Multisystem Langerhans Cell Histiocytosis in Adults Revealed by Skin Lesions.

A 37-year-old woman with no remarkable medical or family history presented with papules and vesicles on an erythematous background involving the neck, sacrum, and folds (postauricular, axillary, inguinal, and under the breasts) (Figure 1). During the previous year, she was treated with local and systemic antifungals without improvement. Her history included a secondary amenorrhea, polydipsia, and polyuria (6 L/d) that started 2 years prior. Physical examination revealed chronic bilateral purulent otorrhea with thick eardrums. Histologic examination of skin biopsy revealed a highly suggestive appearance of multisystem Langerhans cell histiocytosis (LCH) with immunohistochemistry (anti-PS100 and anti-CD1a), which were positive (Figure 2A and 2B). Pituitary magnetic resonance imaging showed a thickening of the pituitary stalk in relation to a location histiocytic (Figure 3). Bone gaps were objectified on two radiographic tibial diaphyseal. Results from computed tomography (CT) scan showed a magma coelio mesenteric, axillary, and inguinal lymph nodes.

[Cutaneous manifestations in hemodialysis patients]. / Manifestations cutanées chez les hémodialysés chroniques.

BACKGROUND: Hemodialysis patients have frequent and various cutaneous manifestations of often hypothetical pathogenesis. Chronic renal failure (CRF) presents with an array of cutaneous manifestations. The objective was to evaluate the prevalence and nature of cutaneous lesions associated with CRF patients on hemodialysis patients. PATIENTS AND METHODS: Transversal and observational study of 53 patients with CRF on regular hemodialysis. RESULTS: There were 28 women and 25 men. Their mean age was 44 year-old. All patients had cutaneous manifestations and 64% complained of dermatological signs. Cutaneous xerosis and pigmentation disorders were found in 96 and 94% of patients, respectively. Other manifestations were pallor (41%), pruritus (20.7%), a diffuse hair loss (35.8%) and nails changes (66%). COMMENTS: Cutaneous manifestations in hemodialysis patients are frequent polymorphous. Their incidence varies from 50 to 100% of the series. Their knowledge deserves a proper management by both dermatologists and nephrologists to improve the life quality of hemodialysis patients.

Recurrent infantile myofibromatosis in a 19-month-old boy presenting as ulcerated plaque.

A 19-month-old boy presented to our department with ulcerated plaque on the right lumbar region. The lesion was known to have been growing for about 5 months. At another center 9 months prior to presenting to our department, the patient's parents reported a history of cutaneous nodules from the same lumbar region. Their total excision was performed, and the pathology report stated that it was infantile calcifying fibromatosis or nodular fasciitis. The patient was healthy with a nonsignificant, medical history including no use of medications, no known drug allergies, and no significant family history of disease.

Curious vascular tumor.

Introduction. Sinusoidal hemangioma is a rare variant of acquired cavernous hemangioma predominantly occurring in females. Very few case reports have been described in the literature. Case Report. We present a case of a 46-year-old woman who noticed a slowly growing, cutaneous nodule on the left breast. Local excision of the lesion was performed and histology allowed to find a sinusoidal hemangioma. No recurrence was noticed. Conclusion. The very few reports of such a lesion in the literature reflect either rarity of such lesions or unfamiliarity of this subset among the pathologists.

[Severe hypertriglyceridemia and macrophage activation syndrome: a case report]. / Hypertriglycéridémie majeure et syndrome d'activation macrophagique.

We report the case of a 36-year-old patient admitted for Macrophage Activation Syndrome (MAS), revealed by inflammatory dermo-hypodermal lesions, tumor syndrome, pancytopenia, biological cholestasis, increased lactate dehydrogenase rates and hemophagocytosis in skin biopsies. The patient also exhibited an exponential increase in triglycerides serum levels that reached 22 g/L. The patient died after multiple organ failure. This case illustrates the value of the hypertriglyceridemia in the diagnosis and monitoring of hemophagocytic syndrome but also highlights the difficulty in understanding its pathophysiology especially with several elements that contribute to its aggravation during the evolution of this syndrome.

Harlequin ichthyosis.

A 2-hour-old newborn boy hospitalized in the neonatal intensive care unit was examined for unusual cutaneous lesions. He had firm plaques covering his body, with fissures especially in flexural areas. Other remarkable findings included edematous hands and feet, ectropion, eclabium, and contractures (Figure). Topical emollients and etretinate were advised, but the newborn died a few hours later. The parents were first-degree relatives. There was no family history of similar lesions. On the basis of clinical features, the diagnosis of harlequin ichthyosis was made.

Granulosis rubra nasi.

An 18-year-old man presented with erythematous lesions over the nose of 1 year's duration without sensitivity to sunlight. He was otherwise well and there was no relevant family history. Physical examination showed excessive sweating of the nose, cheeks, and upper lip, and erythema of the nose covered by beads of sweat and vesicles (Fig. 1). The rest of the physical examination was normal. The skin biopsy showed a discrete mononuclear cell perivascular infiltrate.

Cutaneous tuberculosis in Morocco.

OBJECTIVE: To describe the salient epidemiologic, clinical, histopathologic, and bacteriologic aspects of cutaneous tuberculosis in Morocco. METHODS: A retrospective review of all cases of cutaneous tuberculosis at our hospital from January 1981 through December 2004. RESULTS: Two-hundred and sixteen cases of cutaneous tuberculosis were identified and included. Men and women were equally affected. The mean patient age was 29 years. Major clinical types of cutaneous tuberculosis were scrofuloderma and gumma (72%), lupus vulgaris (12%), tuberculosis verrucosa cutis (7%), tuberculids (6%), orificial tuberculosis (1%), and tuberculous chancre (1%). Systemic involvement was seen in 35%. Where performed (66%), 81% of subjects had positive Mantoux skin tests. Lesion biopsy for histopathologic study was performed in 81% of patients and showed classical tuberculous findings in 57%. Mycobacterium tuberculosis was isolated in culture from 9% of patients. CONCLUSION: Cutaneous tuberculosis is still a common disease in Morocco, and scrofuloderma and gumma are the most common clinical presentations.

[Urachal sinus. Report of 3 cases]. / Le sinus de l'ouraque: à propos de 3 cas.

Urachal sinus is a congenital anomaly secondary to insufficiently high obliteration of the urachus. It can present clinically at any age with purulent umbilical discharge. A combination of ultrasound and fistulography is essential to confirm the diagnosis. There is a risk of adenocarcinomatous degeneration. Treatment consists of resection of the sinus and its implantation base on the bladder. Excision of the umbilicus is not mandatory in children. The authors report a retrospective analysis of 3 cases of urachal sinus in 3 girls with a mean age of 12 years, presenting a 3-month history of purulent umbilical discharge before admission. An umbilical nodule was detected in 2 patients and an infraumbilical spindle-shaped mass was observed in the third patient. Ultrasound showed an infraumbilical echogenic mass and fistulography, performed in 2 patients, showed a blind cavity directed inferiorly, confirming.the urachal sinus. Treatment consisted of complete resection of the sinus in one patient and complete resection of the sinus and its implantation base on the bladder in the other 2 patients. An associated teratoma of the right ovary was found in one patient. The postoperative course was uneventful. Umbilicoplasty, performed in 2 patients with resection of the umbilicus, was satisfactory. No recurrence was observed with a mean follow-up of 12 months. Histological examination did not reveal any signs of malignancy. Purulent umbilical discharge may indicate the presence or urachal sinus, which can be confirmed by a combination of ultrasound and fistulography. Treatment consists of resection of the sinus and its implantation base on the bladder. The objective of this study was to define the diagnostic and therapeutic features of urachal sinus.