Annelies Stockman

SACRAL Syndrome: Spinal Dysraphism, Anogenital, Cutaneous, Renal and Urologic Anomalies, Associated with an Angioma of Lumbosacral Localization

Background: Publications concerning perineal infantile hemangiomas are scarce, and comprise no large series. Objective: Studying clinical features of hemangiomas of the perineal area, complications and associated malformations. Methods: Retrospective analysis of all hemangiomas localized in the perineal area, encountered at the Children’s Hospital in Bordeaux from 1994. Results: Of 49 perineal hemangiomas (34 girls, 15 boys), 5 patients had accompanying malformation, mainly lipomyelomeningocele with tethered cord. The superficial hemangiomas were more represented in males and presented sooner than the nodular counterpart. The average rate of ulceration was 73%, ulcerations developed earlier in the superficial forms than their nodular counterparts (2 vs. 4 months). Conclusion: Superficial perineal hemangiomas are more often complicated by ulceration, and are associated with developmental anomalies. As a counterpart for the PHACE syndrome in facial hemangioma, we propose the acronym SACRAL for perineal hemangiomas: Spinal dysraphism, Anogenital anomalies, Cutaneous anomalies, Renal and urologic anomalies, associated with Angioma of Lumbosacral localization.

Diffuse Plane Normolipaemic Xanthomatosis in a Patient with Chronic Lymphatic Leukaemia and Monoclonal Gammopathy

Diffuse plane normolipaemic xanthomatosis is a well-defined uncommon condition characterized by yellow-orange plaques involving the eyelids, neck, upper trunk, buttocks and flexural folds. Over half of the reported cases are associated with lymphoproliferative disorders. The condition may arise as a result of perivascular deposition of lipoprotein-immunoglobulin complexes. We present a female patient with clear clinical features of diffuse plane xanthomatosis associated with chronic lymphatic leukaemia and monoclonal gammopathy. In this patient, we found evidence of complement consumption supporting the hypothesis that complexes of lipoprotein and paraprotein are formed. Lipid electrophoresis of fasting serum revealed the presence of a faint precipitate at the application slot, also indicative of the presence of protein-lipid complexes.

Pyoderma gangrenosum as an Early Revelator of Acute Leukemia

Bullous pyoderma gangrenosum is an atypical, more superficial variety of the classical pyoderma and is often associated with myeloproliferative disorders. We present the case of a patient who presented initially with subcutaneous nodules and who developed bullous lesions afterwards. Histological evaluation showed the presence of neutrophilic infiltrates in both lesions. A few months after the diagnosis of bullous pyoderma gangrenosum, an underlying leukemia was revealed. Our case illustrates the importance of regular blood and bone marrow examinations in patients with atypical bullous pyoderma gangrenosum, resulting in a rapid diagnosis of the underlying disease.

A case of anaphylaxis caused by macrogol 3350 after injection of a corticosteroid.

Conflicts of interest: The authors have declared no conflicts. Macrogols are polymers of ethylene glycol, and are widely used in food and food packaging, and also in cosmetics and pharmaceutical preparations as solvents, softening agents, and lubricants. There are different types of macrogols, according to their molecular weight (e.g. macrogol 400, 3350, 4000, and 6000). There have been reports of anaphylactic reactions to macrogol

Topical class I corticosteroids in 10 patients with bullous pemphigoid: correlation of the outcome with the severity degree of the disease and review of the literature.

Background  Treatment of bullous pemphigoid (BP) with systemic immunosuppressive agents, in particular with systemic corticosteroids, has many long‐term side‐effects. A dozen reports were published regarding the efficacy of topical corticosteroids in the treatment of bullous pemphigoid.

Harlequin syndrome after thyroidectomy for compressive retrosternal goiter. Case report and review of the literature.

Abstract A 74-year-old woman with known euthyroid multinodular retrosternal goiter necessitated an urgent intubation at home, due to acute respiratory distress evoked by tracheal compression. Extubation after a few days failed, and she underwent an urgent total thyroidectomy. During postoperative extubation the patient developed suddenly unilateral facial flushing and sweating at the left side, without ptosis of the left levator palpebrae superioris. These symptoms persisted during the next 24 hours. The skin at the right side of the face remained uninvolved. In the early postoperative period this appearance recurred at moments of emotions, exercise or heat. Beside this, the patient had a normal recovery. Six weeks later this reaction couldn’t be provoked anymore. ‘Harlequin’ syndrome (unilateral facial flushing and sweating) is caused by a lesion of the contralateral sympathetic chain at the levels T2 and T3. It is unknown if the sweating and vasodilation at the “healthy” side is normal or if it is a reaction of hyperactivity.

A multileveled approach in psoriasis assessment and follow-up: A proposal for a tailored guide for the dermatological practice

Abstract Background: Psoriasis is a complex and heterogeneous disease resulting from interactions between genetic, immunological, and environmental factors. To make the most optimal treatment decision, the dermatologist must therefore have a detailed overview of the patient’s history and lifestyle. Objectives: We sought to offer an overview of the various relevant aspects in clinical dermatological assessment of psoriasis patients, emphasizing the importance of a multidisciplinary and integrated clinical approach. Methods: We gathered information on psoriasis management and developed a tailored checklist covering all health-related aspects associated with psoriasis. Results: Demographics, personal and family history were elaborately described as well as drug history to discuss how they affect psoriasis management. Relevant patient information such as the vaccination status or cardiovascular profile were included in the checklist as well and treatment recommendations were adapted and updated in accordance with evidence-based literature. This checklist also emphasizes the importance of drug surveillance, proper follow-up and specialist referral, and why the dermatologist needs to address these health-related aspects when assessing psoriasis patients, going beyond optimal skin care. Conclusions: Our comprehensive overview can be used as a consultation checklist for good clinical practice in psoriasis patient management and aid in treatment decision.