Susana Machado

Glucagonoma syndrome and necrolytic migratory erythema

The glucagonoma syndrome is a rare disorder, characterized by necrolytic migratory erythema, elevated serum glucagon levels, abnormal glucose tolerance, weight loss, and anemia in association with a glucagon‐secreting alpha‐cell tumor of the pancreas. We present a 67‐year‐old diabetic patient with extensive cutaneous lesions, weight loss, and poor glycemic control. The clinical investigation revealed a pancreatic glucagonoma with resolution of the cutaneous and systemic features after surgical removal. The dermatologic and endocrine approach to this syndrome is discussed here. Early recognition and treatment may prevent metastatic disease and ensure its cure with resolution of the cutaneous and catabolic manifestations.

Cardiovascular comorbidities in childhood psoriasis

BackgroundPsoriasis is a common, chronic, systemic inflammatory skin disease associated with numerous cardiovascular comorbidities. Much evidence of this association exists in the adult population, data available in childhood psoriasis is more limited.ObjectivesTo analyze the prevalence of excess adiposity, cardiovascular risk factors, metabolic syndrome and lipid profile in children with psoriasis comparing to control group with similar age and sex distribution.Materials & methodsA case-control study was conducted with children, 5–15 year-sold, with moderate-to-severe plaque-type psoriasis and a control group comprising children with other skin diseases without systemic inflammatory diseases.ResultsPsoriatic children had a significantly higher prevalence and greater odds of excess adiposity compared to controls: BMI (≥85th percentile; OR 4.4; 95%CI 1.2–15.6), waist circumference (>75th percentile; OR 7.4; 95%CI 2.0–27.7) and waist-to-height ratio (>0.490; OR 4.6; 95%CI 1.3–17.0). A higher prevalence of metabolic syndrome was observed in children with psoriasis compared to controls (25% vs 3.7%;P=0.07), and two components of the metabolic syndrome were significantly higher in the psoriasis group: waist circumference (75% vs 29.6%; P = 0.002) and the high blood pressure component (30% vs 3.7%P=0.032). Finally, an altered and more atherogenic lipid profile was observed among psoriatic patients without excess adiposity.ConclusionThis study demonstrates that comorbidities known to be associated with adult psoriasis are also observed in childhood psoriasis, reinforcing the need for screening cardiovascular comorbidities in children with psoriasis and promoting healthy lifestyle choices in these patients. Moreover, it also suggests that its association with psoriasis may be in part genetically determined rather than uniquely acquired.

Woolly hair generalizado: caso clínico e revisão da literatura

The term woolly hair, or tightly curled hair, refers to a structural anomaly of scalp hair, which can be partial or generalized. Generalized woolly hair variants are rare and can occur in isolation or in association with other cutaneous and extra-cutaneous abnormalities. Thus, it is important to dismiss in these patients the diagnosis of syndromes such as the Noonan syndrome and Cardiofaciocutaneous syndrome, which have high morbidity and mortality rates. We report the clinical case of a 7 year-old boy with generalized woolly hair and keratosis pilaris. After an evaluation of organs and systems, no alteration or characteristics associated with these syndromes were found.

Cutaneous Crohn's disease.

A 42-year-old woman with Crohn’s disease presented with a 2-month history of painful nodules (Fig. 1a) and plaques (Fig. 1b) on the abdominal wall and dorsum of the left foot. The intestinal illness was quiescent. The patient had been treated with mesalazin (3 g/d) for the past 7 years with good control of the gastrointestinal disease. Physical examination revealed erythemato-violaceous nodules and plaques, with peripherical desquamation, some of them with central crusting. There was no fever nor adenomegaly. A complete blood cell count, chemistry profile, serologic studies and skin biopsy specimens were obtained, with bacterial, fungal and mycobacterial cultures of tissue samples. Light microscopic examination of the biopsy specimen revealed an infiltrate of peri-vascular lymphocytes and histiocytes in the papilar dermis. Medium-sized noncaseating epithelioid granulomas composed of numerous foreign bodies and Langhan’s giant cells were observed in the reticular dermis (Fig. 2a,b). The epidermis and cellular subcutaneous tissues were unremarkable. Acid-fast and Ziehl-Neelsen stains were negative for Mycobacterium tuberculosis and atypical mycobacteria, respectively, as well as for periodic acid-Schiff and Gram stains. Serum chemistry studies were normal, including calcium levels and angiotensin-conversion enzyme. Serologic testing revealed negativity for VDRL, TPHA, HIV and B and C viral hepatitis. Prednisone therapy (40 mg/d) was initiated and tapered over 4 months, with complete resolution of the lesions (Fig. 3). At that time the patient experienced an intestinal flare, without new cutaneous lesions. Prednisone dosage was increased and the patient initiated azathioprine (150 mg/d) and metronidazole (800 mg/d), with complete resolution of the bowel disease in 2 months. There was no recurrence of skin lesions after 36 months of follow up.

Frontal fibrosing alopecia: a review of eleven patients

BACKGROUND Frontal fibrosing alopecia (FFA) is a primary lymphocytic cicatricial alopecia with a distinctive clinical pattern of progressive frontotemporal hairline recession. OBJECTIVES Our purpose was to describe the clinical and histopathological features as well as the response to treatment of eleven cases of FFA diagnosed at the Trichology Consultation, over three years. METHODS A retrospective case note review was performed of eleven adult patients with FFA. The clinical data as well the histopathologic findings and laboratory tests were accessed. The patients were treated with different drugs, depending on the stage of the disease. The age of onset of the alopecia ranged from 45 to 80 years. Ten patients (90.9%) were postmenopausal women. All patients had progressive and symmetrical alopecia localized to the frontal and temporal hairline. Seven patients (63.6%) had marked decreased to complete loss of eyebrows and in four patients axillar alopecia was also evident. Laboratory investigations were normal. Scalp biopsy specimens from the anterior hairline showed similar findings. No significant improvement was observed in the majority of cases. CONCLUSION Differential diagnosis should take into account several other conditions. It seems there is no effective treatment proven with an appropriate level of evidence in the management of FFA.

Severe contact allergy to footwear in a young child

A 5-year-old female patient, with plantar juvenil dermatitis and family and personal history of atopic dermatitis, presented with bilateral and symmetrical diffuse erythema, vesicles, bullae and exulcerations on the dorsum of her feet and toes as well as on the plantar surfaces, with severe itching (Figs 1 and 2). The lesions evolved for 1 year and aggravated whenever she wore shoes, especially in summer. The severity of the clinical picture kept her away from school causing a significant impact in her psychosocial development. Patch tests with standard series of Portuguese Contact Dermatitis Group (GPEDC) and shoe series (Chemotechnique) resulted in the reactions summarized in Table 1. We advised the parents to use chromium-free tanned leather footwear over 2 pair of cotton socks. We also stressed the need of having 2 pairs of shoes to alternate day-byday, allowing them to dry. Although not sensitized, the use of rubber shoes was not recommended. Occlusion and moist environment would worsen juvenile plantar dermatitis and could promote new sensitizations. The use of topics containing Balsam of Peru was forbidden. With these strategies and topical corticosteroids, the lesions improved gradually and disappeared in 3 weeks with no recurrence in a 6-month follow-up.

Bart syndrome: Correspondence

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PHACE Syndrome: a new case report

ejd.2011.1262 Auteur(s) : Aristoteles ROSMANINHO1 arisrosmaninho@gmail.com, Susana MACHADO1, Antonio J. BASTOS-LEITE2,3, Manuela SELORES1 1 Department of Dermatology, Centro Hospitalar do Porto-Hospital Santo Antonio, Ex-CICAP, Rua D. Manuel II, 4099-001 Porto, Portugal 2 Department of Medical Imaging, Faculty of Medicine, University of Oporto, Oporto, Portugal 3 Lysholm Department of Neuroradiology, The National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom A [...]

Serpiginous lesion on the foot

Cutaneous larva migrans is a parasitic infection caused by percutaneous penetration and subsequent migration of the larvae of hookworm. The tracks are commonly raised, erythematous, serpiginous, and pruritic. Cutaneous larva migrans is diagnosed based on its clinical characteristics. It is a self-limiting condition because larvae eventually die in humans without being able to infest new hosts. Treatment is used to shorten the disease course, control the intense pruritus, and prevent the risk of secondary infection. Topical thiabendazole is the treatment of choice for mild and localized condition. Systemic treatment such as albendazole, mebendazole, and ivermectin are used in widespread cases or cases recalcitrant to topical treatment.

Refractory leg ulcer successfully treated with hyperbaric oxygen therapy

ejd.2011.1354 Auteur(s) : Aristoteles Rosmaninho arisrosmaninho@gmail.com, Ines Lobo, Susana Machado, Manuela Selores Department of Dermatology, Centro Hospitalar do Porto-HSA, Rua D. Manuel II, 4099-001 Porto, Portugal A 30 year-old woman presented with a one year history of an enlarging leg ulcer that failed to heal despite local wound care and several courses of antibiotics. Her medical history was only remarkable for a juvenile rheumatoid arthritis (RA). She had had no systemic therapy for [...]