Degos Disease with Dermatomyositis-like Phenomenon: A Diagnostic Dilemma and a Therapeutic Challenge

Background: Degos disease is a type of vasculopathy characterized by progressive occlusion of small-sized blood vessels primarily involving the skin, gastrointestinal system, and central nervous system as well as various other systems. Owing to the rarity of the condition, the diagnosis is often a challenge; consequently, management is even more difficult owing to the paucity of experience and literature for the effective treatment of this entity. Case Presentation: We report a case of a 50-year-old male patient with classic skin lesions and rapidly progressive fatal clinical course involving multiple organs associated with dermatomyositis-like features. Therapeutic challenge in such a case of secondary Degos disease with multiorgan dysfunction is discussed. Conclusion: Secondary Degos disease with underlying multisystem dysfunction is a rapidly fatal disease. The patient must be thoroughly investigated to watch for systemic involvement and aggressively treated preferably with antiplatelet drugs and immunosuppresants so as to moderate the severity of the disease. Corticosteroids are best avoided to prevent gastrointestinal perforation.

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Legionnaires’ disease arising with hirsutism: case report of an extremely confusing event

BMC Infectious Diseases ◽

10.1186/s12879-021-06247-9 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Gabriella Serio ◽

Francesco Fortarezza ◽

Federica Pezzuto ◽

Luigi Santacroce ◽

Pietro Nazzaro ◽

...

Keyword(s):

Legionella Pneumophila ◽

Clinical Course ◽

Tracheal Aspirate ◽

Young Female ◽

Case Presentation ◽

Systemic Involvement ◽

Serotype 1 ◽

Autopsy Examination ◽

Legionnaires Disease ◽

The Brain

Abstract Background Legionella bacteria is a common cause of pneumonia, but the infection may affect several organs in the most serious cases. A systemic involvement ab initio could be non-specific, leading to a diagnostic misinterpretation. Case presentation A 33-year-old woman had been complaining of mental confusion, restlessness, aggressiveness, and, subsequently, hirsutism. After 3 weeks, the patient developed pneumonia and died during the hospitalization. The autopsy examination revealed a multi-organ necrotizing exudative disease involving the lung, the heart and the brain. The microbiological tests of tracheal aspirate were positive for Legionella pneumophila serotype 1. Conclusion The Legionella infection may show a proteiform clinical course and an extra-pulmonary manifestation may be the first sign of the disease. Herein, we report a case of Legionella infection in a young female, presenting with non-specific neurological symptoms and hirsutism at onset, misdiagnosed as a metabolic disease.

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Degos disease: a case report and review of the literature

Journal of Medical Case Reports ◽

10.1186/s13256-020-02514-6 ◽

2020 ◽

Vol 14 (1) ◽

Author(s):

Santosh Tummidi ◽

Prabhakaran Nagendran ◽

Swaroopa Gedela ◽

Jami Rupa Ramani ◽

Arundhathi Shankaralingappa

Keyword(s):

Central Nervous System ◽

Specific Treatment ◽

Skin Lesions ◽

Punch Biopsy ◽

Review Of The Literature ◽

Thrombotic Occlusion ◽

Case Presentation ◽

Degos Disease ◽

Skin Punch Biopsy ◽

Hindu Woman

Abstract Background Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. It can affect the skin, gastrointestinal tract, and central nervous system. However, other organs such as the kidney, lungs, pleura, and liver can also be involved. Case presentation A 35-year-old Hindu woman presented to our dermatology outpatient department with complaints of depigmented painful lesions. A skin punch biopsy taken from the porcelain white atrophic papules which revealed features of Degos disease. Conclusion The diagnosis of Degos disease is usually based on the presence of the pathognomonic skin lesions and a tissue biopsy demonstrating a wedge-shaped area of necrosis with thrombotic occlusion of the small arterioles. No specific treatment is currently available for this disease.

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Fever in the returning traveller: Dual infection with SARS-CoV-2 and Plasmodium falciparum malaria

Journal of Medical Laboratory Science & Technology of South Africa ◽

10.36303/jmlstsa.2020.2.2.57 ◽

2020 ◽

pp. 105-108

Author(s):

MA Parker ◽

E Nell ◽

A Mowlana ◽

MS Moolla ◽

S Karamchand ◽

...

Keyword(s):

Plasmodium Falciparum ◽

Falciparum Malaria ◽

Clinical Course ◽

Febrile Illness ◽

Plasmodium Falciparum Malaria ◽

Dual Infection ◽

Pneumocystis Pneumonia ◽

Case Presentation ◽

Presenting Symptoms ◽

Uncomplicated Plasmodium Falciparum Malaria

Background: More than 90% of the global 400 000 annual malaria deaths occur in Africa. The current SARS-CoV-2 pandemic has resulted in more than 830 000 deaths in its first 10 months. Case presentation: This case describes a patient who had travelled from Mozambique to Cape Town, presented with a mild febrile illness, and was diagnosed with both COVID-19 and uncomplicated Plasmodium falciparum malaria infection. She responded well to malaria treatment and had an uneventful COVID-19 admission. Her blood smear showed a low malaria parasitaemia and a relatively high gametocyte load. Conclusion: We postulate that her clinical course and abnormal smear could well be due to reciprocal disease-modifying effects of the infections. The presenting symptoms of COVID-19 may mimic endemic infectious diseases including malaria, tuberculosis, pneumocystis pneumonia and influenza thus there is a need for clinical vigilance to identify and treat such co-infections.

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Complicated cutaneous leishmaniasis caused by an imported case of Leishmania tropica in Japan: a case report

Tropical Medicine and Health ◽

10.1186/s41182-021-00312-4 ◽

2021 ◽

Vol 49 (1) ◽

Author(s):

Hiroyuki Kitano ◽

Chizu Sanjoba ◽

Yasuyuki Goto ◽

Kazumasa Iwamoto ◽

Hiroki Kitagawa ◽

...

Keyword(s):

Cutaneous Leishmaniasis ◽

Skin Lesions ◽

Leishmania Tropica ◽

Case Presentation ◽

Imported Case ◽

Imported Cases ◽

History Of ◽

Hematoxylin And Eosin ◽

Polymerase Chain ◽

Positive Results

Abstract Background Leishmaniasis is not endemic in Japan, and imported cases are rare. However, there are increasing concerns regarding imported cases of cutaneous leishmaniasis from endemic countries to Japan. This report describes a case of imported cutaneous leishmaniasis that was diagnosed and treated in Japan. Case presentation A 53-year-old Pakistani man presented with skin lesions on both malleoli of his right ankle and the dorsum of the left foot. The skin lesions manifested as erythematous nodules surrounding an ulcer in the center of the lesion. The lesions of the malleoli of his right ankle each measured 3 × 3 cm, and the lesion on the top of his left foot measured 5 × 4 cm. He had been living and working in Japan but had a history of a visit to Pakistan for about 2 months in 2018. The skin lesions were biopsied. Giemsa and hematoxylin and eosin staining of biopsy samples showed amastigotes of Leishmania in macrophages, and the presence of Leishmania was confirmed by skin tissue culture. Polymerase chain reaction using biopsy specimens identified Leishmania parasites, and DNA sequence analysis revealed that the species was Leishmania tropica. The patient was treated with intravenous liposomal amphotericin B for 6 days. The erythema disappeared, and the erythematous nodules resolved within 3 weeks. Conclusion This is the first report of imported cutaneous leishmaniasis caused by L. tropica from Pakistan, and it is interesting that all three testing modalities showed positive results in this case.

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Fulminant Multisystem Non–Langerhans Cell Histiocytic Proliferation With Hemophagocytosis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2005-129-e39-fmnchp ◽

2005 ◽

Vol 129 (2) ◽

pp. e39-e43 ◽

Cited By ~ 2

Author(s):

R. Nagarjun Rao ◽

Chung-che Chang ◽

Nevin Uysal ◽

Kenneth Presberg ◽

Vinod B. Shidham ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Clinical Course ◽

Langerhans Cell ◽

Variant Form ◽

Malignant Histiocytosis ◽

Unique Case ◽

Multiple Organs ◽

Biopsy Specimens ◽

Extensive Involvement ◽

Erdheim Chester

Abstract Hemophagocytosis (HP), a feature seen in malignant histiocytosis and infection- and lymphoma-associated disorders, has not been previously emphasized in Erdheim-Chester disease (ECD). Generally, ECD is recognized as a rare, systemic, non–Langerhans cell histiocytosis with a variable clinical course. Herein, we describe a unique case of multisystem non–Langerhans cell histiocytic proliferation with a fulminant clinical course (death occurred within 3 months of presentation) that showed prominent HP and extensive involvement of multiple organs, including the lungs, resulting in respiratory failure. Hemophagocytosis led to severe anemia that required transfusion and thrombocytopenia. Antemortem lung and bone marrow biopsy specimens revealed involvement by a histiocytic infiltrate with features highly suggestive of ECD and HP. Furthermore, the autopsy documented the presence of HP and the histiocytic infiltrate in multiple other organs. This case is best categorized as a variant form of ECD. Recognizing this variant has the following important implications: (1) HP may be a marker for fulminant clinical course in ECD, (2) the presence of HP does not exclude a diagnosis of ECD, and (3) ECD should be considered in the differential diagnosis of HP.

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Leptospirosis presenting as severe cardiogenic shock: A case report

Journal of the Intensive Care Society ◽

10.1177/1751143718754993 ◽

2018 ◽

Vol 19 (4) ◽

pp. 351-353

Author(s):

E Forbat ◽

MJ Rouhani ◽

C Pavitt ◽

S Patel ◽

R Handslip ◽

...

Keyword(s):

Case Report ◽

Cardiogenic Shock ◽

Social History ◽

Rare Case ◽

Clinical Course ◽

Unknown Origin ◽

Case Presentation ◽

Pyrexia Of Unknown Origin ◽

Severe Cardiogenic Shock ◽

Infectious Illness

Background Leptospirosis is a rare infectious illness caused by the Spirochaete Leptospira. It has a wide-varying spectrum of presentation. We present a rare case of severe cardiogenic shock secondary to leptospirosis, in the absence of its common clinical features. Case presentation A 36-year-old woman presented to our unit with severe cardiogenic shock and subsequent multi-organ failure. Her clinical course was characterised by ongoing pyrexia of unknown origin with concurrent cardiac failure. She was initially managed with broad-spectrum antibiotics and inotropes. Percutaneous cardiac biopsy excluded major causes of myocarditis. On day 21 after presentation, she was found to be IgM-positive for leptospirosis. Conclusions This is a rare case of severe cardiogenic shock secondary to leptospirosis infection. The case also highlights the importance of obtaining a thorough social history when assessing a patient with an unusual presentation, as clues can often be missed.

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Photodistributed sarcoidosis: a case report and approach to management

SAGE Open Medical Case Reports ◽

10.1177/2050313x18796877 ◽

2018 ◽

Vol 6 ◽

pp. 2050313X1879687

Author(s):

Cristina Olteanu ◽

Brandon Worley ◽

Iris Teo ◽

Jillian Macdonald

Keyword(s):

Case Report ◽

Ionizing Radiation ◽

Bone Pain ◽

Prior Exposure ◽

Systemic Involvement ◽

Pruritic Rash ◽

Cutaneous Involvement ◽

Multiple Organs ◽

History Of ◽

Idiopathic Disease

Sarcoidosis is an idiopathic disease, characterized by non-caseating granulomas in multiple organs/tissues. Cutaneous involvement occurs in approximately one-quarter of patients with a wide variety of presenting morphologies. This case report describes a case of photodistributed sarcoidosis, a rare cutaneous variant, with systemic involvement. A 42-year-old man presented with a history of a pruritic, rash with photoexacerbated annular plaques along with arthralgias and bone pain. Compared to previous reports of photodistributed sarcoidosis, our case presented with annular plaques rather than papules, and there was no prior exposure to ionizing radiation. He was treated successfully with prednisone and hydroxychloroquine. It is important to include sarcoidosis in the differential of photodistributed dermatoses.

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