Department of Dermatology, New York University School of Medicine, New York, NY 10016, USA. amorkh@pol.net
Pityriasis lichenoides (PL) is an uncommon, acquired spectrum of skin conditions that poses various challenges to patients as well as clinicians. It is a difficult and debatable disorder to diagnose, categorize, and treat. Besides these inherent obstacles, PL merits awareness because of its potential to progress to cutaneous lymphoma or an ulceronecrotic presentation, both of which carry a significant risk of mortality. The scope of PL presentations is delineated along a continuum of multiple variants including pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC), and febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Classification of these presentations as separate subsets is debatable in view of their overlapping clinical, histopathologic, and etiologic features. PLEVA generally presents as an acute-to-subacute skin eruption of multiple, small, red papules that develops into polymorphic lesions and vacillates with periods of varying remissions as well as possible sequelae of hyper/hypopigmentation and varicella-like scars. PLC has a more gradual manifestation of very small red-to-brown flat maculopapules with mica-like scale; it also follows a relapsing course but with long periods of remission. FUMHD is an acute and severe generalized eruption of purpuric and ulceronecrotic plaques with associated systemic involvement and a mortality rate of up to 25%; hence, it should be approached as a dermatologic emergency.Histopathological evaluation of PL usually reveals dermal, wedge-shaped, lymphocytic infiltrate, epidermal spongiosis, parakeratosis, and variable necrosis of keratinocytes. PLC demonstrates more subtle histology whereas, at the other end of the spectrum, febrile ulceronecrotic FUMHD exhibits the most exaggerated histological features. The pathogenic mechanism behind PL is unclear although infectious or drug-related hypersensitivity reactions versus premycotic lymphoproliferative disorder are the mainstay theories. The foremost therapies for PLEVA and PLC are phototherapy, systemic antibacterials, and topical corticosteroids. Aggressive treatment with immunosuppressant and/or immunomodulating agents as well as intensive supportive care are recommended for FUMHD. We first describe a representative case of a 14-year-old boy with PLC who was successfully treated with narrow-band UVB. We then review the pathophysiology, classification, and treatment of PL.
Latest citations:
Dermatology, Pediatrics, and Pathology, New Jersey Medical School, Newark, NJ 07103, USA.
Pityriasis lichenoides et varioliformis acuta (PLEVA), or Mucha-Habermann disease (MHD), is a cutaneous disorder evident with crops of erythematous macules and papules, usually on the trunk and flexural areas of the extremities. Its etiology remains unknown. PLEVA is speculated to be an inflammatory reaction triggered by certain infectious agents, an inflammatory response secondary to T-cell dyscrasia, or an immune complex-mediated hypersensitivity. Histologic examination of a skin biopsy specimen is the standard for the identification of PLEVA, but definitive diagnosis may be difficult. Apart from the febrile ulcerative variant, which may be fatal, PLEVA tends to be self-limited in its course. Treatment is targeted mainly at the symptomatic relief of pruritus.
Department of Dermatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Background: Current knowledge about the efficacy of narrowband UVB therapy for the treatment of pityriasis lichenoides is limited. Objective: The aim of this study was to investigate the efficacy of narrowband UVB therapy for the treatment of pityriasis lichenoides chronica (PLC). Methods: Data were retrospectively collected following a review of patient phototherapy and medical charts, and telephone interviews were performed for follow-up information. Results: The study included 25 patients (14 male, 11 female) with a mean age of 34+/-13 years. Diagnosis was confirmed by histopathological examination in 92%(n = 23) of the patients. The median duration of the disease was 24 months (range: 2-192 months). The median number of sessions until response was 25 (8 weeks)(range: 9-77 sessions), with a median cumulative dose of 15 J/cm(2)(range: 2-158 J/cm(2)). Complete response and partial response were achieved in 48% and 44% of the patients, respectively, while 8% of the patients achieved no response. In those patients for whom follow-up data were available (n = 17), 10 (58%) relapsed within a median of 9.5 months (range: 1-44 months). Conclusions: Our results suggest that narrowband UVB is an effective and well-tolerated treatment option for PLC.
J Dtsch Dermatol Ges. 2008 Sep 19;:
18808379
University Clinic Schleswig-Holstein -Campus Kiel, Clinic for Dermatology, Venerology and Allergology (Director: Prof. Dr. T. Schwarz), Germany.
Viruses cause not only direct infectious exanthems, but also parainfectious exanthems, which provoke skin alterations via interactions with the immune system. These distinct exanthems, for instance Gianotti-Crosti syndrome and pityriasis lichenoides group, do not reflect a specific pathogen but can occur in the course of many viral infections. In addition, some exanthems result from the interaction between viruses and drugs.
Department of Ophthalmology, Mayday University Hospital, London, UK. thibault.verhamme@gmail.com
CLINICAL REPORT: We report a case of a 42-year old lady with recurrent bilateral nodular conjunctival inflammation following a diffuse papulo-vesicular rash, mainly over her trunk and scalp. Slitlamp examination revealed limbal and bulbar inflammatory nodules with secondary corneal involvement. DISCUSSION: Pityriasis lichenoides is considered to be a spectrum of uncommon, acquired maculo-papular skin eruptions. The diagnosis is made by the combination of a typical clinical picture and matching histopathology. In severe cases, there may be associated with mucous membrane involvement. CONCLUSION: Although ocular involvement has been reported in pityriasis lichenoides, this is the first report of conjunctival inflammatory nodules with secondary corneal ulceration as a part of the manifestations of the pityriasis lichenoides spectrum. Treatment with topical steroid drops was required to bring this condition under control.
Department of Dermatology, Faculty of Medicine, Uludag University, Bursa, Turkey. aydogank@uludag.edu.tr
BACKGROUND/PURPOSE Narrowband (NB) UVB (NB-UVB) phototherapy has recently demonstrated high levels of efficacy and tolerability in a variety of skin diseases. The purpose of the present study was to assess the efficacy of NB-UVB phototherapy in the management of pityriasis lichenoides (PL). METHODS The therapeutic response in 31 PL patients (23 pityriasis lichenoides et varioliformis acuta; PLEVA, eight pityriasis lichenoides chronica; PLC) treated with NB-UVB phototherapy between 2000 and 2007 was assessed. RESULTS NB-UVB treatment led to a complete response (CR) in 15 out of 23 PLEVA patients (65.2%) with a mean cumulative dose of 23 J/cm(2) after a mean number of 43.4 exposures and a partial response (PR) in eight patients (34.8%) with a cumulative dose of 15.6 J/cm(2) after a mean number of 32.3 exposures. NB-UVB treatment led to CR in seven out of eight PLC patients (87.5%) with a mean cumulative dose of 18.4 J/cm(2) after a mean number of 45.8 exposures and PR in one patient (12.5%) with a cumulative dose of 9.1 J/cm(2) after a mean number of 19 exposures. Relapses occurred in four PL patients within a mean time period of 6 months. CONCLUSION NB-UVB therapy is an effective, safe and practical alternative treatment modality for the management of PLEVA and PLC.
Department of Clinical Medicine and Immunological Sciences - Section of Dermatology, University of Siena, Italy.
OBJECTIVES Pityriasis lichenoides chronica (PLC) is a skin disease of unknown etiology. Uncertainty about the etiopathogenesis of this skin disease is the reason for the unpredictable and non optimal efficacy of therapies available for its treatment. The aim of the present study was to evaluate the efficacy of bromelain, a crude aqueous extract of the stems and immature fruit of pineapple, in the treatment of PLC. MATERIALS AND METHODS Eight patients (3 males and 5 females) with PLC were enrolled in the study and treated for three months with oral bromelain (40 mg 3 times a day for 1 month, 40 mg twice a day for 1 month and 40 mg/day for 1 month). RESULTS All patients showed complete clinical recovery after treatment. In 12 months of follow up, two patients experienced relapse 5-6 months after suspension of therapy but responded to another brief cycle of therapy. No side effects were encountered during therapy. CONCLUSIONS In conclusion bromelain can be considered an effective therapeutic option for PLC; its efficacy could be related to its anti-inflammatory, immunomodulatory and/or anti-viral properties.
Other papers by authors:
Georgetown University Medical Center, Washington, DC, USA.
Division of Dermatology, Georgetown University Medical Center, Washington DC, USA. Amorkh@pol.net
A 28-year-old man with a 6-year history of subcorneal pustular dermatosis (SPD) is presented. This case reviews the clinical features, differential diagnosis, etiology, and treatment options of SPD. In particular, this case emphasizes the absence of response to dapsone and colchicine and control with PUVA therapy.
Dermatol Online J. 2012 ;18 (7):7
22863629
Boston University Medical Center, Boston, Massachusetts.
Atypical presentations of genital herpes are more commonly described among immuno-compromised patients. Furthermore, verrucous lesions may be attributed to acyclovir resistant strains of herpes simplex virus as well as co-infection with fungi and other viruses in this patient population. We describe the first case of verrucous genital herpes infection in an immuno-competent patient.
Ann Saudi Med. ;32 (3):283-7
22588440
Department of Dermatology, King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Saudi Arabia. kmgderm@yahoo.com
BACKGROUND AND OBJECTIVES Mycosis fungoides (MF) is a rare disease; and to our knowledge, there are no reports on its profile in Arabs. The objective of this study was to preliminarily analyze the clinical characteristics of MF patients seen in our institution. DESIGN AND SETTING Retrospective review of 140 patients with pathologic or clinical diagnosis or differential diagnosis of MF for the period 2000-2006. PATIENTS AND METHODS Pathology reports with diagnosis or differential diagnosis of MF were retrieved and suspected cases were identified and reviewed. For pathologically confirmed cases, sociodemographic, clinical, laboratory, and radiological details were collected. Details of staging, treatment modalities, and disease status at the last follow-up were retrieved. RESULTS A total of 43 pathologically confirmed MF patients (skin phototypes IV and V) with a mean age at diagnosis of 33.5 years were reviewed. This comprised 29 males (M:F ratio, 2:1), and the majority (86%) of patients had early-stage (I and II) MF. Twenty-one (48.8%) patients had classic MF; 18 (41.8%), hypopigmented MF; and 4 (9.3%), other variants. The male-to-female ratio was higher in the hypopigmented (3.5:1) than in the classic variant (1.6:1). The mean age at diagnosis was lower in the hypopigmented compared to the classic variant (25 versus 38.8 years, P=.019). The mean duration of follow-up was 27.6 months (range, 1-98 months). At the final assessment, 4 (9.5%) patients recovered; whereas 35 (83.3%) had MF skin disease; 1 had (2.4%) extracutaneous disease; and 2 (4.8%) died of MF. CONCLUSIONS MF tends to affect younger Saudi patients. The hypopigmented variant constitutes a significant proportion of MF cases, especially in younger patients.
Skinmed. ;9 (5):313-4
22165047
King Faisal Hospital, Saudi Arabia, USA. amoa65@hotmail.com
Claude Huriez (1907-1984) was a well-known French dermatologist. In the 1960s, Huriez and colleagues reported two families from northern France with a rare congenital genodermatosis-later known as Huriez syndrome. This syndrome is characterized by scleroatrophy of the hands and feet, nail hypoplasia, mild palmoplantar keratoderma, and hypohidrosis and is a cancer-prone genodermatosis. This report sheds light on Huriez and the syndrome that bears his name.
Dermatol Online J. 2011 ;17 (9):12
21971277
Albert Einstein College of Medicine.
Glomus tumors (GT) are rare, soft-tissue tumors commonly found on the extremities. Because these tumors occur most often in the subungual region, they can be confused with other subungual pathologies including melanoma. Subungual GTs have a unique presentation and history but biopsy is diagnostic. Various imaging techniques are useful in diagnosis and management. We describe a classical case of subungual GT and discuss its presentation, diagnosis, management, as well as "atypical" or "malignant" variants.
JAAPA. 2011 Jun ;24 (6):20
21682171
University of South Florida, College of Medicine, Tampa, Florida, USA.
Stritch School of Medicine, Loyola University Chicago, Maywood, Illinois, USA.
Honey has been used to treat wounds throughout the ages. This practice was rooted primarily in tradition and folklore until the late 19th century, when investigators began to characterize its biologic and clinical effects. This overview explores both historic and current insights into honey in its role in wound care. We describe the proposed antimicrobial, immunomodulatory, and physiologic mechanisms of action, and review the clinical evidence of the efficacy of honey in a variety of acute and chronic wound types. We also address additional considerations of safety, quality, and the cost effectiveness of medical-grade honeys. In summary, there is biologic evidence to support the use of honey in modern wound care, and the clinical evidence to date also suggests a benefit. However, further large, well designed, clinical trials are needed to confirm its therapeutic effects.
Latest similar papers:
Cutan Ocul Toxicol. 2012 Sep 13;:
22974328
Hospital de Curry Cabral, Dermatology and Venereology Department , Lisbon , Portugal.
Pityriasis lichenoides is a benign disease that includes a continuous spectrum with two polar ends: pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC). Although its benign and self-limited character, treatment is required, both for itch relief and for cosmetic issues. The present study is a retrospective analysis of 13 patients (11 PLC and 2 PLEVA) treated in our institution with psoralen plus ultraviolet A (PUVA) or ultraviolet A combined with ultraviolet B (UVA/UVB) during the period 1998-2011. In the PUVA group, complete response was achieved in five patients and partial response in two. Total cumulative UVA dose was 84.4 J/cm(2). One patient quit therapy without therapeutic response. In the UVA/UVB group, complete response was achieved in two patients and partial response in an equal number of patients. One patient did not reach a significant improvement. Total cumulative doses were: 26.1 J/cm(2) for UVA and 3.62 J/cm(2) for UVB. There were no acute side effects in either therapeutic group. In the present study, PUVA phototherapy was preferred for patients with more widespread or long-evolving disease, while UVA/UVB was selected for patients who presented more recent disease or contraindications for PUVA therapy. Regardless of the absence of clinical guidelines, both therapeutic options proved to be successful, ascertaining phototherapy as an effective and safe option for pityriasis lichenoides patients.
*Kempf und Pfaltz Histologische Diagnostik †Department of Dermatology, University Hospital, Zurich, Switzerland ‡Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Czech Republic §Dermatopathologie Bodensee, Friedrichshafen, Germany ∥Department of Pathology, Necker-Enfants Malades Hospital, APHP, Universite Paris Descartes, Paris, France ¶Department of Dermatology, Medical University of Graz, Graz, Austria.
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.
Department of Pathology, 12 de Octubre University Hospital, Madrid, Spain.
Irritant contact dermatitis is a common cause of genital eruptions representing at least one-fifth of presenting anogenital symptoms. A spectrum of inflammatory reactions have been identified, some of them with features severely mimicking more serious dermatoses, which may lead unnecessary workup and treatments. We report a case of a 10-year-old girl who presented at birth with cloacal atresia involving the rectum and the urethra. A diverting colostomy and a urethral-vaginal fistula were created to correct the deformity. Physical examination reveals numerous shiny, white-gray, pseudoverrucous papules and nodules coalescing into plaques over the vulva and its surrounding skin. Histological examination showed psoriasiform epidermal hyperplasia with a marked reactive acanthosis and altered cornification with parakeratosis, hypogranulosis, and pale keratinocytes in the upper reaches of the epidermis. The lesions regress when the irritating factor was removed. This case represents a peculiar form of presentation of perianal pseudoverrucous papules and nodules, usually secondary to urinary incontinence or encopresis (inability to control the elimination of stool) after surgery for Hirschsprung disease. Because similar findings have not been previously observed in patients with urethral-vaginal fistula, we attempt to extend the spectrum of presentations for a better knowledge of this condition.
Dermatology, Pediatrics, and Pathology, New Jersey Medical School, Newark, NJ 07103, USA.
Pityriasis lichenoides et varioliformis acuta (PLEVA), or Mucha-Habermann disease (MHD), is a cutaneous disorder evident with crops of erythematous macules and papules, usually on the trunk and flexural areas of the extremities. Its etiology remains unknown. PLEVA is speculated to be an inflammatory reaction triggered by certain infectious agents, an inflammatory response secondary to T-cell dyscrasia, or an immune complex-mediated hypersensitivity. Histologic examination of a skin biopsy specimen is the standard for the identification of PLEVA, but definitive diagnosis may be difficult. Apart from the febrile ulcerative variant, which may be fatal, PLEVA tends to be self-limited in its course. Treatment is targeted mainly at the symptomatic relief of pruritus.
Elizabeth Lazaridou,
Christina Fotiadou,
Christina Tsorova,
Maria Trachana,
Anastasia Trigoni,
Aikaterini Patsatsi,
Demetris Ioannides
First Departments of Dermatology & Venereology, Aristotle University Medical School, Thessaloniki, Greece. bethlaz@med.auth.gr
Pityriasis lichenoides et varioliformis acuta (PLEVA) represents the acute clinical subtype of pityriasis lichenoides (PL) and its occurrence is relatively common during childhood. Diagnosis and treatment may sometimes pose certain difficulties. We present the recalcitrant case of a 3-year-old boy with an asymptomatic polymorphic eruption consisting of multiple, scattered, 0.5 cm, round to ovoid, erythematous papules covered in places with a fine scale, vesicles and superficial erosions with thick hemorrhagic crusts. The correlation of the clinical features with the lesional histopathology favored the diagnosis of PLEVA. No first-line treatment scheme succeeded in controlling the eruption of new lesions. The only therapeutic approach that eventually managed to cease the disease evolution was the combination of prednisolone and methotrexate.
Department of Medicine, Waitemata District Health Board, Auckland, New Zealand.
Febrile ulceronecrotic Mucha-Habermann disease is a rare severe variant of pityriasis lichenoides et varioliformis acuta, a disease within the pityriasis lichenoides spectrum of disorders. It remains uncertain whether these disorders are preneoplastic or reactive against infectious or other antigenic stimuli. Febrile ulceronecrotic Mucha-Habermann disease varies in severity and may be accompanied by a range of systemic symptoms. We present a case associated with herpes simplex virus infection. In this case, no significant T cell clone was identified. Clonality may be a prognostic marker, but reports with T-cell receptor polymerase chain reaction results are limited to eight previous cases, and further reports are required. Awareness of the condition is important because of its fulminant potential.
First Dermatologic Clinic, Aristotle University of Thessaloniki, Greece.
Febrile ulceronecrotic Mucha-Habermann disease is a severe variant of pityriasis lichenoides et varioliformis acuta characterized by the sudden onset of ulceronecrotic skin lesions and associated with high fever and systemic symptoms. We report here a case of a 20-year-old woman in whom the disease started as pityriasis lichenoides et varioliformis acuta and evolved to febrile ulceronecrotic Mucha-Habermann disease. Almost 90% of the body surface was involved, together with high fever and malaise. Steroids alone proved to be an insufficient therapeutic procedure. The remission achieved was attributed to the use of methotrexate. To our knowledge, only 39 cases of febrile ulceronecrotic Mucha-Habermann disease have been reported in the literature to date.
Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY 10065, USA.
BACKGROUND The primary function of regulatory T cells (Treg cells) is to regulate the function and proliferation of immunologically responsive T cells; the transcription factor Foxp3 is expressed by this cell populace and is held to be the standard marker for Treg cells. DESIGN A variety of cutaneous T-cell lymphocytic infiltrates were evaluated for Foxp3 expression. RESULTS Of the 95 cases, 33 (35%) were reactive, 40 (42%) were prelymphomatous cutaneous T-cell dyscrasia and 22 were (23%) T-cell lymphoma. The reactive category included dermatomyositis, lupus erythematosus, hypersensitivity reactions and graft-vs.-host disease. The prelymphomatous dyscrasia category was represented chiefly by pityriasis lichenoides chronica (PLC) and pigmented purpuric dermatosis (PPD). The Foxp3 reactivity was less than 10% for cases of dermatomyositis and lupus erythematosus, 23% for hypersensitivity cases, 0% for graft-vs.-host disease, 16% for the dyscrasias and 11% for the lymphomas. Intermediate grade and aggressive lymphomas had very few Foxp3+ cells (< 5%). There were fewer numbers of Foxp3+ T cells in the monoclonal variants of PLC and PPD. CONCLUSIONS T-reg cells may play a role in controlling the extent of T-cell proliferations in the skin with a lack of T-regulatory function permissive to the development of various T-cell disorders.
Department of Dermatology, University Hospital, School of Medicine, Malaga, Spain. norbertoln@hotmail.com
Keratosis lichenoides chronica (KLC) is a rare, acquired disorder of keratinization of unknown aetiology. The disease has a chronic and progressive course and is characterized by a poor response to almost all topical treatments and most systemic regimens. We report the first case of KLC in which there was a marked response in localized areas to photodynamic therapy (PDT) with methyl 5-aminolaevulinic acid (ALA).
Department of Dermatology, Faculty of Medicine, Uludag University, Bursa, Turkey. aydogank@uludag.edu.tr
BACKGROUND/PURPOSE Narrowband (NB) UVB (NB-UVB) phototherapy has recently demonstrated high levels of efficacy and tolerability in a variety of skin diseases. The purpose of the present study was to assess the efficacy of NB-UVB phototherapy in the management of pityriasis lichenoides (PL). METHODS The therapeutic response in 31 PL patients (23 pityriasis lichenoides et varioliformis acuta; PLEVA, eight pityriasis lichenoides chronica; PLC) treated with NB-UVB phototherapy between 2000 and 2007 was assessed. RESULTS NB-UVB treatment led to a complete response (CR) in 15 out of 23 PLEVA patients (65.2%) with a mean cumulative dose of 23 J/cm(2) after a mean number of 43.4 exposures and a partial response (PR) in eight patients (34.8%) with a cumulative dose of 15.6 J/cm(2) after a mean number of 32.3 exposures. NB-UVB treatment led to CR in seven out of eight PLC patients (87.5%) with a mean cumulative dose of 18.4 J/cm(2) after a mean number of 45.8 exposures and PR in one patient (12.5%) with a cumulative dose of 9.1 J/cm(2) after a mean number of 19 exposures. Relapses occurred in four PL patients within a mean time period of 6 months. CONCLUSION NB-UVB therapy is an effective, safe and practical alternative treatment modality for the management of PLEVA and PLC.
|
FP7 Partner
Add BioInfo.PL bioinformatics lab
to Your FP7 application bioinfo.pl
|
|
SME Funding
High-tech startup fundraising, evaluation and profit sharing
picostocks.com
|