome of the MacIntosh lateral-substitution over-the-top anterior cruciate ligament (ACL) reconstruction in 82 patients (84 knees) at an average follow-up of 9.8 years. In this retrospective cohort study patients were evaluated with subjective questionnaires and by clinical and radiographic examination. Using the Lysholm score, 17 knees were rated excellent, 35 good, 19 fair, and 13 poor. The pivot shift test was negative in 74 patients. Altogether, 30 knee radiographs were evaluated. The mean Hospital for Special Surgery ACL radiographic score was 20.9. There was a non-significant association between the radiographic score and the Lysholm score and between a worsening radiographic score and increasing time from injury. The MacIntosh lateral-substitution over-the-top ACL reconstruction shows results comparable to those of previously reported long-term studies. Although endoscopically assisted intraarticular reconstructions with faster rehabilitation protocols have now become popular, this procedure may be considered in patients needing an intra- and extraarticular reconstruction in whom cosmesis is not an issue.
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Morbus Köhlmeier-Degos is a rare systemic disease characterized by pathognomonic cutaneous lesions with typical histology. We report the case of a 22-year-old woman with a benign course of this disease and discuss therapeutical options. The patient presented with whitish papules on neck, trunk and extremities that slowly developed within seven months. Examination of the skin revealed about 20 papules of 2-5 mm size. The lesions were partly skin-colored, partly with an erythematous rim and showed a central porcelain-like atrophy. Histology showed an interface-dermatitis and a wedge-shaped mucin deposition with sclerosis of the upper reticular dermis. Organ manifestation was absent. The patient was started on a daily therapy of 300 mg acetyl salicylic acid. Over the course of 24 months single new lesions appeared at a reduced frequency. Apart from the malignant form of Morbus Köhlmeier-Degos there exists a benign course which may be successfully controlled with anti-platelet therapy. We speculate that the lack of vessel occlusion in the histology could be a hallmark of a benign form of this rare disease.
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Data on clinical features observed in patients with Waldenstrom's macroglobulinemia (WM) during follow-up remain limited. Therefore, we evaluated 860 follow-up procedures in 101 patients. Median age was 66 years and 5-year overall survival 72%, with a median follow-up of 36 months in surviving patients. Sixteen patients presented at diagnosis with two or three cytopenias lasting for at least 3 months (multiple cytopenias [MC]), and MC improved after treatment in eight patients, 4 to 18 months later. MC was observed during at least 6 consecutive months in 23 other patients, 2 to 73 months (median, 24) after diagnosis. MC occurred off-therapy in 12 patients, and on-therapy in 11. Regression occurred in three of the former patients, and in seven of the latter (6 to 24 months after completion of treatment; median, 7). Finally, the 4-year estimated cause-specific cumulative incidence was 40% in the 101 patients. A second malignancy was observed in 11 patients, histological transformation in three, and rapid rise of M-component in only six patients. In conclusion, the present analysis pointed out a high incidence of long lasting MC during the evolution of WM, and a low frequency of rapid rise of M component.
We report an unusual case of congenital melanocytic nevus presenting in a 19-year-old African woman as widespread papules and variably sized nodules and tumors affecting the entire body, including the palms, soles, and oral mucous membrane. Histopathologic examination of 3 representative skin lesions showed mainly dermal aggregations of round to oval, focally pigmented, monomorphous melanocytes, arranged in nodular and plexiform patterns. Scattered areas with spindle-shaped dendritic melanocytes surrounded by fibrosis were also noted in the center of the lesions. The clinical and histopathologic findings were similar to those in 2 other previously reported cases, except that in 1 of the earlier cases the skin nodules were composed of spindle-shaped cells, suggesting a type of blue nevus. The findings in our case indicate a broader spectrum of morphologic features in this condition, with dermal aggregations of melanocytes showing congenital features, representing a common histopathologic denominator. Based on this observation, we suggest the term "widespread congenital dermal nevus with large nodules" to be the most appropriate for this rare, but distinctive, type of congenital nevus.
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Pemphigoid gestationis (PG) is an autoimmune pregnancy-associated subepidermal blistering disease. It usually affects skin and, rarely, mucous membranes. In the vast majority of patients with PG, the autoimmune response is directed to the membrane-proximal NC16A domain of the 180-kd bullous pemphigoid (BP) antigen (BP180) and is mediated by IgG1 and IgG3 autoantibodies. We report the case of a patient with PG associated with extensive lesions on oral mucous membranes. Immunoblotting studies demonstrated the presence of circulating IgA autoantibodies in the patient's serum that were exclusively directed to a 49 amino acid stretch on the C-terminal portion of the BP180 ectodomain located 800 amino acids downstream from NC16A. This C-terminal stretch of BP180 has previously been demonstrated to localize to the lamina lucida/lamina densa interface and to be recognized by IgG and IgA antibodies in a subgroup of patients with cicatricial pemphigoid as well as by IgG autoantibodies in some BP sera. Our patient's lesions healed without scarring within 6 weeks after delivery of a healthy child. The findings in this patient extend the clinical and immunopathologic spectrum of PG.