Dermatological Diseases

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• Spider bite presenting as fever, macrophage activation syndrome and a skin ulcer Marco Fedele, Mariangela Antonelli, Egidio Carbone, Marco Di Stefano, Raffaele Manna, Giovanni Addolorato Views: 236 HTML: 13 PDF: 146

  Introduction: Fever of unknown origin (FUO) refers to a condition of prolonged increased body temperature, without identified causes. The most common cause of FUO worldwide are infections; arthropod bites (loxoscelism) should be considered in view of the spread of the fiddleback spider. Loxoscelism can present in a cutaneous form (a necrotic cutaneous ulcer) or in a systemic form with fever, haemolytic anaemia, rhabdomyolysis and, rarely, macrophage activation syndrome (MAS). For this suspicion, it is important to have actually seen the spider.
  Case description: A 71-year-old man was admitted to our department because of intermittent fever, arthralgia and a necrotic skin lesion on his right forearm that appeared after gardening. Laboratory tests were negative for infectious diseases, and several courses of antibiotics were administered empirically without clinical benefit. Whole-body computed tomography showed multiple colliquative lymphadenomegalies, the largest one in the right axilla, presumably of reactive significance. A shave biopsy of the necrotic lesion was performed: culture tests were negative and histological examination showed non-specific necrotic material, so a second skin and lymph node biopsy was performed. The patient developed MAS for which he received corticosteroid therapy with clinical/laboratory benefit. Cutaneous and systemic loxoscelism complicated by MAS was diagnosed. Subsequently, the second biopsy revealed morphological and immunophenotypic findings consistent with primary cutaneous anaplastic large cell lymphoma (PC-ALCL).
  Conclusions: Skin lesions and lymphadenomegalies of unknown origin should always be biopsied. It is very common to get indeterminate results, but this does not justify not repeating the procedure to avoid misdiagnosis.

• The concomitant occurrence of hidradenitis suppurativa and Fournier’s gangrene Georges Khattar, Elie Bou Sanayeh, Marina Makram, Hussein Rabah, Saif Abu Baker, Salman Khan, Catherine Hong Views: 244 HTML: 9 PDF: 169

  Background: Fournier’s gangrene represents a life-threatening necrotising infection affecting the perineal region, while hidradenitis suppurativa is characterised by a chronic inflammatory skin condition. The simultaneous occurrence of both conditions is exceedingly rare.
  Case description: A 42-year-old female with a documented history of severe untreated hidradenitis suppurativa presented for shortness of breath, fever and lethargy, along with extensive wounds and skin breakdown involving the left axilla, perineum, lower back, lumbosacral region and bilateral gluteal areas, extending to the perineum. Upon presentation, the patient was in a state of septic shock, and a diagnosis of actively manifesting Fournier’s gangrene was established at the site of the pre-existing hidradenitis suppurativa lesions. Despite the implementation of an aggressive multidisciplinary approach incorporating surgical interventions, antibiotic therapy and intensive care measures, the patient’s condition deteriorated, culminating in septic shock, multi-organ failure and eventual demise. In this report, we discuss both clinical entities, their similarities and differences, and the possible mechanisms by which they may have co-occurred.
  Conclusion: The co-existence of hidradenitis suppurativa and Fournier’s gangrene poses unique challenges, given the rapid progression of Fournier’s gangrene within the context of hidradenitis suppurativa, potentially suggesting the latter as a predisposing factor. This case underscores the importance of vigilant screening and management of hidradenitis suppurativa.

• Annular leukocytoclastic vasculitis: a new feature of IgA vasculitis Nabil Belfeki, Nouha Ghriss, Sonia Kammoun, Souheil Zayet, Cyrus Moini, Benjamin Terrier Views: 153 HTML: 11 PDF: 153

  Background: Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases.
  Case description: A 36-year-old woman with no personal medical history presented with annular erythema with target lesions and petechial purpura. The patient had fever and joint arthralgia. A skin biopsy showed leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. The diagnosis of immunoglobulin A vasculitis with annular leukocytoclastic vasculitis was made. The patient showed global improvement with topical steroids without relapse.
  Conclusion: An annular variant of leukocytoclastic vasculitis is a rare manifestation of immunoglobulin A vasculitis.

• Kaposi’s varicelliform eruption: a potentially life-threatening complication of atopic dermatitis María Martín-Galache, Ana María Escalona-Gil, Luis Posado-Domínguez, Alejandro Jiménez-Domínguez, Alicia Arévalo-Cenzual, Francisco Javier López-Ávila, Eduardo Consuegra-Llapur Views: 404 HTML: 53 PDF: 269

  Introduction: Kaposi’s varicelliform eruption (KVE), also known as eczema herpeticum or eczema vaccinatum, is an acute dermatosis that affects patients with chronic dermatopathies. The diagnosis is primarily clinical and is characterised by the presence of a vesicular exanthema on physical examination. The exanthema subsequently evolves into crusted lesions with typical circular ulcerations in ‘punched-out’ areas on the skin affected by the underlying dermatopathy.
  Case description: We present the case of a 6-year-old patient who presented to the Paediatric Emergency department with skin lesions consistent with eczema herpeticum. The patient’s management was initially outpatient; however, due to the slow progression of the condition, hospitalisation and intravenous antiviral treatment were initiated.
  Discussion: KVE affects patients with chronic dermatoses, especially atopic dermatitis. It is important to know the clinical presentation for an early suspicion. KVE is a medical emergency that requires prompt diagnosis and treatment. It can progress to secondary viraemia, which can be fatal in up to 10% of immunocompetent individuals and up to 50% of immunocompromised individuals. It is important to be aware of this condition and to start early treatment with antivirals, especially given the high prevalence of atopic dermatitis in our population. This condition is one of the most serious complications that can occur in these patients.

• Uncommon thigh mass in neurofibromatosis type 1: unveiling aggressive epithelioid sarcoma Mohamed A. Gharbi, Faten Limaiem, Khaled B. Romdhane, Anis Tebourbi, Ramzi Bouzidi, Mouadh Nefiss Views: 155 HTML: 25 PDF: 128

  Background: Patients with neurofibromatosis type I (NF1) have an increased risk of developing soft-tissue sarcomas, particularly those related to the nervous system. Epithelioid sarcoma (ES) is an exceptionally rare subtype of soft-tissue sarcoma, with limited knowledge about its clinical presentation and optimal management in NF1. This report aims to provide insights into the characteristics and outcomes of ES in NF1 patients.
  Case description: A 37-year-old man with a history of NF1 presented with a progressively worsening mass on his right inner thigh. An MRI scan revealed a well-defined tissue mass originating from the adductor magnus muscle, later confirmed as ES through histopathology and immunohistochemistry. Considering poor local and general prognosis, the multidisciplinary team recommended salvage hip disarticulation, however the patient refused and opted for palliative marginal resection to reduce the tumour size. The patient’s condition declined rapidly, and he succumbed six days after the surgery.
  Conclusion: This case highlights the rarity of ES in NF1 patients and underscores the potential for malignant tumour development in this population. Further research is needed to improve our understanding and management of sarcomas in the context of NF1.

• Rumpel-Leede phenomenon following radial artery catheterisation Floyd A. Pirtle, Dylan Maldonado , Pitchaporn Yingchoncharoen, Jerapas Thongpiya, Natnicha Leelaviwat, Annia Cavazos, Cloyce L. Stetson Views: 585 HTML: 253 PDF: 316

  Rumpel-Leede phenomenon is a rarely diagnosed entity that can be seen in patients following the application of tourniquet-like forces to the extremities. This phenomenon describes petechiae and purpura secondary to venous compression and congestion, with its underlying aetiology involving the fragility of capillary vessels within the dermis. This condition is associated with chronic medical conditions such as diabetes mellitus, hypertension, dyslipidemia, peripheral vascular disease and systemic inflammatory diseases, including infections. In addition, patients with coagulopathy including thrombocytopenia or platelet dysfunction from antiplatelet use, or those with thrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura, are predisposed to capillary haemorrhage and petechiae formation. In this report, we present a case of a patient who developed Rumpel-Leede phenomenon following catheterisation of the right radial artery with spontaneous resolution – where only five cases have been reported to date – with the aim to make clinicians aware of this condition and to avoid unnecessary interventions.

• Wells syndrome with generalized oedema Magdalini Manti, Efthymia Pappa, Vasiliki Arkouli, Leonidas Marinos, Constantinos Christopoulos Views: 404 HTML: 62 PDF: 333

  Wells syndrome or eosinophilic cellulitis is a rare and relapsing skin disease which lacks systemic involvement. A skin biopsy is needed to establish a diagnosis. Several precipitating factors have been proposed but no proven causative link has been found. On the other hand, Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), an auto-immune disease, is associated with multiorgan, including cutaneous manifestations. We report a case with overlapping features of Wells and Churg-Strauss syndrome, suggesting that these syndromes could be part of the same nosological entity.

• Disseminated necrotic suppurative ulcers as a manifestation of cocaine abuse Álex García Tellado, Carmen Lasa Teja, María Rodríguez Vidriales, Mercedes De la Fuente Vázquez, Patricia Marín Oliván, Francisco Arnaiz Las Revillas Almajano Views: 352 HTML: 64 PDF: 339

  Introduction/Aims/Background: Pyoderma gangrenosum is an immune-mediated illness that can be caused by several affections, such as inflammatory bowel disease, rheumatoid arthritis, and drug use. We present a rare case of pyoderma gangrenosum induced by levamisole-adulterated cocaine. There have been few cases of this disease reported in the world. Levamisole is an anthelmintic drug used to adulterate cocaine to boost its effect. It also has immune-modulating effects causing, among others, vasculitis and dermatological problems.
  Materials and Methods: Clinical case of a 46-year-old man admitted to the hospital University Marqués de Valdecilla in Santander, Spain, in August 2022. We diagnosed pyoderma gangrenosum based on clinical, analytical, and histological parameters.
  Results: We report a case of pyoderma gangrenosum induced by consumption of levamisole-adulterated cocaine.
  Discussion: This patient suffered from a rare and extensive immune-mediated affection with characteristic primary lesions in the form of suppurative ulcers that responded to immunosuppressive treatment. Behind pyoderma gangrenosum there may be underlying conditions such as inflammatory bowel disease, or pyoderma gangrenosum may be secondary to identifiable causes such as cocaine use as in this patient.

• Facial cellulitis caused by Candida guilliermondi in an immunocompetent patient: a rare case report Ilir Tolaj, Visar Gashi, Gramoz Bunjaku, Murat Mehmeti, Hajrullah Fejza Views: 486 PDF: 370 HTML: 389

  Background: Candida cellulitis is a rare disease, primarily reported in immunocompromised patients. Atypical Candida spp. infections are increasing, largely due to the growing number of immunocompromised patients. This case report describes a 52-year-old immunocompetent patient with facial cellulitis caused by Candida guilliermondi. Candida guilliermondi has not previously been reported as a cause of facial cellulitis in either immunocompromised or immunocompetent patients.
  Case presentation: A 52-year-old, otherwise healthy, male patient presented with facial cellulitis that did not respond to intravenous antibiotics. Culture of the drained pus revealed Candida guilliermondi. The patient was successfully treated with intravenous fluconazole.
  Conclusion: This case highlights the possibility of atypical Candida spp. causing deep facial infections in immunocompetent patients. Candida guilliermondi has not been previously reported as a cause of facial cellulitis in either immunocompromised or immunocompetent patients. Healthcare providers should consider atypical Candida spp. infections in the differential diagnosis of deep facial infections in both immunocompromised and immunocompetent patients.

• Acute onset of bullous skin rash and acute kidney injury after exposure to radiocontrast: Sweet's syndrome Mahmoud Abdelnabi, Annia Cavazos, Corley Pruneda, Jerapas Thongpiya, Pitchaporn Yingchoncharoen, Sierra Sullivan, Jesus Davalos , Michele Tarbox Views: 735 HTML: 430 PDF: 507

  Sweet's syndrome or acute febrile neutrophilic dermatosis is characterized by an acute inflammatory skin eruption of oedematous and erythematous papules, plaques or nodules, accompanied by fever, and leucocytosis with possible extracutaneous involvement. Aetiologies include infections, inflammatory bowel disease, pregnancy or malignancy, or the syndrome may be drug-induced by many classes of medications or very rarely, radiocontrast exposure. Herein, the authors report a case of radiocontrast-induced bullous Sweet's syndrome and contrast-induced acute kidney injury in a woman in her 60s with a complex medical history.