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Dermatology Reports Nov 2022Febrile Ulceronecrotic Mucha- Habermann Disease (FUMHD) is a variant of Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA). Although rare, the condition may progress...
Febrile Ulceronecrotic Mucha- Habermann Disease (FUMHD) is a variant of Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA). Although rare, the condition may progress to involve serious complications and even lead to fatal outcomes if diagnosis and appropriate treatment is delayed. A PubMed search following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRIMSA) guidelines was performed to find cases of FUMHD from the earliest records to October 2021. Treatments, complications, and patient outcomes were extracted from the literature and summarized, while a review of quality was also performed. A total of 63 publications with 68 patients were found. Successful treatment modalities for FUMHD included antibiotics, antivirals, systemic steroids, Methotrexate (MTX), cyclophosphamide, Cyclosporine (CYA), Intravenous Immunoglobulins (IVIG), pentoxifylline, and ultraviolet B phototherapy. Out of 68 patients, 55 patients had their condition fully resolved and 13 cases were fatal. Increased age, systemic involvement, and monoclonal T-cell receptor rearrangement were associated with worst prognosis, but mucosal involvement did not affect mortality risk. Overall, the publications had low risk of bias, but most lacked adequate follow-up periods. FUMHD is a diagnostic and therapeutic challenge due to the lack of clearly defined diagnostic criteria and optimum treatment. Further studies with larger patient populations and longer follow-up periods may lead to refinement of diagnostic criteria, establish an optimum treatment regimen, and better estimate the likelihood of recurrence.
PubMed: 36483219
DOI: 10.4081/dr.2022.9492 -
Italian Journal of Pediatrics Oct 2022Lymphomatoid papulosis (LyP) is a rare condition in pediatrics; LyP histological type D has been reported in only 7 children. The differential diagnosis of LyP in the... (Review)
Review
Type D lymphomatoid papulosis with pityriasis lichenoides et varioliformis acuta-like features in a child with parvovirus infection: a controversial diagnosis in the spectrum of lymphoid proliferations: case report and literature review.
BACKGROUND
Lymphomatoid papulosis (LyP) is a rare condition in pediatrics; LyP histological type D has been reported in only 7 children. The differential diagnosis of LyP in the spectrum of lymphoid proliferation remains controversial.
CASE PRESENTATION
A 6-year-old boy presented to Emergency Department with a 3-week history of an erythematous papulo-vesicular itchy eruption over the submandibular regions, trunk and extremities. History, symptoms and laboratory tests were unremarkable. SARS-CoV-2 antigen was negative. The clinical suspicion of pityriasis lichenoides et varioliformis acuta (PLEVA) was posed, and topical steroids were introduced. One week after, he returned with an extensive painful scaly papulo-erythematous rash, with some ulcerated and necrotic lesions, and fever; therefore the child was hospitalized. Biochemical results were within reference limits, except for high level of C-reactive protein, aspartate aminotransferase, alanine transaminase and bilirubin. Due to a persistently high fever, systemic corticosteroid treatment was administered, with a good clinical response and an improvement of the skin lesions. Anti-PVB-19 Immunoglobulin M was detected. Elevated levels of IL-6, IL-10 and IFN-γ were also recorded. Five days post-admission, most of the lesions had cleared, and the child was discharged. Methotrexate was started, with a positive response. At skin biopsy a "PLEVA-like" pattern was apparent, with a dense, wedge shaped lymphoid infiltrate featuring epidermotropism and morphologically comprising pleomorphic and blastic cells. The pattern of infiltration was highlighted by immunohistochemical stains, which prove the process to feature a CD8+/CD30 + phenotype, the latter being intense on larger cells, with antigenic loss. Polymerase chain reaction for T-cell receptor gamma (TCRG) chain clonality assessment documented a monoclonal peak. A diagnosis of LyP type D was favored.
CONCLUSION
The reported case encompasses most of the critical features of two separated entities-PLEVA and LyP-thus providing further support to the concept of them representing declinations within a sole spectrum of disease. Studying the role of infectious agents as trigger potential in lymphoproliferative cutaneous disorders and detecting novel markers of disease, such as cytokines, could have a crucial impact on pathogenic disease mechanisms and perspective therapies.
Topics: Child; Humans; Male; COVID-19; Lymphomatoid Papulosis; Parvoviridae Infections; Pityriasis Lichenoides; SARS-CoV-2; Cell Proliferation
PubMed: 36307824
DOI: 10.1186/s13052-022-01371-x -
Indian Dermatology Online Journal 2022
PubMed: 36304638
DOI: 10.4103/idoj.IDOJ_637_21 -
Journal of the European Academy of... Feb 2023
Topics: Humans; ChAdOx1 nCoV-19; COVID-19; COVID-19 Vaccines; Pityriasis Lichenoides
PubMed: 36227123
DOI: 10.1111/jdv.18646 -
Archives of Dermatological Research Apr 2023The exact aetiology of pityriasis lichenoides chronica (PLC) remains unknown. While phototherapy is the most investigated therapeutic modality, azithromycin has been... (Randomized Controlled Trial)
Randomized Controlled Trial
The exact aetiology of pityriasis lichenoides chronica (PLC) remains unknown. While phototherapy is the most investigated therapeutic modality, azithromycin has been used scarcely. The aim of this study is to evaluate the therapeutic efficacy of azithromycin in the treatment of PLC compared to NB-UVB and evaluating the presence of streptococcal infection as a possible etiological factor in PLC patients. The study was designed as a randomised controlled trial. Twenty-four patients with PLC were randomly allocated into either azithromycin (n = 13, standard dose every 10 days) or NB-UVB (n = 11, thrice weekly) groups. End of study (EOS) was either complete clearance of lesions or a maximum of 8 weeks. Therapeutic efficacy was defined as percent reduction in lesions and was calculated for the rash as a whole, erythematous papules alone, and hypopigmented lesions alone and graded into complete, very-good, good, poor or no response. Anti-streptolysin O titre (ASOT), anti-deoxyribonuclease B titre (anti-DNaseB) and throat culture were evaluated at day 0. No significant difference existed between both groups as regards therapeutic efficacy. At EOS, NB-UVB achieved significantly more percent reduction in the extent of hypopigmented lesions and consequently in the rash as a whole (p = 0.001, p = 0.034, respectively). The extent of the rash as a whole was significantly less in the NB-UVB at EOS (p = 0.029, respectively). The effect of NB-UVB on hypopigmented lesions appeared early at week 4 of treatment. Only two patients, one from each group, relapsed during the 3 month follow-up. Evidence of recent streptococcal infection was present in 79% of the cases, mainly in the form of elevated ASOT (94.7%). It was significantly more encountered in young children (< 13 years) (p = 0.03) and was associated with more extent of erythematous papules and consequently with more extent of the rash as a whole (p = 0.05 and p = 0.01, respectively). It did not affect outcome of therapy at EOS. Azithromycin did not show more favorable response in patients with recent streptococcal infection. Therapeutic efficacy of azithromycin is comparable to NB-UVB in treatment of PLC; however, NB-UVB is superior in management of hypopigmented lesions. It is highly suggested that PLC could be a post streptococcal immune mediated disorder.Registration number: ClinicalTrials.gov, NCT03831269.
Topics: Child; Humans; Child, Preschool; Azithromycin; Pityriasis Lichenoides; Ultraviolet Therapy; Streptococcal Infections; Exanthema; Antibodies; Treatment Outcome
PubMed: 36129521
DOI: 10.1007/s00403-022-02398-0 -
Clinical, Cosmetic and Investigational... 2022Pityriasis lichenoides et varioliformis acuta (PLEVA) and lymphomatoid papulosis (LyP) are uncommon inflammatory skin disorders that occasionally share...
Pityriasis lichenoides et varioliformis acuta (PLEVA) and lymphomatoid papulosis (LyP) are uncommon inflammatory skin disorders that occasionally share clinicopathological features. Differentiating between the two entities remains problematic, and a definitive diagnosis usually requires multi-step investigations, which is an enormous challenge to physicians. We hereby report a rare case of a 22-year-old female patient diagnosed with PLEVA who later developed LyP type F, a new histological variant of LyP. Our report highlights that long-term follow-up is essential to determine associated hematologic malignancies, particularly in cases with recalcitrant or progressive cutaneous lesions of PLEVA and/or LyP.
PubMed: 36065341
DOI: 10.2147/CCID.S379577 -
Dermatopathology (Basel, Switzerland) Jul 2022Pityriasis lichenoides is an acute and/or chronic skin disease associated with recurrent erythematous papules that self-resolve. While its etiology is unknown, preceding...
Pityriasis lichenoides is an acute and/or chronic skin disease associated with recurrent erythematous papules that self-resolve. While its etiology is unknown, preceding viral infection may play a role. We present an atypical case of a 40-year-old woman with pityriasis lichenoides et varioliformis acuta as a complication of a COVID-19 infection.
PubMed: 35892481
DOI: 10.3390/dermatopathology9030028 -
JAAD Case Reports Sep 2022
PubMed: 35875513
DOI: 10.1016/j.jdcr.2022.07.017 -
Journal of the European Academy of... Dec 2022
Topics: Humans; Pityriasis Lichenoides; COVID-19 Vaccines; BNT162 Vaccine; COVID-19; SARS-CoV-2; Vaccines
PubMed: 35841285
DOI: 10.1111/jdv.18418 -
Pediatric Dermatology Nov 2022A 10-year-old female with a several-year history of pityriasis lichenoides (PL) presented with a new, asymptomatic, large, and necrotic ulcer of her right upper arm....
A 10-year-old female with a several-year history of pityriasis lichenoides (PL) presented with a new, asymptomatic, large, and necrotic ulcer of her right upper arm. Skin biopsy was consistent with lymphomatoid papulosis (LyP) Type D, a recently recognized subtype of LyP that is distinguished histologically by marked epidermotropism and a perivascular infiltrate of medium-sized pleomorphic lymphocytes with a cytotoxic phenotype (CD3+, CD8+). This is only the sixth reported case of LyP Type D in a child, and while the prognosis in children appears favorable, with no reports of progression to lymphoma to date, more experience in children with longer-term follow-up is needed. Our case highlights both the challenging clinical diagnosis, since in our patient the longstanding clinical presentation was indistinguishable from PL, as well as histopathologic diagnosis, which required expert opinion and consensus.
Topics: Female; Child; Humans; Lymphomatoid Papulosis; Pityriasis Lichenoides; Skin; Biopsy; Skin Neoplasms
PubMed: 35817594
DOI: 10.1111/pde.15076