scholarly journals New Described Dermatological Disorders

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Müzeyyen Gönül ◽  
Bengu Cevirgen Cemil ◽  
Havva Ozge Keseroglu ◽  
Havva Kaya Akis
Keyword(s):  
Pyoderma Gangrenosum ◽  
Hidradenitis Suppurativa ◽  
Autoinflammatory Diseases ◽  
Neutrophilic Dermatosis ◽  
Macular Atrophy ◽  
Dermatological Disorders ◽  
Pyogenic Arthritis ◽  
Pachyonychia Congenita ◽  
Unusual Type ◽  
Made In

Many advances in dermatology have been made in recent years. In the present review article, newly described disorders from the last six years are presented in detail. We divided these reports into different sections, including syndromes, autoinflammatory diseases, tumors, and unclassified disease. Syndromes included are “circumferential skin creases Kunze type” and “unusual type of pachyonychia congenita or a new syndrome”; autoinflammatory diseases include “chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome,” “pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome,” and “pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH) syndrome”; tumors include “acquired reactive digital fibroma,” “onychocytic matricoma and onychocytic carcinoma,” “infundibulocystic nail bed squamous cell carcinoma,” and “acral histiocytic nodules”; unclassified disorders include “saurian papulosis,” “symmetrical acrokeratoderma,” “confetti-like macular atrophy,” and “skin spicules,” “erythema papulosa semicircularis recidivans.”

scholarly journals Erythema Nodosum and Pyoderma Gangrenosum in 50 Patients with Crohn’s Disease

2005 ◽  
Vol 19 (10) ◽  
pp. 603-606 ◽  
Author(s):  
Hugh J Freeman
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Pyoderma Gangrenosum ◽  
Complex Disease ◽  
Erythema Nodosum ◽  
Colonic Disease ◽  
Detection Rates ◽  
Dependent Effect ◽  
Age Dependent ◽  
Dermatological Disorders

Erythema nodosum and pyoderma gangrenosum may occur in Crohn's disease. In the present evaluation of consecutive patients with Crohn's disease spanning more than two decades, erythema nodosum was seen in 45 patients and pyoderma gangrenosum was seen in seven patients. Forty-one of 566 women (7.2%) and nine of 449 men (2.0%) were affected. Of these, 45 (4.4%) had erythema nodosum and seven (0.7%) had pyoderma gangrenosum, including two (0.2%) with both dermatological disorders at different times during their clinical courses. Recurrent erythema nodosum was also detected in nine patients (20%) including eight women, while recurrent pyoderma gangrenosum was seen in two patients (28.6%). There was an age-dependent effect on the appearance of erythema nodosum in women, with the highest percentages seen in those younger than 20 years of age. Detection rates for erythema nodosum in women only approached the low mens' rates in Crohn's disease at older than 40 years of age. Most patients with these dermatological disorders had colonic disease with or without ileal involvement as well as complex disease, usually with penetrating complications. The present study documents a sex-based and age-dependent effect on the clinical expression of erythema nodosum in Crohn's disease. This suggests that some components of the inflammatory process in Crohn's disease may be modulated by estrogen-mediated events, particularly in adolescents and young adults.

scholarly journals A Rare Case of Pyoderma Gangrenosum in a HIV Positive Patient

2011 ◽  
Vol 2 (2) ◽  
pp. 128-130
Author(s):  
Eswari Loganathan ◽  
Asima Banu
Keyword(s):  
Pyoderma Gangrenosum ◽  
Rare Case ◽  
Positive Patient ◽  
Neutrophilic Dermatosis ◽  
Hiv Positive ◽  
Medical Sciences ◽  
First Line ◽  
Hodgkins Lymphoma ◽  
Lymphoid Malignancies ◽  
Non Hodgkins Lymphoma

HIV infection can lead to varied spectrum of associated disease conditions. Pyoderma gangrenosum is a neutrophilic dermatosis that may be associated with myeloid malignancies. Less information is available about the association of pyoderma gangrenosum with lymphoid malignancies. We report a rare case of pyoderma gangrenosum in association with Non hodgkins lymphoma(NHL) of diffuse large B cell type. In this case the lesion which showed NHL features occurred in the perianal region, coexisting with pyoderma gangrenosum lesions in the perianal, lower limb and abdominal region. Another interesting feature is the occurrence of both these conditions in a HIV-positive patient with severe immunologic failure to first line antiretroviral therapy contributing to the refractoriness to treatment. Key Words: HIV; pyoderma gangrenosum; Non Hodgkins Lymphoma DOI: http://dx.doi.org/10.3126/ajms.v2i2.4029 Asian Journal of Medical Sciences 2 (2011) 128-130

Systematic evaluation of nine monogenic autoinflammatory diseases reveals common and disease-specific correlations with allergy-associated features

2021 ◽  
pp. annrheumdis-2020-219137
Author(s):  
Daniella Muallem Schwartz ◽  
Moses M Kitakule ◽  
Brian LP Dizon ◽  
Cristhian Gutierrez-Huerta ◽  
Sarah A Blackstone ◽  
...  
Keyword(s):  
High Rate ◽  
Autoinflammatory Diseases ◽  
Th2 Cells ◽  
Systematic Evaluation ◽  
Neutrophilic Dermatosis ◽  
Tumour Necrosis Factor Receptor ◽  
Periodic Syndrome ◽  
Clinical Phenotypes ◽  
Type 2 Immunity

BackgroundMonogenic autoinflammatory diseases (AID) are caused by mutations in innate immune genes. The effects of these mutations on allergic inflammation are unknown.ObjectivesWe investigated allergic, immunological and clinical phenotypes in FMF (familial Mediterranean fever), CAPS (cryopyrin-associated periodic syndrome), TRAPS (tumour necrosis factor receptor-associated periodic syndrome), HIDS (hyper-IgD syndrome), PAPA (pyogenic arthritis, pyoderma gangrenosum and acne), DADA2 (deficiency of adenosine deaminase 2), HA20 (haploinsufficiency of A20), CANDLE (chronic atypical neutrophilic dermatosis, lipodystrophy, elevated temperature) and SAVI (STING-associated vasculopathy of infancy).MethodsIn this cross-sectional study, clinical data were assessed in 425 patients with AID using questionnaires and chart reviews. Comparator data were obtained from public databases. Peripheral blood mononuclear cells obtained from 55 patients were stimulated and CD4+ cytokine production assessed.ResultsClinical laboratory features of Type 2 immunity were elevated in CAPS but reduced in most AID, particularly DADA2. Physician-diagnosed allergic diseases were prevalent in multiple AID, including CAPS and DADA2. T helper 2 (Th2) cells were expanded in CAPS, TRAPS and HIDS; Th9 cells were expanded in HA20.ConclusionsCAPS is characterised by an enhanced Type 2 signature, whereas FMF and CANDLE are associated with reduced Type 2 responses. DADA2 is associated with reduced Type 2 responses but a high rate of physician-diagnosed allergy. Therefore, NLRP3-driven autoinflammation may promote Type 2 immunity, whereas AID like DADA2 may manifest clinical phenotypes that masquerade as allergic disorders. Further investigations are needed to determine the contribution of autoinflammation to allergic clinical and immunological phenotypes, to improve the treatment of patients with AID.

The Importance of Early Diagnosis of Pyoderma Gangrenosum: A Case After Plastic Surgery

2021 ◽  
Vol 33 (7) ◽  
pp. E58-E60
Author(s):  
Gunel Guliyeva ◽  
Ali Kilic
Keyword(s):  
Hospital Stay ◽  
Plastic Surgery ◽  
Pyoderma Gangrenosum ◽  
Reduction Mammoplasty ◽  
Careful Examination ◽  
Neutrophilic Dermatosis ◽  
Unknown Etiology ◽  
Surgical Trauma ◽  
Surgical Interventions ◽  
Low Incidence

Introduction. Pyoderma gangrenosum (PG) is a multifactorial neutrophilic dermatosis of unknown etiology. It can occur in isolation; in association with different inflammatory, autoimmune, or malignant diseases; or as part of various syndromes. Because of its low incidence and the difficulty in distinguishing it from other possible lesions, PG is usually misdiagnosed. As a result, patients may be subjected to unnecessary treatments and surgical interventions that exacerbate the development of PG, as pathergy phenomenon is observed with this skin disorder. Surgical trauma can also lead to the formation of PG with the same mechanism. The occurrence of PG lesions has been reported after plastic surgery as well. In most cases, however, the diagnosis is delayed, resulting in disfigurement, additional surgeries, and extended hospital stay. Case Report. In this article, a case of early detected bilateral PG after reduction mammoplasty in a patient with no personal or family history of autoimmune disorders is presented. Careful examination of the wound and analysis of the clinical picture resulted in the diagnosis of PG. Conclusions. The authors believe that the description of the diagnostic clues considered in this case will aid the plastic surgeon in prompt recognition and management of postoperative PG, with the aim of decreasing patient morbidity and the duration of hospital stay while preventing additional complications.

Applying Intravenous Immunoglobulin and Negative-Pressure Wound Therapy to Treat Refractory Pyoderma Gangrenosum: A Case Report

2020 ◽  
pp. 153473462094045
Author(s):  
Shi-Ying Jin ◽  
Mei Chen ◽  
Feng-Yuan Wang ◽  
Fei Wang
Keyword(s):  
Intravenous Immunoglobulin ◽  
Pyoderma Gangrenosum ◽  
Negative Pressure ◽  
Negative Pressure Wound Therapy ◽  
Lower Leg ◽  
Neutrophilic Dermatosis ◽  
Wound Therapy ◽  
Systemic Corticosteroids ◽  
Painful Ulcer ◽  
The Right

Pyoderma gangrenosum (PG) is a rare chronic neutrophilic dermatosis that causes undermining ulcers. Unfortunately, standardization of PG treatment remains a challenge. In this article, we describe a case in which a 69-year-old man presented with a painful ulcer on the right lower leg. The diagnosis of PG was made after excluding other diseases. He had a history of PG on his left lower leg 2 years earlier and was cured by the treatment of systemic corticosteroids and cyclosporin A for 43 days. However, such a treatment was not effective this time. Hence, we applied intravenous immunoglobulin and negative-pressure wound therapy, and the patient was cured. Altogether, this case supports the use of intravenous immunoglobulin as an effective adjuvant for refractory PG, and indicates negative-pressure wound therapy as a treatment option to advance ulcer healing under adequate immunosuppression.

Sign in / Sign up

Export Citation Format

Share Document