Search

Abstract Erdheim–Chester disease (ECD) is a rare, non‐Langerhans histiocytic disease, with the manifestation of cutaneous lesions becoming further recognised and understood. Most commonly presenting with xanthelasma‐like lesions, cutaneous manifestations are the first noticeable sign of ECD in a significant number of patients. Other commonly reported cutaneous lesions of ECD include panniculitis‐like lesions and granuloma annulare‐like lesions. While previously reported papular lesions of ECD include crusty yellow and erythematous papules, small, pink to fleshy coloured papules, and verruca plana‐like papules, papulonodular eruptions consistent with fibrous histiocytomas are a rare and underreported sequala of ECD. Here, we report an 86‐year‐old male with a history of prostate and bladder cancer who presented with eruptive fibrous histiocytomas, prompting workup that lead to a diagnosis of ECD. The patient received expedited imaging given the rare association of eruptive fibrohistiocytic lesions with malignancy, revealing diffuse perinephric and urothelial soft tissue thickening and enhancement, which was biopsied and found to harbour the BRAF V600E mutation. One could reasonably hypothesise that the pathologic mechanism occurring in the perinephric and urothelial soft tissue areas of this patient bodes similarities to the cutaneous sites consistent with the fibrohistiocytic lesions. This may present a potential clue to the poorly understood origin and pathogenesis of ECD.

Eosinophilic dermatosis of hematologic malignancy (EDHM) is a dermatosis characterized by tissue eosinophilia that has been previously reported as insect bite-like reaction. It is a rare condition with a wide variety of clinical presentations ranging from papules, nodules, or blisters that simulate arthropod bites, to the formation of plaques of differing sizes. We report a case of eosinophilic dermatosis of hematologic malignancy in a patient with a hematoproliferative disorder.

Disseminated cutaneous gout is a rare atypical cutaneous manifestation of gout in which widespread dermal and subcutaneous tophi develop at extra-articular body sites. Given the lack of joint involvement that is typically a feature in tophaceous gout, the diagnosis may not be initially suspected. We present the case of a 50-year-old Hispanic man with poorly controlled gout who was evaluated for several years of firm papulonodules over the trunk and upper and lower extremities, sparing the joints; histopathology confirmed, the diagnosis of disseminated cutaneous gout. Per our literature review, disseminated cutaneous gout presents with polymorphous papules and nodules that can mimic other, more common cutaneous diseases. There is a preponderance of cases in males, Asians, and patients with longstanding gout. The lower extremities are involved in nearly all reports. Uric acid-lowering therapy with allopurinol has been reported to decrease the size and number of lesions in a minority of treated patients.

The article discusses a rare case of disseminated adult xanthogranuloma (MAXG) in a 48-year-old male with B-cell acute lymphoblastic leukemia. MAXG is a form of non-Langerhans cell histiocytosis that presents as multiple orange-brown papules and nodules. The patient's lesions were excised, and although most cases of MAXG are S-100 negative, this case showed a mixture of markers. The article emphasizes the importance of monitoring for hematologic abnormalities in patients with MAXG, as it may be associated with underlying hematologic malignancies. [Extracted from the article]

The article explores the use of skin barrier repair as a novel therapeutic strategy for Chronic Kidney Disease-associated pruritus (CKD-aP), a condition that affects up to 60% of individuals with end-stage renal disease. The authors highlight the importance of addressing skin barrier dysfunction, which contributes to the persistence of itch and inflammation in CKD-aP patients. While skin barrier therapies show promise in alleviating pruritus, a personalized approach to treatment may be necessary due to the heterogeneity of CKD-aP. Further research is needed to optimize these therapies and understand their mechanisms of action within a comprehensive treatment strategy for CKD-aP. [Extracted from the article]

Nodular cutaneous amyloidosis (NCA), the leastcommon form of primary cutaneous amyloidosis, ischaracterized clinically by waxy, purpuric plaques andnodules and histologically by amyloid deposits in thedermis and subcutaneous tissue. We present a patientwho developed multiple, non-contiguous NCA lesionsover a three year period without evidence of systemicdisease. We reviewed the literature and found fewother cases of this unusual presentation.

A Sister Mary Joseph's nodule (SMJN) is characterized by a palpable umbilical nodule and is often a clinical indicator of the metastasis of an advanced abdominal or pelvic malignancy. Observing the cutaneous manifestation of an abdomino-pelvic malignancy is a relatively rare phenomenon due to the appearance of visible changes in the later stages of the disease. With the pancreas being a less common primary tumor site for SMJN, this case report describes a 57-year-old male diagnosed with metastatic pancreatic adenocarcinoma with a SMJN. With a history of alcohol use disorder and alcohol pancreatitis, this patient presented to the Emergency Department with worsening abdominal pain and vomiting. A computed tomography (CT) scan with intravenous (IV) contrast revealed a mass in the patient's rectum and a lobulated mass traversing the anterior abdominal wall, which extended into the umbilicus. A physical exam revealed the presence of an umbilical lesion. A biopsy of the umbilical lesion revealed metastatic adenocarcinoma that favored pancreaticobiliary origin. The results of the umbilical biopsy and CT imaging established the diagnosis of SMJN secondary to pancreatic cancer metastasis to the umbilicus., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Custer et al.)

Background Sweet's syndrome ( SS) is a neutrophilic dermatosis characterized by the abrupt onset of cutaneous, systemic and histopathological alterations in response to different stimuli. Objectives The aim of this study was to assess the epidemioclinical, histological, and therapeutic features and outcomes of SS. Methods A retrospective study of all patients diagnosed with SS over a 20-year period (1993-2012) was conducted. Data were analyzed using a level of significance of 5%. Results Ninety patients (mean age: 46.5 years) fulfilled the inclusion criteria. The ratio of women to men was 5. Significant associations emerged between dermohypodermic nodes and location on the lower limbs ( P = 0.042), and vesiculobullous lesions and location on the legs ( P = 0.030), dorsum of the hand ( P = 0.015), and forearms ( P = 0.003), and paraneoplastic forms ( P = 0.012). The upper extremities were involved in the majority of patients (83.3%). Correlations were found between edema of the superficial dermis and vesiculobullous lesions and between leukocytoclastic vasculitis and atypical targetoid lesions. Sweet's syndrome was associated with cytomegalovirus infection ( n = 1), inflammatory bowel disease ( n = 4), neoplasm ( n = 6), and pregnancy ( n = 3). First-line treatment consisted of colchicine. Conclusions To the best of the present authors' knowledge, this is the largest series of SS to be reported. Clinical presentations are similar to those described in the literature. Colchicine was efficient and facilitates the reduced use of corticosteroids. The association between SS and neoplasms should be considered in the context of vesiculobullous lesions. [ABSTRACT FROM AUTHOR]

Neuromyelitis optica (NMO) is an autoimmune, relapsing central nervous system demyelinating disease. There is a known association between NMO and autoimmune disease. However, cutaneous findings in these cases and in the setting of isolated NMO have rarely been described. We report the case of a 60-year-old Chinese female who presented with nonspecific cutaneous findings and acute onset paraplegia and was subsequently found to be seropositive for aquaporin-4 (NMO IgG) antibodies, consistent with a diagnosis of NMO spectrum disorder. Serologic testing prompted by history and cutaneous stigmata revealed additional humoral derangements, together suggestive of an overlap syndrome with features of amyopathic dermatomyositis, rheumatoid arthritis and lupus erythematosus. We review the existing literature on the cutaneous manifestations of this entity. Awareness of the cutaneous signs and heralding symptoms of this devastating neurologic syndrome by dermatologists will ensure prompt diagnosis and initiation of treatment that can minimize neurologic sequelae. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

We present a case of anti-melanoma differentiation-associated gene 5 (Anti-MDA5) dermatomyositis (DM) in a 30-year-old female. Anti-MDA5 dermatomyositis, previously termed clinically amyopathic dermatomyositis, was first recognized in 2005. Most cases present with varying combinations of cutaneous and oral ulcerations, palmar papules, respiratory symptoms, and minor muscle involvement (most commonly in the shoulders, upper arms, hips, thighs, and neck). This subtype of disease is most notable for its association with an increased risk of rapidly progressive interstitial lung disease. Our patient presented initially with only complaints of cutaneous ulcerations on the dorsal aspect of her hands. Following several months of no true diagnosis, she developed muscle weakness and joint pain. This led to retrieval of a punch biopsy which suggested anti-MDA5 DM at the top of the differential diagnoses. Immunoprecipitation revealed the presence of melanoma differentiation-associated gene 5 (MDA5) antibodies, confirming the diagnosis of anti-MDA5 dermatomyositis. This case demonstrates the importance of pinpointing the diagnosis of this rare disease subtype in a timely manner to prevent a fatal course, and we hope to inform dermatologists, rheumatologists, pulmonologists, and internists alike of the uncommon presentation of anti-MDA5 in an unsuspected, young patient., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Ruppel et al.)