Formation of perfused, functional microvascular tubes in vitro.

These guidelines for management of pemphigus vulgaris have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence-based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1365-2133.2003.05665.x

Guidelines for the management of pemphigus vulgaris

Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript.

Pityriasis lichenoides and its subtypes.

Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment.

The histologic diagnosis of early mycosis fungoides (MF) is one of the most vexing problems in dermatopathology. We reviewed the histopathologic features of 745 biopsy specimens from 427 patients (male:female = 277:150; median age, 52 years; range, 3-95 years) with early (patch) lesions of MF collected from the lymphoma database of the Department of Dermatology of the Medical University of Graz (Austria). In all patients, the diagnosis was established by clinicopathologic correlation. The most common histopathologic pattern consisted of a band-like or patchy lichenoid infiltrate admixed with coarse bundles of collagen in the superficial dermis. Epidermotropism of lymphocytes was observed in most cases in one or more forms (single lymphocyte epidermotropism, 22%; basilar lymphocytes, 23%; Pautrier's microabscesses, 19%; "haloed" lymphocytes, 40%; disproportionate exocytosis, 17%; pagetoid epidermotropism, 3%). In 4% of cases, epidermotropism was completely missing. Atypical lymphocytes were present only in 9% of cases. Features of interface dermatitis were observed in 59% of cases. Other unusual findings were the presence of necrotic keratinocytes (23%), melanophages (8%), and extravasated erythrocytes (4%). In 28 patients, two or more biopsies taken on the same day at different body sites showed different histopathologic aspects, underlying the protean features of MF even in a single patient at a given time. Our study expands previous observations on histopathologic features of early lesions of MF. Although sometimes the histopathologic features are not diagnostic, they should be considered consistent with MF and do not rule out the diagnosis.

Histopathologic features of early (patch) lesions of mycosis fungoides: a morphologic study on 745 biopsy specimens from 427 patients.

Intravenous immunoglobulins for pemphigus vulgaris: adjuvant or first choice therapy?

Schnitzler syndrome is a rare condition characterized by chronic non-pruritic urticaria, recurrent fever, bone pain, osteocondensation, and monoclonal IgM gammopathy without features of lymphoproliferative disease. We describe the case of a 44-year-old man with an 8-year history of bone pain with hyperostosis and a 5-year history of chronic non-pruritic urticaria, associated with fever, hyperleukocytosis, hepatomegaly, serum monoclonal IgM-kappa. Systemic treatment with steroids was effective against bone pain but was ineffective in controlling the urticaria. We also review 35 cases. No adequate treatment has yet been found. The pathogenesis is unclear and the role of the IgM component in the induction of urticaria has not been established.

Schnitzler's syndrome: Report of a new case and a review of the literature

A 43-year-old woman was examined in October 1993 for a widespread, large, ulceronecrotic skin eruption with exception of the mucosa (Fig 1) The skin lesions were round and regular, varied in size from 05 to 2 cm, and were partially covered by a brownish-black crust The patient had a fever ranging from 38 to 40 °C, always preceded by shivering



She had presented with small exfoliating erythematous patches and itching, 8 months before being admitted to our institute, and was treated with topical corticosteroids After five and a half months, together with the beginning of fever, the above lesions became wet and were covered with squamous crusts



For 2 years the patient had been affected by perianal fistula, but had not referred to this pathology before She also reported that she had been treated with antibiotic and corticosterod intravenous therapies



A biopsy specimen from a lesion of the right leg showed a widely ulcerous fragment of the skin, characterized by a strong infiltration of lymphocytes, especially in the upper part of the dermis, with neutrophil granulocytes The epidermis surrounding the ulcer showed acanthosis, orthokeratosic hyperkeratosis, and spreading lymphocyte exocytosis, with a large number of necrotic keratinocytes and the presence of melanophages (Figs 2 and 3), All the above data suggested a diagnosis of pityriasis lichenoides et varioliformis acuta (PLEVA)



Direct immunofluorescence carried out on perllesional skin showed the presence of fluorescent bodies on the superficial dermis using antisera anti-immunoglobulin (anti-IgM) and Ciq



Laboratory tests showed the following values: erythrocyte sedimentation rate (ESR), 55 LVh; C-reactive protein, 85 mg/L; white blood cell (WBC), 9200 g/dL; platelets, 476,000; gamma glutamil transferase, 93 U/L; alkaline phosphatase, 185 IU/L; complement C3 fraction, 709 mg/L; CP4/CD8 lymphocyte ratio, 65; total IgE, 2493 ku/L Human immunodeficiency virus (HIV), Epstein-Barr virus (EVB), cytomegalovirus, herpes simplex 1 and 2, toxoplasma, rickettsia, and hepatitis markers A, B, and C gave negative results, while blood cultures were positive for Staphylococcus aureus and Pseudomonas aeruginosa Circulating immune complexes (as C1 binding system), 80 (im/eq/L (normal value, less than 20) Qhest X-ray and total body computed axial tomography (CAT) scan were negative



Antimicrobial therapy with teicoplanin and trimethoprim + sulfamethoxazole for 15 days resulted in a reduction in microbial count in blood cultures, and therapy with prednisone (60 mg/day) for a further 20 days was started The patient was discharged from hospital in reasonable physical condition, without fever and with an improvement of the skin lesions Surgery for the treatment of her perianal fistula was not performed as her general condition had improved- The patient continued therapy at home with 50 mg prednisone and a broad spectrum antimicrobial for a further 20 days One month later, the patient was admitted to hospital in a poor physical condition with fever (peaks of 40/41 °C), preceded by shivering, relapsing skin ulcers, weak respiration, and polypneia The laboratory tests showed a serious hypoproteinemia, C-reactive protein concentration of 321 mg/L, and high transaminase levels Blood analysis was positive for Staphylococcus aureus Therapy was started with 60 mg prednisone, antimicrobials, and albumin, but 24 h later the patient fell into a coma and died from cardiogenic shock Upon her family's request, no autopsy was performed

Febrile ulceronecrotic Mucha‐Habermann's disease with fatal outcome

Fibroblastic rheumatism is a rare syndrome characterized by the association of multiple cutaneous nodules with symmetric polyarthritis. We report on a patient who presented a 4-year history of pink to skin-coloured nodular lesions symmetrically localized at para-articular sites without evident rheumatological symptoms. Histopathology of a skin nodule led to the diagnosis of fibroblastic rheumatism showing a poorly circumscribed dermal proliferation of spindle and stellate fibroblast-like cells embedded in thickened collagen bundles with a marked reduction of elastic fibres. X-rays of both hands and feet showed metacarpophalangeal, metatarsalphalangeal and interphalangeal erosions, unexpected by patient history. This case of fibroblastic rheumatism appears unique in view of the absence of any clinical manifestation of polyarthritis at 7 years from appearance of skin lesions.

Fibroblastic rheumatism: a case without rheumatological symptoms.

Dermatomyositis is an inflammatory myopathy characterized by proximal symmetrical muscle weakness with a characteristic cutaneous eruption. Population-based cohort studies provide evidence of an increased frequency of cancer in dermatomyositis patients. Many signs and serology tests have been suggested as markers for malignancy in dermatomyositis. We performed a case-control study on the patients admitted in our institutions for dermatomyositis. Clinical and laboratory data were collected and statistical analysis was performed to reveal important predictive signs of malignancy in dermatomyositis. We found no statistical difference in the clinical or laboratory parameters between the dermatomyositis patients with or without malignancy, with the exception of the erythrocyte sedimentation rate. In our study group an erythrocyte sedimentation rate higher than 35 mm/hr was very strongly associated with the presence or the development of a malignancy. Given the high positive and negative predictive values observed in our study, erythrocyte sedimentation rate evaluation using this cut-off point could be very useful in alerting dermatologists to the need for more in-depth diagnostic procedures in dermatomyositis patients.

Usefulness of erythrocyte sedimentation rate as tumor marker in cancer associated dermatomyositis

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