scholarly journals Scleromyxedema (Arndt-Gottron Syndrome): a Case Report

2016 ◽  
Vol 8 (1) ◽  
pp. 28-38
Author(s):  
Danijela Popović ◽  
Mirjana Paravina ◽  
Dragan Jovanović ◽  
Vesna Karanikolić ◽  
Dragana Ljubisavljević

Abstract Lichen myxedematosus, also known as papular mucinosis, is a primary diffuse cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, also known as Arndt–Gottron syndrome, is a rare, confluent, papular and sclerotic variant of lichen myxedematosus, characterized by diffuse thickening of the skin underlying the papules. The condition is associated with systemic, even lethal manifestations, fibroblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients if appropriate or even repeat testing is used. Herein, we present a 67-year-old patient with a 2-year history of skin problems. He had no health problems other than hypertension and diabetes, wich were both diagnosed 15 years before. On examination, the patient exhibited sclerodermoid lesions with diffuse pseudo-sclerodermatous thickening of the exposed skin, microstomia and sclerodactyly-like changes; on the face, there were numerous solid, shiny 2 - 4 mm in diameter skin-coloured lichenoid papules, scattered across the forehead, glabellar area, nasolabial folds, perioral region, ear lobes and the neck. Histopathological examination revealed: highly distinctive collagenosis and fibrosis in the middle dermis, increased fibroblasts; collagen bundles with irregular arrangement and fragmentation; alcian blue-positive deposits with appearance consistent with acid mucins. Serum protein electrophoresis detected IgG lambda paraproteinemia. The patient was treated with systematic corticosteroids during 9 months with subsequent introduction of methotrexate and showed satisfactory results. The etiology of scleromyxedema remains unknown, since the purified IgG paraprotein itself has no direct effects on fibroblast proliferation. In scleromyxedema, numerous therapeutic modalities are proposed, unfortunatelly with limited effects. In colclusion, we report a case of an adult male with lichenoid papules; after a two-year progression, they evolved into scleromyxedema and exhibited well response to conventional therapy.

2017 ◽  
Vol 4 (4) ◽  
pp. 6
Author(s):  
Hendra Gunawan ◽  
Nina Roslina ◽  
Oki Suwarsa

Subcorneal pustular dermatosis (SPD) is a rare, chronic, and recurrent pustular eruption characterized histopathologically by subcorneal pustules that contain neutrophils. SPD has been clearly reported conjunction with other diseases. Leprosy reactions are acute inflammatory process that immunologically driven on the chronic course of leprosy. Erythema nodosum leprosum (ENL) is a type II of leprosy reaction putatively can initiate SPD lesions. We report one case of concomitant SPD and ENL in borderline lepromatous leprosy-relapses. A 41-year-old man with the history of using multidrug therapy-multibacillary for leprosy presented with painful erythematous nodules on the trunk and extremities, accompanied by pustules on erythematous base on the face, arms, buttocks, and legs. There were thickening of both ulnar nerves with gloves and stocking hypesthesia. The bacterial index was 3+ and morphological index was 20\%. Histopathological examination on the pustule revealed subcorneal pustules with exocytosis of neutrophils which supported the diagnosis of SPD. A possible immunologic mechanism has been suggested in the induction of the occurence both SPD and ENL.


Author(s):  
Sahar H. Alsharif ◽  
Reda H. Saifaldeen ◽  
Logain G. Alghanemi

<p class="abstract">Granuloma faciale (GF) is a chronic condition characterized by asymptomatic erythematous plaque with prominent telangiectasia presenting usually over the face. Although the condition is benign, its treatment is often unsatisfactory. Therapeutic modalities that have been tried include topical steroids and topical tacrolimus sometimes enhanced with topical dapsone. Others include intralesional corticosteroids, antimalarials, isoniazid and pulsed-dye laser. We report a case of a 58 years old female with a 1 year history of a solitary slowly progressive plaque over the nose. Diagnosis of GF was made based on the histopathological findings. The patient was started on the combination of topical tacrolimus, intralesional corticosteroids injection and oral doxycycline for 3 months. The patient showed gradual improvement in 3 months without any side effects. This case supports previous papers of successful treatment of GF with topical tacrolimus. There was no recurrence at follow-up 18 months later. It also supports the use of combination therapy especially in resistant cases.</p>


2020 ◽  
Vol 4 (1) ◽  
pp. 20-21
Author(s):  
Bruno de Oliveira Rocha

Introduction: Hemangiosarcoma is a rare vascular malignant neoplasm. Diagnosis is based on both clinical and histopathological features. Case Presentation: An 85-years-old white man presented with a 1-year history of progressive lesions on face and scalp. Physical examination revealed violaceous infiltrative plaques, with ulceration and bleeding, on the face and scalp, associated to numerous violaceous satellites papules. Histopathological examination revealed a neoplastic proliferation represented by slit-like anastomosing vascular channels that dissociated collagen fibers in the dermis, lined by atypical endothelium with moderately hyperchromatic and pleomorphic nuclei. Diagnostic conclusion was cutaneous angiosarcoma. Conclusions: Cutaneous angiosarcoma most often affects males (2:1), mostly Caucasian patients over 70 years old. It may originate from blood or lymphatic vessels. The most common form affects the face and scalp. There are varying degrees of cellular atypia, anastomosing slit-like channels, dissociation of collagen fibers, occasional hyaline globules and areas of hemorrhage. Immunohistochemistry confirmed blood vascular origin with diffuse positivity for CD34 and focally diffuse for factor VIII. We emphasize the need of clinicopathological correlation for early recognition and treatment.


2021 ◽  
Vol 14 (6) ◽  
pp. e241754
Author(s):  
Mafalda Crisóstomo ◽  
Mafalda Casinhas Santos ◽  
Ermelindo Tavares ◽  
Florbela Cunha

A 3-month-old, full term female infant, adequate for gestational age, and exclusively breastfed, was admitted with a 10 day history of generalised scaling erythematous dermatitis, affecting the face (perinasal, nasolabial folds and periauricular), acral and intertriginous areas, with irritability and failure to thrive. Her mother had been treated with isoniazid since the third trimester because of family contact with tuberculosis. Based on a diagnosis of suspected impetiginised eczema, the infant was treated with flucloxacillin and prednisolone, and maternal isoniazid was suspended, with no improvement. Investigations found low serum zinc levels in the infant (33 μg/dL; normal range (NR) >60 μg/dL), normal plasma zinc levels in the mother (111.3 μg/dL; NR 68–120 μg/dL) and lower than the normal range of zinc levels in breast milk (270μg/L; NR 1000–2500 μg/L), suggesting acrodermatitis caused by zinc deficiency. Oral zinc supplementation (3 mg/kg/day) was started with a marked improvement in skin lesions, as well as good weight gain. At the age of 6 months, after food diversification, supplementation was suspended, without any recurrence of symptoms.


2017 ◽  
Vol 8 (1) ◽  
pp. 288-293 ◽  
Author(s):  
Alena Furdova ◽  
Pavel Babal

Purpose: The aim of this report was to report a heroin abuser with nondiagnosed giant uveal melanoma infiltrating the orbit and prolapsing through the face. Methods: We conducted a case report of the patient with giant melanoma. Results: In March 2013, a 39-year-old male with bleeding from the right side of his face was taken to the Emergency Center of the University Hospital Bratislava by ambulance. The heroin abusing, homeless male who had never been treated before reported a 2-year history of a prolapsed, dark colored mass from his eye. Acute computed tomography confirmed a solid mass 20 × 20 cm prolapsing from the orbit. The patient rejected primary care, accepted only 1 transfusion and left the hospital. Two months later, he was admitted to the hospital due to anemia after many collapses. Liver metastasis was present. Surgical palliative therapy was not possible due to his general status. The patient started fractionated external radiotherapy with 8.0 Gy Co60. Histopathological examination of the biopsy specimen showed malignant melanoma of the epitheloid type G2–3. The patient died in December 2013. Conclusion: Extraorbital formation of a melanoma mass may progress to a giant volume, and without treatment, led to metastases in the liver and to death after 9 months in our patient with anemia.


2020 ◽  
Vol 13 (11) ◽  
pp. e236659
Author(s):  
Justin S Bhullar ◽  
Joseph MWS Leung ◽  
Mohammed S Almehthel

A 27-year-old otherwise healthy man of African descent presented to the hospital with initial symptoms of carcinoid syndrome that later evolved into symptoms of hyperinsulinemic hypoglycaemia. Investigations revealed a metastatic neuroendocrine tumour (NET), co-secreting both serotonin and insulin. Management involved a multimodal approach in an attempt to reduce tumour burden and achieve euglycaemia, which proved to be a significant challenge in the face of refractory hypoglycaemia despite the administration of multiple prohyperglycaemic agents in combination. Unfortunately, given the burden of metastatic disease and multiple medical complications that ensued, the patient passed away. This case highlights the clinical history of a NET co-secreting serotonin and insulin, the use of combination therapy in the treatment of refractory hypoglycaemia in a metastatic insulin-producing tumour and emerging therapeutic modalities in the treatment of these rare malignancies.


Author(s):  
Krishnendra Varma ◽  
Shashank Bhargava

<p>Nevus comedonicus is a rare skin disorder due to defect in development of hair follicle presenting as groups of closely set, dilated follicular openings with dark keratin plugs resembling comedones. The lesions may be distributed in a linear, interrupted, unilateral or bilateral pattern and sometimes follow the lines of Blaschko. It may develop inflammatory acne-like lesions leading to cysts, abscesses and scarring. A 36 year-old woman presented with multiple pits on left half of the body involving the face, upper limb, shoulder, back and buttock since the age of 4 years. She also had a painful swelling over the back. There was no history of cranial, skeletal or ocular involvement. Physical examination revealed multiple circular honeycomb-type pitted scars sized from 0.1×0.1 to 0.5×0.5 mm present unilaterally along lines of Blaschko in an interrupted pattern over the left half of the body as mentioned above. Among these pitted scars few scattered comedones were present with interfollicular normal skin. There was an abscess along the margin of one of the lesions over the back which was tender, indurated and with local rise of temperature. Histopathological examination revealed deep, wide invagination of acanthotic epidermis, filled with concentric lamellae of keratin. Hair shafts were occasionally seen in the dermis, below the lower part of invagination. Based on clinical and histopathological picture a diagnosis of nevus comedonicus was made. She was treated with topical tretinoin and oral retinoids without much improvement. Nevus comedonicus in itself is a rare epidermal nevus, its multi-dermatomal unilateral distribution along the lines of Blaschko is even rarer.</p>


VASA ◽  
2011 ◽  
Vol 40 (3) ◽  
pp. 251-255 ◽  
Author(s):  
Gruber-Szydlo ◽  
Poreba ◽  
Belowska-Bien ◽  
Derkacz ◽  
Badowski ◽  
...  

Popliteal artery thrombosis may present as a complication of an osteochondroma located in the vicinity of the knee joint. This is a case report of a 26-year-old man with symptoms of the right lower extremity ischaemia without a previous history of vascular disease or trauma. Plain radiography, magnetic resonance angiography and Doppler ultrasonography documented the presence of an osteochondrous structure of the proximal tibial metaphysis, which displaced and compressed the popliteal artery, causing its occlusion due to intraluminal thrombosis..The patient was operated and histopathological examination confirmed the diagnosis of osteochondroma.


Author(s):  
Sara Lynn Rependa ◽  
Robert T. Muller

This article discusses the case of a male vowed religious clergy, who was also in residential treatment for sexual misconduct and interpersonal difficulties. Importantly, this client also had a childhood history of sexual trauma. The case, difficult and complex in its own right, posed unique clinical challenges. The first author and therapist, a Catholic, feminist, woman often works with child trauma clients. Thus, the experiences of transference and countertransference were particularly important therapeutic considerations working with this client. Themes of power, sex, shame, guilt, and blame needed to be explored and processed in depth from the client’s and therapist’s perspectives both during session and supervision. Concurrent issues include personality disorders, physical disability, and psychosexual disorders. This client was referred by their religious institution and took part in a mandated fourteen to twenty-week residential programme. Therapeutic modalities include trauma-informed, attachment-oriented, and psychodynamic individual and grouporiented psychotherapy.


2016 ◽  
Vol 7 (3) ◽  
pp. 245-255 ◽  
Author(s):  
Michael J. Sandford

This article begins by outlining contemporary anti-work politics, which form the basis of Sandford’s reading. After providing a brief history of anti-work politics, Sandford examines recent scholarly treatments of Jesus’ relationship to work. An examination of a number of texts across the gospel traditions leads Sandford to argue that Jesus can be read as a ‘luxury communist’ whose behaviour flies in the face of the Protestant work ethic. Ultimately, Sandford foregrounds those texts in which Jesus discourages his followers from working, and undermines work as an ‘end in itself’, contextualising these statements in relation to other gospel texts about asceticism and the redistribution of wealth.


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