Dermatology online journal最新文献

Posttransplant lymphoproliferative disorder is an uncommon complication of immunosuppression following solid organ or hematopoietic stem cell transplants. Primary cutaneous posttransplant lymphoproliferative disorder with isolated skin involvement but without systemic involvement is rare. We report a 50-year-old woman, a renal transplant recipient on long-term immunosuppression, who presented with a rapidly expanding ulcer on her right posterior thigh after a skin incision. Although the clinical presentation was similar to that of pyoderma gangrenosum, pathological investigation confirmed Epstein-Barr virus-associated primary cutaneous B-cell posttransplant lymphoproliferative disorder. Initially, we reduced her immunosuppression. As the ulcer rapidly expanded, we initiated R-CHOP chemotherapy, consisting of rituximab, cyclophosphamide, doxorubicin, and prednisolone. Owing to the complications from cytomegalovirus retinitis, we were only able to administer two courses of chemotherapy. However, after continuous administration of ganciclovir, the skin ulcer regressed and completely healed, leaving a scar five months after her first visit. Since pyoderma gangrenosum is a diagnosis of exclusion, ulcerative skin lesions, similar to the clinical presentation of pyoderma gangrenosum, should be subjected to pathological investigation for accurate diagnosis.

Munchausen syndrome (factitious disorder imposed on self), a condition in which the patient intentionally injures themselves to create disease signs and symptoms, is difficult to diagnose.  The affected individual not only seeks attention but also sympathy for their illness. A 31-year-old woman with cutaneous Munchausen syndrome who had persistent cutaneous ulcers and new abscesses is described; she died during her hospitalization and postmortem biopsies of her skin lesions and lungs both demonstrated polarizable foreign bodies. Fatal Munchausen syndrome has not commonly been described; including the woman in this report, we are aware of 19 decedents. The cause, mechanism, and manner of death have varied. Like our patient, the manner of death was most frequently undetermined since the circumstances did not permit accident to be differentiated from suicide. In conclusion, forensic dermatology aided in establishing the diagnosis of fatal cutaneous Munchausen syndrome in the woman we report. A biopsy of her non-healing ulcer showed polarizable foreign material that she had inoculated into her skin. Her condition involved not only self-inflicted skin abscesses, but also similar manifestations in her lungs. To prevent unnecessary laboratory tests and procedures and possibly death, healthcare providers need to consider the possibility of Munchausen syndrome.

Atopic dermatitis is a chronic inflammatory skin condition driven by immune dysregulation, with interleukin-13 playing a central role in its pathogenesis. Recent advances in targeted biologic therapies have shown promising results in treating moderate-to-severe atopic dermatitis. A comprehensive literature review of PubMed and Google Scholar was conducted to identify studies related to interleukin-13 inhibition in atopic dermatitis. An expert panel reviewed and graded the evidence using Strength of Recommendation Taxonomy criteria and utilized a modified Delphi process to formulate consensus statements on the role of interleukin-13 inhibitors. Based on selected literature, the panel developed 14 consensus statements, all receiving unanimous approval. Key findings include the rapid efficacy, sustained benefits, and favorable safety profiles of interleukin-13 inhibitors. Differences between available interleukin-13 inhibitors included pain of injection, speed of onset, durability of efficacy, and number of injections needed to maintain efficacy. Interleukin-13 plays a pivotal role in atopic dermatitis pathogenesis, driving inflammation, pruritus, and barrier dysfunction. Targeted therapies, including interleukin-13 inhibitors, provide rapid, durable, and safe options for managing moderate-to-severe atopic dermatitis. This consensus highlights interleukin-13 inhibition as a cornerstone in advancing atopic dermatitis treatment strategies, offering improved patient outcomes and quality of life.

Background: Hedgehog inhibitors (HHIs) are approved for the treatment of locally advanced BCCs in patients who are not surgical candidates or have had recurrence following surgical treatment. This expert consensus panel further characterizes the efficacy and safety of HHIs while also providing clinical guidance on their appropriate dosing, laboratory monitoring, and supplementation.

Methods: A comprehensive literature review was completed on November 1, 2024 using the keywords "basal cell carcinoma," "hedgehog inhibitor," "sonidegib," and "vismodegib". An expert panel of nine dermatologists reviewed and assigned levels of evidence to the relevant articles and created consensus statements regarding HHIs, with correlating strength of recommendations, using the modified Delphi process.

Results: Of the 304 articles identified, 23 articles met the selection criteria and were included for review. The panel unanimously adopted nine consensus statements and recommendations, of which three were given a strength of recommendation of "A", two were given a "B", and four were given a "C".

Conclusion: Sonidegib and vismodegib have similar efficacy in treating advanced BCC, but sonidegib has lower rates and a greater delay in onset of AEs. Sonidegib has a significantly greater volume of distribution and half-life than those of vismodegib. Dosing interruptions have not been shown to reduce the efficacy of HHIs, and L-carnitine supplementation can help reduce the incidence of muscle spasms.

A 44-year-old woman with type II diabetes mellitus presented with an 8-month history of a painful, ulcerating rash at the area of insulin injection of her left lower abdomen. Topical antibiotics, topical corticosteroids, oral antibiotics, and supportive wound care provided no relief. A similar eruption developed on the right abdomen when she switched injection sites. Wedge biopsy of the violaceous, reticular plaque demonstrated a central dermal scar with surrounding lobular and linear collections of small blood vessels, consistent with reactive angioendotheliomatosis. This condition typically occurs secondary to an underlying systemic disease, but we believe hers was a side effect related to trauma from insulin injection or secondary to an insulin additive hypersensitivity. The patient was switched to a glucagon-like peptide-1 agonist, active lesions were treated with topical timolol 0.5% ophthalmic solution, and oral propranolol was started with upward titration. She continues to see improvement in pain and has not developed any new ulcerations at other sites on the body.

Bullous pemphigoid is a chronic autoimmune blistering disease typically presenting as non-specific pruritis following by the development of vesicles and bullae. A variety of medications and comorbid medical conditions have been associated with bullous pemphigoid. Herein, we present a case describing a potential novel association between immunoglobulin A nephropathy and bullous pemphigoid.

Hidradenitis suppurativa is a chronic inflammatory skin disorder characterized by painful recurring pustules, nodules, abscesses, and sinus tract formation. Secondary lymphedema associated with hidradenitis suppurativa is an uncommon but severe condition, often resulting in significant morbidity. Although the exact link between lymphedema and chronic hidradenitis suppurativa is not fully understood, the chronic inflammatory process with scarring is a likely an important factor. Herein, we reported a 52-year-old man with untreated chronic hidradenitis suppurativa, nonalcoholic fatty liver disease-related cirrhosis, and secondary massive scrotal lymphedema, complicated by cellulitis and sepsis. Despite appropriate treatment, the patient's condition deteriorated and he unfortunately passed away two weeks after hospitalization. Additionally, we reviewed the literature on hidradenitis suppurativa-associated lymphedema, analyzing data from 51 patients. The majority were men with long-standing severe hidradenitis suppurativa. Surgical intervention was identified as the most commonly effective treatment modality, offering substantial improvements in function and appearance. However, some patients experienced postsurgery adverse events. In conclusion, this report highlights the importance of early detection and management of hidradenitis suppurativa and its complications to prevent patients' significant morbidity.

Epithelial sheath neuroma is a cutaneous tumor presenting as an erythematous and often painful lesion on the skin of the back. We report a 74-year-old woman with a 7mm epithelial sheath neuroma located on the right upper central back. The initial clinical impression was a basal cell carcinoma and histopathologically, the tumor mimicked a carcinoma with perineural invasion. She had no prior trauma or surgeries at the surgical site. She reported experiencing pain at the site of the tumor. Pain is a symptom of more than half of the reported epithelial sheath neuroma cases. The papule was excised, and there has been no recurrence at the surgical site. We report the unusual occurrence of this very uncommon neoplasm, a tumor that might be inadvertently confused with an aggressive squamous cell carcinoma.

Morphea is an uncommon inflammatory disorder characterized by progressive sclerosis of the skin and soft tissues. We describe the novel occurrence of generalized morphea profunda arising in close temporal association with the COVID-19 messenger ribonucleic acid vaccination series. An 80-year-old man presented with numerous areas of firm, tight skin across his trunk and extremities with associated itching and burning. He denied characteristic symptoms of systemic sclerosis. Physical examination revealed numerous indurated plaques, some with porcelain-white or sclerotic yellow centers, on the bilateral dorsal forearms, anterior waistline, and bilateral legs. There was no evidence of sclerodactyly or inflammatory arthritis. Punch biopsies showed thickened collagen bundles in the reticular dermis, diminished periadnexal fat, and a perivascular and interstitial mononuclear cell infiltrate. The hyalinizing fibrosing reaction extended into the fat and was manifested by notable expansion of the interlobular septa of the fat by dense collagen. He was started on oral methotrexate with significant improvement in symptoms and lesion induration at 14-month follow-up. This case report provides further insight into the potential dermatologic adverse events associated with COVID-19 messenger ribonucleic acid vaccination. Further investigation is needed to determine any predisposing patient-specific intrinsic factors, unrecognized pathophysiologic mechanisms, and approximate incidence of such adverse events.