Juvenile dermatomyositis with Anti-SAE antibodies in a Moroccan child associated with pseudo-angioedema: a case report.

BACKGROUND: Juvenile Dermatomyositis (JDM) is the leading cause of non-infectious inflammatory myopathy in children. It is a heterogeneous group of autoimmune diseases characterized by a variable combination of muscular, dermatological, and visceral involvement. Myositis-specific autoantibodies help define homogeneous subgroups with common clinical characteristics and prognoses. Anti-SAE (small ubiquitin-like modifier 1 (SUMO-1) activating enzyme) antibodies are among the most recently discovered specific autoantibodies. The presence of these antibodies is very rare, making it challenging to define clinical features and prognosis in the juvenile form. We report the first case of an African patient with juvenile dermatomyositis and positive anti-SAE antibodies. CASE REPORT: A 5-year-3-month-old Moroccan boy presented to the pediatric emergency department with dysphagia that had been evolving for two days, preceded two months earlier by facial erythema associated with fatigue, lower limb pain, difficulty walking, and progressive inflammatory polyarthralgia. On admission, the child had a heliotrope rash with predominant pseudo-angioedema on the lips, periungual telangiectasia, and Gottron's papules over the bilateral interphalangeal and metatarsophalangeal joints. The patient had a more pronounced proximal muscle weakness in the lower limbs. He had no urticaria, fever, arthritis, calcinosis, cutaneous ulcers, or lipodystrophy. The Joint examination was normal, as was the pleuropulmonary examination. The electroneuromyography showed myogenic changes in all four limbs. Laboratory findings showed elevated levels of creatine phosphokinase and lactate dehydrogenase and a mild inflammatory syndrome. The electrocardiogram was normal. The anti-SAE antibodies were positive. The boy was diagnosed with juvenile dermatomyositis. He received methylprednisolone bolus therapy followed by oral prednisone. The latter was gradually tapered in combination with weekly intramuscular methotrexate. As a result, dysphagia disappeared within 48 h. After two weeks, there was an improvement in the muscular score and a significant regression of facial pseudo-angioedema. CONCLUSION: We report the first African patient with anti-SAE autoantibody-positive JDM. He had a typical dermatological manifestation of JDM associated with pseudo-angioedema predominant on the lips; a rarely reported sign in DM and JDM patients. The patient responded well to corticosteroid therapy and methotrexate.

Psychodermatology knowledge, awareness and patterns of practice among Moroccan dermatologists: a national survey study.

We aimed to assess the knowledge, attitudes and patterns of practice of Moroccan dermatologists and their awareness of psychodermatology (PD). A survey questionnaire was distributed to dermatologists and dermatology trainees from May to July 2022. A total of 112 completed surveys were received. Of these, 63.4% were dermatologists and 36.6% were dermatology residents. Of the participants, 72.3% summarized PD as the psychological impact of dermatological conditions and 50.9% reported frequent involvement with PD. Patients with a psychocutaneous condition represented a significant proportion of dermatological consultations (41.1% of dermatologists stated that 10-25% of their consultations were for psychocutaneous conditions). Only 17% felt 'very comfortable' in the management of PD conditions and 56.3% were not confident prescribing psychotropics. A total of 88.4% had never participated in PD training. Moroccan dermatologists lack a full understanding and proper training in PD. We recommend the incorporation of PD curriculum in training programmes and advocate dermatology and psychiatry liaison.

Congenital cutaneous aplasia of the limbs: A case report.

Introduction: Congenital skin aplasia, or aplasia cutis congenita (ACC), is a rare congenital anomaly. The incidence is estimated to be between 0.5 and 1 in 10,000 births. We report an observation of ACC of the limbs in a female newborn at D3 of life. Case report: Female newborn at D3 of life, born at term by vaginal delivery with a birth weight of 2, 900kg. The general examination revealed a hemodynamically and respiratorily stable and apyretic newborn. The dermatological examination noted the presence of translucent exulcerations on both wrists, the inner side of the left knee, and the distal end of the left leg. In view of this clinical aspect, the diagnosis of ACC was evoked. The management was to hospitalize the newborn in a neonatology unit, do a biological check-up with a trans-fontanelle ultrasound, echocardiography, and abdominal ultrasound, associated with daily care. Discussion: Many hypotheses have been put forward to explain the pathophysiological mechanism of CCA, whether isolated or associated with other anomalies. However, at present, the origin of this malformation remains unknown. There is no unambiguous management in the initial phase, as it depends on the type of CCA. Conclusion: Because of the different possible clinical presentations and existing syndromic associations, it can be thought that it corresponds to a phenotypic expression of various origins, which may be interrelated.

Psoriasis of the Lips: A Case Report with Review of the Literature.

Psoriasis is a chronic inflammatory dermatosis, which progresses by relapses-remissions, of a multifactorial etiology which involves genetic, immunological, and environmental factors. Skin lesions are mainly localized in areas of friction: elbows, knees, pre-tibial, lumbosacral regions, scalp, and nails. Although the appearance of the skin lesions is sufficient for a diagnosis, diagnostic difficulties may be found in case of unusual topography. We report a case of a 45-year-old female patient with psoriatic cheilitis with a review of the literature.

Clinical and histological patterns and treatment of pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis for which accurate epidemiological data are limited and therapy remains a challenge. The primary study's aim was to examine all cases of PG observed in our department over a 6-year period in order to describe the relevant characteristics and outcome under therapy. Fourteen patients were included (5 women, 9 men). The average age of our patients was 40,15 years. The classical, ulcerative form was found in 10 cases (71.42%), the pustular form in 4 cases (27.57%) and PG was multifocal in 4 cases. The PG was located preferentially to the lower limbs. Histological examination was realized in all patients and objectified inflammatory infiltrate composed of polymorphonuclear neutrophils in all cases with vasculitis in 4 cases. Six patients (42.85%) had associated disease at diagnosis of PG, including inflammatory bowel disease in two cases (14.28%), a blood disease in 2 cases (14.28%), lymph node tuberculosis and inflammatory arthritis in 1 case (7%). The most frequent first-line treatments were oral corticosteroids (7 cases) and other treatments used were colchicine in 2 cases, topical corticosteroids in 3 cases with good clinical evolution. Our study confirms that PG is a rare disease, associated in almost half of cases with systemic disease already present at diagnosis; in our Moroccan background, it is most often inflammatory bowel disease, hematological or solid cancer and tuberculosis.

Crohn's disease presenting as vulvar edema in a 15-year-old girl.

BACKGROUND: Vulval involvement is an uncommon extraintestinal manifestation of Crohn's disease, and it is very rare in children. Patients with vulval CD typically present with erythema and edema of the labia majora, which can progresses to extensive ulcer formation. Vulval CD can appear before or after intestinal problems or it may occur simultaneously. OBSERVATION: We present a 15-years-old girl with bilateral labial hypertrophy which revealed a Crohn's disease. The course of her lesion was independent of the intestinal disease and responded significantly to medical treatment including Mesalamine, corticosteroid and local care. CONCLUSIONS: We emphasize that although vulval involvement in childhood is uncommon, Crohn's disease must be considered in the differential diagnosis of nontender, red, edematous lesions of the genital area.