[Aquagenic urticaria. A case report]. / Aquagene Urtikaria. Ein Fallbericht.

A 38-year-old woman presented with recurrent episodes of urticaria after contact with water affecting the upper part of the body. We confirmed the diagnosis of aquagenic urticaria. Aquagenic urticaria is a rare form of contact urticaria with small wheals generally affecting the upper part of the body. It has to be distinguished from aquagenic pruritus. The pathogenetic mechanisms are not known in detail. Treatment with antihistamines, ultraviolet therapy or application of protective hydrophobic barrier creams could be effective.

Algorithm for detection of small-bowel metastasis in malignant melanoma of the skin.

BACKGROUND AND STUDY AIM: The aim of this study was to develop an algorithm to detect small-bowel metastasis (SBM) of melanoma by sequential laboratory parameters and pan-intestinal endoscopy (PIE) including video capsule endoscopy (VCE). PATIENTS AND METHODS: A total of 390 melanoma patients (AJCC stage I/II/III/IV, 140/80/121/49) were screened for signs of intestinal blood loss (fecal occult blood test [FOBT] or overt bleeding) in an open, multicenter, prospective study, and those who were positive underwent PIE. Independent of the presence of intestinal bleeding, all stage IV patients were offered PIE. Follow-up was obtained in 357 patients (91.5 %) for a median of 16 months. We undertook to identify possible associations between SBM and clinical and laboratory data. Survival data were analyzed with regard to clinical and laboratory data and small-bowel findings. RESULTS: Intestinal blood loss was suspected in 49 of 390 patients (12.6 %), 38 of whom (77.6 %) agreed to undergo endoscopy. In 10 patients, SBM was detected by VCE (intention-to-diagnose, 20.4 %; AJCC III, n = 2; AJCC IV, n = 8). The SBM was resected in five patients. Total detection rates of SBM were 14 of 49 patients in stage IV (28.6 %, intention-to-diagnose), 2 of 121 in stage III (1.7 %), and 0 in stage I/II. In FOBT-positive patients, SBM detection rates were 72.7 %, 14.3 %, and 0 % in tumor stages IV, III, and I/II, respectively. Positive FOBT proved to be an independent negative prognostic factor for total survival in stage III and IV melanoma. CONCLUSIONS: SBMs are frequent in advanced melanoma. In stage III patients, screening for intestinal blood loss by PIE may help to identify SBMs. In stage IV, indication for PIE should depend on the individual consequences of detecting SBM, but not on bleeding symptoms alone.

Hidradenitis suppurativa/acne inversa: bilocated epithelial hyperplasia with very different sequelae.

BACKGROUND: Hidradenitis suppurativa/acne inversa is a chronic, inflammatory, scarring disease in the terminal hair follicle and apocrine gland-bearing areas (skin folds). There is considerable histological evidence that perifolliculitis and follicular hyperkeratosis precede the rupture of the follicle. The timing of the epithelial hyperplasia at the infundibula of inflamed terminal follicles has not yet been clarified. OBJECTIVES: To clarify the early histopathological life of lesions ('chronology') in hidradenitis suppurativa/acne inversa, focusing on the terminal follicle structure and its surrounding tissue (hyperkeratosis, hyperplasia of follicular epithelium, perifolliculitis and rupture). METHODS: In total, 485 operative specimens obtained from 128 patients with diagnosed hidradenitis suppurativa/acne inversa (all surgically treated by wide excision) were examined histologically. Two to five histological preparations (total 485) per operation area (total 196) were prepared by multiple slicing. RESULTS: Hidradenitis suppurativa/acne inversa showed a heterogeneous histological pattern: hyperkeratosis of the terminal follicles (89%), hyperplasia of follicular epithelium (80%), pronounced perifolliculitis (68%) and follicle rupture (24%). Perifolliculitis, follicular hyperkeratosis and hyperplasia occurred prior to the rupture of the follicle. Other histological criteria were: subepidermal cellular inflammatory infiltrate (82%), epidermal psoriasiform hyperplasia (56%), pronounced acute dermal inflammation (28%), pronounced chronic dermal inflammation (49%), and involvement of apocrine glands (52%) and subcutis (31%). CONCLUSIONS: Infundibular hyperkeratosis, hyperplasia of the follicular epithelium and perifolliculitis are major histopathological characteristics of hidradenitis suppurativa/acne inversa. These apparently precede rupture of the follicle. In particular, hyperplasia of the follicular epithelium probably marks the beginning of sinus formation, which usually spreads horizontally. Psoriasiform hyperplasia of the interfollicular epidermis with subepidermal inflammatory infiltrate might be interpreted as an inflammation-driven process basically identical to that which is evident at the terminal follicle. However, it does not lead to harmful and progressive sequelae like those (rupture, sinus tracts) seen at the terminal follicles.

[37-year old patient with fever, diarrhea and lymphadenopathy]. / 37-jähriger Patient mit Fieber, Durchfall und Lymphknotenschwellung.

A 37-year-old homosexual man was admitted because of oropharyngeal pain, fever, diarrhea, loss of weight and lymphadenopathy since one week. Acute retroviral syndrome (ARS) in primary HIV type 1 infection was diagnosed, associated with Giardia lamblia infection. Antiinfective and combined antiretroviral treatment was established, and the general condition of the patient rapidly improved. The presented report demonstrates that in case of acute HIV-infection with diarrhea other infections should be considered, particularly with regard to enteropathogens like Giardia lamblia.

[Sclerodermiform chronic graft-versus-host disease after allogenic peripheral blood stem-cell transplantation]. / Sklerodermiforme chronische Graft-versus-Host-Erkrankung nach allogener peripherer Blutstammzelltransplantation.

HISTORY AND CLINICAL FINDINGS: A 46-year-old man presented with suberythrodermia and an acral-accentuated sclerosis, which had been progressing over the past 6 months, with extensive, painful ulcers within the sclerotic areas of the calf. Due to acute myelotic leukemia (AML), an allogenic peripheral blood stem-cell transplantation with subsequent immunosupression with mycophenolatmofetil (MMF) and ciclosporin A had been performed 8 years previously. The patient had discontinued treatment on his own after about 2 years, having suffered a cerebroischemic insult in the meantime. INVESTIGATIONS: Histological examinations revealed sclerodermatous changes. Titres of antinuclear antibodies were unremarkable. Laser-Doppler-flowmetry also indicated an active inflammatory and sclerosing process. FACS analysis of the peripheral blood did not reveal signs of AML recurrence. DIAGNOSIS, THERAPY AND COURSE: The histological pattern in conjunction with the anamnesis indicated a cutaneous chronic graft-versus-host disease (GvHD). No further organ involvement was observed. The MMF therapy which the patient had discontinued was restarted. In addition, PUVA therapy was initiated. These measures and intensive physiotherapeutic exercises in parallel prevented further progression of the sclerosis and secondary mobility limitations. The ulcers healed completely with pentoxifylline and anti-infective treatment. CONCLUSION: After stem-cell transplantation, early diagnosis of GvHD is especially important due to possible irreversible sclerodermatous changes and other organ manifestations. Also for this reason, strict clinical follow-up is especially important with respect to compliance and efficacy of the immunosuppression.

[Symptomatic cardiac metastasis. Isolated visceral manifestation of a cutaneous malignant melanoma]. / Symptomatische Herzmetastase. Solitäre viszerale Manifestation eines kutanen malignen Melanoms.

Symptomatic cardiac melanoma metastases are very rare. A 76-year-old woman was admitted because of dyspnea and intrathoracic pain 8 years after surgery of a superficial spreading melanoma and 4 years after resection of in-transit metastases. MRI and echocardiography disclosed an intracavitary right atrial mass. Histologically a cardiac melanoma metastasis was found. Unspecific cardiac symptoms in a patient with elevated risk for distant metastases of melanoma should be further investigated to discover cardiac metastasis early.

[Primary cutaneous manifestation of Wegener's granulomatosis]. / Primär kutane Manifestation einer Wegener-Granulomatose.

HISTORY: A 57-year-old man was admitted with hemorrhagic papules and necrotising nodules on both elbows and upper legs. Recurrent arthralgia occurred. INVESTIGATIONS: The skin biopsy showed a cutaneous necrotising vasculitis. Positive test results for c-ANCA and proteinase 3 antibodies and a slightly increased WBC and a mild proteinuria were noticeable. DIAGNOSIS, TREATMENT AND COURSE: The diagnosis of an early systemic Wegener's granulomatosis was based on elevated proteinase 3-titres and cutaneous histologic findings as necrotising vasculitis and granulomatous inflammation. Treatment with prednisolone followed by methotrexate resolved the cutaneous symptoms and the arthralgia completely. Three months later the patient developed a progredient methotrexate toxicity caused by a glomerulonephritis. CONCLUSION: Wegener's granulomatosis should be considered if a cutaneous necrotising vasculitis is diagnosed. A cutaneous manifestation could be an early symptom. Methotrexate could be used for treatment of mild courses of Wegener's disease without renal involvement.

[Acquired reactive perforating collagenosis associated with diabetes mellitus and renal insufficiency requiring dialysis]. / Erworbene reaktiv perforierende Kollagenose bei Diabetes mellitus und dialysepflichtiger Niereninsuffizienz.

HISTORY: A 60-year-old man with diabetes mellitus and chronic renal insufficiency needing hemodialysis was admitted with a 3 months history of multiple hyperkeratotic papules on the trunk and extremities partly ulcerated with a keratotic central plug. INVESTIGATIONS: Laboratory tests revealed elevated levels of blood urea nitrogen, creatinine, and HbA (1c). Histopathology showed vertical strands of collagen perforating from the ulcerated lesions. COURSE, DIAGNOSIS AND TREATMENT: The biopsy specimen was consistent with acquired reactive perforating collagenosis. The progression was stopped and secondary wound healing was initiated after two weeks of therapy with allopurinol and PUVA. CONCLUSION: Acquired reactive perforating collagenosis should be considered when ulcera with oystershell-like keratotic plugs are found especially in patients with predisposing diseases like diabetes and renal insufficiency. A good interdisciplinary cooperation between internist and dermatologist is crucial for the early recognition by histopathology and the immediate treatment.

[Chronic lupoid leishmaniasis. A rare differential diagnosis in Germany for erythematous infiltrative facial plaques]. / Chronische lupoide Leishmaniose: In Deutschland eine seltene Differenzialdiagnose erythematös-infiltrativer Plaques im Gesicht.

Lupoid leishmaniasis is a unique form of cutaneous leishmaniasis characterized by unusual clinical features and a chronic relapsing course. Clinically and histologically it is similar to lupus vulgaris, which is thus the most important differential diagnostic consideration. All patients with granulomatous facial lesions coming from endemic areas or with a positive travel history should be suspected of having leishmaniasis. We describe a 59-year-old woman with facial lupoid leishmaniasis.

[Steroid-aggravated rosacea: successful therapy with pimecrolimus]. / Steroidaggravierte Rosazea: Erfolgreiche Therapie mit Pimecrolimus.

Steroid-induced erythema in rosacea is a therapeutic challenge because of its tendency to rebound and the local characteristics of the facial skin. We describe 3 cases of steroid-induced rosacea with the typical history of steroid abuse with tachyphylaxis. Steroids with increasing potency had to be used with increasing frequency in the course of treatment in order to achieve a response. Acute exacerbations followed any attempt at withdrawal. The steroid treatment was discontinued and therapy with pimecrolimus cream 1% twice daily initiated. This brought rapid and marked improvement within a few days. The cases show that the calcineurin antagonist pimecrolimus offers an effective and well-tolerated therapy option in the acute therapy of steroid-aggravated facial dermatoses.

[Gold. Effective therapy for mucosal lesions of pemphigus vulgaris]. / Gold. Effektive Therapie bei Schleimhautläsionen des Pemphigus vulgaris.

High-dose steroids in combination with other immunosuppressant substances, usually azathioprine, are the therapy of choice in pemphigus vulgaris. Complete remission can be difficult to achieve, with mucosal lesions often recalcitrant. We describe two patients in whom gold in the form of sodium aurothiomalate was effective in resolving mucosal lesions. Initially both patients were treated with high-dose corticosteroids, one in combination with mycophenolate mofetil. Their oral lesions proved to be stubborn but responded well to intramuscular gold, which also showed a steroid-sparing effect, so that the dosage of corticosteroids could be reduced to a minimum. Sodium aurothiomalate was continued until both patients were in remission without any side effects.

Concomitant traumas influence prognosis in melanomas of the nail apparatus.

BACKGROUND: In nail apparatus melanomas (NAM), the role of standard melanoma prognostic factors is under discussion. The prognostic influence of traumas to the clinically apparent tumour has not been sufficiently examined. OBJECTIVES: To estimate the influence of traumas against the background of standard prognostic factors on the course of NAM. METHODS: In 33 patients (20 women, 13 men, median age 65.0 years) with NAM (median tumour thickness 3.5 mm), we retrospectively examined a detailed history of trauma to the affected extremity at first presentation. Histological or other standard prognostic factors and follow-up were studied prospectively using a standardized protocol. RESULTS: Of 33 patients, 21 had suffered injury to the clinically apparent melanoma primary between 4 and 72 months prior to definitive surgical treatment (diagnostic biopsy up to 3 months ahead of excision of the melanoma was not considered). Eight of these patients had undergone inadequate therapy procedures. In Kaplan-Meier analysis, trauma to the clinically apparent tumour and tumour thickness were found to be significant prognostic factors for recurrence-free survival, and trauma to the clinically apparent tumour, Clark level and tumour thickness were significant prognosis predictors for overall survival. In Cox's proportional hazards model, trauma to the clinically apparent tumour was found to be a significant and independent risk factor with regard to overall survival (hazard ratio 5.39; P = 0.029). An influence of trauma on the primary pathogenesis of NAM could not be confirmed. Only three patients reported trauma prior to the onset of tumour. CONCLUSIONS: From our data, trauma to the clinically apparent tumour might be a considerable risk factor in NAM. The diagnosis of NAM should be considered in lesions of the nail apparatus and further deterioration of the prognosis due to inadequate therapeutic attempts or injury to the NAM should be avoided.

[The lymphatic system and the skin. Classification, clinical aspects und histology]. / Lymphgefässsystem und Haut Orthologie, Klinik und Pathologie.

Dermatology offers many clinical examples of abnormalities in the production, absorption and transport of lymph. Lymphatic thromboses also occur. Many diseases are not named in accordance with their underlying pathogenesis. Malignant lymphedema must be recognized immediately. The differential diagnosis of regional lymphedema is also part of dermatology. Stemmer sign is a clue to chronic lymphedema. Lymphoedema is first divided into primary and secondary forms, depending on the cause, and then subdivided into stages depending on severity. Complications of chronic lymphedema include recurrent erysipelas, superficial lymphangiectases, verrucous lymphedema (papillomatosis lymphostatica) and angiosarcoma, which is often multifocal. Established diagnostic approaches are available to correctly diagnose lymphatic disease. The treatment of chronic lymphedema centers around reducing the edema which then influences all of the other problems. The physical therapeutic approach to reducing blockage is the best way to achieve these goals.

[A rare low-grade malignant scalp tumor. Atypical fibroxanthoma]. / Seltener niedrig-maligner Tumor der Kopfhaut. Atypisches Fibroxanthom.

A 79-year-old patient presented with an asymptomatic scalp tumor which had grown over the past 9 months. Based on clinical, histological and immunohistochemical staining, we made the diagnosis of an atypical fibroxanthoma (AFX). AFX is a rare tumor, but important in the differential diagnosis of other scalp tumors. The routine histological staining allows one to identify a malignant fibrous histiocytoma, while a spindle-cell malignant melanoma can be excluded with immunohistochemical staining. An anaplastic squamous cell carcinoma cannot always be separated with completed certainty from atypical fibroxanthoma. AFX is a low-grade malignant tumor and most cases behave in a benign fashion. After complete excision, we recommend follow-up for a number of years because of the possibility of local recurrence.

[Long-term locally-recurrent melanoma]. / Langfristig lokal rezidivierendes malignes Melanom.

Malignant melanoma is a highly malignant skin tumor with a widely variable individual course. Currently, approximately 75% of German patients can be cured of malignant melanoma by early diagnosis and excision, while more than 20% die of distant metastases of the melanoma. In contrast, there are single cases of long-term locally recurrent melanomas with neither complete cure nor development of distant metastases. We report on a female patient with a nevus-associated melanoma who was treated in 1968 with X-ray radiation (Dermopan, 100 Gy) with curative intent. In the following 31 years, the patient developed four phases of in-transit and satellite metastases in a zosteriform pattern as well as ilioinguinal lymph node metastasis. Since 1999, the patient has been free of recurrence. Besides the unusual biological features of this case, this report reflects the changes of paradigms in the therapy of melanoma over the last forty years.

Unilateral nevoid telangiectasia syndrome.

We report on a 29-year-old patient with a unilateral nevoid telangiectasia syndrome (UNTS). Based on microcirculation examinations, both functional impairments at the blood vessel level (laser-Doppler flowmetry) and morphologically altered capillaries (capillary microscopy) could be observed. We could demonstrate that microcirculatory examinations are suitable to demonstrate pathophysiological defects at the vascular level, especially since subclinical functional defects can also be recorded. To our knowledge, no such observations have yet been published with regards to UNTS.

[The dermatologic consultation]. / Das dermatologische Konsil.

BACKGROUND: Dermatology and venereology, as a cross-sectional discipline, plays an important role in the interdisciplinary care of inpatients. Nonetheless, only a few studies on dermatological consultations have been performed. We reviewed the dermatological consulting service at the University Hospital of the Martin-Luther-University, Halle, Germany. PATIENTS AND METHODS: All consultations performed in 2001 and 2002 were retrospectively assessed. In addition, the time spent in consulting activities was prospectively recorded over a period of six months (January-June 2003). RESULTS: A total of 2390 consultations were evaluated. Most of the consultations (42.8%) were made on internal medicine wards, followed by pediatrics (11.7%), neurology (9.9%) and cardiothoracic surgery (5.7%). Infectious skin diseases accounted for the most frequent diagnosis at 24.4%. The time lapse between request and performance of the consultation averaged 1.1 days. Patients older than 40 years needed dermatological consultation more frequently than younger patients. The duration of consultation averaged 23 minutes (time to site, patient examination, documentation). Thus, the man-hours required for the consulting service is 33.6 per month. CONCLUSION: The study emphasizes that dermatological consultation services make an important contribution to the care of inpatients. In light of increasing specialization in medicine, the consulting service guarantees interdisciplinary treatment of the patients and is thus an important quality parameter for inpatient care.