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Multiple Skin Necrotic Lesions—Diagnosis

Multiple Skin Necrotic Lesions—Diagnosis Diagnosis: Systemic zygomycosis with cutaneous embolization and infarction. Microscopic findings The skin biopsy specimen showed areas of subepidermal hemorrhage with infarction. Within the dermis, numerous large nonseptate branching fungalhyphae were seen invading vessel walls, suggesting an angioinvasive zygomycosis. Discussion Zygomycosis (or mucormycosis) is a rare form of invasive fungal infection that is increasingly being recognized in immunocompromised patients. The fungus,found in soil, belongs to the class Zygomycetes. Rhizopus, Absidia, and Mucor belong to the family Mucorales, hence the use of mucormycosis inthe literature. The organisms are characterized by broad-branching nonseptate hyphae. Five clinical forms have been described: rhinocerebral, pulmonary, gastrointestinal, disseminated, and cutaneous.1 Inhalation,direct inoculation, and ingestion are recognized portals of entry. Pathogenicity is dependent on host immunity, and 40% of cases are described in hematologicmalignancies.2 Diabetes mellitus, immunosuppression (including cases involving chemotherapy and transplantation), surgical woundcontamination,3,4 burns, and trauma5 have been reported as underlyingcauses. About 90% of patients are neutropenic at the time of onset. Fever, chest pain, and respiratory symptoms are frequent presenting symptoms. It is increasingly being recognized that, following candidiasis, aspergillosis, and cryptococcosis, zygomycosis is the most common opportunistic fungal infection.6 Zygomycosis can present in the skin as a primarycutaneous process or as embolic skin lesions caused by systemic dissemination. It can present as erythematous plaques, pustules, nodules, and ulceration.1-8 Asuperficial granulomatous pyoderma pattern has also been reported.4 Our immunocompromised patient could have acquired zygomycosis through invasive procedures carried out in the hospital. As in cases reported by duPlessis et al,6 we cannot say with certainty whether or not she had systemic disease with cutaneous embolic lesions, becausean autopsy was not performed. The extent and rapidity of her disease suggest dissemination. Diagnosis of zygomycosis is made by skin biopsy. Identification of the genus and species can be made by culture, although the results were negativein this case. Only 30% of cases with a biopsy-proved diagnosis demonstrate growth on fungal culture.4 The differentialdiagnosis includes other causes of embolic disease and cutaneous vasculitis. Treatment involves medical andsurgical management. Amphotericin B therapyand surgical debridement are the most widely used modalities. Unfortunately, our patient died before antifungal therapy was initiated. A high index ofsuspicion is needed to make a diagnosis and to aggressively treat this infection. In any case, mortality rates as high as 90% have been reported in cases inwhich patients were treated empirically with amphotericin B.2 References 1. Prevoo RLMStarink TMde Haan P Primary cutaneous mucormycosis in a healthy young girl J Am Acad Dermatol. 1991;24882- 885PubMedGoogle ScholarCrossref 2. Penas PFRios Lde la Camara RFraga JDauden E Cutaneous lesions as the first sign of disseminated mucormycosis Acta Derm Venereol. 1995;75166- 167PubMedGoogle Scholar 3. Akritidis NPapaioannides DKitsiou EKorantzpoulos P Necrotizing cutaneous mucormycosis Hosp Med. 2002;63308- 309PubMedGoogle ScholarCrossref 4. Gellar JDPeters MSSu WPD Cutaneous mucormyocosis resembling superficial granulomatous pyodermain an immunocompetent host J Am Acad Dermatol. 1993;29462- 465PubMedGoogle ScholarCrossref 5. Umbert IJSu WPD Cutaneous mucormycosis J Am Acad Dermatol. 1989;211232- 1234PubMedGoogle ScholarCrossref 6. du Plessis PJWentzel LFDelport JDvan Damme E Zygomycotic necrotizing cellulitis in a premature infant Dermatology. 1997;195179- 181PubMedGoogle ScholarCrossref 7. Adriaenssens KJorens PGMeuleman LJeuris WLambert J A black necrotic skin lesion in an immunocompromised patient Arch Dermatol. 2000;1361165- 1170PubMedGoogle ScholarCrossref 8. Pagano LRicci PTonso A et al. Mucormycosis in patients with haematological malignancies: a retrospective clinical study of 37 cases Br J Haematol. 1997;99331- 336PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Multiple Skin Necrotic Lesions—Diagnosis

Archives of Dermatology , Volume 140 (7) – Jul 1, 2004

Multiple Skin Necrotic Lesions—Diagnosis

Abstract

Diagnosis: Systemic zygomycosis with cutaneous embolization and infarction. Microscopic findings The skin biopsy specimen showed areas of subepidermal hemorrhage with infarction. Within the dermis, numerous large nonseptate branching fungalhyphae were seen invading vessel walls, suggesting an angioinvasive zygomycosis. Discussion Zygomycosis (or mucormycosis) is a rare form of invasive fungal infection that is increasingly being recognized in immunocompromised patients. The fungus,found in...
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Publisher
American Medical Association
Copyright
Copyright © 2004 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.140.7.877-b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Systemic zygomycosis with cutaneous embolization and infarction. Microscopic findings The skin biopsy specimen showed areas of subepidermal hemorrhage with infarction. Within the dermis, numerous large nonseptate branching fungalhyphae were seen invading vessel walls, suggesting an angioinvasive zygomycosis. Discussion Zygomycosis (or mucormycosis) is a rare form of invasive fungal infection that is increasingly being recognized in immunocompromised patients. The fungus,found in soil, belongs to the class Zygomycetes. Rhizopus, Absidia, and Mucor belong to the family Mucorales, hence the use of mucormycosis inthe literature. The organisms are characterized by broad-branching nonseptate hyphae. Five clinical forms have been described: rhinocerebral, pulmonary, gastrointestinal, disseminated, and cutaneous.1 Inhalation,direct inoculation, and ingestion are recognized portals of entry. Pathogenicity is dependent on host immunity, and 40% of cases are described in hematologicmalignancies.2 Diabetes mellitus, immunosuppression (including cases involving chemotherapy and transplantation), surgical woundcontamination,3,4 burns, and trauma5 have been reported as underlyingcauses. About 90% of patients are neutropenic at the time of onset. Fever, chest pain, and respiratory symptoms are frequent presenting symptoms. It is increasingly being recognized that, following candidiasis, aspergillosis, and cryptococcosis, zygomycosis is the most common opportunistic fungal infection.6 Zygomycosis can present in the skin as a primarycutaneous process or as embolic skin lesions caused by systemic dissemination. It can present as erythematous plaques, pustules, nodules, and ulceration.1-8 Asuperficial granulomatous pyoderma pattern has also been reported.4 Our immunocompromised patient could have acquired zygomycosis through invasive procedures carried out in the hospital. As in cases reported by duPlessis et al,6 we cannot say with certainty whether or not she had systemic disease with cutaneous embolic lesions, becausean autopsy was not performed. The extent and rapidity of her disease suggest dissemination. Diagnosis of zygomycosis is made by skin biopsy. Identification of the genus and species can be made by culture, although the results were negativein this case. Only 30% of cases with a biopsy-proved diagnosis demonstrate growth on fungal culture.4 The differentialdiagnosis includes other causes of embolic disease and cutaneous vasculitis. Treatment involves medical andsurgical management. Amphotericin B therapyand surgical debridement are the most widely used modalities. Unfortunately, our patient died before antifungal therapy was initiated. A high index ofsuspicion is needed to make a diagnosis and to aggressively treat this infection. In any case, mortality rates as high as 90% have been reported in cases inwhich patients were treated empirically with amphotericin B.2 References 1. Prevoo RLMStarink TMde Haan P Primary cutaneous mucormycosis in a healthy young girl J Am Acad Dermatol. 1991;24882- 885PubMedGoogle ScholarCrossref 2. Penas PFRios Lde la Camara RFraga JDauden E Cutaneous lesions as the first sign of disseminated mucormycosis Acta Derm Venereol. 1995;75166- 167PubMedGoogle Scholar 3. Akritidis NPapaioannides DKitsiou EKorantzpoulos P Necrotizing cutaneous mucormycosis Hosp Med. 2002;63308- 309PubMedGoogle ScholarCrossref 4. Gellar JDPeters MSSu WPD Cutaneous mucormyocosis resembling superficial granulomatous pyodermain an immunocompetent host J Am Acad Dermatol. 1993;29462- 465PubMedGoogle ScholarCrossref 5. Umbert IJSu WPD Cutaneous mucormycosis J Am Acad Dermatol. 1989;211232- 1234PubMedGoogle ScholarCrossref 6. du Plessis PJWentzel LFDelport JDvan Damme E Zygomycotic necrotizing cellulitis in a premature infant Dermatology. 1997;195179- 181PubMedGoogle ScholarCrossref 7. Adriaenssens KJorens PGMeuleman LJeuris WLambert J A black necrotic skin lesion in an immunocompromised patient Arch Dermatol. 2000;1361165- 1170PubMedGoogle ScholarCrossref 8. Pagano LRicci PTonso A et al. Mucormycosis in patients with haematological malignancies: a retrospective clinical study of 37 cases Br J Haematol. 1997;99331- 336PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Jul 1, 2004

Keywords: zygomycosis,necrosis,skin

References