Recurrent Henoch-Schönlein Purpura with bullous rash and pulmonary nodules.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It has a characteristic rash described as palpable purpura that most frequently affects the distal lower extremities and buttocks. HSP rarely presents with bullous rash nor pulmonary nodules. CASE PRESENTATION: We present a novel case of a 12-years-old female with recurrent pediatric HSP with a combination of the rare manifestations of bullous rash and pulmonary nodules. She initially presented with the bullous rash, chest pain, cough, and abdominal pain. Patient was successfully treated with intravenous pulse corticosteroids followed by a high dose oral corticosteroid taper, with resolution of the bullous rash and pulmonary nodules. CONCLUSION: The rare manifestations of scarring bullous rash and pulmonary nodules can be presenting features of pediatric HSP, the combination of which has not been previously reported. The treatment of intravenous corticosteroid resolved patient's abdominal symptoms, rash and pulmonary nodules.

Adult-onset lichen striatus versus adult blaschkitis: a clinicopathological review of 40 cases of acquired blaschkolinear inflammatory dermatosis.

BACKGROUND: Since the first description of adult blaschkitis (AB), the existence of this entity has been a matter of great debate. OBJECTIVES: To compare clinicopathological features of lichen striatus (LS) and AB cases. MATERIALS AND METHODS: We retrospectively reviewed the clinicopathological features of patients who clinically showed linear inflammatory dermatosis along Blaschko's lines based on a skin biopsy registry. RESULTS: Through a process of clinicopathological differential diagnosis, 27 cases of LS, three of AB, eight of linear lichen planus, and two of linear psoriasis were identified. Clinicopathological differences between LS and AB were mostly insignificant except for age at onset and multiple site involvement. In these cases, females were affected more frequently than males. The mean age at onset was 31.6 years, and the most common involved site was the leg. The lesions lasted approximately 8.3 months with few relapses. The most common histopathological finding was perivascular infiltration followed by peri-appendageal infiltration. CONCLUSION: Distinction between LS and AB appears to be unnecessary given their overlapping features.

Pretibial myxedema without ophthalmopathy: an initial presentation of Graves' disease.

OBJECTIVE: To report a rare case of Graves' disease without ophthalmopathy presenting with pretibial myxedema (PM) as an initial presentation. METHODS: We present the clinical history, physical findings, laboratory studies and biopsy data of a 62-year-old man with a history of uncontrolled type 2 diabetes (DM2) presenting with arm and leg skin lesions in the absence of other physical findings. Histopathology confirmed PM. Graves' disease and its association with PM without Graves' ophthalmopathy and the pertinent literature are reviewed. RESULTS: A 60-year-old man with a history of uncontrolled DM2 presented for glycemic management. He described symptoms of anxiety, insomnia and fatigue for the last 5 to 6 months. He described diffuse chest pain, occasionally associated with palpitations, and a 50-pound weight loss. He also complained of severe itching and burning of his arms and legs for the past several months. Subsequent thyroid studies revealed hyperthyroidism suggestive of Graves' disease. In the interim, he was hospitalized for atrial flutter and was cardioverted. After being started on methimazole, his symptoms abated. His skin lesions were biopsied, and the leg biopsy was consistent with PM. He however had no lid lag or proptosis characteristic of Graves' disease. He subsequently underwent radioiodine ablation. His hyperglycemia was better control led after treatment of his hyperthyroidism. CONCLUSIONS: PM is an autoimmune manifestation of Graves' disease. Almost all cases of thyroid dermopathy are associated with relatively severe ophthalmopathy. Usually ophthalmopathy appears first and dermopathy much later. However, this case represents a rare initial presentation of Graves' disease with PM without ophthalmologic symptoms or findings. Hyperthyroidism is typically associated with worsening glycemic control and increased insulin requirements. In patients with diabetes having hyperthyroidism, deterioration in glycemic control should be anticipated and treatment should be adjusted accordingly. Restoration of euthyroidism will lower the blood glucose level.

Treatment of pretibial myxedema with dexamethazone injected subcutaneously by mesotherapy needles.

Pretibial myxedema (PTM) is a rare extrathyroidal manifestation of Graves' disease that requires treatment when the clinical picture is markedly evident. In addition to topical treatment with steroid ointments, there have been previous reports of subcutaneous injections of steroids. This procedure may cause nodular degeneration of the skin due to fat atrophy when standard needles are used. In the present study, we have tried a novel modality of treatment of PTM by injecting a solution of dexamethasone in the subcutaneous tissue using needles employed for mesotherapy. These needles are ≤4 mm long and deliver the medication within the dermis or the first layer of the subcutaneous fat. We have treated five patients, four with diffuse and one with elephanthiasic PTM. We utilized multiple injections of a solution of dexamethasone, lidocaine, and saline in the PTM plaque and in the pretibial area, both in the PTM plaque and in the area surrounding the lesions, once a week for three consecutive weeks. Two patients with a more severe form of PTM underwent another two cycles four to six weeks after initial treatment. Patients were studied before and after treatment by clinical assessment and ultrasound of the pretibial skin. The treatment was well-tolerated, with only moderate pain upon injection of the solution. One month after treatment, all patients showed improvement of PTM at clinical assessment and a reduction of the thickness of the lesions at ultrasound of ∼15%, involving mostly the dermis. Moreover, all patients reported amelioration of the leg appearance. The present study, although preliminary, shows that intralesion steroid injection with mesotherapy needles in PTM is effective and well tolerated, and does not cause undesired long-term modifications of the skin. More studies are warranted to standardize such treatment in larger groups of patients.

Recurrent Kawasaki disease with strawberry tongue and skin desquamation in a young adult.

A 19-year-old man with history of Kawasaki disease (KD) at age 12 developed intractable fevers, swelling in hands and feet, arthralgias, and conjunctivitis, followed by strawberry tongue and desquamation of distal extremities. Laboratory studies revealed leukocytosis, thrombocytosis, anemia, elevated erythrocyte sedimentation rate and C-reactive protein levels, and mildly elevated liver enzymes. He was empirically treated with broad-spectrum antibiotics without any improvement. Extensive infectious and rheumatologic workup remained negative. Recurrence of KD was diagnosed based on characteristic mucocutaneous changes and systemic inflammatory response. His symptoms and laboratory values responded rapidly to intravenous immunoglobulin and aspirin therapy. An echocardiogram did not show any coronary abnormality. We report the third case of pediatric KD relapsing in adulthood. Similar to childhood and adult KD, these recurrent episodes respond well to intravenous immunoglobulin therapy. Although recurrent KD is rare, our case highlights the importance of considering it in the differential of febrile illness in the appropriate individual.

Pretibial myxedema is associated with polymorphism in exon 1 of CTLA-4 gene in patients with Graves' ophthalmopathy.

Cytotoxic T-lymphocyte associated antigen-4 (CTLA-4) is a well-known molecule that regulates T cell activity, with polymorphisms at different regions of this gene having been associated with autoimmune conditions. Pretibial myxedema (PTM), also called Graves' dermopathy, is an autoimmune extrathyroidal manifestation of Graves' disease. We opted to investigate the relationship between three single nucleotide polymorphisms of the CTLA-4 gene (+49A/G, and -318C/T and -1147C/T) and PTM in Iranian patients with Graves' ophthalmopathy (GO). A total of 105 unrelated Iranian patients with GO from the outpatient endocrine clinic of a large university general hospital as well as 103 healthy controls were studied. The genomic DNA was extracted from venous blood samples by a salting out method, and the polymorphisms at +49, -318 and -1147 positions of the CTLA-4 gene were determined using the polymerase chain reaction-restriction fragment length polymorphism method. The GG genotype (OR = 6.000, 95% CI = 1.805-19.940, P = 0.005) and the G allele (OR = 2.653, 95% CI = 1.314-5.357, P = 0.009) at position +49 were significantly associated with PTM in the patient group. The same genotype and allele were also significantly more common among patients (with or without PTM) than controls. No significant association was found for the other two polymorphisms. In conclusion, the +49G allele is associated with increased risk of PTM in patients with GO. Studies with larger sample sizes are needed to confirm the results of the present study.

Electrical measurement of moisturizing effect on skin hydration and barrier function in psoriasis patients.

Transepidermal water loss (TEWL) in psoriatic skin lesions seems to be related to the severity of the psoriasis, and the electrical capacitance and conductance of the skin are indicators of the hydration level of the stratum corneum. We compared the characteristics of these electrical measurements, in assessing the persistent effect of a moisturizing cream on skin hydration and barrier function in psoriasis patients. Seventeen Korean psoriasis patients were recruited. Their right leg was treated with the moisturizer twice daily for 6 weeks, while their left leg was used as the control site. For each patient, one psoriatic plaque on each leg was selected as the involved psoriatic lesion. Uninvolved psoriatic skin was regarded as the apparently healthy looking skin 4-5 cm away from the periphery of the psoriatic lesion. The TEWL, electrical capacitance and conductance were measured, in order to evaluate the barrier function and hydration level of the stratum corneum. The clinical and biophysical data for each patient were recorded at the start of the study and after 2, 4 and 6 weeks. The degree of skin dryness at the applied area improved progressively. The electrical capacitance at the treated psoriatic lesion increased significantly after 2 weeks, and this improvement was maintained during the entire study period. However, no noticeable change was observed in the electrical conductance. The TEWL showed an inverse pattern to that of the skin capacitance, decreasing during the study period. The skin capacitance and TEWL exhibited good correlation with the visual assessment of skin dryness, but the skin conductance did not. Our data suggest that electrical capacitance and TEWL may be useful in the evaluation of the effect of a moisturizer on the hydration status and barrier function of psoriatic skin.

A case of non-selective phagocytosis of hemosiderin and melanin of dermal histiocytes in stasis dermatitis.

A case study was undertaken to determine whether or not the same dermal histiocytes could phagocytose both melanin and hemosiderin simultaneously. A biopsy specimen was taken from a pigmented lesion of the lower leg of a 57-year-old woman with stasis dermatitis. The specimen was processed for histology, conventional transmission electron microscopy and electron-probe X-ray microanalysis. Histologically, numerous histiocytes with their cytoplasm packed with either Prussian blue-positive granules or Fontana-Masson-positive granules were distributed almost equally in the dermis. Electron microscopically, the dermis had many histiocytes with their cytoplasm containing solitary or compound electron-dense substances. The electron-dense substances were classified into three types according to their degree of electron density. By electron-probe X-ray microanalysis, these electron-dense substances were classified into iron-containing and non-iron-containing substances. Both substances were seen in the cytoplasm of the same histiocytes and even in the same compound electron-dense substance. The former were siderosomes and the latter were probably melanosomes. These results show that the same dermal histiocytes probably phagocytose non-selectively both hemosiderin and melanin granules.

[Erysipelas. A retrospective series of 92 patients in a department of internal medicine]. / L'érysipèle. Une série rétrospective de 92 patients dans un service de Médecine Interne.

Erysipleas, also known as Saint Anthony's fire, is an acute infection of the skin caused, in most of cases, by group A streptococci. In the past, the most common site of involvement was the face and, in the pre-antibiotic era, mortality was high. In this retrospective study, we highlight the clinical and bacteriological features and report follow-up in 92 patients hospitalized in an internal medicine unit between 1st March 1992 and 31st December 1996 for 94 episodes of erysipelas. The involvement of the lower limbs predominated as involvement of the face is becoming very rare. Streptococci from others groups and Staphylococcus aureus have been implicated on occasions. Recovery is usual even if this infection may greatly weaken these often fragilized patients. In this paper, antibiotic treatment as well as the place of anticoagulants and Doppler ultrasound are discussed. Hospitalization is often necessary but it must not be systematic.

T-cell restriction in thyroid eye disease.

Infiltrating immunocompetent cells act to establish and perpetuate the orbital autoimmune process in thyroid eye disease (TED). Until recently, it has remained unclear whether T cells infiltrating the orbital connective/fatty tissue and extraocular muscles represent a primary immune response that is specifically directed against orbital antigens. In addition, despite a close clinical and temporal association of the thyroidal and extrathyroidal manifestations in Graves' disease (GD), it has not been proven whether T cells infiltrating thyroid, orbital, and pretibial tissue in patients with TED and pretibial dermopathy (PTD) are directed against certain antigenic determinants shared between these anatomically distinct tissues. Using polymerase chain reaction (PCR)-based molecular analysis of T-cell antigen receptor (TcR) variable (V) region genes, we have demonstrated marked restriction of orbital and pretibial TcR Valpha and Vbeta genes in patients with active TED and PTD. In addition, molecular analysis of T cells in paired samples of extraocular muscle and orbital connective/fatty tissue revealed usage of similar TcR V genes. In contrast, TcR V gene restriction was either absent or much less pronounced in patients with longstanding TED and PTD. Comparison of TcR V genes in T cells obtained from thyroid gland, orbital tissue, pretibial tissue, and peripheral blood of three individual patients with active GD, TED, and PTD also revealed marked restriction and, in addition, striking similarities of TcR V gene usage. Sequencing of complementarity determining regions 3 (CDR3) and junctional domains of TcR Vbeta genes confirmed oligoclonality of intrathyroidal, orbital, and pretibial T cells. Moreover, several conserved junctional motifs were shared by T cells infiltrating the thyroid gland and the extrathyroidal sites. Taken together, these data suggest that, in patients with GD and extrathyroidal manifestations, similar antigenic determinants may be responsible for recruitment and oligoclonal expansion of T cells both within the thyroid gland and in the involved extrathyroidal sites.

Subbandage pressure measurements comparing a long-stretch with a short-stretch compression bandage.

Forty-three patients with venous leg ulcers were randomized into treatment with either a long- or a short-stretch compression bandage. Subbandage pressure was regularly measured during rest and walking for a period of up to 1 year. The long-stretch bandage was kept on as long as possible, often up to 1 week. It maintained a significantly higher subbandage pressure in the upright position and during passive dependency as well as during walking than the short-stretch bandage after both 2 and 24 h. The difference between maximum and minimum subbandage pressures during walking did not differ between the two groups. Thus, in contrast to general opinion, the short-stretch bandage did not produce a higher peak working pressure than the long-stretch bandage. The pressure decreased in the supine position in both groups.

Sporotrichosis: survey and clinical aspects from Botucatu School of Medicine, state of Säo Paulo - Brazil

FUNDAMENTO - Esporotricose é micose subcutânea de maior ocorrência em regiöes subtropicais e temperadas, com características distintas segundo o país ou regiäoestudada. OBJETIVO - Estudar a casuística de esporotricose do Departamento de Dermatologia da Faculdade de Medicina de Botucatú - UNESP, diagnosticada entre 1976 e 1995. MÉTODOS - Realizou-se estudo transversal sendo utilizados dados primários, obtidos segundo protocolo específico, incluindo dados de anamnese, exame clínico-dermatológico e de seguimento dos pacientes. RESULTADOS - Foram observados 53 casos de esporotricose entre 1976-1995 correspondendo a 0,18 porcento dos casos dermatológicos em primeira consulta no mesmo período; 73,6 porcento dos casos foram do sexo masculino, 56,6 porcento acima de quarenta anos e 54,7 porcento trabalhadores rurais. As lesöes específicas foram mais frequentes nos membros superiores (61,3 porcento) e inferiores (21,0 porcento). A forma linfangítica (50,9 porcento) e a cutânea localizada (41,5 porcento) apresentaram incidência similar. Nos casos da forma localizada da doença, as lesöes ulceradas (46,6 porcento), infiltrativas (26,6 porcento) e vegetante ou verucosa (23,0 porcento foram as mais observadas. CONCLUSÖES - Devem ser salientadas neste estudo: a alta freqüência de lesöoes nos membros superiores, a distribuiçäo pós-contato com gato doméstico infectado pelo sporothix schenckii.