[Challenges in diagnosing familial Mediterranean fever: exploring atypical clinical features. Clinical case].

This clinical case series presents descriptions of 3 patients with familial Mediterranean fever (FMF) who have atypical manifestations and abnormal inheritance mechanisms in terms of Gregor Mendel's laws. Although molecular genetic testing can help with disease diagnosis, it is not always conclusive. The primary need for genetic testing in atypical cases is to explain the mechanism of inflammation and to select the optimal therapy. These clinical observations demonstrate the changes in the spectrum of phenotypic manifestations of FMF in the context of the widespread introduction of molecular genetic methods.

Pyoderma Gangrenosum: A Presenting Feature of Rheumatoid Arthritis.

Pyoderma gangrenosum (PG) is an uncommon inflammatory disorder that exhibits a range of clinical manifestations and levels of severity. It frequently occurs alongside an underlying condition, most often inflammatory bowel disease. PG, Sweet syndrome, palisaded neutrophilic granulomatous dermatitis (PNGD), interstitial granulomatous dermatitis (IGD) and rheumatoid neutrophilic dermatitis may be associated with rheumatoid arthritis (RA). We present a case of a 65-year-old woman with disseminated dermatosis to the hands, abdomen, buttocks, and lower limbs. The dermatosis presented with numerous ulcers of varying shapes, featuring clean bases, undermined edges, and a purplish erythematous appearance. Further investigations, including imaging studies and RA factor and anti-cyclic citrullinated peptide (anti-CCP) levels, led us to the diagnosis of RA. This case indicates that RA may be frequently undiagnosed and untreated in other patients with PG, as ulcers on the lower extremities can often be the main reason for seeking medical attention.

[Pyoderma gangrenosum after orthopedic conservative treatment]. / Pyoderma gangrenosum après traitement conservateur orthopédique.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. Half of the cases are associated with an immune dysfunction and are frequently triggered by pathergy such as a tissular aggression via surgery or burn wounds. A patient with ulcerative colitis presented a PG at the site of an iontophoresis patch for tendinopathy. Treatment in a specialized burn center, corticosteroid therapy and adapted local care contributed to a favourable evolution. PG remains a diagnosis of exclusion and inflammatory phenomena must be differentiated from infectious causes such as necrotizing fasciitis to initiate immunosuppressive treatment. Being rare and difficult to diagnose and to treat as well as associated with potentially severe sequelae, a multidisciplinary team is required for the management of PG.

Le Pyoderma gangrenosum (PG) est une dermatose neutrophilique rare. Il est, dans la moitié des cas, associé à une maladie dysimmunitaire et il est fréquemment déclenché par un phénomène de pathergie, défini comme une agression tissulaire par une intervention chirurgicale ou encore une brûlure. Une patiente avec une rectocolite ulcéro-hémorragique a développé un PG sur le site d'application d'un patch d'ionophorèse pour une tendinopathie. Un traitement par une corticothérapie, un traitement immunosuppresseur local et des soins locaux adaptés ont permis une évolution favorable. Le PG reste un diagnostic d'exclusion et les phénomènes inflammatoires doivent être différenciés de phénomènes infectieux, comme la fasciite nécrosante, afin d'initier rapidement des immunosuppresseurs. Comme il s'agit d'une pathologie rare avec un diagnostic difficile, que des séquelles peuvent être catastrophiques et qu'un traitement immunosuppresseur complexe doit être instauré, une équipe pluridisciplinaire est requise pour la prise en charge de cette pathologie.

Effect of Density and Lineage on Dorsal Surface Temperature, Performance, and Carcass Condemnation of Broiler Grillers.

The aims of this study were (i) to evaluate the effect of density, lineage, age, and time of day on dorsal surface temperature and (ii) to evaluate the effect of density and lineage on performance and carcass condemnations in broiler grillers. The evaluations were carried out in barns with the Dark House system, with two densities, 17 and 19 chickens/m2 and two lineages, Cobb and Ross. The dorsal surface temperature of the chickens was measured by infrared thermography at 7, 14, 21, 23, 25 and 27 days of age, four times a day. The average daily weight gain, feed conversion, mortality, partial carcass condemnations, as well as those due to arthritis and dermatosis were also evaluated. The highest dorsal surface temperatures were observed in Cobbs housed at a density of 17 chickens/m2, and in Ross housed at a density of 19 chickens/m2. Cobbs housed at a 17 chickens/m2 density showed the lowest feed conversion compared to Ross at the same density. Ross showed higher dorsal surface temperatures when compared to Cobbs at 14, 21, and 27 days. Cobbs showed higher percentages of partial carcass condemnation and arthritis compared to Ross. The higher density of broiler grillers in the Dark House system does not influence the dorsal surface temperature, performance, dermatosis, arthritis, and partial carcass condemnations.

An Effective Siddha Management for Dermatosis Papulosa Nigra: A Case Report.

Dermatosis papulosa nigra (DPN) is a noncancerous skin growth that is classified as a subtype of seborrhoeic keratoses. DPN is predominantly found in individuals with darker skin tones, namely, those with Fitzpatrick skin types III-VI. This condition primarily affects women of Asian or African American descent. The non-availability of accurate management for this illness presents a challenge to the medical fraternity. Electrodesiccation, laser therapy, and surgery offer expensive solutions. Siddha external medicine provides a solution through appropriate methods. A 70-year-old woman with Fitzpatrick skin type V appeared with many hyperpigmented papules on the malar region that had been present for five years. The dermatological examination revealed the presence of several brown papular lesions, which were particularly pronounced in the malar areas. The dimensions of the lesions typically varied from 1 to 5 mm; however, in the malar area, their size extended up to 1 cm. The Siddha formulation Pachaieruvai was administered externally for five consecutive days. While undergoing treatment, the patient experienced mild pain, burning, slight redness, and swelling in the area where Pachaieruvai was applied. These symptoms persisted for about an hour during and after the procedure but disappeared within 24 hours with the use of coconut oil. The evaluation of treatment response was determined using the recognised quartile grading methodology. During the first-week follow-up appointment after the last treatment, significant improvements were observed in the cheek lesions, particularly in four large lesions.Throughout the treatment, the patient may encounter mild discomfort, a burning sensation, slight redness, and swelling at the administration site of Pachaieruvai. These reactions are positive indicators of disease regression. No adverse symptoms and recurrence were observed during the follow-up. This research specifically examines the Siddha approach to managing DPN. Based on the findings and observations, it can be concluded that Siddha medicine is effective in treating DPN.

Heparin-Induced Hemorrhagic Bullous Dermatosis: A Rare Complication of Unfractionated Heparin.

We present a case of an 82-year-old female with a significant medical history of hypertension and Alzheimer's disease who developed heparin-induced hemorrhagic bullous dermatosis during treatment for a subsegmental pulmonary embolism. The patient was admitted with lower extremity edema and cyanosis, diagnosed with a subsegmental pulmonary embolism, and started on therapeutic doses of unfractionated heparin. On the sixth day of heparin therapy, she developed abdominal bloating and a diffuse exanthematous rash, which progressed to hemorrhagic bullae on the plantar and dorsal aspects of her feet, alongside extensive purpura on her legs. Laboratory findings revealed thrombocytopenia. Multidisciplinary consultations confirmed the diagnosis of heparin-induced hemorrhagic bullous dermatosis. Management included continuing unfractionated heparin with close monitoring, supportive topical treatments, and a subsequent transition to rivaroxaban. The patient's condition improved significantly, and she was discharged in stable condition. This case highlights the importance of recognizing rare adverse reactions to heparin and raises the question of preventive measures or risk factors related to this manifestation.

The involvement of keratinocytes in pruritus of chronic inflammatory dermatosis.

Frequent itching and incessant scratching are commonly observed in various chronic inflammatory skin conditions, including atopic dermatitis and psoriasis. The persistent and prolonged nature of pruritus can worsen one's quality of life. Keratinocytes (KCs), the predominant cells of the epidermis, have been confirmed to interact with sensory neurons and immune cells and be involved in chronic skin inflammatory diseases associated with pruritus. Initially, KCs and sensory neurons form a unique synapse-like connection within the epidermis, serving as the structural foundation for their interaction. Additionally, several receptors, including toll-like receptors and protease-activated receptor 2, expressed on KCs, become activated in an inflammatory milieu. On the one hand, activated KCs are sources of pro-inflammatory cytokines and neurotrophic factors, such as adenosine triphosphate, thymic stromal lymphopoietin, and nerve growth factor, which directly or indirectly participate in stimulating sensory neurons, thereby contributing to the itch sensations. On the other hand, KCs also function as primary transducers alongside intraepidermal nerve endings, directly initiating pruritic responses. This review summarizes the current literature and highlights the critical role of KCs in the development and persistence of chronic itch in inflammatory skin disorders.

Photodynamic therapy for facial dermatosis papulosa nigra: Efficacy and safety in a preliminary study.

Dermatosis papulosa nigra (DPN) is a type of benign epidermal hyperplasia that affects the appearance of patients and poses a threat to their physical and mental health. Photodynamic therapy (PDT) has been shown to have the advantages of non-invasiveness, efficacy, and low recurrence in the treatment of skin disorders. However, no studies have been reported on the use of PDT for the treatment of DPN. Therefore, we used PDT for the first time to treat DPN and monitor its efficacy. Forty-five patients with DPN diagnosed at the outpatient clinic of Changzhou First People's Hospital were treated with 10 % 5-aminolevulinic acid (ALA) once a week for four consecutive weeks and followed for 3 months. After four treatment sessions, the cure rate among the 45 patients was 71.1 %, and the overall efficacy rate was 93.3 %. The most common adverse reactions were mild erythema, edema, and temporary pigmentation. PDT is expected to become a new and effective treatment for DPN.

Sweet syndrome associated with moderate leukocyte adhesion deficiency type I: a case report and review of the literature.

Sweet syndrome is an acute febrile neutrophilic dermatosis characterized by the infiltration of neutrophils into the skin. It may occur idiopathically or be linked to malignancies, inflammatory or autoimmune diseases. Leukocyte adhesion deficiency type I (LAD-I) is an inborn error immunity wherein leukocytes lack adhesion molecules necessary for migration to infection sites due to mutations in the CD18 gene encoding ß2 integrins. We present a case of a 16-month-old female initially diagnosed and treated for Sweet syndrome based on histopathological findings with recurrent flare episodes. Subsequent workup revealed LAD-I, making this case the first documented association between Sweet syndrome and LAD-I. Moreover, we reviewed the pertinent literatures detailing the concurrence of neutrophilic dermatosis and immunodeficiency disorders. This case underscores the significance of comprehensive evaluation for Sweet syndrome patients who are refractory to conventional treatments.

Neonatal Sweet syndrome with associated rectovestibular fistula and review of the literature.

An otherwise healthy 4-week-old term female of Japanese heritage presented with a 1-week history of asymptomatic progressive, generalized skin lesions. The lesion morphology, distribution, and dermatopathology result was consistent with Sweet syndrome. The patient was found to have a congenital type H rectovestibular fistula. This case highlights the rare association of rectovestibular fistula in neonatal Sweet syndrome which has only been described in neonates of Japanese heritage.

[Palmoplantar dermatoses: Diagnostic approach in primary care]. / Dermatosis palmoplantares: aproximación diagnóstica en atención primaria.

This article addresses the diagnostic challenges of palmoplantar dermatoses (PPD) within the scope of Primary Health Care (PHC). These common skin conditions, encountered in daily practice, exhibit a diverse range of symptoms and morphologies, complicating their diagnosis. They are etiologically classified into infectious inflammatory, non-infectious inflammatory, and hereditary keratodermas. While various dermatoses may affect the palms and soles, few are specific to this area. Notable examples include palmoplantar pustulosis, dyshidrosis, erythema pernio, and Bazex syndrome. Given the high prevalence of dermatological consultations in PHC, this article underscores the significance of PHC professionals' knowledge regarding these conditions. It proposes a diagnostic algorithm to facilitate their management and timely referral.

A Grover-Like Skin Rash: A Potential Indicator of Underlying Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) Infection.

Transient acantholytic dermatosis, also known as Grover's disease, is an acquired dermatological condition characterised by the sudden emergence of pruritic, erythematous papules, or vesicles, primarily affecting the trunk. It is observed most commonly in men older than 50 years. Histology typically demonstrates a pattern of focal acantholysis within the epidermis, dyskeratotic cells including corps ronds and grains, and a variable perivascular lymphocytic infiltrate in the upper dermis. While its aetiology is not well understood, recognised triggers include excessive heat, sweating, sun exposure, and certain drugs, such as chemotherapy agents. More recently, isolated reports of Grover's disease and Grover-like skin eruptions have been described in patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and following COVID-19 vaccination. We report the case of a 65-year-old man who presented to secondary care with a nine-day history of an intensely pruritic rash over his chest and back. On internal medical workup, he was found to have SARS-CoV-2 infection and rapidly deteriorated due to coronavirus disease 2019 (COVID-19)-associated pneumonia, necessitating a 10-day hospital admission for supportive care. Diagnostic workup of his skin lesions confirmed transient acantholytic dermatosis (Grover's disease), which resolved following a course of oral corticosteroids. This case underscores the rare but significant association between Grover's disease and COVID-19, contributing valuable insights to the evolving body of literature on cutaneous lesions associated with SARS-CoV-2 infection, and highlighting the importance of considering SARS-CoV-2 screening as part of the diagnostic workup for patients presenting with Grover-like skin eruptions.

Gender differences in quality of life of vitiligo patients attending a tertiary care center.

Background: Vitiligo is a common depigmenting dermatosis characterized by milky-white macules or patches. Any pathological discoloration of the skin has been linked to an impact on the body image of an individual, and vitiligo has been linked to decreased self-esteem and poor socialization of the affected people. In Indian society, vitiligo is labeled as Shweta-Kushta (i.e., white-colored leprosy) and is compared to leprosy-a more stigmatizing skin condition. Aim: This study aims to study gender-related differences in the quality of life of Indian vitiligo patients. Methodology: In this hospital-based cross-sectional study, a total of 55 vitiligo patients (females 25 and males 30) were enrolled using the purposive sampling technique. The sample was assessed on Dermatology Life Quality Index (DLQI) questionnaire, Rosenberg Self-Esteem Scale (RSS), and General Health Questionnaire-12 (GHQ-12) to assess their quality of life, self-esteem, and psychological well-being, respectively. The gender differences in sociodemographic and clinical details as well as their relationship with the quality of life of the patients were sought with the help of appropriate statistical measures. Results: The patients of both genders were comparable in terms of all sociodemographic and clinical variables except the females being less educated and employed than the males. There was a statistically significant trend of higher mean DLQI total score in females than males (6.6 ± 3.55 vs 4.8 ± 2.71, Mann-Whitney U = 263.5, P =0.058). Significantly, the average DLQI score in female vitiligo patients was negatively related to family income (r s = -.659, P <.001) and it was significantly higher for the patients from a rural background than those from an urban background (8.55 ± 3.30 vs 5.07 ± 3.03, Mann-Whitney U = 33.5, P <.05). The average DLQI score was negatively correlated to RSS score but positively correlated to GHQ-12 score in patients of both genders. Conclusion: The quality of life of female patients with vitiligo is poorer than the same in male patients. The poor financial condition of families and rural living are two factors related to the poorer quality of life of female patients. A poorer quality of life in vitiligo patients of both genders is linked with decreased self-esteem and decreased psychological well-being. Gender-related issues in the quality of life and the overall well-being of such patients are important in their management and policy-making.

Sweet syndrome in pregnancy: A narrative review.

The aim of this review is to increase obstetrician awareness of pregnancy-associated Sweet syndrome. Patients present with fever, leukocytosis, and skin eruption, which can mimic other infectious or inflammatory conditions, but do not respond to antibiotics. A search using PubMed, EMBASE, and Web of Science Core Collection was conducted to review all reported cases of pregnancy-associated Sweet syndrome, an acute febrile neutrophilic dermatosis occurring during pregnancy or postpartum. A total of 33 episodes among 30 patients were identified, with the majority (54.5% [18]) of cases occurring within the second trimester. Among the 30 patients, skin lesions most commonly affected the head and neck (73.3% [22]), with rare oral or ocular involvement. Leukocytosis was the most common laboratory finding, reported in 96.7% [29] of patients, with neutrophil predominance noted in 70.0% [21]. The diagnosis was confirmed for all patients with pathognomonic results of skin biopsies. Of the 27 cases detailing treatment, systemic corticosteroids were most frequently used (19 cases), followed by conservative management (seven cases), and dapsone (one case). The dapsone-treated patient and 15 of the 19 steroid-treated patients experienced resolution, but additional management strategies were required in the remaining four individuals. Spontaneous resolution occurred during pregnancy in six of the seven conservatively managed individuals, with one patient experiencing spontaneous abortion shortly after skin eruption at 10 weeks of gestation. No associated maternal deaths were reported. Obstetric complications of pregnancy-associated Sweet syndrome included endomyometritis, sterile placental abscesses, and abdominal wall necrosis. Delivery of healthy infants occurred in 24 of the 25 cases that presented fetal outcome, which included two infants who underwent medically indicated preterm deliveries.

[Dermatoses of elderly inpatients in Casablanca: epidemiology and factors associated with length of stay]. / Dermatoses du sujet âgé en hospitalisation à Casablanca : épidémiologie et facteurs associés à la durée de séjour.

Geriatric in-patient dermatoses are diverse. Few data in Morocco describe the epidemiological profile and factors associated with average length of stay (LOS). Our aim was to identify these dermatoses and determine the factors associated with LOS.