Childhood pyoderma gangrenosum: diagnosis often missed

We present a 10-year-old male child who presented with 2 months history of fever and extensive ulceration over both lower limbs and buttocks. The new lesions were bullous and pustular, so working diagnosis of bullous impetigo was considered. WBC count showed mild anemia and neutrophilic leucocytosis. Peripheral smear showed neutrophilia, adequate platelets and normal lymphocytes. Blood culture and pus culture were sterile. Stool for occult blood was negative, liver function tests were normal. Mantoux test was negative. Serum immunoglobulins were normal and HIV, HBsAg tested negative. Skin biopsy showed foci of granulomatous inflammation with neutrophilic infiltration. With persistent neutrophilia and neutrophilic infiltration of ulcers, pyoderma gangrenosum was kept as diagnosis. The child was started on local steroid application and oral prednisolone 1 mg/kg/day. Wthin 2 days of oral steroids child became afebrile. Ulcers started healing and neutrophila also normalized. In view of reappearance of fever, immunosuppressive, closporin was started, after which child responded well and ulcers healed completely. So, in conclusion, we report a paediatric case of pyoderma gangrenosum which poses diagnostic difficulty, particularly to non-dermatologists. The dramatic response to steroid and cyclosporine helped cure the ulcers and remit the disease and sufferings.

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Atypical Granulomatosis with Polyangiitis Presenting with Meibomitis, Scleritis, Uveitis and Papillary Bladder Tumor: A Case Report and Literature Review

Diagnostics ◽

10.3390/diagnostics11040680 ◽

2021 ◽

Vol 11 (4) ◽

pp. 680

Author(s):

Takashi Kojima ◽

Murat Dogru ◽

Eisuke Shimizu ◽

Hiroyuki Yazu ◽

Aya Takahashi ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

Early Stage ◽

Topical Steroid ◽

Granulomatous Inflammation ◽

Oral Prednisolone ◽

Meibomian Gland ◽

Clinical Findings ◽

Meibomian Gland Dysfunction ◽

Oral Steroids ◽

Atypical Cells

Granulomatosis with polyangiitis (GPA) presents with a variety of systemic findings, sometimes with ocular findings initially, but is often difficult to diagnose at an early stage. An 85-year-old male had complaints of ocular dryness and redness and was diagnosed with meibomian gland dysfunction with meibomitis. Despite an initial treatment with topical steroid and antibiotics, the meibomitis did not improve and the left eye developed scleritis and iridocyclitis. The patient was administered topical mydriatics and oral steroids. During follow-up, the patient developed left hearing difficulty and reported a darker urine. Urinalysis revealed microscopic hematuria. A blood test showed an elevated erythrocyte sedimentation rate, positivity for perinuclear anti-neutorophil cytoplasmic antibody, and elevations in blood urea nitrogen and serum creatinine. Nasal mucosal biopsy showed a non-necrotizing granulomatous inflammation. Renal biopsy revealed focal glomerulosclerosis. Cystoscopy and bladder wash followed by a planned transurethral resection revealed atypical cells and apical papillary tumors which were resected. Iridocyclitis and scleritis responded well to oral prednisolone with 0.1% topical betamethasone and prednisolone ointment. The patient is tumor free with no recurrences 24 months after resection. GPA may present atypically with meibomian gland dysfunction without showing representative clinical findings. Early detection and treatment are essential for visual recovery.

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Tuberculous Pachymeningitis Presenting as a Diffused Dural Thickening in a Patient with Chronic Headache and Recurrent Neurological Abnormalities for More than a Decade: A Case Report and a Review of the Literature

Case Reports in Infectious Diseases ◽

10.1155/2018/3012034 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5

Author(s):

C. L. Fonseka ◽

T. E. Kanakkahewa ◽

S. D. A. L. Singhapura ◽

J. S. Hewavithana ◽

L. P. Kolambage ◽

...

Keyword(s):

Case Report ◽

Chronic Headache ◽

Facial Nerve Palsy ◽

Extrapulmonary Tuberculosis ◽

Granulomatous Inflammation ◽

Mantoux Test ◽

Resonance Imaging ◽

Neurological Signs ◽

Neurological Abnormalities ◽

Contrast Enhanced

Background. Tuberculous pachymeningitis is a rare form of extrapulmonary tuberculosis usually suspected from the detection of thickening of the dura in contrast-enhanced magnetic resonance imaging. Progressive nature of the disease can lead to chronic headache with focal neurological signs due to compression from the thickened dura. Case Report. We report a 40-year-old female who presented with chronic headache over a decade associated with recurrent neurological abnormalities including optic neuritis, hemisensory loss, migraine, facial nerve palsy, and recurrent vertigo. Although there was an initial perceived response to steroids, the patient had a subsequent progressive course. On investigations, she was found to have a diffused dural thickening on contrast MRI with a strongly positive Mantoux test with caseating necrotizing granulomatous inflammation on dural histology. With initiation of antituberculous medication with steroids, the patient markedly improved, and the medication for tuberculosis was continued for a year with good response. Conclusion. Primary tuberculous pachymeningitis should be suspected in a patient complaining of prolonged headache with focal neurological signs when MRI evidence of dural thickening is detected, and another focus of tuberculosis was not found. Prompt suspicion with image-guided dural biopsy for histology would help to confirm the diagnosis.

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Drug Hypersensitivity Syndrome Caused by Minocycline

Journal of Cutaneous Medicine and Surgery ◽

10.2310/7750.2006.00019 ◽

2006 ◽

Vol 10 (3) ◽

pp. 131-135 ◽

Cited By ~ 10

Author(s):

Daisuke Tsuruta ◽

Yukiko Someda ◽

Junko Sowa ◽

Hiromi Kobayashi ◽

Masamitsu Ishii

Keyword(s):

Drug Hypersensitivity ◽

Human Herpesvirus ◽

Oral Prednisolone ◽

Liver Function Tests ◽

Hypersensitivity Syndrome ◽

Skin Lesions ◽

Japanese Woman ◽

Barr Virus ◽

Epstein Barr ◽

Drug Hypersensitivity Syndrome

Background: Minocycline is a commonly prescribed drug for the treatment of acne. Its use is generally not associated with systemic side effects. Objective: To describe a case of minocycline-induced drug hypersensitivity syndrome in a 20-year-old Japanese woman. Methods and Results: Following 2 months of minocycline treatment, the patient developed skin lesions composed of exudative maculopapules, purpuratous macules, and target-like, erythema multiforme-like plaques over most of her body. In addition, she had fever, abnormal liver function tests, eosinophilia, and atypical lymphocytosis. Laboratory tests indicated no elevation of antibody titers against cytomegalovirus, Epstein-Barr virus, and human herpesvirus 6. Her ongoing exposure to minocycline was stopped, and treatment with oral prednisolone was begun. Her signs, symptoms, and laboratory abnormalities then began to resolve. Subsequently, the syndrome was observed to return briefly in response to an oral challenge with minocycline. Conclusions: Minocycline is able to elicit a drug hypersensitivity syndrome that can resemble infectious mononucleosis. This drug reaction can be treated effectively by cessation of exposure to this drug and steroid therapy.

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088 Cryoglobulinemic neuropathy associated with lymphoplasmacytic lymphoma

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-anzan.87 ◽

2018 ◽

Vol 89 (6) ◽

pp. A35.3-A36

Author(s):

Yew Li Dang ◽

Emma Foster ◽

Moira Finlay ◽

Andrew Evans

Keyword(s):

Peripheral Neuropathy ◽

Upper Limb ◽

Early Recognition ◽

Oral Prednisolone ◽

Livedo Reticularis ◽

Lower Limbs ◽

Lymphoplasmacytic Lymphoma ◽

Hepatitis C Infection ◽

Limb Weakness

IntroductionA 64 year old woman presented with livedo reticularis and peripheral neuropathy secondary to Type 1 (IgM paraproteinemia) cryoglobulinemia, associated with lymphoplasmacytic lymphoma. Type 1 cryoglobulinemia is rare and remains poorly studied. However, given the common association of Type 1 cryoglobulinemia with lymphoproliferative diseases, it is important to consider Type 1 cryoglobulinemia as a differential diagnosis in presentations of a rash and peripheral neuropathy, and search for underlying malignancy.CaseOur patient presented with a leukocytoclastic vasculitic rash over bilateral lower limbs, which improved with topical betamethasone and oral prednisolone. Two months later, she had rapidly progressive right upper limb and bilateral lower limb weakness, absent reflexes in these limbs, and right arm reduced sensation. Nerve conduction studies revealed generalised axonal sensorimotor peripheral neuropathy in all four limbs, most prominent in the right upper limb. She then developed a livedo reticularis rash over her right forearm and punch biopsy revealed luminal pseudo-thrombi in small vessels consistent with Type 1 cryoglobulinemia. Her cryoglobulin (1558 mg/L, N 0–50 mg/L) and IgM paraprotein levels (4 g/L, N 0.4–2.3) were elevated. Vasculitic and other infective screens were unremarkable. Computer tomography imaging of chest, abdomen and pelvis found widespread lymphadenopathy. Subsequent lymph node core biopsy and bone marrow aspirate revealed lymphoplasmacytic lymphoma—the likely underlying cause of the Type 1 (IgM paraproteinemia) cryoglobulinemia.ConclusionPeripheral neuropathy is commonly associated with Type 2 and 3 cryoglobulinemia, especially in the presence of hepatitis C infection. However, reports of vasculitic peripheral neuropathy due to Type 1 cryoglobulinemia are limited. Early recognition is essential to allow the identification and treatment of the underlying haematological malignancy, commonly associated with Type 1 cryoglobulinemia. Treatment of the underlying cause indirectly treats the cryoglobulinemia, and avoids or reduces the associated; sometimes severe; cutaneous, neurological, and renal manifestations of this condition.

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EVALUATION RISK FACTORS OF NECROTIZING ENTEROCOLITIS

The Professional Medical Journal ◽

10.29309/tpmj/2018.25.05.300 ◽

2018 ◽

Vol 25 (05) ◽

pp. 647-653

Author(s):

Abdur Rehman Malik ◽

Ahmed Iqbal Quddusi ◽

Imran Iqbal ◽

Mukhtar Hussain Bhatti

Keyword(s):

Risk Factors ◽

Blood Culture ◽

Necrotizing Enterocolitis ◽

Occult Blood ◽

Liver Function Tests ◽

Cross Sectional Study ◽

High Morbidity ◽

Cross Sectional ◽

Maternal Risk ◽

First Feeding

Introduction: Necrotizing enterocolitis (NEC) is a condition where the intestinesbecome infected and can begin to die. Necrotizing enterocolitis is a serious condition that mayrequire surgery, and has a high morbidity and mortality rate. Objectives: To evaluate the riskfactors of necrotizing enterocolitis among neonates at children hospital Multan. Study Design:Descriptive Cross-sectional study. Setting: Neonatal Unit of Children Hospital and Institute ofChild Health Multan. Period: October 2015 to September 2016. Material and Methods: A total of79 neonates presented with necrotizing enterocolitis were enrolled for the possible causes. Theparameters studied included gestational age at birth, birth weight, maternal risk factors, patientrisk factors, age when feeding was started, type of feed, age when signs of NEC appeared,per feed increment, presence of any antecedent associations, clinical features, radiologicalfeatures, blood investigations (complete blood counts, blood glucose, serum sodium, serumpotassium, serum creatinine, liver function tests), ABGs, stool for occult blood and culture,septic screening and blood culture, management(medical or surgical) and outcome. The datawas analyzed using SPSS-20. Results: Out of a total of 79 neonates, 48 (60.8%) were maleand 31(39.2%) female. There were 71% infants who were younger than 32 gestational weeksand 67.7% under 1500 grams. The majority of neonates 62 (78.5%) commenced enteral feedswithin the first 24 hours. First feeding was started at a mean 5.6 ± 3.85 (2-17) days. Prematuritywas the commonest factor and present in 63 (79.9%) neonates. Abdominal distention was thecommonest symptoms 55% followed by bilious vomiting in 15%. Blood culture was positive in22 (27.8%) with predominance of gram negative microorganisms. According to Bell’s staging,54 (68.35%) neonates had stage I, 17 (21.5%) stage II, and 8 (10.1%) neonates were in stageIII. Conclusion: In most of the cases, the causes of necrotizing enterocolitis were presentand prematurity was the main etiological factor. Cautiously introducing enteral feeds usingexpressed maternal breast milk and increasing feed volumes slowly is important in reducingthe incidence amongst high risk individuals.

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A study on laboratory features of scrub typhus

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20183515 ◽

2018 ◽

Vol 5 (5) ◽

pp. 1828

Author(s):

R. Ramanathan ◽

E. Yazhini

Keyword(s):

Scrub Typhus ◽

Liver Function Tests ◽

Elisa Test ◽

Emerging Infections ◽

Rickettsial Infections ◽

Laboratory Investigations ◽

Hematological And Biochemical Parameters ◽

Wbc Count ◽

Hematological Changes ◽

Pediatric Icu

Background: Rickettsial infections are the most common re-emerging infections in the recent times especially Scrub typhus. If untreated, fatality rate is as high as 30-35%. Scrub typhus can cause changes in hematological and biochemical parameters. The need for this study is to correlate the laboratory investigations in patients with scrub typhus, so that early diagnosis and appropriate treatment can be done.Methods: This prospective observational study was carried out in children admitted in the pediatric ward/ pediatric ICU of RMMCH who were diagnosed to have Scrub typhus by positive ELISA test. Their basic laboratory investigations are categorized and correlated.Results: Normal Leukocyte count was seen in 48% (n=24) of the cases, leucocytopenia 30% (n=15), leucocytosis 22% (n=11). Platelets <1.5 lakhs were seen in 72% (n=36) and none of the cases had platelets less than 80000. Hb< 9gm/dl was seen in 44% (n=22). Hyponatremia in 24% (n=12). CRP was positive in 28% (n=14). Hypoalbuminemia was seen in 22% (n=11). Renal function test was normal in all the cases.Conclusions: This study discusses the various biochemical and hematological changes in patients with Scrub typhus. Pancytopenia is rare in patients with Scrub typhus. Abnormalities in renal and liver function tests may indicate the progression to complications. Normal WBC count with hyponatremia and hypoalbuminemia with related symptoms and signs can be used to diagnose the disease at earlier stages.

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Pyoderma gangrenosum – an interdisciplinary approach to the disease

HIGHER SCHOOL’S PULSE ◽

10.5604/01.3001.0012.7978 ◽

2018 ◽

Vol 12 (4) ◽

pp. 61-64

Author(s):

Aleksandra Kapuśniak ◽

Aleksandra Czachor ◽

Grażyna Wąsik

Keyword(s):

Pyoderma Gangrenosum ◽

Rapid Development ◽

Calcineurin Inhibitors ◽

Skin Lesions ◽

Neutrophilic Dermatosis ◽

Unknown Etiology ◽

Neutrophilic Infiltration ◽

Inflammatory Infiltration ◽

Laboratory Findings ◽

Immunological Mechanisms

Pyoderma gangrenosum is a rare dermatosis of unknown etiology. It is classified as being a neutrophilic dermatosis, in which there is inflammatory infiltration consisting primarily of mature polynuclear leukocytes. Its pathogenesis is multifactorial and is thought to involve neutrophilic dysfunction, inflammatory mediators in combination with a genetic predisposition for the disease. Neutrophilic infiltration is observed in new lesions, while necrosis associated with fibrosis and granulomas are seen in chronic lesions, however these findings are not pathognomonic. Pyoderma gangrenosum can occur at any age. However, it most commonly develops in young and middle-aged adults predominantly women between the second and fifth decades of life. Grossly, pyoderma gangrenosum is characterized by skin lesions in the form of rapidly spreading ulcers, with cylindrical edges and necrotic bottoms. These ulcers are painful and crusted but have undermined borders. Pyoderma gangrenosum commonly presents with the rapid development of one or more purulent ulcers with undermined borders on sites of normal or traumatized skin. Pyoderma gangrenosum is often associated with other systemic diseases such as ulcerative colitis, Crohn’s disease, monoclonal gammopathies, IgG or IgA myelomas and tumors of internal organs and hematopoietic system diseases, which supports the immunological mechanisms involved in the pathogenesis of the disease. Of note, neutrophilic infiltration associated with other extracutaneous manifestations and different systemic disorders can co-exist with pyoderma gangrenosum. Despite the recent development of immune modulating drugs in the treatment of skin conditions, steroid therapy still plays a pivotal role. For patients with mild pyoderma gangrenosum, the local application of topical corticosteroids or calcineurin inhibitors can be sufficient. Systemic therapy is necessary in patients with more extensive disease. The role of surgery is controversial, as it is associated with the induction of pathergy. The clinical, histopathologic and laboratory findings in pyoderma gangrenosum are non-specific, and a diagnosis can only be made once other diagnoses have been excluded.

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Sacral Tuberculosis: An Atypical Manifestation

IIUM Medical Journal Malaysia ◽

10.31436/imjm.v17i1.914 ◽

2018 ◽

Vol 17 (1) ◽

Author(s):

Faisal Amir Si Mirah ◽

Ahmad Faizal Roslan ◽

Ed Simor Khan Mor Japar Khan ◽

Rajandra Kumar Karupiah ◽

Zamzuri Zakaria @ Mohamad

Keyword(s):

Correct Diagnosis ◽

Lower Limbs ◽

Mantoux Test ◽

Ct Guided ◽

Walking Aids ◽

Grade 5 ◽

History Of ◽

Prolapsed Intervertebral Disc ◽

The Right ◽

High Index Of Suspicion

Sacral tuberculosis (TB) is extremely rare and its unusual entity might delay the diagnosis and treatment of this treatable disease. A 38-year-old lady presented with a 1 year history of lower back pain with radiculopathy more to right lower limb. The patient was initially treated as Prolapsed Intervertebral Disc (PID), but showed no improvement despite regular physiotherapy and medication. Subsequently, the pain was confined to the right gluteal area and became more severe. Quality of life was impaired in which patient started using walking aids and stopped working. There was a weight loss of 20kg. No other symptom of TB infection or history of contact with TB patient. Bowel and urinary functions were normal. Examination showed localized tenderness at the right gluteal area. Neurological assessment of both lower limbs were MRC grade 5. Blood investigations were normal including the ESR level (17mm/hour). Mantoux test was positive with 18mm induration. MRI revealed a large rim enhancing paravertebral collection at pre-sacral space which extended into bilateral piriformis and gluteal muscles. The patient underwent CT-guided drainage of both gluteals and specimens taken to confirm the diagnosis of TB. Patient showed significant improvement clinically within 1 week after the drainage procedure and initiation of antituberculous chemotherapy. The initial presentation of this patient mimics PID due to irritation of sciatic nerve at piriformis level. However a change in the presentation and failure of conservative treatment should raise a high index of suspicion and necessitates further investigation to establish correct diagnosis hence proper treatment can be initiated.

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Pyoderma gangrenosum with renal and splenic impairment - case report

Anais Brasileiros de Dermatologia ◽

10.1590/abd1806-4841.20132448 ◽

2013 ◽

Vol 88 (6 suppl 1) ◽

pp. 150-153 ◽

Cited By ~ 7

Author(s):

Luciana Rabelo de Carvalho ◽

Virgínia Vinha Zanuncio ◽

Bernardo Gontijo

Keyword(s):

Case Report ◽

Renal Impairment ◽

Pyoderma Gangrenosum ◽

Lower Limbs ◽

Neutrophilic Dermatosis ◽

Internal Organs ◽

Cutaneous Manifestations ◽

Neutrophilic Infiltrate ◽

Multisystemic Disease

Pyoderma gangrenosum is an uncommon and recurrent neutrophilic dermatosis of unknown cause. The lesions usually start as tender sterile papulopustules or erythematous nodules that undergo necrosis followed by ulceration. The lower limbs are most commonly affected and around half of the cases are associated with systemic disorders. Although rare, cases of pyoderma gangrenosum with extramucocutaneous sterile neutrophilic infiltrate have been reported, with the lungs being the most commonly affected organ. We report a case of pyoderma gangrenosum with splenic and renal impairment. Pyoderma gangrenosum should be considered a multisystemic disease with classic cutaneous manifestations and potential involvement of internal organs.

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