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A case of Chediak-Higashi syndrome is reported in a four-year-old boy who presented with recurrent chest infection, partial albinism, hyperpigmentation of the extremities and presence of giant granules in leucocytes and melanocytes in the skin. Parental consanguinity was present. Though uncommon, hyperpigmentation of sun exposed areas may be the initial symptom in Chediak-Higashi syndrome.
A patient with sarcoidosis was treated with thalidomide for disfiguring and painful steroid unresponsive sarcoidal granulomas of the skin. The duration of the therapy was 14 months, during which time, the skin lesions resolved almost completely. The initial dosage was 200 mg a day, which was increased to 400 mg a day after 4 months. Episodic paresthesia of the finger tips and one lower extremity was the only side effect noted, which resolved promptly after discontinuation of the drug. The dramatic response of sarcoidal granulomas of the skin to thalidomide observed in this patient demonstrates the usefulness of this drug as a possible long-term monotherapeutic or steroid-sparing agent in the treatment of sarcoidosis.
In the recent years, the use of motion tracking systems for acquisition of functional biomechanical gait data, has received increasing interest due to the richness and accuracy of the measured kinematic information. However, costs frequently restrict the number of subjects employed, and this makes the dimensionality of the collected data far higher than the available samples. This paper applies discriminant analysis algorithms to the classification of patients with different types of foot lesions, in order to establish an association between foot motion and lesion formation. With primary attention to small sample size situations, we compare different types of Bayesian classifiers and evaluate their performance with various dimensionality reduction techniques for feature extraction, as well as search methods for selection of raw kinematic variables. Finally, we propose a novel integrated method which fine-tunes the classifier parameters and selects the most relevant kinematic variables simultaneously. Performance comparisons are using robust resampling techniques such as Bootstrap 632+ and k-fold cross-validation. Results from experimentations with lesion subjects suffering from pathological plantar hyperkeratosis, show that the proposed method can lead to approximately 96% correct classification rates with less than 10% of the original features.
Based on the opinion that 5-aminolevulinic acid-based photodynamic therapy (ALA-PDT) may be disadvantageous to Oriental patients with dark skin, including Japanese, because the competing chromophore melanin inhibits the photochemical reaction, we assessed the therapeutic effects when Japanese AK were treated with ALA-PDT. Fifty-three lesions in ten patients were followed up for over one year. The cure rates of AK on the face including the neck and on extremities were 81.8% and 55.6%, respectively. The results were approximately the same cure rates as those reported for AK in Caucasian patients. However, a larger number of treatment sessions were required. In the red light range, incoherent light including 630 nm and 670 nm is superior to 630 nm laser light.
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Most leg ulcers are of venous or arterial origin (85%). Advanced chronic venous insufficiency is the most common underlying condition (65%), followed by advanced peripheral arterial occlusive disease (10%), and combined chronic venous insufficiency and peripheral arterial occlusive disease (10%). Chronic ulcers in diabetic feet (5%) are of great socio-economic importance, as well. They are a consequence of diabetic polyneuropathy which in part of the patients may be combined with peripheral arterial occlusive disease, usually of the calf arteries. However, a leg ulcer can also be caused by a large array of other underlying conditions, such as ulcerating skin tumours, trauma followed by disturbed wound healing, infectious ulcerations, ulcerations in angiodysplasias, vasculitic ulcerations, pyoderma gangrenosum, cholesterol-embolism, idiopathic livedo reticularis with ulceration, primary and secondary antiphospholipid-antibody-syndrome, coumarin-necrosis, calciphylaxis in chronic renal insufficiency, necrobiosis lipoidica, different forms of panniculitis, hematologic disorders, autoimmun diseases and autoimmun-bullous dermatoses. The following article discusses the differential diagnosis, examination and treatment of leg ulcers in these less common underlying conditions.
UNLABELLED: Pyoderma gangrenosum (PG) is a rare, poorly understood skin disease that occurs in all age groups. Less than 0.4% of patients are infants and represent a diagnostic challenge as early lesions may resemble other skin disorders. Here we report for the first time three siblings affected with PG all presenting during infancy. Unlike the older age group, the ulcers spared the legs but involved the buttocks, thighs and perianal area in all the infants. CONCLUSION: This is the first reported family with PG affecting three siblings suggesting autosomal recessive inheritance. The diagnosis may be more difficult in infants due to absence of underlying associated disorders and the tendency of the lesions to appear in areas where infants frequently have other dermatoses. PG characteristically involves the buttocks, thighs and perianal area and spares the legs.
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Sweet's syndrome (acute febrile neutrophilic dermatosis) and erythema nodosum are reactive dermatoses whose pathogenesis is currently undefined. Although there are several associated conditions that both of these disorders have in common, the appearance of Sweet's syndrome and erythema nodosum in the same person is rare. Only 8 individuals with biopsy confirmed lesions of both dermatoses were described in the world literature. We describe a 30-year-old woman with biopsy confirmed lesions of Sweet's syndrome on her hand and erythema nodosum on her legs. She had been taking an oral contraceptive and an appetite suppressant during the 5 months before these dermatoses appeared. Both cutaneous lesions rapidly responded to topical corticosteroid ointment and have not recurred since her medications were discontinued. The association of Sweet's syndrome and erythema nodosum is reviewed, their similarities discussed, and a pathogenesis is proposed.
Cryoglobulins are serum immunoglobulins that precipitate reversibly when the serum is cooled below 371 degrees C. In cryoglobulinemic syndrome, a multitude of organs can be involved, but many patients suffer from a moderate, chronic disease consisting of palpable purpura, arthralgia and fatigue. Skin involvement is especially useful in aiding early recognition of this entity. We present four patients with cryoglobulinemic syndrome and skin lesions, in whom it was not possible to find an etiological or associated factor. We discuss the etiopathogenesis, clinical and pathological findings, treatment and prognosis of this entity.
Idiopathic eruptive macular pigmentation (IEMP) is a rare disease. IEMP is characterized by asymptomatic, pigmented macules involving the neck, trunk, and proximal extremities. This study describes 10 cases of idiopathic eruptive macular pigmentation seen during a 9-year period at the Asan Medical Center, Seoul, Korea. We present these characteristic consecutive cases to provide more insight into the clinical picture and course of IEMP. Skin lesions of 8 patients were multiple brown macules involving the trunk, face, neck, and extremities. In 2 patients, multiple dark brown macules and patches were noted. The age of onset varied from 1 to 20 years. Tentative diagnoses were usually ashy dermatosis (erythema dyschromicum perstans), fixed drug eruption, or mastocytosis. The history of any erythema and drug medication was absent. Darier's sign was absent. Skin biopsy specimens showed increased pigmentation of the basal layer in an otherwise normal epidermis. Pigmentary incontinence, melanophages, and mild perivascular lymphohistiocytic infiltrate in the papillary dermis were also revealed. Mast cells could not be found. The lesions gradually disappeared during a period of several months to years. The alleged rarity of IEMP may be partially caused by medical unfamiliarity with this entity, despite its clinical and histopathologic characteristic picture. Treatment of IEMP is unnecessary because spontaneous resolution of the lesions can be expected within several months to a few years.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.