Judit Hársing

Wegener's granulomatosis presenting as pyoderma gangrenosum

A 59‐year‐old male patient developed a necrotizing ulceration on the right shin. Both clinical and histopathologic examinations suggested pyoderma gangrenosum. After temporary improvement of skin symptoms under peroral glucocorticoid treatment, a hemorrhagic‐purulent discharge started from the nose, he began to have fever, malaise, cough, and a chest X‐ray revealed inflammation in the lung. Cerebral CT and MRI disclosed midline bone loss within the nasal septum and granulomatosus tissue masses protruding into the right orbit. The c‐ANCA test was positive, serum IgA was elevated, and he had microhaematuria and proteinuria. In this severe case of Wegeners granulomatosis prolonged methylprednisone and cyclophosphamide treatment was initiated. Both the skin symptoms and the granulomatosus infiltrations resolved.

Folliculotropic mycosis fungoides: clinicopathological analysis of 17 patients

Folliculotropic mycosis fungoides (FMF) represents a variant of MF characterized by hair follicle invasion of mature, CD4‐positive small lymphoid cells with cerebriform nuclei. The disease displays resistance to standard treatment modalities and has an unfavourable course.

Dirofilaria repens infection case in Hungary: a case report

A 51‐year‐old female developed urticarial lesions of her right forearm which progressed into transient edema and subcutaneous swelling. Later a small infiltrated subcutaneous nodule also appeared and was removed in toto. Histopathological examination revealed the presence of Dirofilaria repens.This worm is the cause of an endemic zoonosis in the Mediterranean area. In the past decade many cases have been reported worldwide, but the condition appears rare in Hungary and skin findings have not been described.

Coexistence of primary cutaneous anaplastic large cell lymphoma and mycosis fungoides in a patient with B-cell chronic lymphocytic leukaemia

with discoid lupus erythematosus. Dermatology 1993; 187:178–81. 7 Hayakawa K, Nagashima M. Systemic sclerosis associated with disseminated discoid lupus erythematosus. Int J Dermatol 1993; 32:440–1. 8 Chan HL, Lee YS, Hong HS, Kuo TT. Anticentromere antibodies (ACA): clinical distribution and disease specificity. Clin Exp Dermatol 1994; 19:298–302. 9 Aragone MG, Balestrero S, Parodi A, Rebora A. Anti-centromere antibodies in 2 patients with discoid lupus erythematosus and no signs of systemic sclerosis. Acta Derm Venereol (Stockh) 1999; 79:165. 10 Katano K, Kawano M, Koni I et al. Clinical and laboratory features of anticentromere antibody positive primary Sjögren’s syndrome. J Rheumatol 2001; 28:2238–44.

Angiolymphoid hyperplasia with eosinophilia in pregnancy.

To the Editor Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign disorder of young adults with female predominance, characterized by solitary or multiple nodules on the head and neck region. Some cases associated with pregnancy have been reported earlier.1 During the first semester of the second pregnancy, the 28year-old female patient developed two subcutaneous, livid nodules 1–1.5 cm in diameter at the lateral side of the left eyebrow and in the left temporal region, as well as several smaller lesions on the scalp of the left parietal region. Sometimes they were spontaneously bleeding. The overlying skin was purplish or normal, larger lesions were pulsating (fig. 1). The two largest nodules were surgically removed. The histological picture showed circumscribed, lobuled lesions, oedematous endothelial cells in the dermal blood vessels and lymphocytic and eosinophilic infiltration (fig. 2). Routine laboratory investigations, peripheral eosinophil count revealed negative results. Direct immunofluorescent histology did not show overexpression of oestrogen or progesterone receptors in the lesions. Polymerase chain reaction (PCR) investigations of human herpesvirus 8 (HHV8) and human T cell lymphoma virus (HTLV) were also negative. After the patient has delivered, the lesions showed spontaneous regression. ALHE was originally described by Wells and Whimster in 1969.2 This rare benign tumour occurs with a female predominance, and most often affects the head and neck region. The typical clinical signs are solitary or multiple purplish, brownish papules and subcutaneous nodules. The histological picture is characterized by the proliferation of endothelial cells associated with lymphocytic and eosinophilic infiltrate. Results of immunohistological examinations, showed that perivascular infiltrating lymphocytes are mostly CD4 + positive. Proliferating endothel cells express adhesion molecules: VLA-1, -3, -5, ICAM-1, ELAM-1.3 Sometimes marked peripheral eosinophilia can be detected. Kimura’s disease was earlier thought to be related to ALHE, but nowadays it is considered a different clinical entity. The main differences between the two diseases were described by Chum et al. in 1992.4,5 Some earlier clinical entities with other names are identified as ALHE, some authors suggested the nomenclature of epithelioid haemangioma.6 The origin of the disease is not known, infection (HTLV or HHV8), hormonal background, or possible role of trauma are the mostly discussed possibilities. Increased expression of vascular endothelial growth factor (VEGF) and interleukin 5 (IL-5) was detected in some cases.7 Associated cases of the disease to pregnancy were already described. Moy et al. found oestrogen and progesterone receptor overexpression in the lesional skin of pregnant women.1 In our case, direct immunofluorescent histology did not show overexpression of oestrogen or progesterone receptors in the lesions. HHV8 and HTLV PCR investigations were also negative. The possible treatment modalities were intralesional steroid or IFN α, surgical excision, cryotherapy and laser therapy.8 Association of ALHE with nephrotic syndrome has also been described.9 The interest of our case makes the typical clinical picture and outcome of the rare disease. Its association with pregnancy suggests the pathogenetic role of immunological and endocrinological changes in the background.

Grayish discoloration of the face – argyria

The 54 year-old vegetarian patient has taken oral colloidal silver for two years to stimulate his immune system. The silver intake resulted in diffuse grayish discoloration on the face. His laboratory values were in the normal range, and no internal organ disease was detected. The histology of the forehead skin confirmed the diagnosis of argyria. Argyria is a condition associated with chronic local or systemic exposure to silver-containing products. The silver is typically deposited in skin, fingernails, oral mucosa and conjunctival membranes. There is no effective treatment for this condition. With the availability of pharmacologic alternatives, physician-directed use of silver-containing products had significantly declined. We review the literature and call attention to the adverse effects and dangers of the widely used paramedicinal colloidal silver products.Az argyria ezustvegyuletek tartos lokalis vagy szisztemas alkalmazasa nyoman kialakulo korkep. A tunetek leggyakrabban a bőron, kormokon, conjunctivan, szajnyalkahartyan jelentkeznek, kezelesuk mind a mai napig kihivast jelent. Bar napjainkban ezusttartalmu gyogyszerek, gyogyaszati eszkozok hasznalatara mersekelt gyakorisaggal kerul sor, az argyria előfordulasaval megis szamolnunk kell. A korkep leggyakoribb okava mara a paramedicinalis gyogyaszatban szeles korben alkalmazott ezustkolloid tartos fogyasztasa valt. Kozlemenyunkben 54 eves ferfi beteg esetet ismertetjuk, aki evek ota vegetarianus eletmodot folytatott. A beteg immunrendszere erősitese celjabol fogyasztott ezustkolloidot kozel ket even at, amelynek hatasara kialakult az arc diffuz, szurke elszineződese. Az anamnezis es a klinikai kep alapjan az argyria diagnozisa merult fel. A beteg kivizsgalasa soran laboratoriumi es kepalkoto diagnosztikus vizsgalatokkal belső szervi elterest nem talaltunk. Az argyria diagnozisat a tunetes bőrből vegzett hisztologiai vizsgalat alatamasztotta. A szerzők az irodalmi adatok tukreben felhivjak a fi gyelmet a paramedicinaban az ezustkolloid napjainkban tapasztalt terhoditasara, mellekhatasaira, a szerrel kapcsolatos teves informaciokra, az ezustkolloid-fogyasztas veszelyeire. Kulcsszavak: ezustkolloid, paramedicina, szurke bőrszin, argyria Grayish discoloration of the face – argyria The 54 year-old vegetarian patient has taken oral colloidal silver for two years to stimulate his immune system. The silver intake resulted in diffuse grayish discoloration on the face. His laboratory values were in the normal range, and no internal organ disease was detected. The histology of the forehead skin confi rmed the diagnosis of argyria. Argyria is a condition associated with chronic local or systemic exposure to silver-containing products. The silver is typically deposited in skin, fi ngernails, oral mucosa and conjunctival membranes. There is no effective treatment for this condition. With the availability of pharmacologic alternatives, physician-directed use of silver-containing products had signifi cantly declined. We review the literature and call attention to the adverse effects and dangers of the widely used paramedicinal colloidal silver products.

Secondary ALK negative anaplastic large cell lymphoma in a patient with lymphomatoid papulosis of 40 years duration.

A 49 year-old man presented to our clinic. He had a history of lymphomatoid papulosis since childhood. At age 44, regional lymph node manifestation of anaplastic lymphoma kinase (ALK) anaplastic large cell lymphoma (ALCL) developed. Chemotherapy resulted in complete remission of the lymphadenopathy. Four years later, systemic relapse was detected which was refractory to therapy. Histology and immunohistochemistry showed congruent characteristics of multiple skin and lymph node biopsies: diffuse mixed infiltrate with large, anaplastic CD30 cells. Immunophenotype and microscopic morphology suggested a common origin of the different manifestations-however, this could not be proven due to lack of T-cell receptor (TCR) gamma gene rearrangement in most of the samples. The diagnosis of ALK-negative systemic ALCL with cutaneous symptoms was set up at the second flare up, however, the possibility of primary cutaneous ALCL was not excluded steadily. Lymphomatoid papulosis, primary cutaneous ALCL, and systemic ALK ALCL are 3 different entities but the separation of them cannot be solved without distinctive diagnostic tools.

CD30-positive anaplastic large T-cell lymphoma of the tongue

© 2008 The Authors JEADV 2009, 23, 169–243 Journal compilation

Intravascular large B-cell lymphoma: Remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy

Intravascular lymphoma is an uncommon, very aggressive extranodal non-Hodgkin lymphoma that most frequently involves the skin and central nervous system. Most cases are of B-cell origin; T-cell phenotype is extremely rare. Malignant cells proliferate within the lumens of capillaries, arterioles, venules, and small arteries; vascular occlusion is responsible for the clinical signs and symptoms. The prognosis of this high-grade B-cell lymphoma has improved since the introduction of the anti-CD20 monoclonal antibody, rituximab. We describe a case of B-cell intravascular lymphoma successfully treated with rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisolone.

Expression of G protein-coupled oestrogen receptor in melanoma and in pregnancy-associated melanoma

The hormone sensitivity of melanoma and the role of ‘classical’ oestrogen receptor (ER) α and β in tumour progression have been intensively studied with rather contradictory results. The presence of ‘non‐classical’ G protein‐coupled oestrogen receptor (GPER) has not been investigated on human melanoma tissues.