Case for diagnosis. A pruritic eruption of keratotic papules over the face and neck

Abstract Generalized eruptive keratoacanthoma of Grzybowski is a rare variant of multiple keratoacanthomas counting with about 40 cases reported. It is a chronic and progressive disease for which none of the described therapeutic options has been entirely satisfactory. We report a case of an 83-year-old female who presented with a 3-month history of extremely pruritic, multiple, skin-coloured to erythematous to brownish, millimetric papules, with a keratotic centre. Histological examination of an incisional biopsy was consistent with the diagnosis of keratoacanthoma. The patient started acitretin 25 milligrams daily with a complete resolution of pruritus and regression of numerous lesions.

Harlequin Ichthyosis in a Filipino newborn: Management pearls in a resource-limited setting

Introduction@#Harlequin ichthyosis (HI) is a rare type of autosomal recessive congenital ichthyosis. There are approximately 200 documented cases worldwide, with less than five published reports in the Philippines. Despite its rarity, current literature suggests a better prognosis for these patients. @*Case description@#We describe a preterm male newborn who presented at birth enclosed in a thick hyperkeratotic armor-like scale plates with areas of fissures, with associated ectropion, conjunctiva dehiscence, and eclabium. The thickened encasement also covered the hands and feet, causing severe contractures. A diagnosis of harlequin ichthyosis was given based on the clinical features. The patient was managed through a multidisciplinary approach, including referral to the tele-ichthyosis platform of a US-based foundation for patients with ichthyosis. Thermoregulation, nutrition, and hydration were carefully managed. Bland emollients were applied generously following normal saline soaks to improve barrier protection. Acitretin was administered on day 2 of life to facilitate the desquamation of the thickened encasement. A marked decrease in erythema and the thickness of the hyperkeratotic skin, and reduced conjunctival dehiscence were noted after one week of therapy. However, the constrictions on the hands and feet showed bluish discoloration and signs of necrosis. Linear band excision was performed to release the constrictors. Despite aggressive management, the patient succumbed to sepsis on day 12 of life. @*Conclusion@#Improved prognosis amongst HI patients is correlated with optimal quality of care regardless of resource limitations. A multidisciplinary approach and early administration of retinoids cannot be overemphasized. Linear band excision within the first week of life is suggested for constrictions on the extremities that do not improve with retinoids to avoid necrosis and autoamputation.

Moderate to severe plaque psoriasis - treatment with drugs of the classic scheme

The Guidelines Project, an initiative of the Brazilian Medical Association, aims to combine information from the medical field in order to standardize producers to assist the reasoning and decision-making of doctors. The information provided through this project must be assessed and criticized by the physician responsible for the conduct that will be adopted, depending on the conditions and the clinical status of each patient.

The Effects of Systemic Psoriasis Therapies on the C-Reactive Protein and the Neutrophil-Lymphocyte Ratio

BACKGROUND: Currently, no generally accepted laboratory marker for monitorizing the disease activity and therapy response of psoriasis is known. OBJECTIVE: The aim of the study is to evaluate the effects of systemic therapies on C-reactive protein (CRP) and the neutrophil-lymphocyte ratio (NLR) in psoriasis. METHODS: One hundred patients with psoriasis treated with narrow band ultraviolet B, acitretin, cyclosporine, methotrexate, adalimumab, etanercept, and ustekinumab were prospectively evaluated. At baseline and at week 12, CRP, NLR, and Psoriasis Area and Severity Index (PASI) were evaluated. RESULTS: A statistically significant decrease was observed in PASI scores, CRP, and NLR values from the baseline to the 12-week visit (p=0.001, p=0.001, p=0.001, respectively). The reduction in PASI scores and NLR values was positively correlated (r=0.460, p=0.001). The comparisons between treatment groups revealed that the median decrease in NLR values was statistically higher in the adalimumab group than in the methotrexate group (p=0.007). And the median decrease in PASI scores was significantly higher in the adalimumab group compared with the methotrexate and acitretin therapy group (p=0.007, p=0.042, respectively). CONCLUSION: In the present study, systemic therapy of psoriasis was demonstrated to decrease the levels of CRP and NLR, which are known to be indicators of systemic inflammation and cardiovascular comorbidities.

A Case of Elephantiasis Nostras Verrucosa Treated with Oral Acitretin / 대한피부과학회지

Elephantiasis nostras verrucosa (ENV) is an uncommon condition caused by repeated inflammation and lymphatic obstruction. It occurs mainly in the lower extremities and is characterized by skin changes, including hyperkeratotic mossy papules and plaques, non-pitting edema, and cobblestone-like appearances. ENV can be diagnosed based on its typical clinical manifestations (pseudoepitheliomatous hyperplasia with enlarged lymphatic spaces, fibrous tissue proliferation, and chronic inflammation) and by skin biopsy. Although ENV is difficult to treat, reduction of lymphedema and use of keratolytic agents may be helpful. To our knowledge, only three cases of ENV have been reported. However, the case of ENV treated with oral acitretin has not been reported in domestic literatures. Therefore, we report the case of a 45-year-old woman who developed ENV. She had a past history of cellulitis and was successfully treated with oral acitretin.

Three Fibrotic Conditions Treated with 5% Topical Imiquimod Cream as an Antifibrotic Agent / 대한피부과학회지

Imiquimod, a toll-like receptor agonist, is a topical immunomodulator that induces the production of several cytokines including interferon-alpha, which shows antifibrotic properties. We hypothesized that the antifibrotic effect of imiquimod would soften fibrotic skin lesions. Therefore, we applied topical imiquimod with topical tacrolimus or systemic acitretin in patients with refractory lesions of myxedema, angiolymphoid hyperplasia, and generalized discoid lupus erythematosus and confirmed improvement in fibrotic lesions in these patients. Thus, we conclude that use of imiquimod improves fibrotic skin lesions. This report describes our experience with the treatment of this condition along with a review of the related literature.

A Case of SAPHO Syndrome Associated with Lytic Bone Lesions Resembling Metastases / 대한피부과학회지

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.

Case for diagnosis. Linear Darier's disease

Abstract: We present a different and rare manifestation of Darier's disease, namely linear Darier's disease. Only a few cases have been described in the literature. The case report is a male patient, 60 years old, presenting brown to red papules and plaques with hyperkeratosis distributed on the abdomen, following Blaschko's lines, with 6 years' evolution. It was a difficult diagnosis until the dermatological workup and biopsy.

Erythrodermic psoriasis and human immunodeficiency virus: association and therapeutic challenges

Abstract: Erythrodermic psoriasis is a rare but severe type of psoriasis that may be triggered by human immunodeficiency virus infection. We describe the case of a 65-year-old male patient with chronic psoriasis who presents an exacerbation of his condition over a period of two weeks. Because of the severity of his case and subsequent need for systemic therapy, human immunodeficiency virus enzyme immunoassay was performed and tested positive. He thus began antiretroviral therapy combined with acitretin, showing good clinical response after 8 weeks of treatment. There is little evidence regarding the management of erythrodermic psoriasis associated with HIV infection, so antiretroviral therapy and systemic retinoid remain as the first-line treatment.

Harlequin Ichthyosis in a 4-year-old male: Case report

@#Harlequin ichthyosis (HI) is a rare type of congenital keratinization disorder that, when left untreated, usually leads to early neonatal demise. A clinical diagnosis of HI is considered when a patient presents with thick plate-like scaling of the skin together with eclabium, ectropion, and nasal hypoplasia. The diagnosis can be confirmed by genetic testing to determine mutation in the adenosine triphosphate-binding cassette A12 (ABCA12) gene. Early administration of systemic retinoids to promote desquamation and emollients to control excessive scaling and dryness of the skin lead to better prognosis in most cases of HI. We present the case of a 4-year-old male with HI who has been successfully managed with bland emollients and systemic acitretin therapy, which we started when he was 1 year old.

Systematized verrucous epidermal nevus partially responsive to acitretin: A case report

@#<p style="text-align: justify;">A 5-year-old female presented a 4-year history of generalized well-demarcated asymptomatic brown to dark brown thin verrucous plaques with a Blaschkoid distribution. Histopathology was consistent with an epidermal nevus. Patient was diagnosed to have systematized verrucous epidermal nevus. Due to the extent of the lesions, surgical management was not feasible. Hence acitretin was given which showed partial decrease in the thickness of the lesions.</p>

Low-dose Acitretin and Cyclosporine Combination Therapy in Patients with Psoriasis: A Retrospective Analysis of 37 Cases / 대한피부과학회지

No abstract available.

A case of disseminated granuloma annulare successfully treated with acitretin and narrowband UV-B phototherapy

Introduction@#Granuloma annulare(GA) is a benign, inflammatory skin condition characterized by asymptomatic, flesh colored or erythematous papules. The disseminated variant of granuloma annulare is uncommon, usually affecting women older than 40 years.@*Case Summary@#We report a case of a 52-year-old female, who presented with a 4-year history of skin colored to erythematous papules and annular plaques covering the entire skin surface. Correlation of the clinical presentation, histopathology and dermoscopy findings established the diagnosis of disseminated granuloma annulare. The patient was treated with 25 sessions of narrowband ultraviolet-B (NB-UVB) phototherapy and acitretin 20mg/day for 5 months.@*Conclusion@#Both histopathology and dermoscopy were noted to be valuable tools in the diagnosis of disseminated GA, as well as in monitoring response to the combined therapy.

Hipercromia pós-inflamatória associada à psoríase. Relato de caso / Post-inflammatory hyperchromia associated with psoriasis. Case report

A Hipercromia pós-inflamatória (HPI), desordem de pigmentação da pele decorrente da produção exagerada de melanina, apresenta-se sob a forma de manchas hipercrômicas e representa uma sequela importante da psoríase, uma dermatose inflamatória. Objetiva-se, neste artigo, um relato de caso, descrever a HPI surgida após um quadro de psoríase, em paciente do gênero masculino, 54 anos de idade, cor parda, marceneiro de profissão, sem histórico de tabagismo, obesidade ou doença de base, atendido em uma Unidade Estratégia Saúde da Família (ESF), no município de Vassouras, região Centro Sul do Estado do Rio de Janeiro. A psoríase é uma doença imunológica crônica, resultante da estimulação persistente de células T por imunógenos de origem epidérmica, envolvendo a imunidade inata e a adquirida. A conduta terapêutica da HPI incluiu a prescrição de corticosteróides, despigmentantes e fotoprotetores. O acompanhamento do paciente por psicoterapia do Núcleo de Apoio à Saúde da Família (NASF), da ESF e pelo dermatologista da média complexidade da Rede de Atenção à Saúde (RAS) representou um diferencial e revelou-se imprescindível para a integralidade do cuidado em saúde.(AU)

Post-inflammatory hyperchromia (IPH), a pigmentation disorder of the skin resulting from the exaggerated production of melanin, is manifested by hyperchromic patches and is an important consequence of psoriasis, an inflammatory dermatosis. The objective of this article is to describe a case of IPH that appeared after psoriasis in a patient attended at a Family Health Strategy Unit (FHU), in the city of Vassouras, in the central region of the State of Rio de Janeiro. Psoriasis is a chronic immune disease that results from the persistent stimulation of T cells by immunogens of epidermal origin, involving innate and acquired immunity. The therapeutic management of IPH consists in prescribing of corticosteroids, despigmentants and photoprotectors. The follow-up of the patient through Psychology of the Family Health Support Center, the FHU and the dermatologist of the medium complexity of the Health Care Network represented a differential and proved to be essential for the integrality of health care.(AU)

Clinical features of von Zumbusch type of generalized pustular psoriasis in children: a retrospective study of 26 patients in southwestern China

Abstract Background: Von Zumbusch type of generalized pustular psoriasis is a rare variant of psoriasis in children. It can occur in patients with or without psoriasis vulgaris. Objective: The aim of the study was to discuss the precipitating factors, clinical manifestations, laboratory data and therapy of von Zumbusch type of generalized pustular psoriasis in children from southwestern China and to improve the diagnosis and treatment level. Methods: A retrospective analysis was conducted for inpatients aged 14 years old or less with von Zumbusch type of generalized pustular psoriasis in our department from 2005 to 2014. Results: A total of 26 patients were included, of whom four (15.38%) had previous history of psoriasis vulgaris and one (3.85%) had previous history of psoriasis arthropathica. Mean onset age was 6.90 years. Gender distribution was equivalent. Incidence of the disease in summer and autumn was higher than that in winter and spring. Nineteen (73.08%) cases were triggered by infection, two (7.69%) cases were caused by sudden discontinuation of systemic use of corticosteroid. Twenty-four (92.31%) cases had concomitant fever. The initial lesion manifested as non-follicular sterile pustules on erythema. Sixteen patients responded well to acitretin, 11 to Tripterygium wilfordii Hook F (TwHF), two to cyclosporine, and one to methotrexate. Study limitations: This study is a retrospective one and the number of cases is small. CONCLUSION: Von Zumbusch type of generalized pustular psoriasis is a rare disease in children, infection is the most common precipitating factor, acitretin is the first-line therapy, traditional Chinese medicine TwHF also can be used.

Low-dose systemic retinoids in preventing subsequent non-melanoma skin cancers (NMSC) in patients with history of NMSC: A systematic review

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Non-melanoma skin cancers (NMSC) consists of  basal-cell carcinomas (BCC) and squamous-cell carcinomas (SCC).Certain populations are predisposed to develop  NMSC,  including  patients  with  previous  history  of  NMSC.Systemic  retinoids  have  shown  promising  results  in chemoprevention of recurrence of NMSC in other high-risk populations (xeroderma pigmentosum and renal-transplant patients).We  assessed  the  efficacy  and  safety  of  low-dose  systemic  retinoids  compared  with  placebo,  as  a  chemopreventive agent for NMSC in patients with previous NMSC.<br /><strong>METHODOLOGY:</strong> Electronic  databases  were  systematically searched for this study. Participants in the studies selected must have had a biopsy-proven NMSC, over 18 years of age, with  no  exclusion  of  other  demographic  characteristics. All  types  of  systemic  retinoids  were  included  with  no restriction on dosage. Two authors independently performed standardized  eligibility  assessment  and  data-extraction.Differences in opinion were resolved by consensus with the third author. Statistical analysis was done using the Review Manager 5 software.<br /><strong>RESULTS:</strong> Eleven full-text studies were assessed for eligibility out of 178 studies found. Five studies were excluded because of the different population, while the two articles used topical retinoids. Four articles were included. The interventions were 10.0 mg isotretinoin, 25,000IU retinol and 25.0 mg acitretin,compared with placebo. Meta-analysis produced RR of 0.94 (95% CI, 0.89-1.00), with moderate heterogeneity (34%) due to the difference in interventions used. There are significantly more  adverse  events  in  the  retinoids  group,  especially  in the  incidence  of  mucocutaneous  adverse  events,  and deranged lipid profile and liver enzymes.<br /><strong>CONCLUSION:</strong> There is insufficient evidence to support the use of low-dose systemic retinoids as chemoprevention for patients with previous NMSC. Furthermore, adverse events may limit their use. Topical preparations with less side-effects may be investigated.</p>

Low-dose systemic retinoids in preventing subsequent non-melanoma skin cancers (NMSC) in patients with history of NMSC: A systematic review

BACKGROUND: Non-melanoma skin cancers (NMSC) consists of  basal-cell carcinomas (BCC) and squamous-cell carcinomas (SCC).Certain populations are predisposed to develop  NMSC,  including  patients  with  previous  history  of  NMSC.Systemic  retinoids  have  shown  promising  results  in chemoprevention of recurrence of NMSC in other high-risk populations (xeroderma pigmentosum and renal-transplant patients).We  assessed  the  efficacy  and  safety  of  low-dose  systemic  retinoids  compared  with  placebo,  as  a  chemopreventive agent for NMSC in patients with previous NMSC.METHODOLOGY: Electronic  databases  were  systematically searched for this study. Participants in the studies selected must have had a biopsy-proven NMSC, over 18 years of age, with  no  exclusion  of  other  demographic  characteristics. All  types  of  systemic  retinoids  were  included  with  no restriction on dosage. Two authors independently performed standardized  eligibility  assessment  and  data-extraction.Differences in opinion were resolved by consensus with the third author. Statistical analysis was done using the Review Manager 5 software.RESULTS: Eleven full-text studies were assessed for eligibility out of 178 studies found. Five studies were excluded because of the different population, while the two articles used topical retinoids. Four articles were included. The interventions were 10.0 mg isotretinoin, 25,000IU retinol and 25.0 mg acitretin,compared with placebo. Meta-analysis produced RR of 0.94 (95% CI, 0.89-1.00), with moderate heterogeneity (34%) due to the difference in interventions used. There are significantly more  adverse  events  in  the  retinoids  group,  especially  in the  incidence  of  mucocutaneous  adverse  events,  and deranged lipid profile and liver enzymes.CONCLUSION: There is insufficient evidence to support the use of low-dose systemic retinoids as chemoprevention for patients with previous NMSC. Furthermore, adverse events may limit their use. Topical preparations with less side-effects may be investigated.

Multiple Giant Keratoacanthoma Treated with Acitretin / 대한피부과학회지

Giant keratoacanthoma is an uncommon variant of keratoacanthoma, which may increase to a diameter of several centimeters. Although keratoacanthomas usually resolve spontaneously, giant keratoacanthoma can be invasive and destructive. A 49-year-old man presented with a 5-year history of multiple large hyperkeratotic and crusted plaques and nodules on sun-exposed areas such as the face, ear, hand, and forearm. Some lesions involuted spontaneously, whereas others became rather enlarged. The biopsy specimen revealed horn-filled crater formation, epidermal extending resembling a buttress, and an eosinophilic glassy appearance in the keratinocytic cytoplasm. We treated the lesions with acitretin, and they almost completely resolved after 13 weeks. Here we describe a case of multiple giant keratoacanthoma treated with acitretin.

Low-dose systemic retinoids in preventing subsequent non-melanoma skin cancers (NMSC) in patients with history of NMSC: A systematic review

BACKGROUND: Non-melanoma skin cancers (NMSC) consists of  basal-cell carcinomas (BCC) and squamous-cell carcinomas (SCC).Certain populations are predisposed to develop  NMSC,  including  patients  with  previous  history  of  NMSC.Systemic  retinoids  have  shown  promising  results  in chemoprevention of recurrence of NMSC in other high-risk populations (xeroderma pigmentosum and renal-transplant patients).We  assessed  the  efficacy  and  safety  of  low-dose  systemic  retinoids  compared  with  placebo,  as  a  chemopreventive agent for NMSC in patients with previous NMSC.METHODOLOGY: Electronic  databases  were  systematically searched for this study. Participants in the studies selected must have had a biopsy-proven NMSC, over 18 years of age, with  no  exclusion  of  other  demographic  characteristics. All  types  of  systemic  retinoids  were  included  with  no restriction on dosage. Two authors independently performed standardized  eligibility  assessment  and  data-extraction.Differences in opinion were resolved by consensus with the third author. Statistical analysis was done using the Review Manager 5 software.RESULTS: Eleven full-text studies were assessed for eligibility out of 178 studies found. Five studies were excluded because of the different population, while the two articles used topical retinoids. Four articles were included. The interventions were 10.0 mg isotretinoin, 25,000IU retinol and 25.0 mg acitretin,compared with placebo. Meta-analysis produced RR of 0.94 (95% CI, 0.89-1.00), with moderate heterogeneity (34%) due to the difference in interventions used. There are significantly more  adverse  events  in  the  retinoids  group,  especially  in the  incidence  of  mucocutaneous  adverse  events,  and deranged lipid profile and liver enzymes.CONCLUSION: There is insufficient evidence to support the use of low-dose systemic retinoids as chemoprevention for patients with previous NMSC. Furthermore, adverse events may limit their use. Topical preparations with less side-effects may be investigated.

Acitretin as a Therapeutic Option for Chronic Hand Eczema

No abstract available.