Characteristics of DRESS Syndrome in the Elderly: A Comparative Study of 55 Patients.

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare drug reaction characterized by a skin rash, eosinophilia, and organ involvement. Objective: Our purpose is to focus on the clinical and epidemiological characteristics of DRESS in the elderly and to identify the incriminated drugs. Methods: This is a retrospective study including patients, hospitalized for DRESS with a RegiSCAR ≥4. The population was divided into 2 groups according to age: 65 years or older (G1) and <65 years (G2). The statistical study was performed using the comparative and multivariate analysis. Results: We included 55 patients (30.9% G1 and 69.1% G2). Skin manifestations were comparable in both groups. Lymphadenopathy was less common in G1 with a statistically significant difference (P = 0.012). Renal impairment was more frequent in the elderly with a statistically significant result (P = 0.005). DRESS in the elderly group was significantly associated with the occurrence of sepsis (P = 0.008). Allopurinol was the most common culprit associated with DRESS in G1 (P = 0.001). Relapses and recurrences were comparable in both groups (P = 0.71). Conclusions: DRESS in the elderly is associated with a high risk of complications, mainly kidney involvement and sepsis. Allopurinol is the most incriminated drug.

Lichenoid lesions around the anus: An unusual site of Hailey-Hailey disease.

The anal region is an unusual site of Hailey-Hailey disease. It manifests with lichenoid lesions with crusted erosions around the anus. It should be differentiated from condylomata acuminata, extramammary Paget disease, and bowenoid papulosis.

Gingival Recession: An Unusual Oral Presentation of Morphea "en coup de sabre".

A 21-year-old woman presented with a 13-year history of a linear lesion on the lip. She experienced no pain and only had an esthetic complaint. Her personal and family history was otherwise unremarkable. She had no history of photosensitivity, Raynaud's phenomenon, arthralgias, dry eyes, fever, trauma, or exposure to irradiation.

Erysipeloid cutaneous leishmaniasis: a study of 40 cases of an unusual variant.

BACKGROUND: Erysipeloid cutaneous leishmaniasis (ECL) is known as the chronic form of cutaneous leishmaniasis (CL). However, keeping its clinical presentation in view, there is a need to revisit this form of the disease. AIMS: To describe ECL in view of clinical features and treatment modalities. METHODS: We include a case series seen in Sfax (Southern Tunisia) from January 2017 to January 2021. All patients clinically suggestive and laboratory confirmed with a diagnosis of CL were registered. Patients of all age groups and of either gender having cutaneous lesions resembling erysipela on the face were included in the study. Different demographic features of the patients and clinical aspects were identified. Descriptive statistics were used for analysis. RESULTS: Of 1300 registered patients with CL, 40 (3%) were diagnosed as ECL. Ages ranged from 15 to 65 years, and duration of lesions varied from 15 to 180 days. All patients had lesions over the face. Clinically, a painful infiltrated inflammatory placard of the central facial area with a butterfly shape was observed in 14 cases, as well as zones of the cheekbone (11 cases), cheekbone and nose (5 cases), cheekbone and eyelid (8 cases), and cheekbone with ear (2 cases). Several therapeutic methods were prescribed with a sufficient result with no recurrence. CONCLUSION: ECL is a rare presentation that typically occurs on the face, looking like erysipelas, in patients who are native from an endemic region of CL.

[A rare cause of AA amyloidosis: Hereditary epidermolysis bullosa]. / Une cause rare de l'amylose AA : les épidermolyses bulleuses héréditaires.

INTRODUCTION: Recessive dystrophic epidermolysis bullosa is a rare genetic condition characterized by fragile skin and mucous membrane, caused by mutations in the COL7A1 gene. AA amyloidosis is a rare complication of these genodermatosis. OBSERVATIONS: Two patients with recessive dystrophic epidermolysis bullosa, generalized severe in the first case and generalized intermediate in the second case, developed at the age of 38 and 28, respectively, nephrotic syndrome. The diagnosis of secondary renal amyloidosis was confirmed by renal biopsy in the first case and by minor salivary gland biopsy in the second case. Death occurred 2 months after diagnosis in both cases. CONCLUSION: Renal involvement is quite common in AA amyloidosis in patients with recessive dystrophic epidermolysis bullosa. Nephrotic syndrome and rapid decline in renal function renal are characteristic features. The prognosis is poor due to underlying conditions and the lack of an etiological treatment.

Hydroxychloroquine-induced acute generalized exanthematous pustulosis: a series of seven patients and review of the literature.

BACKGROUND: Hydroxychloroquine (HCQ)-induced acute generalized exanthematous pustulosis (AGEP) is poorly described in the literature. The aim of our study was to characterize the clinical, laboratory, allergological, and genetic features of HCQ-induced AGEP. METHODS: We conducted a retrospective study of patients with HCQ-induced AGEP diagnosed between 2011 and 2019. We performed molecular analysis to identify variations in the IL36RN gene. We also reviewed similar cases reported between 1991 and March 2020. RESULTS: Seven female patients were included. The mean age was 47 years old, and the average time from HCQ start to onset of symptoms was 40 days. All patients received topical steroids with a full resolution of the rash within an average of 39 days after HCQ withdrawal. Patch tests were performed for three patients with positive results in one case. Genetic analyses were performed for three patients, and no mutation in the IL36RN gene was identified. CONCLUSION: The latent period and the duration for resolution of HCQ-induced AGEP may be longer than with other drugs due to the metabolic characteristics of HCQ. Mutations in the IL36RN gene were not identified in our patients.

Adverse Reactions Due to the Bacillus Calmette-Guerin Vaccine: Twenty Tunisian Cases.

BACKGROUND: Bacillus Calmette-Guérin (BCG) vaccine is a widely used vaccine. Management of local BCG complications differs between clinicians, and the optimal approach remains unclear. AIMS: We aim to describe the epidemiological, clinical and therapeutic aspects of the BCG vaccine side effects in Sfax. PATIENTS AND METHODS: This was a retrospective study of all the cases of BCG vaccine adverse reactions recorded in the Dermatology and Paediatrics Departments of Hedi Chaker University Hospital of Sfax over a period of 10 years (2005-2015). RESULTS: Twenty cases of BCG adverse reactions were notified during the study period. Actually, 80% of the patients presented local adverse reactions. The outcome was good in all the followed patients. The rate of disseminated BCG disease was 20%. Biological tests of immunity showed a primary immunodeficiency in three cases, whereas the outcome was fatal in two cases. CONCLUSION: BCG vaccine adverse reactions range from mild to severe. However, the management of benign local reactions remains unclear. Disseminated BCG disease must alert clinicians to the possibility of a primary immunodeficiency.

Angiolymphoid hyperplasia with eosinophilia: report of nine cases.

BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferation characterized by dermal or subcutaneous red or brown papules or nodules, most commonly on the head and neck. OBJECTIVE: The aim of this study was to review the epidemiological and clinical characteristics of ALHE, focusing particularly on the histological and therapeutic features. METHODS: We performed a retrospective study of all cases of ALHE diagnosed in our dermatology and pathology departments between 2004 and 2015. RESULTS: Over 12 years, we collected nine cases of ALHE (0.75 case/year). There were four men and five women. The mean age was 43 years. Lesions presented as erythematous or violaceous papules or nodules in all cases, multiple in five cases, and localized on the head in eight cases or other sites in four cases. The diagnosis of ALHE was clinically suspected in only two cases. The histopathological findings showed an ill-circumscribed, intradermal slightly lobular proliferation of capillary-sized vessels around several central vessels. In all cases, the blood vessels were lined by large endothelial cells. An inflammatory infiltrate around the vessels was formed mainly of lymphocytes and eosinophils with isolated plasma cells and histiocytes. Surgery was the most common treatment in our series. Other local or general treatment has also been used with varying responses. CONCLUSION: Angiolymphoid hyperplasia with eosinophilia is a rare epithelioid vascular tumor with a challenging clinical and histological diagnosis. Despite its benign nature, ALHE causes a therapeutic dilemma.

Congenital hemangioma.

Congenital hemangiomas (CHs) are fully developed hemangiomas that are present at birth. There are 2 possible types: rapidly involuting CHs (RICHs) and noninvoluting CHs (NICHs). We conducted a retrospective study (2008-2012) of 6 patients (2 females, 4 males) with CHs (mean age, 16 days). We analyzed the epidemiology, clinical characteristics, and clinical outcome of CHs over this 5-year period.

Sweet's syndrome: a retrospective study of 90 cases from a tertiary care center.

BACKGROUND: Sweet's syndrome (SS) is a neutrophilic dermatosis characterized by the abrupt onset of cutaneous, systemic and histopathological alterations in response to different stimuli. OBJECTIVES: The aim of this study was to assess the epidemioclinical, histological, and therapeutic features and outcomes of SS. METHODS: A retrospective study of all patients diagnosed with SS over a 20-year period (1993-2012) was conducted. Data were analyzed using a level of significance of 5%. RESULTS: Ninety patients (mean age: 46.5 years) fulfilled the inclusion criteria. The ratio of women to men was 5. Significant associations emerged between dermohypodermic nodes and location on the lower limbs (P = 0.042), and vesiculobullous lesions and location on the legs (P = 0.030), dorsum of the hand (P = 0.015), and forearms (P = 0.003), and paraneoplastic forms (P = 0.012). The upper extremities were involved in the majority of patients (83.3%). Correlations were found between edema of the superficial dermis and vesiculobullous lesions and between leukocytoclastic vasculitis and atypical targetoid lesions. Sweet's syndrome was associated with cytomegalovirus infection (n = 1), inflammatory bowel disease (n = 4), neoplasm (n = 6), and pregnancy (n = 3). First-line treatment consisted of colchicine. CONCLUSIONS: To the best of the present authors' knowledge, this is the largest series of SS to be reported. Clinical presentations are similar to those described in the literature. Colchicine was efficient and facilitates the reduced use of corticosteroids. The association between SS and neoplasms should be considered in the context of vesiculobullous lesions.

Aplasia cutis congenita: report of 22 cases.

BACKGROUND: Aplasia cutis congenita (ACC) is a rare malformation characterized by absent or scarred areas of skin at birth. Although most commonly found on the scalp, ACC can also involve other locations. Its etiology and pathogenesis remain unclear. OBJECTIVE: To describe the epidemiologic, clinical, therapeutic, and evolutionary aspects of ACC through a hospital series. METHODS: We conducted a retrospective study from 1995 to 2012 and reported all cases of ACC. RESULTS: We enrolled 22 cases (14 girls and eight boys) of ACC during 18 years. The mean age at diagnosis was 5.7 years. Sixteen ACC involved the scalp, five the trunk, and one the left buttock. ACC was oval-shaped in 20 cases, triangular in one case, and linear in one case. The mean size was 4 cm. ACC was associated with bone defects in two cases, various malformations in eight (37.1%), and with syndromic malformation in three (Adams-Olivier syndrome: two cases; Goltz syndrome: one case). Conservative treatment consisting of wound dressing with vaseline was indicated in six cases. Bone reconstruction was performed in two cases. Regular follow-up and no treatment was recommended in 14 cases. CONCLUSION: Our study emphasizes the frequent association of ACC with malformations (37.1%) and bone defects (9%).