Regression Of Skin Lesions Research Articles

Detection of Leishmania infantum in a naturally infected horse in an endemic area for Leishmaniasis in Northeast Brazil

Introduction: Leishmaniasis is an anthropozoonosis caused by a protozoan of the genus Leishmania and transmitted by vectors of the species Lutzomyia longipalpis. The disease can present in two forms: the cutaneous form (Cutaneous Leishmaniasis) and the visceral form (Visceral Leishmaniasis). Its main hosts are domestic canids, but it can parasitize other diverse species such as rodents, possums, foxes, non-human primates, edentulous primates, domestic cats, and even equids, although this is infrequent. Study objective: Due to the scarcity of reports on Leishmaniasis in horses. Added to this, taking into account the susceptibility of horses to infection by many species of Leishmania and the fact that the vast majority of cases are asymptomatic, the objective of this study was to report a case of equine leishmaniasis in the state of Pernambuco. Results and discussion: A male horse, of the Manga Larga Machador breed, four years old, residing in the municipality of Bezerros- PE, Northeast of Brazil, was treated. On physical examination, the presence of ulcerated and non-ulcerated nodular lesions was observed on the face close to the masseter muscle, in the ventrodorsal region, and below the humeroradiounar joint. Presence of papules in the neck region. In the blood count, normocytic normochromic anemia, and leukocytosis due to neutrophilia and eosinophilia were observed. In the parasitological diagnosis through immunohistochemistry, the presence of Leishmania sp. amastigotes was observed. As a therapeutic protocol, injectable marbofloxacin was used for this patient at a dosage of 2mg/kg once a day, intramuscularly for 28 days. Conclusion: Horses infected by L. infantum may present dermatological lesions. Therefore, early diagnosis and treatment are recommended, as it is suggested that the use of marbofloxacin provides good results in the regression of skin lesions in these cases. However, more studies are needed to investigate the epidemiology and pathophysiology of this disease in horses.

Leucoderma in Buffaloes (Bubalus bubalis) in the Amazon Biome.

Leucoderma is a condition that affects the skin and hair of animals, causing depigmentation and acromotrichia. In buffaloes, this condition results in significant economic losses for the production chain due to its impact on the leather trade. This study aimed to investigate the epidemiological and clinicopathological aspects of leucoderma in buffaloes in the Amazon biome and describe the prophylactic treatment to control the disease. The study included 40 buffaloes, 16 males and 24 females, aged between 1 and 10 years, and were of the Murrah, Jafarabadi, Mediterranean, and Murrah × Mediterranean crossbreed breeds. The animals were raised without mineral supplementation. The clinical signs observed in the animals included acromotrichia and depigmentation, with varying degrees and distribution of skin lesions. Histological examination of the epidermis showed interrupted melanin production, mild dermal fibrosis, mild perivascular mononuclear inflammatory infiltrate, and pigmentary incontinence. None of the animals had the genotype for albinism. After 120 days of mineral supplementation based on the use of copper sulfate, the clinical signs of leucoderma regressed. There was no predisposition by breed, sex, or age for the occurrence of the disease. The regression of skin lesions after proper mineral supplementation suggests that copper deficiency may be considered an important factor for the occurrence of leucoderma in buffaloes in the Amazon biome.

Bisphosphonates for the Treatment of Calcinosis Cutis-A Retrospective Single-Center Study.

(1) Background: Calcinosis cutis is a frequent symptom of autoimmune connective tissue diseases leading to pain, transcutaneous expulsion of calcified material and bacterial superinfection. There is a high need for new therapeutic options as no standardized treatment algorithm is established. While case reports indicate beneficial effects of bisphosphonates, standardized evaluation of treatment effects is missing. (2) Methods: In this retrospective analysis we evaluate the effects of intravenous pamidronate, a second-generation bisphosphonate, in seven patients with calcinosis cutis using consecutive clinical pictures, radiological examinations and patient’s subjective evaluation. (3) Results: 5/6 patients reported a reduction of pain, improvement of general condition and cessation of calcinosis progression. Regression of skin lesions was detectable in clinical pictures of 2/6 patients, while 1/6 patients had stable disease. Radiological examination revealed improvement or stable disease in 3/5 patients. Fever was the most common side effect. One out of seven patients developed osteonecrosis of the jaw. (4) Conclusions: Bisphosphonates appear to have beneficial effects in a subgroup of calcinosis cutis patients. While patient’s subjective evaluation was mainly positive, objective assessments showed improvement in approximately half of the cases. With regard to potential severe side effects, a careful risk-benefit evaluation is necessary before treatment initiation.

S3064 Henoch-Schӧnlein Purpura in the Context of Sars-CoV-2 Infection—Practice Management

Introduction: Henoch–Schönlein purpura (HSP), is a self-limiting vasculitis involving the small vessels of the skin, gastrointestinal tract, kidneys, joints, and rarely the lungs and central nervous system (CNS). The severe acute respiratory syndrome coronavirus-2 (Sars-CoV-2) infection may be a condition associated with the development of vasculitis sustained by an exacerbated IgA-mediated immune response. Case Description/Methods: In the context of the current pandemic, we report the case of a 32-year-old patient who showed clinical features of HSP that appeared in a few days after the diagnosis of Coronavirus Disease 2019 (COVID-19) mild form, confirmed by PCR test. He presented an acute onset with marked asthenia, edema and painful bilateral swelling of the knee, associated with difficulty walking. Small flat lesions distributed on the upper and lower extremity and on the posterior-chest have been identified, one day later. He needed hospitalization in a medical center where he received corticosteroid therapy, anticoagulant, antiviral with regression of skin lesions and joint swelling. After five days the skin lesions expand and after 48 hours there is intense abdominal pain accompanied by accelerated intestinal transit with blood. The objective examination reveals a rash on both lower extremity, which extends from the soles to the ankles (Figure 1). Examination of the knee joints shows no local erythema, but a painful limitation of passive movements is noted. Laboratory data indicate a biological inflammatory syndrome, proteinuria and hematuria. The immunological profile does not indicate the presence of non-COVID-19 reactive arthritis. Upper gastrointestinal examination performed at 4 days reveals gastric vascular spots. There are no evidence of suggestive lesions for inflammatory bowel disease (IBD) observed both on colonoscopy and abdominal CT based on 7 mm parietal thickening of the descending colon and sigmoid. Clinical, laboratory, imaging and endoscopic data establish the diagnosis of HSP with nephritic syndrome and reactive arthritis in the context of Sars-CoV-2 infection. Under cortisone treatment, purpura disappears completely after 10 days. Discussion: In our patient the presence of purpura in the absence of thrombocytopenia, as a mandatory criterion, in association with other criteria, namely arthritis reactive at onset, renal and digestive impairment, support according to EULAR / PRINTO / PRES criteria, the diagnosis of Henoch–Schönlein purpura.Figure 1

Unsatisfactory agreement using current classification of maculopapular cutaneous mastocytosis.

Mastocytosis is a disease characterized by abnormal accumulation and expansion of tissue mast cells in the skin, bone marrow or other organs.1 Cutaneous (CM) and systemic forms (SM) of mastocytosis have been distinguished.1 CM is usually diagnosed in childhood and has a good prognosis with regression of skin lesions during puberty. Recently, many adults with mastocytosis are identified because of hymenoptera venom anaphylaxis together with increased baseline serum tryptase levels, even without skin lesions. However, in most adults, mastocytosis is still diagnosed because of their cutaneous manifestations.2 The 2001 WHO classification divided CM into maculopapular CM (urticaria pigmentosa, UP, MPCM), the most common form in children and adults, diffuse cutaneous mastocytosis (DCM) and solitary mastocytoma in childhood-onset mastocytosis, which is still valid. Skin lesions differ between children and adults with mastocytosis3 and may have some value in making a prognosis for disease progression or regression2: although the extent and maximum density of MPCM skin lesions are comparable between children and adults, children have a significantly higher diameter of lesions, a different predominant distribution more often involving the head and show a more variable clinical picture.3 Based on dermatological morphological criteria, a plaque and a nodular form have been delineated in children and a telangiectatic subvariant (also referred to as telangiectasia macularis eruptiva perstans, TMEP) in adults.4 The existence of the latter as singular entity has meanwhile been questioned by our expert group, but has brought attention to the existence of more erythematous less pigmented lesions in adult MPCM. In 2016, a prognostic simplistic consensus subclassification for MPCM has been proposed defining two variants: (i) a monomorphic variant, typically seen in adult patients, presenting with macules or papules of 3–5 mm diameter or (ii) a polymorphic variant with larger lesions of variable size, colour and shape, often asymmetric and typically seen in children.5 The hypothesis, backed up by initial evidence, is that monomorphic lesions in children indicate the presence of systemic disease and persistence of mastocytosis into adulthood, whereas polymorphic lesions might correspond to a good prognosis. The study by Torrelo et al.6 in this issue of the Journal describes their experience with the validation of this classification. They presented pictures of 19 sample cases with childhood mastocytosis to 10 European or US experts experienced with mastocytosis and asked them to classify these cases. Pictures were also displayed to 129 general dermatologists after explaining, reading and discussing the relevant text in the consensus report for 15 min and showing the examples presented in it. The value of kappa interobserver variability coefficient for the 10 experts was ‘fair’ and for the group of 129 dermatologists ‘slight’. This has been considered inadequate because of missing agreement among experts and dermatologists far below expectations for a satisfactory classification system. This is highly disturbing as unambiguity is needed for a good classification. The outcome signals a need for readjustment or for a different classification system. The definition of polymorphic and monomorphic variants has been insufficiently explained, is mostly intuitive and may be misleading. Clear instructions on how (i) shape, (ii) colour and (iii) size should be measured objectively and exactly which criteria clearly distinguish polymorphic from monomorphic are lacking. For dermatologists, this terminology may be confusing. ‘Polymorphism’ describes the occurrence of two or more clearly different morphs or forms. The diagnosis ‘polymorphic light dermatosis’ depicts that the same disease may show different morphological patterns in different patients. The term ‘polymorphic lesions’ describes lesions of different shapes and sizes in one given patient. However, the degree of difference needed to call lesions ‘polymorphic’ remains undefined, as reflected by the results of the study.6 We dermatologists make our diagnoses based on morphology and on our nomenclature of patterns and features. Thus, we specify distribution and lesion morphology, such as macules, papules, plaques, nodules or tumours, often with objective parameters such as sizes, colours and shapes to describe a patient precisely. Although using monomorphism versus polymorphism of shape, colour and/or size in the same patient may be a key for MPCM classification, it may not present the best possible system, as it does not follow the dermatologist's way of thinking and leads to disagreement among observers. For dermatologists, it would come much more natural to use the commonly used terminology defining subcategories of macular, papular, nodular or plaque forms for describing the phenotype of a given patient. One simple possibility for a two-category approach might be by size alone, for example small-sized individual lesions ≤6 mm also allowing confluence to larger patches versus individual lesions >6 mm, which at least would be measurable, understandable and lead to a better agreement among observers. The author declares no conflicts of interest. No external sources of funding.

Grover’s disease in a patient with atopic dermatitis - a case report

Introduction. Grover?s disease is characterized by pruriginous polymorphic rash with a variable course and duration. Although the etiology is still unknown, the disease is often associated with other dermatoses, malignant diseases, use of certain medications, as well as immunosuppression. Case Report. We report a case of a 70-year-old male patient who was referred for examination to the Clinic of Dermatovenereology Diseases, Clinical Center of Vojvodina, due to a rash that lasted for nine months. The first lesions on the skin appeared around the nipples as exudative eczematous plaques. A few months later, identical lesions appeared on the lower legs. During treatment with systemic antihistamines and topical corticosteroids, there were episodes of transient improvements and re-exacerbations. In the meantime, erythematous brownish, round and oval papules appeared on the abdomen and the back, accompanied by intense itch. Laboratory findings revealed eosinophilia and elevated serum immunoglobulin E levels. A skin biopsy of the back lesion was performed and the histopathological examination confirmed the diagnosis of Grover?s disease. After the systemic treatment using corticosteroids and antihistamines, with gradual dose reduction and application of topical corticosteroids and emollients, complete regression of the skin lesions was achieved. Conclusion. Since the clinical manifestations of the disease may be nonspecific and discrete, dermatopathological analysis is of crucial importance in making the correct diagnosis. In patients with atopy, the treatment with systemic corticosteroids, antihistamines and topical agents may lead to regression of skin lesions with a significant improvement in the quality of life.

Kaposi’s Disease (KS) in a Senegalese Child Living with HIV

Kaposi’s Disease or Kaposi’s Sarcoma (SK ) is a multifocal malignant proliferation induced by viral growth factors, including interleukin 6 of human herpes virus type 8 (HHV8). We describe four forms of this disease who poses a real public health problem in East and Central Africa. The purpose of our observation was to report a rare condition in a Senegalese HIV-positive child. It was an 11-year-old girl from a region in central Senegal. She was an orphan of both parents, tested and monitored since the age of 5 for HIV infection 1. She was on the 1st line protocol. Due to a lack of support and good observance, she was referred to us at the age of 11 for follow-up in our structure in the suburbs of Dakar. The initial follow-up assessment showed a very low CD4 count and a very high viral load. Before the lack of clinical and immune-virological response, a genotypic resistance test was performed and showed immunological and virological failure. The initial development was marked by the appearance of lesions which were highly suggestive of Kaposi’s disease. She was on 2nd line treatment. The histopathological aspect of cutaneous biopsy was very suggestive of Kaposi’s disease. The subsequent course after ART and bleomycin treatment was clinically marked by regression of skin lesions. Virologically, it was marked by a fall in the viral load. Immunologically there was a gradual recovery of CD4 levels which came back to normal. Our observation demonstrates that absence of effective antiretroviral therapy for HIV increases the risk to develop Kaposi’s sarcoma.

Childhood molluscum contagiosum treated with 585 nm pulsed dye laser: a clinical observation

Objective To evaluate the efficacy and safety of 585 nm pulsed dye laser for the treatment of molluscum contagiosum in children. Methods Between September 2016 and February 2017, 50 children with confirmed molluscum contagiosum were enrolled from Maternal and Child Health Care Hospital of Zibo. These children were treated with 585 nm pulsed dye laser at energy of 6.0 - 9.0 J/cm2, pulse width of 0.5 ms and spot size of 5 mm. If a patient was not cured after 1 session of the laser treatment, another session of the laser treatment would be performed 1 month after the first treatment. If the proportion of regressed skin lesions was less than 30% after more than 2 consecutive sessions of the laser treatment, the treatment would be stopped. After the treatment, the patients were followed up for 6 months. During the follow-up, clinical efficacy was evaluated according to regression of skin lesions, and adverse reactions and recurrence were recorded. Results All the patients completed the trial. After the treatment, 44 (88%) patients were cured, and the response rate was 94% (47/50) . After 1 session of the treatment, 32 (64%) patients were cured. After the laser treatment, purpura occurred instantly, hyperpigmentation and hypopigmentation occurred in a short-term period, and no obvious long-term adverse reactions were observed. During the follow-up, no new wart was found at the site of regressed warts after the treatment. Conclusion The 585 nm pulsed dye laser shows good efficacy, safety and tolerance in the treatment of molluscum contagiosum. Key words: Lasers, dye; Treatment outcome; Adverse reactions; Molluscum contagiosum

Subcutaneous fat necrosis of the infant

Subcutaneous fat necrosis of the newborn and infant is a rare disease, with still unknown incidence, which usually occurs in term or post-term newborns that have experienced perinatal stress. It usually occurs within the fi six weeks of newborn’s life; howe ver, onset of the disease may be delayed for several months. A 6-week-old female infant was admitted to our department due to failure to thrive, irritability and vomiting. Physical examination in the area of the inner thighs, hips, back and shoulders, revealed the presence of subcutaneous infi ltrations, which were fi rm, slightly livid, and did not seem painful to touch. Laboratory analysis showed hypercalcemia, ultrasonographic review of body fat revealed hyperechogenicity, while abdominal ultrasound revealed nephrocalcinosis. Computerized tomography detected the presence of calcifi cations in the brain. Deep skin biopsy confi rmed the diagnosis o f subcutaneous fat necrosis. Treatment included fl loading, termination of vitamin D substitution, and low calcium diet. Singl e doses of calcitonin and pamidronate were administered. After this therapy, calcium levels returned to normal range. Subcutaneous infi ltrates gradually decreased and became softer. In most reported cases, regression of skin lesions is expected after a few m onths, often without any residue on the skin. Elevated serum calcium may persist long after the withdrawal of cutaneous lesions, which is the reason for continuous monitoring of serum calcium and appropriate treatment in case of hypercalcemia in order to prevent metastatic calcifi cation.

Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic study of 7 cases

To improve the clinicopathological understanding of blastic plasmacytoid dendritic cell neoplasm (BPDCN). A total of 7 BPDCN patients were recruited from 2009 to 2013. And their clinicopathological and immunohistological features, treatment and prognosis were retrospectively analyzed. There were 6 males and 1 female. The median age range was 60 (6-72) years at the time of diagnosis. The skin lesion at the time of diagnosis presented as generalized (n = 6) or solitary (n = 1) plaques or nodules. Histologic examination showed a diffuse or nodular infiltrate composed of intermediate-sized cells in dermis sparing epidermis area. The tumor cells contained round to ovoid nucleus, finely dispersed chromatin and 1 or 2 small-sized nucleoli. Most neoplastic cells were positive for CD4, CD56 and CD123 while negative for CD3, CD20, CD30, CD34, CD79a, MPO and EBER. Two patients received chemotherapy.One had no response while another relapsed rapidly. Two patients had a spontaneous regression of skin lesions and one died 2 years later. And no systematic infiltration was detected at diagnosis. BPDCN is a rare hematopoietic malignancy with specific immunophenotypes.It primarily affects elderly males and frequently presents initially as cutaneous involvement.It may be easily misdiagnosed and has a rapid course and a poor prognosis.

Intérêt de l’échographie rénale dans la cytostéatonécrose sous-cutanée

Subcutaneous fat necrosis is an uncommon disease that may be complicated with potentially fatal hypercalcemia or with nephrocalcinosis. We report on the case of a patient with a history of significant perinatal asphyxia, hospitalized for a urinary tract infection. Lesions of subcutaneous fat necrosis were noted, with asymptomatic hypercalcemia at 3.9mmol/L. A renal ultrasound was performed and showed echogenic medullary pyramids bilaterally, consistent with nephrocalcinosis and left nephrolithiasis. The treatment of hypercalcemia included hyperhydration, a diuretic and corticosteroids. Progression was characterized by the total regression of skin lesions and normalization of serum calcium. Hypercalcemia is a rare complication of subcutaneous fat necrosis. It develops within days to weeks after the appearance of skin lesions. Nephrocalcinosis appears after several weeks or months. Hypercalcemia must be treated in due time to avoid the impact on the kidney.

Subacute cutaneous lupus erythematosus in the course of rheumatoid arthritis: a relationship with TNF-α antagonists and rituximab therapy?

Introduction: Drug-induced subacute cutaneous lupus erythematosus (DI-SCLE) is caused by different medicines, first of all: calcium channel blockers, angiotensin converting enzyme inhibitors, thiazides, terbinafine, statins and antagonists of tumor necrosis factor-α (TNF-α). DI-SCLE does not distinguish from idiopathic form of the disease, clinically, histopathologically and immunologically. However, receding of symptoms is observed after recapture of the provoking drug.Aim: To present a patient with rheumatoid arthritis (RA), who developed SCLE after treatment with TNF-α antagonists and rituximab.Case report: In a 31-year-old woman with RA leucopenia due to treatment with etanercept and adalimumab was observed. Therefore, the treatment was changed to rituximab, but after starting the therapy, erythematous and oedematous skin lesions of an oval or annular shape appeared on the cheeks, auricles, lips and the decolette. Histopathological evaluation of the skin lesions revealed SCLE. Ro/SS-A and La/SS-B antibodies were detected in serum. Regression of skin lesions and hematologic disturbances was achieved after starting corticosteroid therapy.Conclusions: Co-existence of SCLE with RA should be considered in some patients. The role of TNF-α antagonists and rituximab therapy in induction of idiopathic form of SCLE requires further investigations.

Primary Cutaneous Hydroa Vacciniforme–Like Lymphoma With Indolent Clinical Course

Primary cutaneous hydroa vacciniforme (HV)-like lymphoma is a rare, Epstein-Barr virus-associated cutaneous neoplasm characterized by photosensitive papulovesicular eruption and usually associated with poor prognosis. This report presents 2 cases of primary cutaneous HV-like lymphoma with unusual indolent clinical course and favorable prognosis during the follow-up periods of 2 and 3 years, respectively. Both patients presented with erythema, papulovesicles, scars, ulcerations, and edema on the face and extremities. Skin biopsies revealed epidermal vesicle with small- to medium-sized atypical lymphoid cells infiltrating in the dermis and subcutis. The lymphoid cells were strongly immunoreactive to CD8 and CD56. The Epstein-Barr virus genomes were also found in both skin biopsies. By genetic analysis, one of patients showed T-cell receptor-γ gene clonal rearrangement. The patients underwent glucocorticoid treatment and obtained remarkable clinical improvement with regression of skin lesions. No sign of recurrence and extracutaneous manifestation was found during the period of follow-up. A long-term follow-up is suggested to be performed to inspect the progression for this tumor.

Topical nitrogen mustard therapy in patients with Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is characterized by abnormal proliferation and infiltration of Langerhans cells in different organs. The skin is frequently involved either as unisystem or multisystem disease. To review the clinical response and side-effects of nitrogen mustard therapy in LCH in children and adults with unisystem or multisystem disease. This retrospective study includes 10 children and four adults with LCH, treated with nitrogen mustard from 1975 to 2010. The median extent of skin involvement was 46% (range 5-100%). Overall, 13 patients had complete or partial response. Although eight patients achieved a complete response with a median time of 12·3months (range 36 days to 1·9 years), six of these patients ultimately relapsed. One patient, who had unisystem disease limited to the skin, initially showed progression of her cutaneous lesions with nitrogen mustard treatment. Although subsequently the cutaneous lesions completely regressed, concomitant systemic involvement was noted. Four other patients similarly experienced improvement of their skin lesions with treatment, but also exhibited progression of the LCH systemically. The patients were treated with other therapies prior and adjunctive to nitrogen mustard. However, five patients had progression to other organs, despite regression of skin lesions, which supports that the treatment effect in the skin is related to topical nitrogen mustard. Six patients developed contact dermatitis to nitrogen mustard. Topical nitrogen mustard can be an effective and safe therapy in both children and adults with cutaneous LCH, although relapses are common.

Hydrogels containing rutin intended for cutaneous administration: efficacy in wound healing in rats

Objective: Development of a hydrogel containing rutin at 0.025% (w/w) and evaluation of its in vivo efficacy in cutaneous wound healing in rats.Methods: Hydrogels were prepared using Carbopol Ultrez® 10 NF and an aqueous dispersion of rutin in polysorbate 80. Hydrogels were characterized by means of pH measurement, rheological and spreadability analysis and rutin content determination by liquid chromatography. The in vivo healing effect was evaluated through the regression of skin lesions in rats and by analysis of oxidative stress.Results and discussion: Hydrogels showed adequate pH values (5.50–6.50) and pseudoplastic non-Newtonian behavior. After 5 days of treatment of wounds, hydrogels containing rutin presented a higher decrease in the wound area compared to the control hydrogels. Analysis of the oxidative stress showed a decrease in lipid peroxidation and protein carbonyl content as well as an increase in catalase activity after the treatment with the hydrogel containing rutin. Furthermore, this treatment increased total protein levels.Conclusion: This study shows for the first time the feasibility of using dermatological formulations containing rutin to improve skin wound healing.

Pyoderma gangrenosum as a initial manifestation of ulcerative proctocolitis

AbstRACt: pyoderma gangrenosum is a rare inflammatory skin condition characterized by progressive and recurrent skin ulcer - ation of destructive course. It is usually associated with rheumatoid arthritis, paraproteinemia, myeloproliferative diseases and in - flammatory bowel diseases, especially non-specific ulcerative proctocolitis. In these situations, skin lesions are described as concurrent with the intestinal condition. However, reports on pyoderma gangrenosum preceding intestinal findings are less frequent. The authors describe a case of a woman with febrile condition associated with skin lesions diagnosed by biopsy as pyoderma gangrenosum. Two weeks later, she developed diarrhea, arthralgia and sepsis, being diagnosed as ulcerative proctocolitis. After the administration of the treatment for ulcerative proctocolitis, she showed improvements in sepsis care, remission of diarrhea and regression of skin lesions. This case highlights the importance of considering pyoderma gangrenosum as a manifestation associated with inflammatory bowel disease, regardless of its timing in relation to intestinal symptoms.

Primary cutaneous blastic plasmacytoid dendritic cell neoplasm without extracutaneous manifestation: Case report and review of the literature

Blastic plasmacytoid dendritic cell (BPDC) neoplasm is a rare, highly aggressive hematopoietic malignancy with involvement of bone marrow and peripheral blood. We present 2 cases of primary cutaneous BPDC neoplasm without extracutaneous manifestation during the course of disease. A 36-year-old and a 51-year-old male presented with erythematous patches, purple plaques, and nodules on the head, trunk, and extremities. Skin biopsies revealed that the lesions of both cases were composed of diffusely medium-sized monomorphic blastoid cells infiltrating into the dermis and subcutis. The neoplastic cells were strongly positive for CD4, CD56, CD123, and TdT, whereas other T-cell markers and EBV markers were not expressed. The patients underwent polychemotherapy with hyper-CVAD regimen and obtained a remarkable clinical response with regression of skin lesions. No sign of recurrence and extracutaneous manifestation was found during the period of follow-up. We presume that the favorable prognosis of our cases might result from the presentation only with a skin lesion, diffuse TdT expression in tumor cells, and aggressive chemotherapy with hyper-CVAD regimens. Laboratory examination for blood and bone marrow should be performed every 3–6 months during the first period of follow-up to monitor the progression of disease even if the patients had complete remission at initial chemotherapy.

Long-term follow-up study of clear cell papulosis

Clear cell papulosis (CCP) was described as a new entity in 1987. Since then, only case reports or small case series have appeared in the literature and the long-term outcome of CCP remains unknown. The aim of this study was to review cases of CCP diagnosed at our institution and to investigate their outcome. Nineteen patients given a diagnosis of CCP more than 6 years previously were identified. Their medical records and histopathologic findings were reviewed. With a median follow-up duration of 11.5 years, regression of skin lesions was observed in 85.7% of patients. Persistence of skin lesions 11.5 years after diagnosis was confirmed histopathologically in one case, with a reduction in clear cell density. Retrospective nature of the study is a limitation. No treatment is necessary for CCP because the skin lesions are asymptomatic and most patients experience at least partial regression.

Evaluation of immunological profile and clinical characteristics in patients with vitiligo

Objective: Assess the presence of immune disorders in patients with vitiligo and the effects of low dose azathioprine and steroid as treatment. Methods: We enrolled 190 Taiwanese vitiligo patients at a rheumatology out-patient clinic, all of whom had previously failed to respond to topical corticosteroids and photochemotherapy. We recorded clinical features, disease course, and serological data commonly used in the diagnosis of autoimmune diseases. The response to low dose of azathioprine 25mg qd and prednisolone 5mg qd were assessed in each of four dimenions, including skin lesion progression, new skin lesions development, less distinct vitiligo margin and pigment regeneration by physician and patient visual analog scale. Results: 145 patients (76.32%) had simple vitiligo (no serological abnormality) and 45 patients (23.68%) had at least one serological abnormality. Of these 45 patients, 22 patients (11.58%) had systemic rheumatic disease (5 patients with systemic lupus erythematosus, 4 patients in each of psoriasis, spondyloarthropathy, and cryoglobulnemia, 2 patients with rheumatoid arthritis, and 1 patients in each of dermatomyositis, Behcet's disease, and hypersensitive angitis), 9 patients (4.74%) had thyroid disease, and 14 patients (7.37%) had serology abnormalities without diagnosis of specific disease. Most patients (174 patients, 91.58%) showed no more vitiligo progression and no development of new skin lesions within 2.3±1.1 months. Furthermore, 64 patients (33.68%) experienced a regression of skin lesions in 5.4±1.6 months, 52 patients (27.37%) experiencing a less distinct margin and 33 patients (17.37%) experiencing pigment regeneration. None of the patients reacted adversely to the treatment. Conclusion: Our study showed a possible immune dysregulation in patients with vitiligo as evidenced by presence of abnormal immune prfiles and association with other autoimmune diseases. Our data also disclosed that low dose systemic azathioprine and steroid were effective in treating this disorder.

Acanthosis nigricans as a paraneoplastic syndrome. Case reports and review of literature

Acanthosis nigricans (AN) is a skin disorder characterized by focal or diffuse hyperkeratosis symmetric hyperpigmentation of the skin and oral cavity mucosa. Various neoplasms, especially gastrointestinal adenocarcinomas are associated with acanthosis nigricans (AN malignant). Chemotherapy may cause regression of skin lesions. The etiology of AN is not clear. A role of growth factors such as melanocyte stimulating hormone alpha, transforming growth factor alpha, and insulin-like growth factor 1 has been discussed. Two cases of AN have been reported in this paper. Both have been associated with gastric adenocarcinoma. In the first case skin lesions were sensitive to chemotherapy (until cancer progression), while in the second case treatment had to be discontinued because of cardiotoxity without regression of skin lesions.