Post‐filarial cutaneous aspergillosis

Background  Aspergillus species are widely distributed in nature; however, cutaneous infections due to A. terreus are particularly rare. In this report, we describe a very uncommon case of primary cutaneous aspergillosis presenting as cauliflower‐like skin lesions caused by A. terreus in a patient with preexisting bilateral filarial elephantiasis of the legs. Materials and methods  Histopathological examination of the lesions revealed narrow, septate, branching, acute‐angled hyphae with delicate chitinous walls and multinucleated giant cells in the dermis. Results  The fungal isolate was identified as A. terreus using standard laboratory procedures. Conclusion  Here we report an extremely rare and unusual manifestation of primary cutaneous aspergillosis.     

Primary cutaneous aspergillosis caused by Aspergillus tamarii in a premature infant with extremely low birthweight: A case report with short review

Primary cutaneous aspergillosis is a rare, life‐threatening fungal infection in premature infants. We report a case of primary cutaneous aspergillosis caused by Aspergillus tamarii in an extremely low birthweight infant. The infant was delivered by cesarean section with complications from an intrauterine infection, brain intraventricular hemorrhage, tension pneumothorax and cardiac tamponade. On the 12th day of life, he developed erythematous maceration with erosion on his back. Septate hyphae were detected on two occasions from specimens of the skin lesion. The manifestations of the colony and slide culture showed the characteristics of A. tamarii. The nucleotide sequences of internal transcribed spacer regions of the ribosomal RNA gene, partial sequences of β‐tubulin and calmodulin gene were compatible with those of A. tamarii. Of the known Aspergillus species, Aspergillus fumigatus and Aspergillus flavus have been reported in previous studies as the major causative agents in primary cutaneous aspergillosis, whereas human infection by A. tamarii is rare. We consider that A. tamarii is important as an unusual opportunistic human pathogen among premature infants.     

Cutaneous, pulmonary and bone aspergillosis in a patient presumed immunocompetent presenting subacute cutaneous lupus erythematosus

INTRODUCTION: We report a case of cutaneous, pulmonary and bone aspergillosis successfully treated after many years of progression in a patient presumed immunocompetent presenting subacute cutaneous lupus erythematosus. CASE-REPORT: A 43-year-old man, treated with thalidomide for subacute cutaneous lupus erythematosus, presented chest pain with haemoptysis and dyspnea. A pulmonary nodule was detected but the microbiological investigation was negative. The histological examination showed granuloma with round structures. No cause was found. Three years later, skin lesions appeared on the patient's face concomitantly with a pulmonary relapse. Histopathological examination of these lesions demonstrated septate hyphae. Aspergillus fumigatus was isolated in skin and lung. Disseminated aspergillosis was then diagnosed as spondylodiscitis developed. Treatment with combined voriconazole and caspofungin produced significant and rapid improvement of lesions. DISCUSSION: While aspergillosis is commonly seen in immunocompetent patients, angiotropic dissemination points to cellular immunodepression. Our patient, however, was not presenting immunodepression. We discuss the possible contributory role of thalidomide in dissemination of aspergillosis given that the literature to date contains only one reported case of cutaneous aspergillosis secondary to A. fumigatus in an immunocompetent patient. We would also point out the specific histopathological pattern of this disseminated aspergillosis with both septate hyphae and round structures. Invasive aspergillosis is highly lethal but the chances of recovery are now greater thanks to new antifungal agents.     

Cutaneous aspergillosis and acquired immunodeficiency syndrome

BACKGROUND: Primary cutaneous aspergillosis is an uncommon finding in patients with acquired immunodeficiency syndrome (AIDS); only 13 cases have been reported in the literature. OBSERVATIONS: We describe 11 patients with primary cutaneous aspergillosis and AIDS. There does not seem to be an age, sex, race, or human immunodeficiency virus risk factor predisposition. This is a late manifestation of AIDS; patients typically have low CD4 counts (<0.050 x 10(9)/L [<50/microL]) and other AIDS-defining illnesses. The most frequent presentation is in patients with cytomegalovirus disease and neutropenia caused by ganciclovir therapy. Lesions developed at the site of occlusive dressings for an indwelling intravenous catheter site in 10 patients. Neutrophil counts may be normal at the time of diagnosis. A minor presentation is in the patient without neutropenia as a result of traumatic inoculation. Histological findings and/or culture results are required for diagnosis. Patients develop cutaneous lesions despite prophylactic therapy with fluconazole. Lesions can be treated with excision and lifelong therapy with itraconazole. CONCLUSION: Because of the potential morbidity and mortality of cutaneous aspergillosis, a high level of suspicion and prompt institution of therapy is required.     

Primary cutaneous aspergillosis in a leukemic child

Primary cutaneous aspergillosis is rare. In this report we describe a primary skin infection by Aspergillus flavus in a child with leukemia. The lesions were characterized by erythematous macules and papules associated with pain and itching, followed by a rapid progression to ulcers and central black eschars with a raised erythematous border. A favorable response to topical nystatin therapy was observed. The multiple cutaneous lesions seen in our patient were most likely due to primary inoculation near the site of intravenous infusion with subsequent local lymphatic spread.     

Primary Cutaneous Aspergillosis in an Immunocompetent Patient: Successful Treatment with Oral Voriconazole

Abstract:   We report a case of primary cutaneous aspergillosis in an immunocompetent child that responded rapidly to oral voriconazole therapy. Voriconazole may be considered as a treatment option for pediatric patients with primary cutaneous aspergillosis.     

Nonhealing scalp wound infected with Aspergillus niger in an elderly patient

Cutaneous aspergillosis is a rare infection most often seen in immunocompromised patients. We report a case of primary cutaneous aspergillosis infection in a nonhealing scalp wound of an immunocompetent elderly patient. The patient had a cutaneous malignancy of the scalp treated with surgical excision but complicated by poor wound healing. Fungal culture of the nonhealing wound revealed Aspergillus niger. The nonhealing wound subsequently resolved with retapamulin ointment 1% and ketoconazole gel 2%.     

Cutaneous nocardiosis: report of two cases and review of the literature

Background Cutaneous nocardiosis is an uncommon infectious disease that presents as a primary cutaneous infection or as a disseminated disease. It is often misdiagnosed because of its rarity and nonspecific clinical picture. Methods We report a case of each type. The first case is an immunocompetent patient who was infected by Nocardia while gardening and developed a superficial skin infection – one of the three clinical manifestations of primary cutaneous nocardiosis. The second case is an immunocompromised patient with pulmonary nocardiosis that extended to the skin as part of a disseminated disease. Results The immunocompetent patient with primary cutaneous nocardiosis had the classical features of a superficial skin infection. He had a nodular–pustular lesion on the right arm, which appeared 7 days after gardening with bare hands. Nocardia was identified in a skin culture taken from a pustule, unfortunately not to the species level. Treatment with minocycline for 3 months resulted in full remission of the lesion. The immunocompromised patient with disseminated nocardiosis had high fever, productive cough, hemoptysis, and erythematous nodules and pustules on the extremities. N. brasiliensis was isolated from bronchial samples and skin. Treatment with a high dose of trimethoprim and sulfamethoxazole for five months resulted in full recovery from cutaneous and pulmonary complaints. No relapse of the infection was found on follow‐up in either patient. Conclusion These cases demonstrate the need for a high degree of suspicion, focused clinical search, and appropriate laboratory procedures in the diagnosis and management of cutaneous nocardiosis.     

Primary cutaneous aspergillosis in a patient with a solid organ transplant: case report and review of the literature

GOAL: To understand primary cutaneous aspergillosis to better manage patients with the condition. OBJECTIVES: Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Describe risk factors for Aspergillus infection. 2. Identify diagnostic methods for primary cutaneous aspergillosis. 3. Discuss treatment options for primary cutaneous aspergillosis. Primary cutaneous aspergillosis is an uncommon disease in immunocompetent individuals that often affects immunosuppressed hosts. We present the first reported case of primary cutaneous aspergillosis in a solid organ transplant recipient caused by Aspergillus niger. Fruiting bodies were isolated from a necrotic ulcer arising in a surgical wound. Debridement alone failed to resolve the infection, emphasizing the need for early antifungal treatment combined with surgical management of this infection.     

ALK‐positive (2p23 rearranged) anaplastic large cell lymphoma with localization to the skin in a pediatric patient

Anaplastic large cell lymphoma (ALCL) either as primary cutaneous or nodal disease is rare in children and difficult to distinguish, which is important both prognostically and for treatment purposes. We present a case of anaplastic lymphoma kinase (ALK)+ skin‐limited ALCL that highlights this challenge and draws attention to pitfalls in assessing ALK status. The patient was an 11‐year old girl with a twice recurrent nodule on her right shoulder. Each biopsy revealed a deep infiltrate of atypical lymphocytes that expressed CD3, CD4, CD43, CD45RO and CD30. The initial biopsy was epithelial membrane antigen (EMA)+ with vague cytoplasmic ALK‐1 positivity by immunohistochemistry, while the second biopsy was EMA+ and nuclear ALK‐1+. Fluorescence in situ hybridization analysis for an ALK (2p23) rearrangement of the first specimen was negative, while an ALK gene rearrangement was present in the second specimen. Therefore, this case was treated as nodal ALCL, despite negative bone marrow and radiographic imaging studies. The patient was treated with combination chemotherapy and remains disease‐free. Demonstration of nuclear ALK‐positivity, ALK (2p23) gene rearrangement is suggestive of systemic ALCL. Without evidence of systemic disease, this case highlights challenges of skin‐limited ALCL, whose clinical behavior as either cutaneous ALCL systemic ALCL may not be immediately apparent.     

Cutaneous malignant fibrous histiocytoma. A rare but serious malignancy

We report 3 cases of malignant fibrous histiocytoma occurring as primary neoplasms of the skin. The first case developed in a leg ulcer of traumatic origin. The second developed on the lower lip at the site of a squamous cell carcinoma which had been treated by radiotherapy. The third arose on a calf at a site of previous surgery. The literature on this malignancy is reviewed, with emphasis on cutaneous involvement.     

Primary cutaneous T‐cell lymphoproliferative disorder of donor origin after allogeneic haematopoietic stem‐cell transplantation

A 56‐year‐old male patient had a history of mantle‐cell lymphoma, which was treated with polychemotherapy and reduced‐intensity conditioning allogeneic haematopoietic stem cell transplantation (ASCT) from his healthy sister with an identical human leucocyte antigen profile. Six years after transplantation, the patient developed asymptomatic eczema‐like cutaneous lesions. Histologically the lesions contained a dense superficial lichenoid infiltrate, mainly consisting of CD4+ atypical medium to large lymphocytes showing indented hyperchromatic nuclei. In situ hybridization for Epstein–Barr virus was negative. PCR amplification of the T‐cell receptor‐γ chain gene from several lesions revealed a monoclonal rearrangement without clonal variation. Two‐colour fluorescence in situ hybridization (X and Y chromosomes) and microsatellite genotyping were used to compare samples from the patient (transplant recipient), his sister (donor) and the skin biopsy sample, which confirmed that the origin of the neoplastic cells was the donor graft. To our knowledge, this is the first case of post‐transplant primary cutaneous T‐cell lymphoproliferative disorder after ASCT.     

Septolobular panniculitis in disseminated Lyme borreliosis

Lyme disease classically evolves through clinical manifestations according to the stage of illness. Because many of the systemic symptoms are non‐specific, and because serology may yield false negative results, cutaneous findings merit even greater importance to diagnosis. The prototypical skin lesion, erythema migrans (EM), occurs early and is the only independent diagnostic clinical feature according to the guidelines of the Infectious Diseases Society of America. EM itself has protean guises, being, at times, vesicular, indurated, necrotic, purpuric, solid, or targetoid, but it is not the sole Borrelia‐associated skin lesion. Acrodermatitis chronica atrophicans and borrelial lymphocytoma cutis are other well‐known skin manifestations. A rare cutaneous manifestation that is increasingly reported in Lyme patients is panniculitis, which develops after dissemination of the spirochete. We present such a case in a patient who was initially treated for cellulitis as well as neck and radicular leg pain, thereby expanding the cutaneous spectrum of Lyme disease.     

JAK2‐positive cutaneous myelofibrosis presenting as sclerosing extramedullary hematopoietic tumors on the scalp: case presentation and review of the literature

We report the second case of cutaneous myelofibrosis with a documented JAK2 activating mutation involving the scalp of a 67‐year‐old woman with primary myelofibrosis in her marrow. In contrast to the previous case, the biopsy revealed extensive lesional collagen deposition and closely mimicked a fibrohistiocytic proliferation. Similar rare lesions occurring in the setting of myeloproliferative neoplasms have been called sclerosing extramedullary hematopoietic tumors. These entities appear histomorphologically and etiologically distinct from extramedullary hematopoiesis, and their diagnosis should prompt the workup for a myeloproliferative neoplasm in the absence of an antecedent diagnosis. The presence of the JAK2 mutation in our case confirmed that the lesions represented skin involvement by a neoplastic myeloid proliferation and not compensatory extramedullary hematopoiesis. Our patient died of disease several months following the appearance of her lesions, which is in keeping with other reports that suggest that cutaneous myelofibrosis may serve as an independent poor prognostic sign in otherwise advanced primary myelofibrosis. A review of the literature further emphasizes the importance of distinguishing this entity from mesenchymal neoplasms and acute myeloid leukemia involving the skin.     

ALK‐positive primary cutaneous anaplastic large cell lymphoma: a case report and review of the literature

Anaplastic large cell lymphoma (ALCL) limited to the skin is a distinct disease that is designated primary cutaneous ALCL (pcALCL). It has an indolent course with a significantly better prognosis compared to systemic ALCL (sALCL). Anaplastic lymphoma kinase (ALK) expression in lesions of cutaneous ALCL is classically considered to be a marker for skin involvement by sALCL. However, recent reports of patients with ALK‐positive pcALCL challenge this concept and raise prognostic and therapeutic dilemmas. Herein, we report a case of ALK‐positive pcALCL in a 45‐year‐old woman who was treated with local radiotherapy. We review previously reported cases in the literature to better characterize this rare variant. Overall, the rates of cutaneous recurrence, systemic dissemination, and disease‐related mortality in ALK‐positive pcALCL do not differ from those previously reported in pcALCL. ALK‐positive pcALCL is diagnosed at younger age and has a better disease course in children compared to adults with lower incidences of skin recurrence and progression to systemic disease. We conclude that ALK‐positivity in cutaneous ALCL does not necessarily imply systemic disease. ALK‐positive pcALCL has an excellent prognosis and should be treated by excision and/or radiotherapy. However, patients must remain under close long‐term follow‐up as recurrence and progression to systemic disease may occur.     

Sporotrichoid Aspergillosis in an Immunocompromised Child: A Case Report and Review of the Literature

Abstract:  Primary cutaneous aspergillosis is an uncommon, opportunistic infection. Atypical presentations have recently emerged with the expanding range of primary and acquired diseases that cause immunosuppression. Primary cutaneous aspergillosis may invade the deep lymphatic structures and present in a sporotrichoid pattern. In pediatric patients with an otherwise normal previous medical history, primary cutaneous aspergillosis should raise the suspicion of an immunodeficiency and prompt referral to immunology and infectious disease specialists should be made. Early diagnosis and management of primary cutaneous aspergillosis prevents invasive aspergillosis, minimizing morbidity and mortality in the immunocompromised patients.     

Pseudotumoral cutaneous aspergillosis in chronic granulomatous disease, report of a pediatric case

ABSTRACT:: Invasive aspergillosis is a life-threatening condition in patients with chronic granulomatous disease (CGD). Skin invasion by Aspergillus occurs most commonly by contiguity to a neighboring cavity. We describe an unusual case of invasive cutaneous aspergillosis presented as a large burgeoning tumor in a 4-year-old girl with CGD who underwent surgical treatment for bifocal osteomyelitis of the left leg. The skin invasion occurred 4 months after a "successful" treatment of invasive pulmonary aspergillosis. Atypical presentation and diagnostic difficulties are discussed. Invasive cutaneous aspergillosis may be polymorphic. The diagnosis should be considered early in the etiological investigation of any suspicious skin lesions in CGD even in uncommon aspects such as burgeoning tumors.     

Erysipelas-like dermatitis of the legs revealing aspergilloma of the maxillary sinus

BACKGROUND: Skin signs is associated with Aspergillus are rare and are seen principally in immunodepressed patients. Distinction is generally made between primary skin aspergillosis, caused by direct cutaneous inoculation with the offending organism, and secondary skin aspergillosis, associated with peripheral emboli from an area of chronic pulmonary or sinus mycetoma. There have been rare reports of indirect satellite skin signs resulting from Aspergillus infection, and below we present such a case. PATIENTS AND METHODS: A 40 year-old immunocompetent man consulted for erysipeloid plaques on the lower limbs recurring over a period of seven months. X-rays and CAT scans of the sinus demonstrated asymptomatic axillary sinusitis probably caused by Aspergillus. The diagnosis was confirmed by surgery, which resulted in cure without additional antifungal treatment. The inflammatory syndrome subsided and after 15 months, there was no recurrence of lesions. DISCUSSION: The absence of relapse following treatment of the focus of aspergillosis forms a major argument in favour of a causal relationship between the erysipeloid dermatitis and the sinus mycotic infection. The hypothesis of a septic embologenic mechanism within the sinus was abandoned in favour of a mechanism similar to streptococcal nodular erythema, seen in diseases involving immune complexes, possibly caused by allergy to Aspergillus proteins. This case history demonstrates the existence of satellite skin signs of Aspergillus infection indicative of neither primary nor secondary aspergillosis.     

Lymphoma or pseudolymphoma: A report of six cases and review of the literature

To explore and compare the clinical presentations and pathologic features of cutaneous pseudolymphomas (CPL) with primary cutaneous lymphomas. Review literature in order to improve the treatment of CPL. Six cases of CPLs were collected. The clinical, pathologic, and immunohistochemical features were performed and analyzed in Peking Union Medical College Hospital in 2018. Of six patients, the distributions and clinical manifestations of skin lesions are varied. The pathologic features consisted of atypical prominent lymphocytes infiltration. Of them, two cases imitated mycosis fungoides, one case mimicked primary cutaneous aggressive pidermotropic CD8+ cytotoxic T‐cell lymphoma, one case was diagnosed as Jessner‐Kanof lymphocyte infiltration and two cases primary cutaneous CD4+ small/medium T‐cell lymphoproliferative disorder. With respect to etiology, one was result from insects bite and the others were on account of drugs. All cases were treated with systemic or local glucocorticoid. The skin lesions and systemic symptoms showed notable improvement after treatment. Follow‐up visits were 2 years, half a year, and months, respectively, without relapse. These unique types of CPL were similar to cutaneous lymphomas in clinical manifestation and pathology. They were all sensitive to the treatment of externally or orally using glucocorticoid. The prognosis is generally good but needs long‐term follow‐up.     

The molecular skin pathology of familial primary localized cutaneous amyloidosis

Please cite this paper as: The molecular skin pathology of familial primary localized cutaneous amyloidosis. Experimental Dermatology 2010; 19: 416–423. Abstract: Familial primary localized cutaneous amyloidosis (FPLCA) is an autosomal dominant disorder associated with chronic itching and skin lichenification. In lesional skin, there are apoptotic basal keratinocytes and deposits of amyloid material on degenerate keratin filaments in the upper dermis. The genetic basis of FPLCA involves mutations in the OSMR and IL31RA genes but the disease pathophysiology is not fully understood. In this study, we identified new pathogenic heterozygous missense mutations in the OSMR gene (p.Val631Leu and p.Asp647Tyr) in two Dutch FPLCA families. We then compared gene expression profiles between FPLCA lesional skin (n = 4) and site‐matched control skin (n = 6). There was twofold or greater upregulation of 34 genes and downregulation of 43 genes. Most changes in gene expression (verified by quantitative RT‐PCR) reflected alterations in epidermal differentiation and proliferation consistent with lichenification, but we also noted a reduction in several interfollicular keratinocyte stem cell markers in FPLCA skin. Differences in gene expression were also noted for proteins involved in apoptosis and nerve conduction. Collectively, this study expands the molecular basis of FPLCA and provides new insight into the skin pathology of this condition.