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Indian Journal of Dermatology 2017Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta, is an inflammatory dermatosis of unknown etiology...
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta, is an inflammatory dermatosis of unknown etiology manifested by ulcerative and necrotic lesions accompanied by systemic manifestations. The mortality rate of FUMHD is about 15%. It is reported here a case of FUMHD presenting as toxic epidermal necrolysis that resulted in multiple organ failure and death.
PubMed: 29263553
DOI: 10.4103/ijd.IJD_631_16 -
Indian Journal of Dermatology,... 2019Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose...
Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose as in the early course they can mimic erythema multiforme or lymphomatoid papulosis. We report a case of a 38-year-old woman who presented with 90% body surface area involvement, fever, diarrhea, malaise and associated comorbidities. She was treated with systemic steroids and methotrexate but suffered a fatal outcome. So far, a total of 65 cases are reported in the literature.
Topics: Adult; Fatal Outcome; Female; Herpes Simplex; Humans; Pityriasis Lichenoides; Rheumatic Heart Disease; Sepsis; Tuberculosis
PubMed: 30860167
DOI: 10.4103/ijdvl.IJDVL_552_17 -
Journal of Clinical Pathology Oct 2003This report describes the case of a 76 year old man who suffered from febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Despite this patient's typical clinical and... (Review)
Review
This report describes the case of a 76 year old man who suffered from febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Despite this patient's typical clinical and histological findings, the fulminating course led to death. Polymerase chain reaction (PCR) analysis of the skin lesions showed that the infiltrating cells were monoclonal in origin and were from an aberrant clone. FUMHD is a very rare, febrile variant type of pityriasis lichenoides et varioliformis acuta, and is characterised by necrotic cutaneous ulcerations associated with high fever and systemic manifestations. Including this present case, only 18 cases of FUMHD have been reported. FUMHD can occur in both adults and children, although there are several differences between the manifestations of the disease in the two groups. One major difference is prognosis: all cases resulting in fatality are of the adult type, whereas no fatal cases have been reported among children. The aberrant clone detected by PCR may be responsible for host responses, resulting in the severe symptoms observed in this disorder.
Topics: Aged; Clone Cells; Fatal Outcome; Fever; Gene Rearrangement, T-Lymphocyte; Humans; Male; Necrosis; Pityriasis Lichenoides; Polymerase Chain Reaction; Shock; Skin Ulcer; T-Lymphocytes
PubMed: 14514790
DOI: 10.1136/jcp.56.10.795 -
Dermatology Online Journal Nov 2017Lymphomatoid drug reactions can mimic endogenous T and B cell lymphoproliferative diseases.
BACKGROUND
Lymphomatoid drug reactions can mimic endogenous T and B cell lymphoproliferative diseases.
OBJECTIVES
We present a novel form of cutaneous drug reaction with features of pityriasis lichenoides (PL), a recognized form of T cell dyscrasia.
METHODS
Ten cases were studied where a cutaneous eruption exhibiting semblance to PL within a few weeks to months after starting a particular drug.
RESULTS
The patient cohort comprised 7 females and 3 males with the mean age of 60 years. Widely distributederythematous cutaneous lesions were present in 6 cases whereas a more localized distribution was seen in three cases. The most frequently implicated drugsassociated with the eruption were antidepressants and statins. Histologic examination showed a morphologic picture identical to PL including marked epitheliotropism of mildly atypical lymphocytes, psoriasiform epidermal hyperplasia, dyskeratosis, hemorrhage, and a thick parakeratotic scale. Therewas a significant reduction in the expression of CD7 and CD62L amid the T cells. Regression of the eruption occurred in all cases excluding one.
CONCLUSION
Thefindings conform the categorization of this process as a form of T-cell dyscrasia albeit one that is reversible, dependent on the drug withdrawal. The limitationof our study includes the retrospective design of the study.
Topics: Adult; Aged; Aged, 80 and over; Antidepressive Agents; Diagnosis, Differential; Drug Eruptions; Female; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Lichenoid Eruptions; Male; Middle Aged; Pityriasis Lichenoides; Retrospective Studies; Skin; T-Lymphocytes
PubMed: 29447634
DOI: No ID Found -
Romanian Journal of Morphology and... 2014Parapsoriasis represents a group of cutaneous disorders that shows variable clinical aspects somehow resembling to psoriasis, how is reflecting by its name. It was first...
BACKGROUND
Parapsoriasis represents a group of cutaneous disorders that shows variable clinical aspects somehow resembling to psoriasis, how is reflecting by its name. It was first named by Brocq, in 1902, as an entity with three components: pityriasis lichenoides, small plaque parapsoriasis and large plaque parapsoriasis. Nowadays, under the name of parapsoriasis are included only the last two categories, that are considered disorders characterized by the presence of a mononuclear infiltrate in the dermis, composed of T-cells. Until now, there were not established pathognomonic histopathological features to diagnose parapsoriasis.
AIM
The aim of the study was to investigate the epidemiological and morphological data of parapsoriasis cases diagnosed at Emergency City Hospital, Timisoara, Romania for a period of 12 years.
MATERIALS AND METHODS
The study had two parts; one was retrospective and another one prospective. For the retrospective part, we searched 210111 patient files recorded in our Pathology Service for a period of 11 years, from January 2002 to December 2012. The slides were searched from the archive and re-read by two individual pathologists. For prospective part of the study, we reviewed 11815 histological slides read between January and June 2013. After inspection of the recorded files, the pathologists noted, were available, the localization and number of the lesions, together with symptoms. The biopsied specimens were initially processed with routine histological technique, the archive slides being stained with Hematoxylin and Eosin. While reading the slides, the pathologists paid attention to the architecture of the epidermis, the presence of epidermotropism and interface dermatitis, type of the dermal infiltrate and its distributions.
CONCLUSIONS
In the present study, we emphasized the histopathological aspects of parapsoriasis in order to create a basic line that could help in the establishment of a uniformly accepted definition of parapsoriasis on histopathological grounds.
Topics: Age Distribution; Biopsy; Coloring Agents; Female; Humans; Male; Parapsoriasis; Retrospective Studies
PubMed: 25607389
DOI: No ID Found -
BMJ Case Reports Dec 2014Primary cutaneous lymphomas (PCLs) are exceedingly rare in children and adolescents, with mycosis fungoides (MF) being the most frequent PCL diagnosed in childhood....
Primary cutaneous lymphomas (PCLs) are exceedingly rare in children and adolescents, with mycosis fungoides (MF) being the most frequent PCL diagnosed in childhood. There are numerous unusual clinical variants of MF, including the hypopigmented type form (HMF). HMF is exceptional overall, but comparatively common among children. We present an 8-year-old boy with a 3-year history of progressive, generalised, scaly, hypopigmented round patches and few erythematous papules. He was first diagnosed with pityriasis alba (PA), and moisturisers were prescribed with no improvement. Skin biopsy showed typical features of MF, and the patient was successfully treated with narrowband ultraviolet B. HMF may simulate atopic dermatitis, PA, pityriasis lichenoides, tinea versicolour, vitiligo, postinflammatory hypopigmentation or leprosy. Therefore, persistent and unusual hypopigmented lesions should be biopsied to rule out this rare variant of MF.
Topics: Biopsy; Child; Delayed Diagnosis; Humans; Hypopigmentation; Male; Mycosis Fungoides; Pityriasis Lichenoides; Skin; Skin Neoplasms
PubMed: 25538219
DOI: 10.1136/bcr-2014-208306 -
Anais Brasileiros de Dermatologia 2024
Topics: Humans; Pityriasis Lichenoides; Dermoscopy; Pityriasis
PubMed: 37661463
DOI: 10.1016/j.abd.2022.04.017 -
Dermatologic Therapy Jun 2022
Topics: COVID-19; Humans; Pityriasis Lichenoides; RNA, Messenger; Vaccination
PubMed: 35286000
DOI: 10.1111/dth.15445 -
Case Reports in Dermatology 2023The febrile ulceronecrotic Mucha-Habermann disease is a rare and potentially lethal variant of pityriasis lichenoides et varioliformis acuta (PLEVA). It is characterized...
The febrile ulceronecrotic Mucha-Habermann disease is a rare and potentially lethal variant of pityriasis lichenoides et varioliformis acuta (PLEVA). It is characterized by a sudden onset of ulceronecrotic skin lesions associated with high fever and systemic symptoms. Herein, we report a 23-year-old male, not known to have any medical illnesses, presented with a month-long history of persistent fever of unknown origin associated with a sudden onset of progressive diffuse necrotic ulcers and widespread papulosquamous lesions. Pan CT showed enlarged lymph nodes in the cervix, chest, and abdomen. Unfortunately, a skin biopsy was done late, showing features consistent with PLEVA. Few days after admission, despite being on intravenous methylprednisolone, our patient rapidly deteriorated by showing severe acute respiratory symptoms and consequently died. In spite of the continuous addition of new case reports to the literature, no definite diagnostic criteria have been established, leading to late or missed cases, and an optimum treatment is still waiting.
PubMed: 36686043
DOI: 10.1159/000528500 -
Case Reports in Dermatology 2021Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by...
Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by intermittent pyrexia, acute onset of generalized ulceronecrotic lesions, and histopathology suggestive of PLEVA. Prompt diagnosis and treatment are necessary to halt the progression of this potentially fatal disease; however, the widely variable presentation of FUMHD in addition to its rarity poses a diagnostic challenge. We report the case of a previously healthy 43-year-old woman who presented to the emergency department with 1 month of generalized rash and intermittent fevers. Her only reported new exposure were elective intravenous vitamin infusions received at a medi-spa 1 week prior to onset of lesions. Initial evaluations were inconclusive, and confluent ulceronecrotic, hemorrhagic lesions appeared on approximately 90% of her body despite steroid, antibiotic, and cyclosporine therapy. Repeat histopathology was consistent with PLEVA, and in the context of her clinical presentation she was diagnosed with FUMHD. The patient rapidly attained remission with methotrexate therapy but sustained residual scarring.
PubMed: 33613228
DOI: 10.1159/000511537