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The American Journal of Surgical... Jul 2012Pityriasis lichenoides comprises a clinicopathologic spectrum of cutaneous inflammatory disorders, with the 2 most common variants being pityriasis lichenoides et...
Pityriasis lichenoides et varioliformis acuta with numerous CD30(+) cells: a variant mimicking lymphomatoid papulosis and other cutaneous lymphomas. A clinicopathologic, immunohistochemical, and molecular biological study of 13 cases.
Pityriasis lichenoides comprises a clinicopathologic spectrum of cutaneous inflammatory disorders, with the 2 most common variants being pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica. The aim of the study was to describe 13 cases of a unique PLEVA variant characterized in the conspicuous CD30 component and thus mimicking lymphomatoid papulosis (LyP), a condition currently classified in the spectrum of CD30 lymphoproliferative disorders. The cohort included 10 female and 3 male patients whose ages at diagnosis ranged from 7 to 89 years (mean 41 y; median 39 y). The clinical manifestation was that of PLEVA, with small erythematous macules quickly evolving into necrotic papules. No waxing and waning was seen on follow-up in any of the cases. Histopathologically, typical features of PLEVA were present, but an unusual finding was occurrence of a considerable number of CD30 small lymphocytes as detected immunohistochemically. Over half of the cases also displayed a large number of CD8 cells and showed coexpression of CD8 and CD30 in the intraepidermal and dermal component of the infiltrate. Of the 11 cases of PLEVA studied for T-cell receptor gene rearrangement, 6 evidenced a monoclonal T-cell population, and 5 were polyclonal. Parvovirus B19 (PVB19) DNA was identified in 4 of 10 cases investigated, and positive serology was observed for PVB19 in 2 patients, altogether suggesting that PVB19 is pathogenetically linked to PLEVA at least in a subset of cases. The presence of CD30 lymphocytes and CD8 lymphocytes would be consistent with an inflammatory antiviral response, as CD30, even atypically appearing lymphoid cells have been identified in some viral skin diseases. The main significance of the PLEVA variant is, however, its potential confusion with LyP or some cytotoxic lymphomas. Admittedly, the CD30 PLEVA variant described herein and LyP show considerable overlap if one takes into account all known variations of the 2 conditions recognized in recent years, thus suggesting that LyP and PLEVA may be much more biologically closely related entities than currently thought or can even occur on a clinicopathologic spectrum.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antibodies, Viral; Biomarkers; CD8-Positive T-Lymphocytes; Case-Control Studies; Child; DNA, Viral; Diagnosis, Differential; Female; Gene Rearrangement, T-Lymphocyte; Genes, T-Cell Receptor; Humans; Immunohistochemistry; Ki-1 Antigen; Lymphomatoid Papulosis; Male; Middle Aged; Parvovirus B19, Human; Pityriasis Lichenoides; Polymerase Chain Reaction; Predictive Value of Tests; Skin; Skin Neoplasms
PubMed: 22472952
DOI: 10.1097/PAS.0b013e31824f4f66 -
Acta Dermato-venereologica Sep 2021
Review
Topics: Humans; Papillomavirus Vaccines; Pityriasis Lichenoides
PubMed: 34515802
DOI: 10.2340/00015555-3921 -
Indian Journal of Dermatology 2022
PubMed: 36578738
DOI: 10.4103/ijd.ijd_279_22 -
BMJ Case Reports Oct 2013The Mucha-Habermann disease is an inflammatory disease of the skin and is a variant of pityriasis lichenoides et varioliformis acuta. We describe the case of a...
The Mucha-Habermann disease is an inflammatory disease of the skin and is a variant of pityriasis lichenoides et varioliformis acuta. We describe the case of a 64-years-old woman who was admitted for erysipelas of the face. Despite treatment, evolution was marked by the appearance of a necrotising ulcerative area in the centre of the erysipelas associated with local oedema and headache. A skin biopsy revealed a pityriasis lichenoides et varioliformis acuta. Corticosteroids led to a rapid stabilisation of lesions, and after 6 months the patient shows only a small area of frontal hypopigmentation. The aetiology remains uncertain. There is no established standard treatment. We would like to draw attention of the medical and surgical specialists to this rare disease. The diagnosis should be considered in a necrotic lesion associated with rapid expansion of systemic and peripheral cutaneous signs. Diagnosis must be considered to avoid unnecessary debridement and extensive scars.
Topics: Diagnosis, Differential; Erysipelas; Female; Forehead; Humans; Middle Aged; Necrosis; Pityriasis Lichenoides; Skin Ulcer
PubMed: 24127370
DOI: 10.1136/bcr-2013-009739 -
JAAD Case Reports Oct 2023
PubMed: 37701883
DOI: 10.1016/j.jdcr.2023.07.038 -
Case Reports in Dermatology Jan 2012We report a case of a 63-year-old man hospitalized for a polymorphous generalized eruption consisting of maculopapules with peripheral scaling, vesicopustules, and...
We report a case of a 63-year-old man hospitalized for a polymorphous generalized eruption consisting of maculopapules with peripheral scaling, vesicopustules, and ulceronecrotic and crusted lesions measuring 5-20 mm, localized on his trunk and extremities, particularly exuberant in the flexural area. Histopathology showed necrotic keratinocytes with exocytosis of red blood cells and lymphocytes and a dermal perivascular and periadnexal inflammatory infiltrate, composed of CD8+/CD4-/CD30- T cells, indicating the clinical diagnosis of pityriasis lichenoides et varioliformis acuta. He was treated with erythromycin and methylprednisolone reduced gradually over 5 months, with a slow but complete response; the patient was without lesions after 2 years of follow-up. The authors want to remind of this rare entity which may present difficulties in diagnosis and therapy.
PubMed: 22548038
DOI: 10.1159/000337745 -
Cureus Dec 2022Pityriasis lichenoides chronica (PLC) is an uncommon inflammatory skin condition of unknown cause that ranges from a mild chronic form to a more severe acute eruption....
Pityriasis lichenoides chronica (PLC) is an uncommon inflammatory skin condition of unknown cause that ranges from a mild chronic form to a more severe acute eruption. Both forms usually involve the skin of the trunk and proximal extremities, and visceral involvement is not a well-described phenomenon. Here, we report a case of PLC that presented with esophageal involvement that occurred after a period of discontinuation of PLC treatment. The histological pattern of involvement is in the form of lymphocytic esophagitis, a non-specific pattern with a broad differential diagnosis. Awareness of the potential involvement of the esophagus and attention to certain endoscopic and morphological details may better help classify esophagitis biopsies and the diagnosis of this rare non-neoplastic chronic inflammatory disease. To our knowledge, this is the first-ever case of PLC with esophageal involvement, and nothing has been reported in the English literature earlier.
PubMed: 36628008
DOI: 10.7759/cureus.32290 -
Journal of Dermatological Science May 2018Classifying inflammatory skin diseases is challenging, especially for the expanding group of disorders triggered by genetic factors resulting in hyperactivated innate... (Review)
Review
Classifying inflammatory skin diseases is challenging, especially for the expanding group of disorders triggered by genetic factors resulting in hyperactivated innate immunity that result in overlapping patterns of dermal and epidermal inflammation with hyperkeratosis. For such conditions, the umbrella term "autoinflammatory keratinization diseases" (AIKD) has been proposed. AIKD encompasses diseases with mixed pathomechanisms of autoinflammation and autoimmunity, and includes IL-36 receptor antagonist (IL-36Ra)-related pustulosis, CARD14-mediated pustular psoriasis, pityriasis rubra pilaris (PRP) type V, and familial keratosis lichenoides chronica (KLC). Mechanistically, the entities include generalized pustular psoriasis (GPP) without psoriasis vulgaris, impetigo herpetiformis and acrodermatitis continua, which are IL-36Ra-related pustuloses caused by loss-of-function mutations in IL36RN; GPP with psoriasis vulgaris and palmoplantar pustular psoriasis which are CARD14-mediated pustular psoriasiform dermatoses with gain-of-function variants of CARD14; PRP type V which is caused by gain-of-function mutations in CARD14; and, familial KLC in which mutations in NLRP1, an inflammasome sensor protein predominantly expressed in skin, have been identified. It is likely that further inflammatory keratinization disorders will also fall within the concept of AIKD, as elucidation of novel pathogenic mechanisms of inflammatory keratinization diseases emerges. A better understanding of the pathophysiology of AIKD is likely to lead to innovative, targeted therapies that benefit patients.
Topics: Adaptor Proteins, Signal Transducing; Apoptosis Regulatory Proteins; Autoimmune Diseases; CARD Signaling Adaptor Proteins; Dermatitis; Guanylate Cyclase; Humans; Immunity, Innate; Interleukins; Membrane Proteins; Mutation; NLR Proteins; Skin; Skin Diseases, Papulosquamous
PubMed: 29422292
DOI: 10.1016/j.jdermsci.2018.01.012 -
Human Pathology Oct 2023Eosinophils are known to be present in inflammatory skin diseases, but their diagnostic utility is not well established. Upon review of the published status of lesional...
Eosinophils are known to be present in inflammatory skin diseases, but their diagnostic utility is not well established. Upon review of the published status of lesional eosinophils, several categories were identified. 1) Lesional eosinophils highly characteristic such that, in their absence, the pathologist may question the diagnosis. These include arthropod bite reactions and scabies, urticarial dermatitis, and other eosinophilic dermatoses. 2) Lesional eosinophils rare or absent, such that, in their presence, the pathologist may question the diagnosis. These include pityriasis lichenoides, graft versus host disease, and connective tissue disorders. 3) Lesional eosinophils variable and, while in some cases expected, are not required for diagnosis. These include drug reactions, atopic dermatitis and allergic contact dermatitis. 4) Lesional eosinophils variable and not expected but may be seen to a limited extent. These include lichen planus and psoriasis.
Topics: Humans; Eosinophils; Diagnosis, Differential; Psoriasis; Lichen Planus; Dermatitis, Atopic; Skin; Skin Diseases
PubMed: 37003367
DOI: 10.1016/j.humpath.2023.03.017 -
Dermatology Practical & Conceptual Jan 2017Pityriasis lichenoides is an uncommon skin disease that presents in three different forms: pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides...
BACKGROUND
Pityriasis lichenoides is an uncommon skin disease that presents in three different forms: pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC) and febrile ulceronecrotic-Mucha-Habermann disease. These represent a spectrum of a disease. PLEVA presents as skin eruption of multiple, small, red papules that develop into polymorphic lesions with periods of varying remissions, as well as possible sequels of hyper/hypopigmentation and varicella-like scars. Diagnosis of this condition is mainly clinical, and sometimes clinical differentiation from other conditions may be a difficult task that often requires histological analysis. In this study, PLEVA lesions were examined by dermoscopy, and the significance of specific dermoscopic findings was investigated in order to facilitate their differentiation from other inflammatory conditions.
OBJECTIVES
To evaluate dermoscopic patterns in PLEVA and to correlate these patterns with histopathology.
MATERIALS AND METHODS
The study was conducted at S. Nijalingappa Medical College, Bagalkot. It was an observational case series study and patients were selected randomly. Ethical clearance and informed consent were obtained. PLEVA lesions in early and late phases were evaluated. A manual DermLite 3 (3Gen, San Juan Capistrano, CA) dermoscope attached to a Sony (Cyber Shot DSC-W800, Sony Electronics Inc., San Diego, California, USA, digital, 14 mega pixels) camera was employed. Histopathology was done to confirm the diagnosis. Data was collected and analyzed. Results were statistically described in terms of frequencies and types of dermoscopic patterns.
RESULTS
There was a total of 14 patients; 8 males and 6 females. Mean age of patients was 19 years. Mean duration of disease was 7 months. Dermoscopy in early-phase lesions revealed amorphous brownish areas around the hair follicles, dotted vessels, and scaling. Dermoscopy in late-phase lesions showed whitish-structureless areas and central white crust within whitish-structureless rim with scale, focal bluish-grayish areas or centrifugal strands irregularly distributed along the periphery and yellow structures. Red dots and hemorrhage were seen at the center and glomerular vessels at the periphery.
CONCLUSION
PLEVA demonstrates specific dermoscopic patterns that correlate well with histologic changes. New dermoscopic findings are described. Thus, dermoscopy is a good diagnostic tool in the clinical diagnosis of PLEVA.
PubMed: 28243491
DOI: 10.5826/dpc.0701a05