Worldwide Distribution and Extracutaneous Manifestations of Henoch-Schönlein Purpura in Adults: Narrative Review.

BACKGROUND: Henoch-Schönlein purpura (HSP), a leukocytoclastic small vessel vasculitis, exhibits both cutaneous and systemic manifestations. While predominantly observed in childhood, it may manifest in adults with more pronounced systemic involvement. Furthermore, HSP is a global phenomenon showcasing epidemiological and systemic variances. OBJECTIVE: This study aims to scrutinize extracutaneous manifestations in adults with HSP, discerning distinctions according to geographical regions on a worldwide scale. METHODS: A comprehensive search encompassing PubMed, Embase, Cochrane Library, and Web of Science was executed, covering papers published from January 1, 1970, to December 1, 2019. Keywords used included "Henoch-Schönlein purpura," "henoch schonlein purpura+adult," "IgA vasculitis+adult," "HSP+adult," and "IgAV." A total of 995 publications were identified, from which 42 studies encompassing 4064 patients were selected, with a predominant focus on cases reported in Asia, Europe, and the Americas. RESULTS: Among adults afflicted with HSP, European patients exhibited a higher propensity for male predominance (P<.001), gastrointestinal involvement (P<.001), and musculoskeletal complications (P<.001). Conversely, patients from the Americas were least likely to experience genitourinary involvement (P<.001). CONCLUSIONS: HSP demonstrates a variance in distribution and extracutaneous manifestations within distinct geographical boundaries. In the adult population, European patients exhibited a higher prevalence of male gender and gastrointestinal and musculoskeletal involvement. Asian patients were more predisposed to genitourinary involvement when compared to their American counterparts. The establishment of prospective studies using standardized reporting measures is imperative to validate the relationships unveiled in this investigation.

Incidence of Squamous Cell Carcinoma in Situ Upstaged to Invasive Squamous Cell Carcinoma During Mohs Surgery.

Objective: We sought to record the incidence and risk factors associated with upstaging squamous cell carcinoma in situ (SCCIS) to squamous cell carcinoma (SCC) during Mohs surgery with the largest sample size to date. Methods: Patient records of preoperative biopsy-proven SCCIS being treated with Mohs between January 2019 to March 2022 were identified and reviewed. Postoperative diagnoses of invasive SCC proven by dermal infiltration on pathology were identified as upstaged SCCIS. Results: From 2,043 cases of preoperative diagnosed SCCIS, 47 (2.3%) were upstaged to SCC during Mohs surgery. Of the 47 invasive tumors, a large proportion on the hands (29.8%) and lesions with larger preoperative sizes had a higher risk of being upstaged to invasive SCC in this study. Limitations: All of the patients included were from rural and suburban areas of North Carolina. The only sections obtained were those reviewed for margin analysis, which may significantly underestimate the actual number of invasive SCC, as only the deepest and furthest portions were examined. Conclusion: This retrospective study concluded that 2.3 percent of preoperatively diagnosed SCCIS were upstaged to SCC during treatment with Mohs surgery. Large lesions (>2cm) and lesions on the hand were more likely to be upstaged (29.8%). Treatment must be individualized considering the size of the lesion, the anatomic location, and the possibility that in some cases the initial biopsy may not have been able to accurately distinguish SCCIS from SCC. Although there is a myriad of treatment options for SCC, select patients with increased risk factors for upstaged SCC must be considered for margin assessed treatment modalities.

Cutaneous Manifestations of Sarcoidosis Seen in a Patient with a History of Tuberous Sclerosis.

This case report details a patient with a history of tuberous sclerosis presenting with new-onset cutaneous lesions that turn out to be sarcoidosis. There may be a shared dysfunction of mTOR present in sarcoidosis and tuberous sclerosis. As a dermatologist, it is worth understanding the cutaneous manifestations of both diseases and maintaining a wide differential when new lesions arise in a patient with a history of either disorder.

Cutaneous Plasmacytosis Associated with Ehrlichiosis in an African-American Patient.

Cutaneous plasmacytosis is a rare disease that presents clinically with multiple red-brown papules and plaques with minimal to no epidermal change. Histopathologic findings include a perivascular dermal infiltration of polyclonal plasma cells. The etiology of cutaneous plasmacytosis is unknown, but hypothesized to be due to persistent or repeated antigenic stimulation. Ehrlichia represents a family of obligate intracellular bacteria that have been associated with the development of plasma cell dyscrasias in the veterinary literature. We present a case of a 67-year-old male patient with the development of progressively worsening cutaneous plasmacytosis following prolonged hospitalization secondary to ehrlichiosis sepsis. The patient initially presented with isolated cutaneous involvement and normal laboratory findings that eventually progressed to include multiple laboratory abnormalities, including anemia, hyperproteinemia, and elevated serum creatinine. Further diagnostic workup was declined by the patient despite evidence of progression to systemic plasmacytosis or multiple myeloma.

Topical Imiquimod and Subsequent Erythema Multiforme.

Topical imiquimod is commonly used in the nonsurgical management of actinic keratosis and superficial basal cell carcinoma. Although adverse effects have been limited primarily to local irritation, another rare adverse reaction is erythema multiforme. We present a case of erythema multiforme involving the oral mucosa, trunk, and extremities that followed broad application of topical imiquimod for the management of suspected superficial basal cell skin cancers and actinic keratosis. The patient had used imiquimod previously without complication. Cessation of use and systemic corticosteroids resulted in prompt clearance.

Adult Henolch-Schonlein purpura: multiorgan failure in the setting of a purpuric rash.

We report a 66-year-old man with a history of congestive heart failure, atrial fibrillation on warfarin therapy and chronic kidney disease that presented with acute dyspnoea. He had multiple palpable purpuric lesions on his bilateral lower extremities. Laboratory findings supported acute anaemia with no obvious bleeding source, supratherapeutic international normalised ratio and acute on chronic kidney injury. Oesophogastroduodenoscopy and colonoscopy initially suggested ischaemic colitis. The patient's legs were treated symptomatically with topical steroids. He later developed acute large volume bloody diarrhoea that made him haemodynamically unstable. Punch biopsy of the skin was consistent with leucocytoclastic vasculitis and direct immunofluorescence demonstrated immunoglobulin A and C3 deposits consistent with Henoch-Schonlein purpura. The patient was treated with oral steroids. Bleeding stabilised and rash resolved. Steroids were successfully tapered. The patient was discharged on haemodialysis but ultimately this was able to be discontinued.

Vancomycin-associated drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome: masquerading under the guise of sepsis.

A patient presented with what appeared to be severe urosepsis. After admission and antibiotic administration, a newly developed rash and subsequent facial swelling appeared to be a reaction to penicillin class antibiotics. However, despite changing class of therapy with continued antimicrobial coverage, end organ damage continued, the rash worsened and facial oedema developed. Drug reaction with eosinophilia and systemic symptoms was ultimately diagnosed and was consistent with clinical and histopathological findings, as well as meeting all criteria for scoring systems. The patient was started on intravenous methylprednisolone 125 mg per 8 hours with rapid improvement of rash, swelling and end organ damage. Initial challenge to decrease dose failed, but the patient was ultimately able to be discharged on an extended taper.

Trichosporon fungemia in a pediatric patient with acute lymphoblastic leukemia.

Trichosporon fungemia is a life-threatening opportunistic infection that is increasing in frequency. Invasive disease occurs almost exclusively in immunocompromised hosts, particularly in neutropenic adults with hematological malignancies and uncommonly in children. We report the case of a pediatric patient where disseminated trichosporonosis progressed while on micafungin, between treatments with voriconazole and amphotericin B, demonstrating the difficulty with and importance of prolonged and continuous treatment.