Dataset Information

A pyoderma gangrenous-like cutaneous leishmaniasis in a Libyan woman with rheumatoid arthritis: a case report.

ABSTRACT: Several case reports describe diseases presenting with skin ulcerations, which resemble pyoderma gangrenosum especially in immune-compromised patients, often proven on further workup, to have an infective or malignant etiology. However, treatment of pyoderma gangrenosum by systemic steroids or other immunosuppressive agents may worsen the condition.We report here, a 45 year-old Libyan woman with rheumatoid arthritis on low dose steroids with pyoderma gangrenosum-like skin lesions and positive pathergy. Slit-smear was positive for Leishmania amastigotes and histopathological examination confirmed the diagnosis of cutaneous leishmaniasis. The lesions healed completely by parenteral sodium stibogluconate (Pentostam) 600 mg daily.We report for the first time, a rare and unusual presentation of pyoderma gangrenosum like-cutaneous leishmaniasis in a patient with rheumatoid arthritis. Atypical cutaneous leishmaniasis should not be ruled out in the differential diagnosis of unresponsive skin diseases, with slit/smear and a skin biopsy is required.

SUBMITTER: Al-Dwibe H

PROVIDER: S-EPMC5831575 | biostudies-literature | 2018 Mar

REPOSITORIES: biostudies-literature

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A pyoderma gangrenous-like cutaneous leishmaniasis in a Libyan woman with rheumatoid arthritis: a case report.

Al-Dwibe Hamida H Amro Ahmad A Gashout Aisha A El-Zurghany Ali A El-Zubi Said S El-Hashme Mohamed M Hamarsheh Omar O Maree Mokhtar M

BMC research notes 20180301 1

<h4>Background</h4>Several case reports describe diseases presenting with skin ulcerations, which resemble pyoderma gangrenosum especially in immune-compromised patients, often proven on further workup, to have an infective or malignant etiology. However, treatment of pyoderma gangrenosum by systemic steroids or other immunosuppressive agents may worsen the condition.<h4>Case presentation</h4>We report here, a 45 year-old Libyan woman with rheumatoid arthritis on low dose steroids with pyoderma ...[more]

PMID: 29490707

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Project description:BackgroundAutoinflammatory phospholipase Cγ2 (PLCγ2)-associated antibody deficiency and immune dysregulation (APLAID) is a rare autoinflammatory disease caused by gain-of-function mutations in the PLCG2 gene. Here we report a rare case of APLAID patient carrying a novel heterozygous missense PLCG2 I169V mutation with gangrenous pyoderma and concomitant high serum immunoglobulin (Ig) E level.MethodsThe patient was diagnosed as APLAID and has been treated in our department. His phenotype and genotype were carefully documented and studied. We also conducted a comprehensive literature review on APLAID.ResultsA 23-year-old Chinese Han man presented with recurrent fever for 18 years and vesiculopustular rashes for 9 years, along with chronic bronchitis, leukocytosis, increased C-reactive protein, immunodeficiency and high serum IgE. Skin biopsy showed chronic inflammatory cells infiltration. A paternal heterozygous missense variant in exon 6 of the PLCG2 gene p. I169V was identified. His vesiculopustular and IgE level responded to medium dose corticosteroids. After withdrawal of steroids, he developed severe arthritis and a large deteriorating ulceration resembling pyoderma gangrenosum on the left knee. Large dose corticosteroids were suboptimal. Then he received adalimumab with satisfactory response for arthritis and skin lesion. But he got an immunodeficiency-associated lymphoproliferative disorder 2 months later. Through literature review, there were a total of 10 APLAID patients reported by six English-language publications. Vesiculopustular rashes, sinopulmonary infection and immunodeficiency were the most frequent symptoms of APLAID patients. Glucocorticoids, intravenous immunoglobulin and biologics were clinically used to treat APLAID but none of these patients had a complete recovery.ConclusionsThe rarity and diversity of APLAID make it difficult to be diagnosed. Our study reported the first case of APLAID with gangrenous pyoderma and concomitant high IgE carrying a novel PLCG2 mutation, which may expand the clinical phenotype and genotype of APLAID.

Project description:BackgroundLeishmaniasis is a common neglected tropical disease in Ethiopia. Visceral leishmaniasis (VL) caused by Leishmania donovani presents in the lowlands, while cutaneous leishmaniasis (CL) affects people living in the highlands. Although CL is described as being caused by Leishmania aethiopica, there is also evidence of L. tropica and L. major isolated from a patient, sand flies and potential reservoirs. Information on species causing CL in Ethiopia is patchy, and no nation-wide study has ever been done. Understanding which species are causing CL in Ethiopia can have important implications for patient management and disease prevention.MethodsWe analyzed stored routine samples and biobanked DNA isolates from previously conducted studies of CL patients from different centers in the north, center and south of Ethiopia. Species typing was performed using ITS-1 PCR with high-resolution melt (HRM) analysis, followed by HSP70 amplicon sequencing on a selection of the samples. Additionally, sociodemographic, clinical and laboratory data of patients were analyzed.ResultsOf the 226 CL samples collected, the Leishmania species could be determined for 105 (45.5%). Leishmania aethiopica was identified in 101 (96.2%) samples from across the country. In four samples originating from Amhara region, northwestern Ethiopia, L. donovani was identified by ITS-1 HRM PCR, of which two were confirmed with HSP70 sequences. While none of these four patients had symptoms of VL, two originated from known VL endemic areas.ConclusionsThe majority of CL was caused by L. aethiopica, but CL due to L. tropica and L. major cannot be ruled out. Our study is the first to our knowledge to demonstrate CL patients caused by L. donovani in Ethiopia. This should spark future research to investigate where, how and to which extent such transmission takes place, how it differs genetically from L. donovani causing VL and whether such patients can be diagnosed and treated successfully with the currently available tools and drugs.

Dataset Information

A pyoderma gangrenous-like cutaneous leishmaniasis in a Libyan woman with rheumatoid arthritis: a case report.

Publications

A pyoderma gangrenous-like cutaneous leishmaniasis in a Libyan woman with rheumatoid arthritis: a case report.

Similar Datasets

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