Cryptococcoid Sweet syndrome: a case report.

Cryptococcoid Sweet syndrome (cSS) is a recently described clinical and histological variant of Sweet syndrome (SS). Its cutaneous presentation is similar to the classical form of SS but it includes atypical findings, such as capsular and yeast-like structures on microscopy that are reminiscent of Cryptococcus species. However, in cSS, fungal staining and cultural examination are negative, whereas myeloperoxidase (MPO) staining on biopsy specimens is typically positive. Due to the rarity and the diagnostic challenge represented by this disease, its extracutaneous involvement, and the latency in its diagnosis, this condition is frequently associated with poor prognosis. In this study, we report the case of a cSS patient with a positive outcome.

Clinicopathological Profile of Pyoderma Gangrenosum: A 10-Year Retrospective Study from a Tertiary Care Center in South India.

Background: Pyoderma gangrenosum (PG) is a reactive neutrophilic inflammatory dermatosis with a varied clinicopathologic presentation. It commonly manifests as rapidly progressive painful ulcers, mimicking varied conditions including infections, vasculitis, and malignancies, and is a diagnosis of exclusion. There are scarce data on PG from the Indian subcontinent. Aim and Objectives: The aim of the study was to study the clinicopathologic profile of patients with PG and their underlying systemic associations. Materials and Methods: A retrospective observational study was done between 2011 and 2021, and patients diagnosed as PG based on the diagnostic tool proposed by Maverakis et al. were recruited and their demographic, clinical, and histological findings were obtained. Results: Among 54 patients with suspected PG, 17 patients (eight males and nine females) fulfilled the diagnostic criteria, and the mean age of disease onset was 32.1 years (range: 3-60 years). Ulcerative variant was the most common type (9/17, 52.9%), and 29.4% had systemic associations including autoinflammatory syndromes. The onset at atypical sites such as face and hand were noted in one patient each. Histopathology revealed a polymorphous dermal infiltrate with neutrophilic predominance in the majority (94.1%). Systemic steroids (dose ranging from 0.5-1 mg/kg prednisolone equivalent) were used in 11/17 (64.7%) patients. The commonly used alternative drugs included clofazimine (47%), minocycline (29%), thalidomide (23.5%), adalimumab and mycophenolate mofetil in 17.6% each, dapsone and ciclosporine in 11.7% each. Remission was achieved between two weeks and three months in 10 (58.8%) patients after treatment initiation and two mortalities (11.7%) were recorded. Conclusion: PG can affect any age group and may be localized to rarer, atypical sites. The possibility of underlying autoinflammatory conditions should be considered in addition to the evaluation of other disorders like inflammatory bowel disease, hematological disorders, and rheumatological disorders.

Sweet Syndrome Presenting with Features of Cellulitis Shortly after Femoral Angioplasty.

Neutrophilic dermatosis, or Sweet syndrome, is a cutaneous disorder caused by neutrophilic infiltration in the upper dermis. It has been associated with medications, infections and malignancies but to date it has not been associated with femoral arterial angioplasty or stenting. We present the case of a 75-year-old female who, after angioplasty and stent placement of the right superficial femoral artery, developed right heel pain with ulceration that did not respond to broad antibiotics. She underwent incision and drainage twice without improvement; both times produced negative cultures. She then underwent a punch biopsy by dermatology, which was consistent with acute spongiotic and other neutrophilic dermatoses. She was started on prednisone with immediate improvement of her symptoms. She was discharged to a rehabilitation centre with a prednisone taper and antibiotics. This report highlights the importance of maintaining Sweet syndrome on the differential for cellulitis as it is a rare mimicry of other infectious and non-infectious aetiologies, which are common in the perioperative space. Early treatment is crucial to improve symptoms, outcomes, healthcare cost and potentially the length of stay. LEARNING POINTS: Sweet syndrome, a rare skin condition related to neutrophil infiltration, may be triggered by angioplasty.Sweet syndrome is easily misdiagnosed as infectious conditions such as cellulitis.

Clinical features, treatment, and outcome of granulocyte colony stimulating factor-induced sweet syndrome.

BACKGROUND: Sweet syndrome (SS) is a rare dermatological adverse reaction caused by granulocyte colony-stimulating factor (G-CSF), but the characteristics of G-CSF-induced SS are unclear. This study aims to elucidate the characteristics of G-CSF-induced SS and offer guidance for its prevention and management. METHODS: We collected relevant case reports of G-CSF-induced SS by searching pertinent databases until June 30, 2024, and synthesized the data for retrospective analysis. RESULTS: A total of fifty patients were analyzed, with a median age of 44 years (1.7-77). The onset of SS occurred between 2 and 90 days post-administration, with a median onset time of 7 days. The predominant cutaneous manifestations included papules/plaques (74.0%), nodules (32.0%), and vesicles/bullae (24.0%). Fever presented in 74.0% of cases, while extra-cutaneous symptoms appeared in 32% of patients. Skin biopsy revealed key findings such as dermal diffuse neutrophil infiltration (97.8%), leukocytoclasis (19.1%), and dermal papillary edema (27.7%). Following both the cessation of G-CSF and systemic corticosteroids treatment, patients showed symptomatic improvement at a median interval of 7 days (2-70). CONCLUSION: Clinicians should remain vigilant for the risk of SS during G-CSF administration. Skin biopsy plays a crucial role in confirming SS diagnosis. G-CSF-induced SS exhibits a favorable response to corticosteroids, and re-administration of G-CSF should be avoided due to the risk of symptom recurrence.

Chronic Treatment-Resistant Annular Rash: A Case of Neutrophilic Figurate Erythema.

Neutrophilic figurate erythema (NFE) is a rarely reported figurate erythema that clinically presents similarly to erythema annulare centrifugum (EAC) with neutrophil-predominant perivascular and interstitial infiltrate in the dermis on histopathology. We present the case of a 32-year-old active-duty military male who presented with a chronic treatment-resistant skin rash. The rash began on his thighs five years previously and was treated with topical and oral antifungals repeatedly without improvement. The patient was deployed overseas during the rash onset, but the rash persisted upon his return stateside. No triggers were identified. His persistent skin eruption consisted of erythematous polycyclic annular plaques with a "trailing edge" scale. Histologic examination revealed perivascular neutrophils and perivascular and interstitial eosinophils without signs of vasculitis or infection. With only 15 reported cases, it can be difficult to recognize leading to long delays in diagnosis and treatment. Despite having a clinical course similar to EAC, NFE may require anti-neutrophil therapy to resolve completely.

Longitudinal Presentation and Management of Neutrophilic Dermatosis of the Dorsal Hand: A Case Report Over Six Visits.

Neutrophilic dermatosis of the dorsal hands (NDDH) is a rare, challenging condition characterized by its acute febrile onset, neutrophilic dermal infiltrate seen on histology, and frequent association with hematologic disorders. This case report presents a longitudinal follow-up of NDDH in an 80-year-old male with significant comorbid conditions, including atrial fibrillation, chronic systolic heart failure, venous insufficiency, and gastroesophageal reflux disease. Over six clinic visits, we document the patient's clinical progression, highlighting key changes in presentation, diagnostic findings, and the multifaceted approach to management, including the use of systemic and topical therapies. Notably, the treatment strategy was adapted in response to diagnostic tests revealing methicillin-resistant Staphylococcus aureus (MRSA) and Group B Streptococcus (GBS), complicating the patient's condition. Despite the challenges posed by the patient's comorbidities, which limited the use of certain medications, the patient showed significant improvement with a treatment approach primarily involving topical and systemic steroids. The observed improvement emphasizes the effectiveness of modifying therapy based on lesion regression and the patient's overall condition. This case underscores the critical need for accurate diagnosis, the complexities of managing NDDH in patients with multiple health conditions, and the value of adaptability in treatment plans. Through this detailed longitudinal case study, we contribute insights into the progression and management of NDDH, emphasizing the need for ongoing research and a flexible approach to treatment.

Disseminated Yersinia enterocolitica infection associated to Sweet's syndrome.

We report the case of a young woman sough care for disseminated form of Yersinia enterocolitica infection (pseudoappendicitis with mesenteric lymph node, arthralgia, glomerulonephritis and hepatitis) diagnosed on Western Blot method for the detection of Yersinia antibodies. The patient also presented a rare concomitant cutaneous manifestation, as Sweet's syndrome, confirmed histologically. Neutrophilic dermatosis is an exceptional skin features among post-infectious autoimmune disorders when encountering Yersinia enterocolitica infection in clinical practice.

Decoding the Histopathology of Pustular Pyoderma Gangrenosum: A Multidisciplinary Approach to Diagnosis.

This article discusses the rare neutrophilic dermatosis pustular pyoderma gangrenosum (PG), characterized by necrotizing skin lesions. It highlights the importance of thorough histological examination in diagnosing this variation, which resembles other pustular dermatoses. The case study of a 54-year-old female highlights the unique histological aspects of PG, including epidermal erosion, neutrophilic infiltration, sterile abscesses, and no vasculitis. The article emphasizes the need for differential diagnosis and clinical correlation, emphasizing the importance of collaboration between physicians and pathologists for accurate diagnosis.

[Challenges in diagnosing familial Mediterranean fever: exploring atypical clinical features. Clinical case].

This clinical case series presents descriptions of 3 patients with familial Mediterranean fever (FMF) who have atypical manifestations and abnormal inheritance mechanisms in terms of Gregor Mendel's laws. Although molecular genetic testing can help with disease diagnosis, it is not always conclusive. The primary need for genetic testing in atypical cases is to explain the mechanism of inflammation and to select the optimal therapy. These clinical observations demonstrate the changes in the spectrum of phenotypic manifestations of FMF in the context of the widespread introduction of molecular genetic methods.

Pyoderma Gangrenosum: A Presenting Feature of Rheumatoid Arthritis.

Pyoderma gangrenosum (PG) is an uncommon inflammatory disorder that exhibits a range of clinical manifestations and levels of severity. It frequently occurs alongside an underlying condition, most often inflammatory bowel disease. PG, Sweet syndrome, palisaded neutrophilic granulomatous dermatitis (PNGD), interstitial granulomatous dermatitis (IGD) and rheumatoid neutrophilic dermatitis may be associated with rheumatoid arthritis (RA). We present a case of a 65-year-old woman with disseminated dermatosis to the hands, abdomen, buttocks, and lower limbs. The dermatosis presented with numerous ulcers of varying shapes, featuring clean bases, undermined edges, and a purplish erythematous appearance. Further investigations, including imaging studies and RA factor and anti-cyclic citrullinated peptide (anti-CCP) levels, led us to the diagnosis of RA. This case indicates that RA may be frequently undiagnosed and untreated in other patients with PG, as ulcers on the lower extremities can often be the main reason for seeking medical attention.

Pyoderma Gangrenosum in a 75-Year-Old Male With Follicular Diffuse Large B-cell Lymphoma.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that characteristically presents with progressive ulcerative lesions. The association of PG with hematological malignancies remains unclear due to its varied clinical presentation. Herein, we report the unusual case of PG in a 75-year-old male with stage III follicular diffuse large B-cell lymphoma. Seven days subsequent to his first dose of rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone (R-CHOP) therapy, he presented to the emergency department with generalized malaise, bilateral lower extremity edema, and ecchymoses with ulcerative wounds on the dorsal of his feet. Due to the rapid progression of the patient's dermatological manifestations and declining clinical status, he required serial surgical wound debridement and a biopsy, which revealed an occlusive vasculopathy with dermal and epidermal necrosis. These pathological findings, along with the patient's clinical presentation, led to the diagnosis of PG. The patient was treated with negative pressure wound therapy, steroids, and tacrolimus ointment, which led to a marked improvement in the appearance of the patient's dermatological features and clinical status.

Crusted plaques on the face.

Facial neutrophilic dermatosis is a relatively recent and rare entity. Herein, we report an original case with a clinical appearance of crusty pyoderma gangrenosum and limited face involvement, classifying it as neutrophilic dermatosis (ND) of the face. The latter is an infrequent entity and forms part of the ND spectrum. The few reported cases in the literature are characterized by a restricted distribution on the face, as in our case. Nevertheless, this entity remains controversial: some consider it a variant of Sweet's syndrome; others see it as a truly independent entity. Matthews et al. reported an association between ND of the face, as in the case of our patient with a crusty appearance, and ulcerative colitis. Our observation and that of Matthews et al. underline the wide and varied potential for the clinical presentation of this entity.

[Pyoderma gangrenosum after orthopedic conservative treatment]. / Pyoderma gangrenosum après traitement conservateur orthopédique.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. Half of the cases are associated with an immune dysfunction and are frequently triggered by pathergy such as a tissular aggression via surgery or burn wounds. A patient with ulcerative colitis presented a PG at the site of an iontophoresis patch for tendinopathy. Treatment in a specialized burn center, corticosteroid therapy and adapted local care contributed to a favourable evolution. PG remains a diagnosis of exclusion and inflammatory phenomena must be differentiated from infectious causes such as necrotizing fasciitis to initiate immunosuppressive treatment. Being rare and difficult to diagnose and to treat as well as associated with potentially severe sequelae, a multidisciplinary team is required for the management of PG.

Le Pyoderma gangrenosum (PG) est une dermatose neutrophilique rare. Il est, dans la moitié des cas, associé à une maladie dysimmunitaire et il est fréquemment déclenché par un phénomène de pathergie, défini comme une agression tissulaire par une intervention chirurgicale ou encore une brûlure. Une patiente avec une rectocolite ulcéro-hémorragique a développé un PG sur le site d'application d'un patch d'ionophorèse pour une tendinopathie. Un traitement par une corticothérapie, un traitement immunosuppresseur local et des soins locaux adaptés ont permis une évolution favorable. Le PG reste un diagnostic d'exclusion et les phénomènes inflammatoires doivent être différenciés de phénomènes infectieux, comme la fasciite nécrosante, afin d'initier rapidement des immunosuppresseurs. Comme il s'agit d'une pathologie rare avec un diagnostic difficile, que des séquelles peuvent être catastrophiques et qu'un traitement immunosuppresseur complexe doit être instauré, une équipe pluridisciplinaire est requise pour la prise en charge de cette pathologie.

Sweet syndrome associated with moderate leukocyte adhesion deficiency type I: a case report and review of the literature.

Sweet syndrome is an acute febrile neutrophilic dermatosis characterized by the infiltration of neutrophils into the skin. It may occur idiopathically or be linked to malignancies, inflammatory or autoimmune diseases. Leukocyte adhesion deficiency type I (LAD-I) is an inborn error immunity wherein leukocytes lack adhesion molecules necessary for migration to infection sites due to mutations in the CD18 gene encoding ß2 integrins. We present a case of a 16-month-old female initially diagnosed and treated for Sweet syndrome based on histopathological findings with recurrent flare episodes. Subsequent workup revealed LAD-I, making this case the first documented association between Sweet syndrome and LAD-I. Moreover, we reviewed the pertinent literatures detailing the concurrence of neutrophilic dermatosis and immunodeficiency disorders. This case underscores the significance of comprehensive evaluation for Sweet syndrome patients who are refractory to conventional treatments.

Bowel-associated dermatosis-arthritis syndrome, a diagnostic challenge in a young child with very early-onset inflammatory bowel disease.

A 14-month-old girl with very early-onset inflammatory bowel disease (VEO-IBD) was admitted with a flare of her bowel disease and subsequently developed high fevers, joint pain, and skin lesions during her hospitalization. Workup demonstrated bowel-associated dermatosis-arthritis syndrome in the setting of VEO-IBD, a neutrophilic dermatosis rarely reported in children that can be challenging to diagnose and treat, with limited literature for patients under 2 years of age.

Neutrophilic eccrine hidradenitis from granulocyte-stimulating factor in a patient with ANCA vasculitis.

Neutrophilic eccrine hidradenitis (NEH) is one of the cutaneous manifestations of chemotherapy side effects. However, it can arise from other non-chemotherapy medications. The granulocyte-colony-stimulating factor is a medication reported to trigger NEH.