Sevgi Bahadir

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.

Plasma polymorphonuclear leukocyte elastase levels and its relation to disease activity in psoriasis

Although some increased enzyme activities in PMN leukocytes isolated from the circulation of psoriatic patients have been previously described, none of these enzymes had been studied in plasma for determination of disease activity. The purpose of this study was to evaluate whether plasma PMN elastase levels might be a good marker for determination of disease activity of psoriatic patients. Plasma PMN elastase and some acute phase reactants including alpha-1 antitrypsin, alpha-2 macroglobulin, fibrinogen, erythrocyte sedimentation rate(ESR), and transferrin were studied in thirty-nine patients with psoriasis, including 16 controlled patients, and forty healthy control subjects. PMN elastase and the acute phase reactants except transferrin were found to be significantly higher in the psoriatic patients than in control subjects. The PMN elastase level in the patients was about 6-fold higher than that of the control group. The PMN elastase levels in the patients in the inactive period was two-fold higher than in the control subjects. PMN elastase was found to correlate significantly with PMN leukocyte count and alpha-1 antitrypsin in active and inactive periods of the disease, but with fibrinogen and psoriasis area and severity index (PASI) only in the active period of the disease. We conclude that plasma PMN elastase level may be a more specific and sensitive inflammatory marker than alpha-1 antitrypsin, alpha-2 macroglobulin, ESR, and may be a good marker for diagnosis and follow up of the disease activity of the psoriatic patients.

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.

Possible role of Mycobacterium tuberculosis complex in Melkersson–Rosenthal syndrome demonstrated with Gen‐Probe amplified Mycobacterium tuberculosis direct test

Melkersson–Rosenthal (MRS) syndrome is characterized by a classical triad of recurrent or persistent orofacial swelling, peripheral facial nerve paralysis and lingua plicata. Granulomatous cheilitis (GC) is regarded as a monosymptomatic form of MRS. The exact aetiologies of MRS and GC are unknown. In this study we investigated the possible role of mycobacteria in these two conditions. A ribosomal RNA amplification‐based Gen‐Probe amplified Mycobacterium tuberculosis direct test was used to investigate the presence of M. tuberculosis complex in paraffin‐embedded skin biopsy specimens from five patients with MRS and one patient with GC. Three of the six specimens were shown to be positive using this system; one of the positive specimens also showed positive Ziehl–Neelsen staining. These results suggest a possible mycobacterial aetiology for MRS and GC.

Polymorphonuclear leukocyte elastase levels in patients with Behçet's disease

Behçets disease is a chronic multisystem disorder characterized by a relapsing inflammatory process of unknown aetiology. The increased activity of polymorphonuclear leukocytes (PMN) in Behçets disease has been intensively studied. PMN elastase, an acute phase reactant, was investigated to determine whether it may serve as a biochemical marker in Behçets disease. Erythrocyte sedimentation rate, protein electrophoresis, immunoglobulins (IgA, IgG, IgM), complement components (C3, C4) as well as PMN elastase were evaluated in 42 patients with Behçets disease and 40 healthy subjects. The mean PMN elastase levels were found to be 244.2 micrograms/l (median 210 micrograms/l, S.D. 126.8) in patients with Behçets disease and 44.3 micrograms/l (median 45, S.D. 19.2) in healthy subjects (P < 0.001). In addition, the mean PMN elastase levels were found to be 321.5 micrograms/l (median 300, S.D. 117.9) in the acute phase and 159 micrograms/l (median 162, S.D. 59.3) in remission (P < 0.001). It was concluded that PMN elastase may be a good biochemical marker for diagnosis and therapy control in patients with Behçets disease.

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.

Cutis laxa-like pseudoxanthoma elasticum with ossification

Pseudoxanthoma elasticum (PXE) is a heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the flexural area, which is loose and wrinkled. A 35‐year‐old woman presented cutis laxa‐like marked wrinkling and yellowish papules, and a skin biopsy revealed ossification and fragmentation of elastic fibers in the dermis. Ophthalmologic examination revealed a peau d’orange appearance of the retina. She was also found to have a mitral stenosis and hypertension. This is an unusual case of concurrent ossification and cutis laxa‐like PXE.

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.