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Anais Brasileiros de Dermatologia 2024
Topics: Humans; Pityriasis Lichenoides; Dermoscopy; Pityriasis
PubMed: 37661463
DOI: 10.1016/j.abd.2022.04.017 -
Clinics in Dermatology 2023This is the second installment of a three-part contribution that highlights the achievements of Jewish dermatologists as reflected by eponyms that honor their names. It...
This is the second installment of a three-part contribution that highlights the achievements of Jewish dermatologists as reflected by eponyms that honor their names. It covers the period 1933-1945 when the Nazis took over Germany and how the lives of 14 notable Jewish physicians, mostly in Germany, were impacted during the Holocaust. Many of them fled from the persecution, bringing their academic talents to other lands such as the United States. At least one committed suicide (Fritz Juliusberg), and three others perished in the Holocaust (Abraham Buschke, Lucja Frey-Gottesman, and Karl Herxheimer). They are remembered by eponyms including Neisser-Juliusberg pityriasis lichenoides chronica, Buschke-Ollendorff syndrome, Frey syndrome, and Jarisch-Herxheimer reaction. It made little difference to the Nazis that several of the 14 physicians had converted to Christianity. All were persecuted by the Nazis and had their professional careers destroyed. Two of the 14 physicians lived outside of the Third Reich (Bruno Bloch and Emanuel Libman) and were spared the suffering endured by the other 12. This tragic account of Jewish dermatologists during the Holocaust, and the eponyms that honor them, will continue in part three of this contribution.
Topics: Humans; United States; Jews; Dermatologists; Eponyms; Holocaust; Physicians; Germany
PubMed: 37777142
DOI: 10.1016/j.clindermatol.2023.09.006 -
The British Journal of Dermatology Apr 2024Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce.
BACKGROUND
Lymphomatoid Papulosis (LyP) is a rare cutaneous T-cell lymphoproliferative disorder. Comprehensive data on LyP in the paediatric population is scarce.
OBJECTIVES
To characterize epidemiological, clinical, histopathological, and prognostic features of paediatric LyP.
METHODS
This was a retrospective, multicentre international cohort study including 87 cases of children and adolescents with LyP diagnosed between 1998 and 2022. Patients aged ≤ 18 years old at disease onset were included. Diagnosis was made in each centre based on clinical-pathological correlation.
RESULTS
Eighty-seven patients from 12 centres were included. The mean age at onset was 7.0 years (range 3 months-18 years) with a male to female ratio of 2:1. The mean time between onset of first cutaneous lesions and diagnosis was 1.3 years (range 0-14 years). Initial misdiagnosis concerned 26.4% of patients. Initially, LyP was most often misdiagnosed as Pityriasis lichenoides et varioliformis acuta (PLEVA), insect bites, or mollusca contagiosa. Erythematous papules or papulonodules were the most frequent clinical presentation. Pruritus was specifically mentioned for 20.7% of patients. The main histological subtype was type A in 55.1% of the cases. If analysed, monoclonal TCR rearrangement was found in 76.5% of the skin biopsies. The overall survival rate was 100% with follow up at 5 years available for 33 patients and at 15 years for 8 patients. A development of associated haematological malignancy (HM) occurred in 9.6% of the cases (7/73), including four mycosis fungoides (MF) cases, one primary cutaneous anaplastic large cell lymphoma (pc-ALCL), one systemic ALCL and one case of acute myeloid leukaemia. If we compare incidence rates of cancer with the world 0-19 years old population from 2001-2010, we estimate a significantly higher risk of associated malignancy in general, occurring before the age of 19 years old with incidence rate ratio of 87.49 (CI 86.01-88.99).
CONCLUSIONS
We report epidemiological data from a large international cohort of children and adolescents with LyP. Overall the prognosis of the disease is good, with excellent survival rates for all patients. Due to increased risk of associated HM, a long-term follow-up should be recommended for LyP patients.
PubMed: 38595050
DOI: 10.1093/bjd/ljae150 -
The American Journal of Dermatopathology Apr 2024Mucha-Habermann disease (MHD) is an inflammatory skin disease characterized by polymorphous eruptions of erythematous, necrotic macules that have been reported for... (Review)
Review
Mucha-Habermann disease (MHD) is an inflammatory skin disease characterized by polymorphous eruptions of erythematous, necrotic macules that have been reported for similarities to cutaneous T-cell lymphoma. Febrile ulceronecrotic MHD (FUMHD) represents a severe variant of MHD, marked by ulcers, hemorrhagic bullae, and systemic symptoms. Herein, we report a case of a severely atypical lymphomatoid expression of FUMHD associated with hemophagocytic lymphohistiocytosis (HLH). A previously healthy 21-year-old woman was admitted to the hospital with a rapidly progressive necrotic papular rash. Physical examination revealed right orbital swelling, bilateral hemorrhagic auricular bullae, and multiple ulcerative purpuric papulonodules on the trunk, face, and extremities. Biopsy indicated a dermal and subcutaneous infiltrate of atypical CD8 + lymphocytes with loss of CD5 and reduction in CD7 expression, along with features of lymphomatoid vasculitis. A diagnosis of a severely atypical lymphomatoid expression of FUMHD was made. The patient also met 7 of 9 HLH-2004 criteria, leading to a diagnosis of HLH. Positron emission tomography/computed tomography, flow cytometry, and rheumatologic workup were unremarkable. Treatment with an eight-week course of etoposide and dexamethasone for HLH led to rapid clinical improvement. Over time, her skin lesions regressed and eventually scabbed over to leave hyperpigmented scars, confirming the diagnosis of MHD. She has remained stable, off therapy for 4 years. Although potentially fatal, FUMHD often exhibits favorable outcomes and may resolve without recurrence, as in our patient. FUMHD should be considered in the differential diagnosis for patients presenting with cutaneous CD8 + necrotizing angiocentric lymphoproliferative disease complicated by HLH.
Topics: Female; Humans; Young Adult; Blister; Fever; Herpes Simplex; Lymphohistiocytosis, Hemophagocytic; Necrosis; Pityriasis Lichenoides; Skin Neoplasms; Skin Ulcer
PubMed: 38457671
DOI: 10.1097/DAD.0000000000002619 -
Pediatric Dermatology 2024Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a lymphocyte-mediated inflammatory skin disorder, is considered a severe variant of pityriasis lichenoides et...
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a lymphocyte-mediated inflammatory skin disorder, is considered a severe variant of pityriasis lichenoides et varioliformis acuta that can lead to a fatal outcome if not managed in a timely fashion. Children with FUMHD can have systemic complications involving various organs. The scarcity of reported cases and the absence of well-designed studies or randomized clinical trials to evaluate different therapeutic modalities pose a major challenge in treating this potentially life-threatening disorder. We report a five-year-old child with FUMHD and seizures treated unsuccessfully with a combination of systemic steroids, methotrexate, dapsone, and oral erythromycin, who improved rapidly and achieved disease control with just a single infusion of low-dose intravenous immunoglobulin.
Topics: Humans; Immunoglobulins, Intravenous; Pityriasis Lichenoides; Child, Preschool; Male; Immunologic Factors; Fever
PubMed: 38234081
DOI: 10.1111/pde.15516 -
Journal of Cutaneous Pathology May 2024Cytophagic histiocytic panniculitis (CHP) is associated with a number of systemic conditions and is characterized by the presence of benign phagocytic histiocytes ("bean...
Cytophagic histiocytic panniculitis (CHP) is associated with a number of systemic conditions and is characterized by the presence of benign phagocytic histiocytes ("bean bag cells"), including phagocytosed erythrocytes, leukocytes, and platelets. We describe a case of a 72-year-old female who presented with a papular eruption that clinically mimicked pityriasis lichenoides et varioliformis acuta (PLEVA). Given that her skin biopsy had multiple features concerning PLEVA, this diagnosis was classified as a superficial pityriasis lichenoides-like variant of CHP. The histopathologic presence of cytophagic histiocytosis prompted workup for a systemic malignancy, leading to a diagnosis of underlying acute monocytic leukemia of myeloid lineage.
PubMed: 38769706
DOI: 10.1111/cup.14659 -
JAAD Case Reports May 2024
PubMed: 38576898
DOI: 10.1016/j.jdcr.2024.02.021