Savaş Yayli

Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology

Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or generalized bullous lesions. In up to 20% of affected patients, bullae may be completely absent, and only excoriations, prurigo‐like lesions, eczematous lesions, urticated lesions and/or infiltrated plaques are observed. The disease is significantly associated with neurological disorders. The morbidity of bullous pemphigoid and its impact on quality of life are significant. So far, a limited number of national treatment guidelines have been proposed, but no common European consensus has emerged. Our consensus for the treatment of bullous pemphigoid has been developed under the guidance of the European Dermatology Forum in collaboration with the European Academy of Dermatology and Venereology. It summarizes evidence‐based and expert‐based recommendations.

Pemphigus. S2 Guideline for diagnosis and treatment – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)

Pemphigus encompasses a group of life‐threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, the prognosis of pemphigus was almost fatal. Due to its rarity, only few prospective controlled therapeutic trials are available.

Erythema dyschromicum perstans: Response to dapsone therapy

Erythema dyschromicum perstans (EDP) is a rare disorder characterized by asymptomatic, slowly progressive, ash‐gray macular pigmentation of the skin, which usually occurs from age 5 years through adult life. Most cases reported to date are of Latin American and Indian patients. Rare cases has been reported from Turkey. No treatment of choice is presently available. Various therapies have been tried, including sun protection, chemical peels, antibiotics, corticosteroids, vitamins, isoniazid, griseofulvin, and chloroquine, without any benefit. Some authors have suggested the therapeutic efficacy of clofazimine and dapsone on EDP. We report a case of EDP that responded remarkably well to treatment with dapsone.

Detection of linear IgE deposits in bullous pemphigoid and mucous membrane pemphigoid: a useful clue for diagnosis

Summary Background  Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease of the skin associated with IgG autoantibodies to BP180 and BP230, while mucous membrane pemphigoid (MMP) comprises a heterogeneous group of autoimmune blistering diseases characterized by a predominant mucous membrane involvement and scarring tendency associated with an autoantibody response to various autoantigens, including BP180. While the pathogenicity of IgG autoantibodies to BP180 has been demonstrated in BP, the role of IgE autoantibodies in mediating tissue damage in BP and MMP is unclear.

Relevance of autoimmune thyroiditis in children and adolescents with vitiligo

Background  Vitiligo is the most common pigmentation‐related disorder worldwide. An autoimmune etiology is widely considered, and genetic factors may play an important role in its pathogenesis. The purpose of this study was to assess the incidence of thyroid dysfunctions and autoimmune thyroiditis in children with vitiligo and to identify related factors.

Pyoderma Gangrenosum in Association with Juvenile Rheumatoid Arthritis

A 17‐year‐old girl presented with multiple, painful, erythematous blisters and ulcerated lesions on the shins and buttocks. She also had arthralgia. She had suffered from juvenile rheumatoid arthritis (JRA) and recieved anti‐inflammatory agents and oral glucocorticoids for eight years. A biopsy of a lesion showed epidermal ulceration with marked neutrophilic infiltrates in the dermis. The patient was diagnosed with pyoderma gangrenosum (PG). PG is an uncommon cutaneous ulceration within the spectrum of the neutrophilic dermatoses that is reported in association with a number of systemic disorders, including inflammatory bowel disease, hematologic disease, internal malignancies, arthritis, immune abnormalities, and solid tumors. To our knowledge, this is the first reported case of PG associated with JRA.

A case of juvenile subcorneal pustular dermatosis successfully treated with acitretin

this is speculative because the medications used in this case have never been reported as a causal factor in the appearance of oral white lesions other than oral lichen planus. The hypothesis that SLE medications act as a trigger is, however, reinforced by the fact that the patient did not have any other family member afflicted with WSN. This patient was successfully treated with topical tetracycline for WSN. Tetracycline has been found to be beneficial in various inflammatory skin disorders including pyoderma gangrenosum and bullous pemphigoid. Tetracycline inhibits protein synthesis in bacteria but also has other actions including the inhibition of neutrophil chemotaxis, lymphocyte mitogenic response, and prostaglandin synthesis. It is possible that the beneficial effect of tetracycline is due to modulatation of epithelial keratinization. Based upon the improvement observed in this case as well as those from other reports, the use of topical tetracycline should be considered as an option for management of WSN.

Lipoid proteinosis: A case with ophthalmological and psychiatric findings

Lipoid proteinosis (LP) is an uncommon, recessively inherited disorder. The disease usually has its onset in the newborn period and is manifested by hoarseness. The skin and mucous membrane involvement arises between the first and second year of age. A 14‐year‐old male presented with the complaint of blistering on various sites of his skin, from the age of 12 months, resulting in scarring. Ophthalmological and psychiatric findings also appeared during the clinical course. The histological findings of skin biopsy included extensive deposits of amorphous eosinophilic material in the papillary dermis. No known therapy exists for LP.

A case of juvenile hyaline fibromatosis

Juvenile hyaline fibromatosis (JHF) is a rare, autosomally‐recessive disease characterized by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. Its onset is in infancy or early childhood. The most commonly affected sites are the nose, chin, ears, scalp, back and knees. The accumulation of an amorphous, hyaline material is typical in the skin and the other organs. Herein, we report a 14‐month‐old boy who presented with confluent pink papules on the paranasal folds and the chin, and nodular lesions on the periauricular and perianal regions. He had gingival hypertrophy and contractures of the shoulders, knees and elbows. He also had third‐degree consanguineous parents. Histopathological studies confirmed the diagnosis of JHF with the presence of increased numbers of fibroblasts embedded in a hyalinized connective tissue stroma.

Coexistence of Basal Cell Carcinomas and Multiple Sebaceous Gland Hyperplasias in a Cyclosporine (Ciclosporin)-Treated Renal Transplant Recipient

A 55-year-old man presented with multiple, asymptomatic, yellowish papules on his face with a 4-year history, and two non-healing tumoral lesions on his nose with a 7-month history. He was a renal transplant recipient and had been treated with cyclosporine (ciclosporin) for 9 years. A biopsy from the asymptomatic, yellowish papule on the face showed sebaceous gland hyperplasia, and biopsies from the lesions on the nose revealed basal cell carcinomas. The lesions on the nose were excised.Sebaceous gland hyperplasia and skin cancers are among the cutaneous neoplasms observed in renal transplant recipients receiving cyclosporine. To our knowledge, this is the third reported case of the coexistence of basal cell carcinomas and multiple sebaceous gland hyperplasias in a cyclosporine-treated renal transplant recipient.