Search

Cell adhesion molecule 1 (CADM1) is one of the immunoglobulin super family adhesion molecules, that is proposed to contribute in the pathogenesis of various types of cutaneous T-cell lymphoma, including mycosis fungoides (MF). In this work, we decided to examine the immunohistochemical expression of CADM1 in MF specimens compared to premycotic parapsoriasis, benign inflammatory dermatosis and normal control skin specimens. 125 participants were enrolled (50 MF, 25 parapsoriasis, 25 inflammatory dermatosis, and 25 healthy controls). Patients were selected from the Outpatient Clinic of Dermatology and Venereology Department, Tanta University Hospitals. From all, 4 mm punch skin biopsies were taken and examined for CADM1 immunohistochemical expression. The current study revealed statistically significant upregulation of CADM1 expression in MF specimens in comparison to parapsoriasis, inflammatory dermatosis, and normal control specimens. Additionally, there was statistically significant positive correlation between CADM1 expression and progression of TNMB staging of MF disease. Therefore, it is possible to recommend CADM1 as a beneficial diagnostic immunohistochemical marker for differentiation between early stages of MF and both the premycotic parapsoriasis and benign inflammatory dermatosis. Moreover, it may be of value in early detection of neoplastic transformation of parapsoriasis as well as in assessment of MF progression., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Poikilodermatous mycosis fungoides (pMF) is characterized by poikiloderma areas, typically involving the major flexural areas and trunk. Its presentation can be generalized or admixed with other forms of MF. Previous studies fail to correlate the clinical presentation with prognosis and laboratory findings. Some reports show pityriasis lichenoides chronica (PLC) preceding the poikiloderma., Objectives: Correlate prognostic, histopathological and molecular aspects of pMF with its clinical presentation., Methods: Retrospective analysis of 14 cases of generalized pMF (GpMF), 22 of localized pMF (LpMF) and 17 of pMF admixed with other forms of MF (mix-pMF)., Results: Female predominance and lower age at diagnosis was found in all groups compared to classic MF, a high prevalence of PLC-like lesions in the GpMF group and a high rate of hypopigmented lesions in the mix-pMF group. There were 2 deaths within the GpMF group. Histology was similar to previously reported findings, as was the prevalence of CD4 T-cell infiltrate, compared to CD8. The T-cell clonality positivity was lower in the GpMF group, compared to other groups (27% GpMF, 80% LpMF and 100% mix-pMF)., Discussion: This is the first article to categorize the different forms of pMF and correlate them with clinical and laboratory findings. The dermatological presentation differs among the groups. There was a high frequency of PLC-like lesions within the GpMF group and of hypopigmented lesions in mix-pMF. The histological and immunohistochemical findings were similar to those previously reported. Aggressive treatments are not recommended due to the good prognosis of all pMF forms. The low positivity of T-cell clonality in the GpMF group should be investigated., (© 2019 S. Karger AG, Basel.)

There is a broad differential diagnosis for the presentation of fever and maculopapular rash in an adult. Although some causative conditions are benign, others are medical emergencies that require prompt diagnosis. We describe various conditions that result in a fever and maculopapular rash in adults. These include infectious processes (meningococcemia, infectious mononucleosis, West Nile virus, zika virus, rubella, primary human immunodeficiency virus, parvovirus B19, ebolavirus), tick-borne illnesses (Rocky Mountain spotted fever, ehrlichiosis), and hypersensitivity reactions (exanthematous drug reactions). We also provide an algorithm to aid in the diagnosis of the patient with fever and maculopapular rash. Such conditions that can occur in adults but are seen predominantly in children are discussed in the article "Rash with maculopapules and fever in children" of this issue., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Several medical conditions can cause children to present with fever and a maculopapular rash Although some presentations are benign, others may be medical emergencies, which warrant a prompt diagnosis. We review some of the more common causes of fever and maculopapular dermatitirs, rash including infectious processes (roseola; rubeola; rubella; parvovirus B19; hand, foot, and mouth disease; scarlet fever; meningococcemia; Epstein-Barr virus infection), hypersensitivity reactions (exanthematous drug reactions), and vasculitis syndromes (Kawasaki disease). We have included a diagnostic algorithm to facilitate rapid identification of the etiology of the rash and fever. Those conditions that can occur in children but are seen predominantly in adults are discussed in the contribution "Rash with maculopapules and fever in adults" in this issue., (Copyright © 2018. Published by Elsevier Inc.)

Mycosis fungoides (MF) is the most common primary cutaneous epidermotropic T-cell lymphoma (80%). The accurate diagnosis of MF confirmed only by clinical, histological and immunohistochemical signs amounts to 50-75%., Objective: To investigate genetic markers (FOXP3, STAT4, IL-12B) for the early diagnosis of MF, to estimate the informative value of used diagnostic techniques (histology, immunophenotyping), and to determine clonality by the T-cell receptor γ-chain genes., Material and Methods: Fifty patients with MF and plaque parapsoriasis (PP) who had been treated at the V.A. Rakhmanov Clinic of Skin and Venereal Diseases and at the National Medical Research Center for Hematology were followed up. A MF group consisted of 27 patients; a PP group included 23 patients, and a control group comprised 10 healthy individuals. The expression of the FOXP3, STAT4, and IL-12B genes was analyzed by TaqMan real time-PCR. The objectives of the study were affected skin portions from patients with MF or PP and healthy individuals., Results: The investigation revealed a increase in the expression level of STAT4 mRNA transcripts by 9 times in patients with MF compared with those with PP and by 553 times in healthy individuals. There was also a statistically significant predominance of the expression level of STAT4 mRNA transcripts in patients with spotted and plaque stages of MF (180; 318) compared with those with PP and healthy individuals, as well as a sharp decrease in those with erythrodermic MF, which was statistically significant., Conclusion: MF cannot be diagnosed without comprehensively assessing the clinical, anamnestic, histological, immunophenotypic, and molecular genetic data. The expression level of STAT4 mRNA transcripts is of great importance for the early diagnosis of MF. Inclusion of the level of STAT4 expression in the list of diagnostic signs increases the accuracy of differential diagnosis of MF and PP from 59.1 to 81.8%, respectively.

Background: Mycosis fungoides (MF) and parapsoriasis are characterized by malignant proliferation and chronic inflammation, which may affect the risk for venous thromboembolism (VTE)., Objectives: To examine the risk for VTE in patients with MF and parapsoriasis., Methods: We conducted a nationwide population-based cohort study in Denmark to examine the relative risk (RR) of VTE in 525 patients with MF and 634 patients with parapsoriasis compared with that in sex- and age-matched controls from the general population., Results: In patients with MF, the 10-year absolute risk for VTE was 3.4% (95% confidence interval [CI], 2.0-5.4). The adjusted RRs were 2.41 (95% CI, 1.49-3.90) for VTE and 4.01 (95% CI, 2.16-7.46) for pulmonary embolism. Notably, within the first 5 years after diagnosis with MF, the RR of pulmonary embolism was increased 6.7-fold (to 6.71 [95% CI, 2.86-15.72]). Patients with parapsoriasis had a 2.7-fold increased RR of VTE (to 2.67 [95% CI, 1.32-5.40]) in the absence of other established VTE risk factors., Limitations: We had no information regarding disease stage of MF and prescribed drugs., Conclusion: Patients with MF and parapsoriasis had an increased RR of VTE, although the absolute risk remained low. These findings should increase awareness of comorbidities in patients with MF and parapsoriasis., (Copyright © 2017 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Staphylococcal enterotoxins have been shown to promote lymphoma-associated immune dysregulation. This study examined changes in the skin microbiome of parapsoriasis compared with intact skin. Swab microbiome specimens were taken of the parapsoriasis lesions of 13 patients. Control samples were taken from contralateral healthy sides of the body. Microbiotas were characterized by sequencing the V1-V3 region of the 16S ribosomal RNA bacterial genes on the Illumina MiSeq platform. The most common genera in the microbiome data were Propionibacterium (27.13%), Corynebacterium (21.20%) and Staphylococcus (4.63%). Out of the Staphylococcus sequences, 39.6% represented S. epidermidis, with the rest including S. hominis, S. capitis and unidentified species. No significant differences were observed between the patients' parapsoriasis and contralateral healthy skin or between large- and small-plaque parapsoriasis. Notable interpersonal variation was demonstrated. These results suggest that parapsoriasis is not associated with significant alterations in the cutaneous bacterial microbiome.

Background: Inpatients with cutaneous adverse drug reactions (CADR) with overlapping features between maculopapular exanthema (MPE) and drug reaction with eosinophilia and systemic symptoms (DRESS) were examined., Objectives: To characterize patients with exanthema and few systemic symptoms not meeting the criteria for DRESS [overlapping MPE-DRESS (MP/DR)]., Methods: We undertook a comparative analysis of clinical and laboratory features of patients with MPE, MP/DR and DRESS (2008-12)., Results: We identified 132 inpatients (85 women/47 men, mean age 64·0 ± 17·7 years) with CADR, 37 with DRESS, 28 with MPE, 34 with MP/DR and 33 with other patterns. There were no significant differences in sex, age or concomitant diseases. Allopurinol was the main cause of DRESS (40·5%) and MP/DR (29·4%); antimicrobials were the main cause in MPE (35·7%). In MP/DR the latency period (18·06 ± 13·17 days) was significantly longer than in MPE but shorter than in DRESS. Although hospitalization time was similar to DRESS (13·26 ± 7·41 days), duration of therapy and follow-up in MP/DR was shorter. Exanthema/erythroderma were frequently associated with facial oedema in MP/DR (73·5%) and DRESS (89·2%) but only in 42·0% of patients with MPE. MP/DR histopathology showed keratinocyte vacuolization and perivascular and interstitial infiltrate of lymphocytes, eosinophils and neutrophils, similar but milder than in DRESS, with less interface dermatitis, exocytosis and spongiosis. DRESS was associated with liver involvement (78·4%) and eosinophilia (78·4%), but only in 64·7% and 11·8%, respectively, of patients with MP/DR., Conclusions: An overlapping pattern between MPE and DRESS was identified and characterized. There may be a continuum spectrum between MPE and DRESS., (© 2016 British Association of Dermatologists.)

Mycosis fungoides (MF) and parapsoriasis display increased inflammation, which may be associated with increased risk of arterial cardiovascular events. The aim of this Danish nationwide population-based cohort study was to assess the relative risk (RR) of acute myocardial infarction (AMI) or stroke in patients with MF and parapsoriasis. In patients with MF, the RR of AMI or stroke was 1.0 (95% confidence interval (95% CI) 0.7-1.3). In the second half of the study period, the RR was 1.8 (95% CI 1.1-2.9) during the first 5 years of follow-up. In men with parapsoriasis, the RR of AMI or stroke was 1.7 (95% CI 1.1-2.7) within the first 5 years of follow-up, whereas the RR of AMI during the first 5 years of follow-up was 2.0 (95% CI 1.2-3.4). In conclusion, patients with MF and parapsoriasis have an increased RR of AMI or stroke within the first 5 years of follow-up.

Introduction: Primary cutaneous lymphomas are lymphoproliferative skin infiltrates of T-, B- or NK-cells, classified according to the World Health Organization - European Organization of the Research and Treatment of Cancer (WHO-EORTC) criteria. They are the second most common group of extranodal non-Hodgkin lymphomas, that present in the skin with no evidence of systemic involvement at the time of diagnosis., Aims: The aim of the study was the analysis of clinical profile of cutaneous lymphomas in the tertiary referral center in Poland., Material and Methods: We analyzed case records of 63 patients (26 women, 37 men aged 19 - 86) referred to the Department of Dermatology, University Hospital in Cracow for the diagnosis and treatment of cutaneous lymphoma., Results: After analysis of clinical and histological data, the final diagnoses were: mycosis fungoides (42 patients), primary cutaneous CD30+ lymphoproliferative disorder (7), Sezary syndrome (3), parapsoriasis (3), primary cutaneous B-cell lymphoma (1), acute myeloid leukemia (1), Hodgkin lymphoma coexistent with mycosis fungoides (1), generalized allergic contact dermatitis (2) and erythema elevatum diutinum (1). We excluded 2 patients due to incomplete data. The most common location of skin lesions was the lower limb (52.46%) and most common clinical presentation was raised erythematous lesion (26.23%). Pruritus was present in 45.9% of the patients and 39.3% had extracutaneous symptoms, with lymphadenopathy as the most common symptom. 37.7% of patients presented with mild eosinophilia and another 37.7% with mild monocytosis. Prior to referral to our center, general practitioners misdiagnosed the lymphomas commonly as: atopic and contact dermatitis, borreliosis, drug-induced exanthema., Conclusions: The diagnosis of cutaneous lymphoma is often delayed due to their indolent, often recurring course, non-specific symptoms and uncommon appearance. The cooperation of a clinician and pathologist is essential in the diagnostic process.

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.

Mycosis fungoides (MF) is the most prevalent cutaneous lymphoma, characterized by uncontrolled growth of T cells within the skin. The diagnosis in the early stages may be difficult, since, in these stages, the disease can imitate benign inflammatory processes, both clinically and histologically. One of the difficulties in determining diagnostic criteria for early MF is the existing terminology of parapsoriasis. There is disagreement concerning the exact definition and whether it is a chronic benign condition with similar clinical and histological characteristics as MF, or rather it is part of the disease spectrum itself. There is a need for uniformity in the definition for treatment decisions, for epidemiological purposes and for clinical and experimental research. This article reviews the different approaches to parapsoriasis as part of MF over the years.

We report a case of extensive tinea corporis in an 80-year-old woman on her forearms, thighs, legs, buttocks and trunk, mimicking parapsoriasis due to Trichophyton schoenleinii, without scalp involvement. Diagnosis of Trichophyton schoenleinii was confirmed by microscopy and mycological culture specimens., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

In the presented article we discuss the problems of lichenoid and plaque parapsoriasis. The difference in Russian and English classifications are discussed in the historical aspect, as well as review of the literature, and personal authors' observations of nine patients with "small plaque parapsoriasis".

Parapsoriasis is a chronic dermatosis whose biological distinction from early mycosis fungoides, the most frequent form of cutaneous T-cell lymphoma, is still not clearly defined. Two types of parapsoriasis have been delineated: large-plaque parapsoriasis and small-plaque parapsoriasis. The lack of clinical and histological features, which may allow distinguishing parapsoriasis from early mycosis fungoides has prompted several investigations to assess the role of immunohistochemistry in establishing a conclusive diagnosis of these conditions. However, the additional data obtained by immunohistochemical analysis concerning the CD4/CD8 ratio, the aberrant expression of T-cell antigens and the expression of proliferation markers has not generally helped establish a more definitive diagnosis. This review critically discusses these immunohistochemical markers and their use in diagnosis of parapsoriasis., (Copyright © 2010 Elsevier GmbH. All rights reserved.)

Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it difficult to diagnose mycosis fungoides in the early patch stage. Early diagnosis of mycosis fungoides (MF) is important for deciding on type of therapy, prognosis and for further follow-up. However, until recently, there has been no consensus on criteria that would help in diagnosing the disease early. Some believe that large plaque parapsoriasis (LPP) should be classified with early patch stage of MF and should be treated aggressively. However, there is no firm clinical or laboratory criteria to predict which LPP will progress to MF and we can only discuss about statistical probability. Moreover, long-term outcome analysis of even patch stage of MF is similar to that of control population. We therefore believe that LPP should be considered as a separate entity at least to prevent the patient from being given a frightening diagnosis. We also feel that patients need not be treated with aggressive therapy for LPP and will need only a close follow-up. This article emphasizes the criteria for diagnosing early MF and has highlighted the importance of considering LPP as a distinct benign entity.

Background: It is unsettled whether small plaque parapsoriasis (SPP) represents an inflammatory dermatosis or has a potential to transform into mycosis fungoides (MF) or is, in fact, MF. The literature contains no fully documented example of progression of SPP into MF., Objective: The purpose of our study was to present a patient with clinical features of SPP who later developed plaque-stage MF, as seen both clinically and pathologically and to compare the clinicopathologic features of this unique case with 27 "nonprogressive" SPP cases., Methods: This study is a prospective and retrospective evaluation of 28 patients, using light microscopy, immunohistochemistry, and molecular biology., Results: A 56-year-old man with a 3-year history of persistent SPP with typical small (<5 cm), elongated and "digitate" lesions presented with newly developed larger patches and plaques. Whereas histologic examination of the patch lesion revealed relatively nonspecific features, a specimen of the crusted plaque showed a dense lymphoid infiltrate composed of small cerebriform lymphocytes, medium-sized lymphoid cells, and occasional large hyperchromatic cells that infiltrated the basal layer of the epidermis and formed small collections. There were atypical mitotic figures. Immunohistochemically, an aberrant immunophenotype with the loss of CD5 expression was found in the plaque specimen. T-cell receptor (TCR)-gamma gene rearrangement studies detected clones in the plaque and in the peripheral blood (biallelic in blood), while the patch tested polyclonal. The 27 SPP patients included 23 men and 4 women, ranging in age from 29 to 75 years. They were followed up and treated for 1.2 to 52 years (mean 10); no patient's SPP progressed into MF. All patients presented with small patch lesions measuring 3 to 6 cm lengthwise and 0.5 to 2 cm in width. Histologic features were nonspecific. Molecular genetic studies revealed the following results: two cases tested polyclonal, 3 cases demonstrated the oligoclonal pattern, whereas the remaining 13 specimens showed a pattern which can be interpreted as oligoclonal or pseudomonoclonal., Limitations: Oligoclonal and monoclonal patterns were overrepresented in the SPP group, which may be due to the low amount and, probably, suboptimal quality of DNA used in the TCR-gamma rearrangement studies., Conclusions: Occasionally patients with the clinical and pathologic presentation of SPP may develop typical features for MF. This event seems to be extremely rare; at present there appears to be no means to predict such a course. The vast majority of SPP patients will never have disease progression to MF.

Small plaque parapsoriasis (SPP) is one of the cutaneous T-cell lymphoproliferative disorders. The aim of the present study was to show the antigenic profile of a subset of dendritic cells and lymphocytes in SPP in comparison with normal cells to provide data on the role of these two cell types in the pathogenesis of SPP. Skin biopsy specimens of lesions were obtained from 8 patients with SPP. Biopsies of the healthy skin from 9 control individuals were also analyzed. Immunohistochemistry was performed on the frozen tissue sections to reveal binding of anti-HLA Class II, anti-CD1a, anti-CD4, anti-CD8, anti-CD44, anti-CD45, and anti-CD68 monoclonal antibodies. There was a statistically significant increase in the number of CD1a(+), Langerhans cells (LCs), HLA-DR-immunoreactive and, CD1a-positive dermal dendritic cells and CD68(+) macrophages in the SPP group (p=0.008, 0.008, 0.002 and <0.0009, respectively). The number of lymphocytes positive for CD4, CD8 and CD45 was significantly higher than normal in the SPP group (p=0.015, <0.0009 and <0.0009, respectively). Our study demonstrates that both peptide- and lipid-based antigens are involved in the persistent antigenic exposure in SPP. Dendritic cells play a pivotal role in SPP by presenting antigens by both LC and dermal dendritic cells via MHC Class II and CD1a molecules. The CD68(+) macrophages are thought to be involved in the immune response in this pathology as an antigen-presenting cell.

Inverse psoriasis, rare in clinical practice, refers to psoriasis only or mainly occurring at flexural sites, such as the axilla, antecubital fossae, popliteal fossae, and inguinal creases. It is also known as flexural psoriasis. With a total collection of psoriatic cases from September 2002 to December 2003 at Xijing hospital, we made a retrospective analysis of the disease history, clinical characteristics, and treatment of the patients affected with inverse psoriasis. The results showed that the major clinical manifestations of inverse psoriasis were sharply demarcated erythematous plaques with varying degrees of infiltration and minimal or no scales. Affected areas often involve the groin, axilla, genitals, and umbilicus. The disease responds well to the narrow band UVB therapy. Compared with common psoriasis, inverse psoriasis has similar and unique characteristics in terms of the affected areas, clinical symptoms, and responses to the treatment.

Background: Erythromelanosis follicularis of the face and neck, originally described in Japan by Kitamura et al. in 1960, is characterized by a clinical set of three: well-demarcated erythema, hyperpigmentation and follicular papules. It affects the face and the neck generally on both sides. Since the original description, it has seldom been reported in the literature., Case Reports: This paper reports two patients with unilateral presentation., Discussion: Having discussed the various differential diagnoses all published cases were listed and analyzed. The prevalence of this disease appears higher than is shown by the limited number of cases reported in the literature. It deserves more recognition. Its nosologic and aetiologic frames still remain to be clarified.

A study investigating the causes of rash diseases using systematic laboratory testing was conducted in Niterói, Rio de Janeiro, between January 1994 to April 1998. Sera from 327 patients were tested for evidence of anti-rubella virus, measles virus, human parvovirus B19 and dengue fever virus specific immunoglobulin IgM and anti-human herpes virus type 6 (HHV-6) IgG antibodies. A laboratory confirmed diagnosis was achieved in 71.3% of the cases investigated: dengue fever (33.0%), rubella (20.2%), parvovirus B19 (9.2%), measles (6.7%) and HHV-6 (2.1%). No diagnosis was established for 94 cases (28.7%). An outbreak of measles was detected during 1997, with a peak in September and October. All of the diseases studied here presented with clinical features similar to measles and classical symptoms were found in all measles confirmed cases. The large overlap of combinations of signs and symptoms seen in this study highlights the difficulties of diagnosing a rash illness on clinical grounds alone.

Introduction: Small plaque parapsoriasis is a relatively rare, chronic, idiopathic dermatosis, most often seen in middle age people. This disease shows a definite male predominance of approximately 3-4: 1. It is characterized by presence of round or oval erythematous, slightly scaly plaques on the limbs and trunk, which histologically reveal mild eczematous changes., Case Report: A male patient, 61 years of age, was admitted to the Clinic of Dermatovenereology in Novi Sad due to long persisting erythematous patches on his upper and lower limbs. Plaques were of oval and round shape, pretty well marginated. They were of light red colour, covered with fine scales with a slightly wrinkled surface. He complained of itching. Laboratory findings showed no abnormalities. Histopathologic examination of the skin specimen revealed epidermal atrophy, focal parakeratosis, perivascular dermal infiltrate of mononuclear cells with exocytosis in the epidermis. This finding was compatible with the clinical diagnosis. After treatment with topical corticosteroid cream combined with whole body exposure to sunlight irradiation, vast majority of skin lesions regressed., Discussion: The clinical course of small plaque parapsoriasis is very long. The plaques are remarkably stubborn, responding to treatment with steroid creams or to natural or artificial sunlight, but usually reappearing promptly when treatment is discontinued. The patches increase in number for a time, and then remain relatively constant for a long time. A small minority of cases clears entirely. Recent studies provided evidence of monoclonality and immunophenotypic abnormalities. Rearrangement of T-cell receptor genes was demonstrated by using PCR method. Detection of monoclonal T-cell populations in skin lesions, as a characteristic of lymphoproliferative diseases, forced some authors to include this dermatosis into a group of abortive cutaneous T-cell lymphomas., Conclusion: This case deserves a long and probably life-long clinical and histological assessment, especially due to new knowledge about the possible nature of this disease.

Cutaneous T-cell lymphomas represent a group of malignant lymphoproliferative disorders characterised by the occurrence of a monoclonal population of T-lymphocytes. Diagnosis of early stages of this disease is a difficult challenge for both the dermatologist and the dermatopathologist. With the aid of the polymerase chain reaction it is possible to amplify specific regions of the T-cell receptor gamma gene. The amplification products can then be separated by denaturing gradient gel electrophoresis in order to detect a monoclonal population of T-lymphocytes in the infiltrate. We studied 4 patients with the clinicopathologic diagnosis of mycosis fungoides and 2 patients diagnosed as large plaque parapsoriasis. A monoclonal population was detected in 3 of the 4 mycosis fungoides cases and in 1 of the patients with large plaque parapsoriasis. This indicates that our analysis can help us establishing a diagnosis, and it can also help us to identify patients with a possible early stage of the disease, which clinically or histologically is not yet recognised as such.

Four patients suffering from small plaque parapsoriasis were treated successfully with balneophototherapy. Within 4 weeks salt-water baths and UV irradiation resulted in clinical clearing of more than 90% of lesions with a duration of total clinical response between 8 and 12 weeks without further maintenance treatment.

Xanthoerythrodermia perstans (XEP) is a distinctive variant of large-plaque parapsoriasis. Along with alopecia mucinosa and lymphomatoid papulosis, there is a strong association between large-plaque parapsoriasis and evolving cutaneous T-cell lymphoma (CTCL). In some reports, large-plaque parapsoriasis was suggested to be a precursor lesion that converted to CTCL in 10 to 30 percent of cases. We describe a patient who presented clinically with both XEP and alopecia mucinosa and was subsequently shown to have CD-30 CTCL.

Adult onset Still's disease is a rare condition presenting with high spiking fever, transient maculopapular rash, myalgias, polyarthralgias or arthritis, lymphadenopathy, hepatosplenomegaly and a sore throat, associated with leucocytosis and neutrophilia. Early diagnosis is difficult because clinical features are non-specific. We report a 33-year-old Chinese female with this condition who presented with recurrent high spiking fever and rash over a 4-month period. We highlight the sequence of events leading to this diagnosis with emphasis on the cutaneous changes.

We report on a 34-year-old male patient who developed generalized parakeratosis variegata lesions 4 years after suffering from pityriasis lichenoides et varioliformis acuta. For further investigation of a possible interrelationship between these two diseases of the parapsoriasis group and their relationship to the T-cell type of cutaneous non-Hodgkin-lymphoma, histological, immunohistological and molecular-biological techniques were applied. We were able to demonstrate typical morphological features common to both diseases, and a polyclonal T-cell infiltrate in both. It is concluded that pityriasis lichenoides et varioliformis acuta and parakeratosis variegata are separate entities without monoclonal rearrangement or signs of malignancy.

The clinical features, histopathology, immunopathology, and management of pityriasis lichenoides and lymphomatoid papulosis are discussed, with particular emphasis on the pediatric aspects of these conditions. The difficulties in logically separating pityriasis lichenoides into an acute (pityriasis lichenoides et varioliformis acuta) and a chronic (pityriasis lichenoides chronical) form are addressed. The development of lymphoreticular malignancy in patients with lymphomatoid papulosis has been well documented, but pityriasis lichenoides has characteristically been regarded as a benign condition. However, recent reports of the development of large plaque parapsoriasis in patients with pityriasis lichenoides have led to a reconsideration. Some of these patients were in the pediatric age group. Although there are significant clinical, histopathological, and immunopathological differences between pityriasis lichenoides and lymphomatoid papulosis, the demonstration of similar clonal T cell receptor gene rearrangements and the confirmation of the potentially premalignant nature of both suggests that there may indeed be an interrelationship between these two controversial entities. Close follow-up of patients with both of these conditions is recommended, with observation being discontinued only when the patient has been free of lesions for several years.

A case is reported of rarely observed skin changes in a girl aged 5 years. The changes resembled those observed in acute parapsoriasis (p. lichenoides et varioliformis of Mucha-Habermann). The diagnosis was established after finding characteristic polymorphic lesions in the form of papulae, necrotizing vesicles, ulcerations, desquamation of certain papulae typical of p. guttata, long-term persistence of the lesions and good general condition of the child. The lesions were situated on the trunk, and in a lower degree on the face and extremities. Before the disease the girl hand contact with insecticides (Ovadofox) and detergents.

The authors report a case of Mucha-Habermann disease in childhood. Mucha-Habermann disease is not a very well known, though not infrequent, disease. It is characterized by recurrent erythematous-papular-vesicular skin lesions associated with arthralgia or arthritis or large joints. Prognosis is generally favourable although an evolution towards Pityriasis Lichenoides Chronica and/or Mycosis Fungoides is possible. There are not specific laboratory findings for this form. Diagnosis is essentially based on histology showing an immunopathogenetic vasculitis. At the present time there is not a safe therapy for the disease; there are however indications for the use of Erythromycin and we followed these in our therapy with positive results.