Confluent And Reticulated Papillomatosis Research Articles

Clinico-pathological Spectrum of Rare Skin Syndromes and Diseases: A Series of Five Cases

A significant threat to patients’ well-being, mental health, capacity to function, and social participation- a measure of disability- is posed by skin diseases, resulting in significant mortality and morbidity worldwide. Despite the fact that the majority of rare diseases are complex, disabling, and life-threatening, little knowledge has been gained in this area. The diagnosis and classification of these rare skin syndromes with pre-determined sets of symptoms present a challenge. To diagnose a rare skin syndrome, one frequently has to correlate histologic findings with clinical symptoms, as there is a vast range of skin disorders, many of whose histologic traits overlap with just slight variances. However, histologic knowledge alone makes it difficult to diagnose these; proper diagnosis demands appropriate clinical knowledge. In the present study institute, 675 skin biopsies were performed over the course of five years, from 2018 to 2022. Based on clinico-pathological analysis, various rare skin syndromes were diagnosed, as depicted in present case series of five cases (10 years old boy, 40 years old male, 27 years old male, 44 years old male, 52 years old male patients) for: a) Griscelli Syndrome; b) Gougerot-Carteaud Syndrome, considered rare as it is one of the underdiagnosed and misdiagnosed syndromes due to its similarity with Pityriasis versicolour; c) Kyrle’s disease, a rare perforating dermatosis occurring in 10% of chronic renal failure patients who are on dialysis; d) Nekam’s disease; e) Sweet syndrome, an uncommon syndrome occurring in about 10-20% of malignancies.

Confluent and reticulated papillomatosis (CARP) of gougeret and carteaud

Confluent and reticulated papillomatosis (CARP) of gougeret and carteaud

Confluent and Reticulated Papillomatosis in Monozygotic Twins: A Case Series

Introduction: Confluent and reticulated papillomatosis (CARP) is a non-inflammatory hyperkeratotic dermatosis that commonly affects teens and young adults. While the exact pathophysiology remains unknown, multiple theories exist including familial inheritance. The present literature on genetic predisposition remains limited to intergenerational relatives and siblings. Case: We describe a case of recurrent CARP with identical presentation and clinical course in two monozygotic 16-year-old female twins. Discussion: Though familial cases of CARP have been reported, the full pathogenesis of this dermatosis remains unknown. Metabolic conditions related to insulin resistance, thyroid and pituitary derangements, and pregnancy, have also been reported in patients with CARP. Thus, there may be a gene that links CARP to the development of other endocrinologic disorders. Furthermore, the simultaneous recurrence of CARP in our monozygotic twins supports an environmental trigger theory. Thus, we hypothesize that the pathophysiology of CARP is multifactorial. Conclusion: This case confirms a strong genetic predisposition, suggesting a greater inheritance component than previously considered.

Confluent and reticulated papillomatosis with eruptions on the trunk and extremities.

Dear Editor, Confluent and reticulated papillomatosis (CRP) is a rare skin disorder in young individuals. It is clinically characterized by slightly hyperkeratotic or verrucous grayish-brown papules coalescing to form a reticulated pattern peripherally with confluent plaques centrally. Sites of predilection are the neck, interscapular, intermammary regions, and abdomen. CRP commonly arises during puberty or in early adulthood. Herein, we describe an atypical case of CRP in which the skin lesions developed not only on the abdomen, as a site of predilection, but also on broad areas of the extremities.

Focal hypohidrosis in lesional skin in a probable case of confluent and reticulated papillomatosis: A case report with insight into the pathomechanism of recurrence.

Confluent and reticulated papillomatosis (CRP) is a rare skin disorder that develops in young adults and presents as persistent brown papules and plaques predominantly affecting the intertriginous areas, however, its etiopathogenesis remains elusive. Herein, we report a probable case of CRP with lesional hypohidrosis as detected by sweat test and provide insight into the pathomechanism. A 23-year-old man presented with nine-months history of painful sensation on his trunk without any skin change. The result of sweat test was compatible with acquired idiopathic generalized anhidrosis. Topical heparinoid and physical exercise improved the symptoms. However, he started to notice asymptomatic brownish reticulated macules on the trunk. Intriguingly, focal hypohidrosis, as detected by sweat test was evident on the macules. In histology, the lesional skin demonstrated hyperkeratosis, acanthosis, basal melanosis, mild papillomatosis, and obstruction of the sweat duct in the upper dermis, which were not observed in the peri-lesional skin. Accumulation of the sweat in the luminal aspect of the secretory portion and dilation of the sweat duct in the deeper dermis was detected in the lesional skin, as highlighted by anti-dermcidin staining. Aquaporin 5 expression in the secretory portion was more confined to the cell membrane in the lesional skin. Both brownish macules and lesional hypohidrosis simultaneously improved in summer and exacerbated in winter. Literature review found nine reports on recurrent CRP, and obesity was thought to be a major comorbidity in recurrent CRP cases. Obesity is often associated with sweat dysregulation. This, together with the findings in our case, implied the possible contribution of focal sweating abnormality in the pathogenesis of reticulated skin lesion in our case.

Confluent and reticulated papillomatosis in pediatric patients at an urban tertiary care center

Confluent and reticulated papillomatosis (CARP) is a dermatosis that often presents during adolescence. Prior studies have linked CARP to metabolic syndrome and comorbidities associated with insulin resistance, such as acanthosis nigricans and type 2 diabetes. Despite this, few studies have evaluated the clinical relationship between glucose dysmetabolism and CARP. In this report, we describe the characteristics of a large cohort of pediatric patients with CARP to further evaluate the potential relationship between CARP and metabolic syndrome in children.

Prurigo Pigmentosa and Confluent and Reticulated Papillomatosis a Spectrum of One Disease: A New Case Report

Background: Prurigo Pigmentosa (PP) is a rare inflammatory dermatitis first discovered in 1971. Characterized by a sudden eruption of pruritic reticulated, pink-brown papules coalescing into plaques distributed symmetrically over shoulders, neck, chest, and back. Various triggers have been identified, including the ketogenic diet. Clinicopathological presentation looks similar to confluent and reticulated papillomatosis (CARP) which is a rare dermatosis of unknown etiology characterized by hyperkeratotic pigmented papules & peripheral reticulation involving seborrheic areas. Aim: To document a new case presentation of PP caused by a low-carbohydrate restricted diet and discuss the comparison with CARP clinically, pathologically, and treatment modalities. Case report: A 15-year-old childhood male developed PP 3 weeks after self-initiating a low carbohydrate-restricted ketogenic diet for weight management. Clinically and histopathologically the lesion looks similar to CARP, treated successfully with re-introduction of high carbohydrates in his food, a short course of systemic steroids in combination with oral doxycycline capsules for the one-month duration. Conclusion: PP & CARP have been considered a spectrum of one disease, and PP is a pruritic variant from CARP caused by a low carbohydrate-restricted diet.

Confluent and Reticulate Papillomatosis: A Retrospective Study from southern India.

Background:Confluent and reticulate papillomatosis (CRP) is an uncommon benign, acquired keratinization disorder. Studies on this disorder are lacking except for a few case reports and there is a paucity of Indian literature on the condition.Objectives/Methods:To study and describe the various morphological patterns and histopathological findings, as well as assess the response to treatment of 30 patients diagnosed with CRP.Results:Thirtypatients with a diagnosis of confluent and reticulate papillomatosis were included in the study. The male to female ratio was 1:1.5. Mean age at onset of skin eruptions was 27.3 years and mean duration of skin eruptions was 8.2 months. Most of the patients (60%) were asymptomatic. The majority (66.7%) had lesions distributed over upper trunk. Two-thirds of patients had typical brown macules in confluent and reticulate pattern. KOH mount was done in 24 cases and was positive in three cases (12.5%) for yeast-like hyphae. Biopsy demonstrated variable degrees of hyperkeratosis, papillomatosis, and moderate acanthosis. Thirteen out of eighteenpatients on minocycline showed complete clearance within 3 weeks andthreepatients had more than 50% improvement at the end of 3 weeks. Doxycycline showed satisfactory response but results were less satisfactory with azithromycin.Conclusion:CRP is an uncommon condition. There is a paucity of large studies in Indian literature. The present study highlightssuch a large cohort of cases. Prevalence of CRP was more in female in contrast to western studies. Association of CRP with hyperthyroidism was described in many studies but the present study highlights the association with hypothyroidism. Morphological variants like shiny atrophic lesions, verrucous lesions, and involvement of atypical sites like forearm have been described. Role of minocycline in the management of chronic and recurrent cases has been reinforced.

Papilomatose Reticulada e Confluente de Gougerot- -Carteaud: Melhoria Significativa e Sustentada das Lesões com Minociclina Oral

Apresentamos um exuberante caso de papilomatose reticulada e confluente de Gougerot-Carteaud em adolescente masculino previamente saudável, tratado com minociclina via oral 100 mg durante 30 dias, com clareamento expressivo das lesões e resposta sustentada após 6 meses de seguimento. A literatura recente relacionada com o tema é discutida.

Antiretroviral-responsive confluent and reticulated papillomatosis: a case report of an unusual association.

This is the case of a 29-year-old male newly diagnosed with advanced HIV (CD4 < 35cells/mm3), presenting to us with hyperpigmented and scaly non-pruritic macules over his chest and upper abdomen of several weeks duration. Woodlamp examination was negative, but a skin biopsy suggested confluent and reticulated papillomatosis (CRP). Given his lack of any of the condition’s identifiable triggers and the unusually rapid resolution of his lesions shortly after antiretroviral therapy initiation, an immunodeficiency-related etiology for his CRP was entertained. Autoimmune disorders and atopic conditions have been well reported previously as possible triggers of CRP. However, in this report, we raise immunodeficiency as a possible trigger of CRP as well, such that immune dysregulation overall (autoimmunity or immunodeficiency) can contribute to CRP ontogenesis. To our best knowledge, this is the first report to date suggesting a possible association between CRP, a rare dermatological condition, and acquired immunodeficiency syndrome.

A case of confluent and reticulated papillomatosis of gougeret and carteaud

&lt;p class="abstract"&gt;Confluent and reticulated papillomatosis (CRP) of Gougerot and Carteaud, a rare skin disorder of unknown etiology, was first described in 1927. It is an uncommon but distinctive icthyosiform dermatosis seen in young adults. It is characterized by persistent brown, scaly macules, papules, patches and plaques, localized predominantly on the neck, intermammary, interscapular regions and axillae. There are numerous therapeutic options - topical retinoids and systemic retinoids, oral antibiotics but none are very effective. We report this case because of its rarity.&lt;/p&gt;

Confluent and reticulated papillomatosis limited to both forearms

Confluent and reticulated papillomatosis limited to both forearms

Clinical and Histopathologic Study of Confluent and Reticulated Papillomatosis by Anatomic Site and Age.

BackgroundConfluent and reticulated papillomatosis (CRP) is an uncommon dermatosis characterized by hyperpigmented scaly macules or papillomatous papules coalescing into confluent patches or plaques centrally with a reticular pattern peripherally. Few studies regarding this have been reported in the literature.ObjectiveThe purpose of this study was to investigate the clinical and histologic characteristics of CRP in Korean patients according to anatomic site and age.MethodsWe retrospectively reviewed the medical records, clinical photographs, and 40 histopathologic slides of 29 patients diagnosed with CRP.ResultsSixteen adults and 13 adolescents were included. The predominantly involved anatomic sites were the trunk (89.7%) and axillae (27.6%); flexural area involvement was mainly found in the adolescent group. The mean disease duration was 2.3 times longer in the adult group than in the adolescent group. The histopathologic features included hyperkeratosis (95.0%), acanthosis (87.5%), and papillomatosis (77.5%) in the epidermis. In the adult group, only 10 specimens (52.6%) showed mild to moderate papillomatosis. There was no relationship between the histologic findings and disease duration; however, the former was associated with the anatomic site.ConclusionThe present study is the first to present the clinical and histologic features of CRP according to age and anatomic site in Korean patients. The patients in this study more frequently had fungal infections (31%) than patients in a previous study (12.8%). Histologic changes were more prominent in the adolescent group than in the adult group and in the flexural area than in the non-flexural area.

Gougerot-Carteaud syndrome treated with acitretin: A case report

Gougerot-Carteaud syndrome treated with acitretin: A case report

A case of confluent and reticulate papillomatosis complicated by pseudo-acanthosis nigricans

A case of confluent and reticulate papillomatosis complicated by pseudo-acanthosis nigricans

Confluent and reticulated papillomatosis: diagnostic and treatment challenges.

Confluent and reticulated papillomatosis (CRP) of Gougerot and Carteaud was first typified in 1927. With the help of electron microscopy, it has been elucidated that CRP arises due to aberrant keratinization. However, till date, there is no clear consensus on the etiologic trigger for CRP. Prevailing postulates include a bacterial trigger by Dietzia papillomatosis (type strain N 1280T), an exaggerated cutaneous response to Malassezia furfur, an endocrine basis stemming from insulin resistance, ultraviolet light-induced epidermal change, amyloid deposition, and a loss-of-function mutation in keratin 16. CRP typically presents as asymptomatic hyperpigmented papules and plaques with peripheral reticulation over the nape, axillae, upper chest, and upper back, occasionally with extension superior to the forehead and inferior to the pubic region. Dermoscopy may be used in the evaluation of CRP, but its diagnosis is made on clinical grounds given its nonspecific histopathological findings. Although successful treatment with topical keratolytics, retinoids, or antifungals has been reported, antibiotics, such as minocycline, at anti-inflammatory doses have emerged as a preferred therapeutic option. In this article, we review the diagnostic considerations in CRP and its therapeutic options.

Differentiating confluent and reticulated papillomatosis from acanthosis nigricans.

Confluent and reticulated papillomatosis (CRP) is an uncommon dermatosis with a reticular pattern. As differentiation between CRP and benign acanthosis nigricans (AN) can be challenging because of their similar clinicopathological features, we aimed to distinguish the two diseases. We retrospectively reviewed the clinical characteristics of 60 CRP and 30 AN patients. Histological examinations were conducted on 33 CRP and 30 AN lesions. While CRP was concentrated on the trunk, AN mostly appeared in the axilla. In the AN group, the number of obese patients was higher, and acanthosis and papillomatosis were more evident. In both group, increases in Ki-67 and keratin 16 expression were similar. Bacterial infection was detected at a higher rate in CRP lesions than in AN lesions.AN lesions had greater pigmentation because of a high number of melanocytes than CRP lesions. The location of skin lesions and body mass index are the main clinical factors that enable differentiation between CRP and AN. The epidermal histological changes in CRP are milder than those in AN. AN lesions also showed a greater degree of pigmentation and melanocytic proliferation.

A Teen Lesion of Confluent and Reticulated Papillomatosis ( CRP )

Confluent and reticulated papillomatosis (CRP) is also known as Gougerot-Carteaud syndrome; it is a rare skin disease typically affecting teenagers. CRP is characterized by small grayish blue hyperkeratotic papillomatosis located on the trunk 1 . Lesions typically begin as hyperkeratotic small papules (1-2 mm) which enlarge and coalesce (4-5 mm) to form grayish brown papules and plaques in a confluent pattern centrally (on the midline region) and with a reticular pattern peripherally. Commonly, it begins on the skin of the intermammary or epigastric region, spreading over a period of weeks or months to axillae, groin, face, neck, arms, trunk, cheeks, pubic area, interscapular region, shoulders, the proximal extremities, and forehead 1-4 . The mucus membranes are spared. Skin markings are sometimes exaggerated, especially in the neck and the axillae (dirty neck and axillae), where the skin may be thickened. Scraping of the lesions produces a fine powdery scale 1 .

Confluent Reticulated Papillomatosis Accompanied by Obesity: Three Cases

Ankara Ataturk Egitim ve Arastirma Hastanesi, Deri ve Zuhrevi Hastaliklari Klinigi, Ankara, Turkiye *Ankara Ataturk Egitim ve Arastirma Hastanesi, Patoloji Klinigi, Ankara, Turkiye @Telif Hakki 2014 Turk Dermatoloji Dernegi Makale metnine www. turkdermatolojidergisi.com web sayfasindan ulasilabilir. @Copyright 2014 by Turkish Society of Dermatology Available on-line at www.turkdermatolojidergisi.com Duriye Deniz Demirseren, Ankara Ataturk Egitim ve Arastirma Hastanesi, Deri ve Zuhrevi Hastaliklari Klinigi, Ankara, Turkiye E-posta: ddemirseren@yahoo.com Gelis Tarihi/Submitted: 05.05.2013 Kabul Tarihi/Accepted: 10.05.2013 Yazisma Adresi/ Correspondence: Confluent and reticulated papillomatosis (CRP) is a rare skin disorder characterized by reticulated, erythematous-brownish macules, patches and plaques. The CRP lesions mostly emerge initially on intermammarian region and then extend to the abdominal area. Although many theories have been proposed, etiopathogenesis of CRP is not clear. In this report, we describe 3 female patients with similar clinical features. All patients were at the ages of twenties, had obese, ,and were successfully treated with doxycycline treatment.

Updated diagnosis criteria for confluent and reticulated papillomatosis: a case report.

Dear Editor: Confluent and reticulated papillomatosis (CRP), a rare skin disorder of unknown etiology, is characterized by hyperkeratotic papules confluent in the central area and reticulated hyperpigmented patch in the peripheral area1. Since Gougerot and Carteud first identified CRP in 1927, this disease has been diagnosed by using clinical courses, histological findings, and treatment results. However, other diseases, such as fungal infection or other pigmentary skin diseases, were frequently misdiagnosed as CRP because of the lack of diagnostic criteria for CRP. Therefore, Davis et al.1 proposed the diagnostic criteria for CRP on the basis of a study on 39 patients, as follows: (i) clinical findings of scaly brown macules and patches, with at least some appearing reticulated and papillomatous; (ii) involvement of the upper trunk and neck; (iii) negative fungal staining of scales; (iv) no response to antifungal treatment; and (v) excellent response to minocycline. However, a few cases of CRP sparing the upper trunk have also shown the typical histopathological findings, clinical findings, and treatment responses of CRP. A 42-year-old Korean woman presented to our clinic with a 4-year history of asymptomatic, brownish, and reticulated skin lesions in her antecubital and axillary regions (Fig. 1A). She reported that her skin lesions had worsened in May and improved slightly in October without any specific treatment. We performed a potassium hydroxide test to rule out fungal infection. Because the test result was negative at the antecubital fossa but positive at the axilla, we began treatment with oral itraconazole and topical flutrimazole cream for 2 weeks. Despite the 2 weeks of treatment, her skin lesions persisted. We then performed an axillary skin biopsy. Histopathological evaluation revealed papillomatosis and hyperkeratosis of the epidermis, and downward growth of the epidermis between the rete ridges (Fig. 2). Periodic acid Schiff staining did not reveal any fungal organism. On the basis of these findings, we diagnosed her condition as CRP limited to the flexural areas. After 2 months of doxycycline treatment (100 mg twice a day), her skin lesions completely disappeared (Fig. 1B). Fig. 1 (A) Hyperpigmented scaly macules with a reticular pattern on the axilla and antecubital fossa. (B) Complete resolution of the skin lesions after 2 months of doxycycline treatment. Fig. 2 Histopathological findings of the axillary lesion showing an orthokeratotic epidermis with acanthosis and papillomatosis plus mild perivascular lymphocytic infiltrate in the superficial dermis (H&E, ×200). Our patient exhibited only limited lesions at the antecubital fossae and axilla, which is inconsistent with the previous diagnostic criteria for CRP1. Several cases of CRP had involved various flexural areas alone, including the axilla2,3, popliteal fossa2, or antecubital regions4. These lesions fulfilled other diagnostic criteria for CRP. Therefore the criteria related to the primary affected sites should be revised. In addition, the criteria for fungal staining and response to antifungal treatment should be modified. Occasionally, CRP and fungal infection coexist5, as in this patient, however an important diagnostic consideration is that CRP lesions cannot be improved with antifungal treatment even in the presence of a positive fungal examination. Finally, minocycline3,4 has been known as the treatment of choice for CRP; however, other antibiotics have also been efficacious3. The exact reason for the effect of antibiotics is unclear, but many dermatologists speculate that their anti-inflammatory effect may be more predominant than their anti-bacterial effect1. As mentioned above, the previous diagnostic criteria for CRP had several limitations in cases of unusually involved location, co-infection with fungus, and use of antibiotics other than minocycline. Therefore, we propose a modified diagnostic criteria, as follows: (i) clinical findings includeing scaly brown macules and patches, with at least some appearing reticulated and papillomatous; (ii) involvement of the upper trunk, neck, or flexural areas; (iii) negative fungal staining of scales or no response to antifungal treatment; and (iv) excellent response to antibiotics.