Lymphedematous HIV-associated Kaposi's sarcoma

BACKGROUND: Advanced Kaposi's sarcoma is frequently associated with chronic lymphedema (cLO). The histopathological features of lymphedematous HIV-associated KS (KS) are poorly documented and the co-existence of fibroma-like nodules in lymphedematous KS is under-recognized. The aims of this study were to assess the clinicopathological spectrum and diagnostic difficulties associated with lymphedematous KS and to highlight the clinicopathological profile of fibroma-like nodules. In addition, the pathogenesis of fibroma-like nodules and cLO is revisited. MATERIALS AND METHODS: Prospective 17-month clinicopathological study of all biopsies from patients with lymphedematous KS. RESULTS: Seventy-four biopsies, the majority from the lower limbs, from 41 patients were evaluated. Nineteen, 14, five and three patients had one, two, three or four biopsies each, respectively. In 14 biopsies, there was poor clinicopathological correlation of KS stage. Exclusive lesional KS (patch, plaque, nodule or lymphangioma-like) was identified in 29 biopsies; 23 and eight biopsies demonstrated KS or fibroma-like morphology and the adjacent dermis demonstrated cLO. There was variable intratumoral and peritumoral venous compression and lymphatic dilatation. Fourteen biopsies demonstrated cLO exclusively. Smaller fibroma-like nodules lacked KS spindle cells, whereas >5 mm nodules demonstrated focal KS spindle cell proliferation and aggregation on extensive sectioning. The subcutis of 42 biopsies demonstrated variable fibrosis, hemosiderin deposits, lymphocytes, plasma cells, KS, interstitial granular material and pools of lymph fluid. Subcutaneous abscesses were identified in six biopsies. All biopsies had variable epidermal features of cLO. CONCLUSIONS: cLO influences clinicopathological correlation of KS stage and may also mask the presence of KS and the co-existence of subcutaneous abscesses. Smaller fibroma-like nodules are hypothesized to be a manifestation of cLO that have the potential to acquire the characteristics of KS. Lymphatic and venous obstruction, protein-rich interstitial fluid, tissue hemosiderin and subcutaneous infection are hypothesized to play a combined role in the evolution and perpetuation of cLO.     

A Report of 10 Individuals with Weathering Nodules and Review of the Literature

Weathering nodules are a benign skin condition that usually present as papules on the helices of patients with significant prior sun exposure. They are easily recognized clinically and blanch upon application of pressure to the adjacent helical rim: a positive blanch sign. We describe the clinical presentation of weathering nodules in 10 patients, nine men and one woman, aging from 38 to 70 (median 59), and their associated risk factors. Eight patients had a history of actinic keratosis, three had a history of nonmelanoma skin cancer, and all patients had increased Sun exposure through outdoor activities. Weathering nodules are rarely mentioned in the literature and may be confused with other cutaneous disorders. Therefore, it is paramount for clinicians to become familiar with weathering nodules and include them in the differential diagnosis of ear nodules. Appropriate diagnosis will help avoid unnecessary biopsies while reassuring the patient that the lesions are benign.     

Necrobiotic granulomas localised to the penis: a possible variant of subcutaneous granuloma annulare

Three young men developed multiple firm asymptomatic nodules confined to the shaft of the penis. Skin biopsies from all 3 cases showed prominent necrobiotic granulomas indistinguishable from granuloma annulare. One of the patients had repeated recurrent nodules confined to the penis which were removed surgically over a period of 20 months. This unusual, but benign, presentation of granuloma annulare has to be distinguished from other granulomatous and malignant processes which may present as penile nodules.     

Follicular occurrence of prurigo nodularis

Thirty-eight biopsies of prurigo lesions (22 large, mature nodules and 16 smaller, intermediately developed nodules) were obtained from 17 patients with idiopathic prurigo nodularis Hyde who had not been bitten by insects, nor had other underlying diseases. Each biopsy was serially sectioned to reveal its histological structure. In 88% (14/16) of the smaller nodules and in 55% (12/22) of the large ones, a hair follicle was observed in the center of the prurigo lesion. The follicular epidermis and the adjacent surface epidermis showed various degrees of acanthosis and spongiosis. The dermal infiltrates extended from the superficial dermis into the middle dermis in a triangular fashion around the involved hair follicle. It is suggested that idiopathic prurigo nodularis most frequently occurs in connection with a hair follicle.     

Panniculitis in association with apomorphine infusion

This study was undertaken to ascertain the histopathology and aetiology of cutaneous nodules observed in Parkinson's patients treated with continuous subcutaneous apomorphine. Ten patients were recruited, answered questionnaires, and underwent skin biopsies and full blood count, and nine were patch tested to apomorphine and its preservative. Six had serum IgE levels measured. A florid panniculitis was seen in all biopsies; five were predominantly eosinophilic, three lymphocytic and two neutrophilic; in seven cases the panniculitis was mixed and in three it was septal. Patch testing was universally negative and the IgE levels were normal.     

Cutaneous manifestations of Takayasu arteritis. A retrospective study of 80 cases

The aim of this retrospective study is to delineate in Europe the frequency and type of cutaneous manifestations associated with Takayasu arteritis (TA). Eighty patients with TA were analyzed. Symptoms suggestive of Raynaud's syndrome were noted in 11 patients (14%) and could be directly related to large vessel involvement. Other skin lesions were observed in 10 patients (12.5%). Five had acute tender erythematous nodules on the legs with a clinical diagnosis of erythema nodosum; 2 had subacute ulcerated nodules of the legs; 1 had pyoderma-gangrenosum-like ulcerations of the four limbs which resulted from the breakdown of subcutaneous nodules; 1 had lupus-like malar flush, and the last one had urticarial lesions with livedo reticularis. Skin samples were obtained from 4 patients. Three of them agreed that reiterated biopsies be done on recurrent lesions. A granulomatous vasculitis was observed in 2 cases involving hypodermal arterioles in one case and veins in the other. The other pathological findings were septal and lobular panniculitis which can be associated with granulomatous vasculitis. Different histological findings on reiterated biopsies were frequently found. The absence of any other etiology and chronological arguments suggested a relationship between these skin lesions and TA. Tuberculosis was probable in 1 case but apparently was not related to the skin lesions.     

Rhabdomyosarcoma presenting as a cutaneous nodule

Two patients with rhabdomyosarcoma presented clinically with dermal nodules on the face. The first patient was a 12-month-old girl with a 2 X 3-cm-diameter red nodule on her left cheek that had begun as a small red papule when she was 3 months old and had progressively increased in size. The second patient was a 19-year-old girl who presented with an enlarging mass on her right cheek that had begun as a pea-sized nodule three months previously. In both cases, biopsies revealed a rhabdomyosarcoma in the underlying soft tissue with extension into the overlying dermis. Rhabdomyosarcoma presenting as a dermal nodule is rare. It usually presents as an asymptomatic papule without distinctive clinical features and therefore may result in delayed diagnosis unless a biopsy is performed.     

Histopathology of necrobiotic xanthogranuloma with paraproteinemia

Fifty-three cutaneous biopsies from 22 patients with necrobiotic xanthogranuloma (NXG) were reviewed. One or more biopsies from each patient displayed a typical pattern of palisading histiocytic xanthogranuloma with bands of hyaline necrobiosis. Multiple foam cells and Touton giant cells were present, and atypical, bizarre-appearing foreign body giant cells were characteristic. Cholesterol clefts were found in 18 specimens, and lymphoid nodules were found in 24, 8 of which had germinal center formation. Twenty-one specimens contained foci of plasma cells, and these were located perivascularly, at the periphery of lymphoid nodules, and, as infiltrates, between dermal collagen bundles. Unique features were xanthogranulomatous panniculitis, often appearing as Touton cell panniculitis, and a rare but distinctive palisading cholesterol cleft granuloma. The cytoplasm of giant cells and histiocytes contained PAS-positive, diastase-resistant polysaccharide. Examination of 3 cases by electron microscopy revealed dendritic cells in the epidermis and vacuolated dermal histiocytes. S-100 antibody was negative in 3 cases. Leukocyte monoclonal antibody studies in 6 patients showed predominantly T-helper lymphocytes within the granuloma. NXG is a T-helper cell, non-X histiocytic granuloma with a distinctive histopathology.     

Histopathology of panniculitis--aspects of biopsy techniques and difficulties in diagnosis

Background: Clinical and histologic diagnosis of panniculitis may be difficult. The patients usually present with erythematous subcutaneous nodules with or without additional symptoms. If a skin biopsy does not include enough subcutaneous fat, histopathologic assessment is limited and the correct diagnosis may be delayed and require further sampling. Patients and Methods: To illustrate the difficulties in the diagnosis of panniculitis, we performed a retrospective examination of four patients with different forms of panniculitis. Results: In two patients with subcutaneous panniculitis‐like T cell lymphoma and lupus panniculitis, the correct diagnosis could only be ascertained after a delay of several months because repeated biopsies had to be obtained throughout the course of disease. In two further patients with cold panniculitis and pancreatic panniculitis, clinicians did not even suspect an inflammatory process in the subcutaneous tissue. The correct diagnosis was made with a deep punch biopsy that included subcutaneous fat. Conclusions: On the one hand, these examples demonstrate the importance of sampling subcutaneous tissue when obtaining routine punch biopsies. On the other hand, in cases where the diagnosis is uncertain, it is necessary to perform large and deep incisional biopsies.     

Angiolipoma in brothers

Two brothers with angiolipoma are presented. The patients, 33- and 37-year-old men, have multiple subcutaneous nodules on the trunk and extremities. Skin biopsies have confirmed the diagnosis of angiolipoma.     

Chronic acquired dyskeratotic papulosis of the face

A Vietnam veteran who had been exposed to Agent Orange presented with a 28-year history of a papulonodular disease mainly affecting the face. Over those years, pin-sized papules had developed into dark nodules. Multiple biopsies of both fresh and mature papulonodules had been performed for routine histopathology and electron microscopy. Results had been positive for spongiosis with exocytosis, acanthosis, dyskeratotic keratinocytes, and marked incontinence of pigment, which together are suggestive of a previously unreported clinical entity that we term chronic acquired dyskeratotic papulosis.     

单发性成人黄色肉芽肿2例

报告2例单发性成人黄色肉芽肿。两例患者均在面部出现黄红色结节,皮损组织病理示真皮层泡沫样组织细胞浸润,CD68(+),S-100(-)。手术切除皮损后未见复发。     

Disseminated Mycobacterium marinum infection with extensive cutaneous eruption and bacteremia in an immunocompromised patient

Mycobacterium marinum can cause fish tank granuloma (or swimming pool or aquarium granuloma) in immunocompetent patients. Dissemination of Mycobacterium marinum-infection is a rare condition which occurs mainly in immunocompromised patients and can be life-threatening. We report the case of an 87-year-old woman who was treated with oral corticosteroids for polymyalgia rheumatica for many years and developed erythema nodosum-like lesions on the right forearm and arthritis of the right wrist. By increasing the steroid dosage and adding methotrexate only short-term remission was achieved. Seven months later painful erythematous nodules occurred on all extremities which became necrotic, ulcerative and suppurative. Ziehl-Neelsen staining revealed acid-fast bacilli and Mycobacterium marinum was cultured from skin biopsies, blood, and urine. The critically ill patient was treated with clarithromycin and ethambutol resulting in a dramatic improvement of the general condition. After four months, doxycycline had to be added because of new skin lesions. This case illustrates the impact of Mycobacterium marinum infection in immunocompromised patients.     

Immunofluorescence Studies in Scabies

Direct and indirect immunofluorescence investigations performed for 11 patients with scabies. All patients had punch biopsies taken from (1) a lesion containing Sarcoptes scabiei, (2) an inflammatory papule which did not contain a mite, and (3) normal skin. In four patients IgE deposits were found in the vessel walls of the upper dermis both in biopsies containing mits and biopsies of inflammatory papules with no mites. No IgE deposits were found in biopsies of normal skin from the same patients. Two patients had IgM and/or C3 deposits along the basal membrane in biopsies containing mites and one of them also had C3 in this area in the biopsy from a papule with no mite, as well as normal skin.     

A Case of Eccrine Poromatosis in a Patient with a History of Chemotherapy Due to Stomach Cancer

An eccrine poroma (EP) is a benign adnexal tumor that typically presents as a single lesion. Eccrine poromatosis defined as multiple EPs is an uncommon presentation. A 54-year-old male had undergone operation for stomach cancer 10 years prior and insisted that he had also taken adjuvant chemotherapy. The patient presented with six reddish papules and nodules scattered on the trunk and extremities for 3 years. The histopathologic findings from all six lesions were consistent with EP. No local recurrence was observed after complete removal through punch biopsies. We report a rare case of eccrine poromatosis in the patient with gastric cancer, suspected of being caused by chemotherapy.     

Lipomatous metaplasia after severe and chronic cutaneous inflammation

A 69-year-old woman with a history of acute generalized exanthematic pustulosis (AGEP) caused by metamizole is described. Furthermore, she had suffered from an untreated psoriasis since the age of 20. After an adequate therapy of both psoriasis and AGEP, yellow-brownish, static, coalescing, lucent nodules on the thighs and upper arms became apparent. Histology of skin biopsies revealed a prominent band of mature adipocytes in the dermis. We diagnosed a lipomatous metaplasia of the dermis and hypothesize that this metaplasia occurred as a consequence of the severe and chronic inflammation of the skin.     

Iatrogenic Mycobacterium abscessus infection: histopathology of 71 patients

The histopathology is described of 190 skin biopsies from 71 patients who presented during an epidemic of nodules and abscesses in the periumbilical region and buttocks, secondary to the application of xylocaine injections. Positive cultures for Mycobacterium abscessus were obtained from the specimens and from the xylocaine solution. The severe inflammatory lesions involved the dermis and the hypodermis and had three main histopathological patterns: (i) granulomatous nodular or diffuse inflammation with mixed granulomas in 57 (80%) of the cases; (ii) prevailing abscesses with mild granulomatous reaction in 28 (15%) of the biopsies; and (iii) deep dermal and subcutaneous granulomatous inflammation with no neutrophil component in three (4%) of cases. Bacilli were detected in 51 (27%) of the specimens, frequently forming small clumps at the centre of clear spaces or vacuoles and which were lipid-like structures in 156 (82%) of the abscesses or granulomas. This series represents one of the largest reported outbreaks caused by atypical mycobacteria and in which the source of infection was confirmed. The results emphasize the essential role of skin biopsies in determining the histopathological substrate, in helping to detect the atypical mycobacterial origin and in encouraging the practice of cultures for the identification of micro-organisms.     

Juvenile case of multiple morphea profunda resulting in joint contracture that was successfully treated with cyclosporin A: A case report and review of the published works

Morphea profunda refers to inflammatory and sclerotic lesions that start primarily from the deep dermis, subcutaneous fat and fascia. Its pediatric case published work is limited. Here, we report the case of an 8‐year‐old girl with a 5‐year history of multiple subcutaneous nodules on her extremities and a right wrist joint contracture who had been previously diagnosed with juvenile idiopathic arthritis and treated with salazosulfapyridine, low‐dose prednisolone (PSL) and methotrexate. We performed biopsies of two subcutaneous nodules, which revealed the typical morphology of morphea profunda. She was administrated a tapered course of oral PSL then cyclosporin A (CyA) for 20 weeks which completely resolved her joint contracture and subcutaneous nodules. We reviewed 11 previously reported cases of morphea profunda and found that some include circumscribed/linear morphea that develop into subcutaneous tissues, indicating that “classical” morphea profunda arising within the deep tissues has rarely been reported. Our report is the first to demonstrate the efficacy of CyA for treatment of morphea profunda, and the possibility of CyA as a treatment option to reduce oral steroid doses in juvenile cases.     

The role of liver biopsies in psoriatic patients receiving long-term methotrexate treatment. Improvement in liver abnormalities after cessation of treatment

Liver biopsy specimens from 168 patients who underwent a total of 364 biopsies were examined. Of 83 patients receiving biopsies before methotrexate treatment, 14 had one or more risk factors predictive of liver abnormality but they had normal pretreatment biopsy specimens. Among 17 patients with abnormal biopsy specimens before methotrexate treatment, only 1 had an identifiable risk factor and 5 had abnormal results of liver function tests. The probability of a normal biopsy specimen after methotrexate treatment dropped below 50% at a cumulative methotrexate dose of 3115 mg for the 31 patients with biopsy specimens from before and after methotrexate treatment and 5776 mg for those who had biopsies only after methotrexate treatment; this difference was statistically significant and is thought to be related to the fact that the patients who had biopsies before and after methotrexate treatment had received most of their medication by the parenteral rather than the oral route. A significant association existed between biopsy grade after methotrexate treatment and obesity. Other risk factors were not correlated with biopsy grade. Blood chemistry tests were not predictive of histopathologic findings. Eight of 11 patients with fibrosis or cirrhosis showed meaningful improvement in liver histologic findings after methotrexate treatment had been withdrawn for 6 months or more; none had progression of abnormalities.     

Aluminium allergy in patients hyposensitized with aluminium-precipitated antigen extracts

During hyposensitization therapy with aluminium-precipitated antigen solutions, a small % of patients develop persistent subcutaneous nodules at the injection site; the existence of delayed sensitivity to aluminium has been implicated in the pathogenesis of these nodules. We studied the prevalence of aluminium sensitivity (using patch, prick and intradermal tests) and common contact allergens (TRUE Test™) in 20 healthy subjects, and in 40 patients treated with aluminium-containing extracts, 20 of whom had persistent subcutaneous nodules that remained for more than 2 months, the other half having no nodular reactions or nodules that remained for less than 2 months. Aluminium sensitivity was found only in those patients of the treated group who had persistent nodular reactions, 4 cases of positivity to an aluminium chloride patch test being found. All 4 cases were women, nodules remained for more than 6 months, and intracutaneous tests were negative. 3 of them also had contact sensitivity to nickel. In 2 cases, nodules were removed for histological and histochemical examination, showing non-specific inflammatory granulomas, and aluminium crystals being found in only 1 case. It is concluded that delayed sensitivity to aluminium appears to be implicated in the pathogenesis of persistent nodular reactions, but sensitivity to aluminium was not found in patients treated with aluminium-precipitated extracts without persistent nodular reactions.