Can Baykal

Orale DMSO-Therapie bei 3 Patienten mit Lipoidproteinose Ergebnisse einer Langzeittherapie

ZusammenfassungWir berichten über 2 Schwestern und einen 30jährigen Mann mit Lipoidproteinose (Hyalinosis cutis et mucosae, Morbus Urbach-Wiethe), bei denen wir eine orale Dimethylsulphoxide-(DMSO-)Therapie (60xa0mg/kg/Tag) eingeleitet haben. Die Krankheit hat i.allg. einen benignen Verlauf, und systemische Therapiemaßnahmen – abgesehen von der versuchsweise durchgeführten oralen DMSO- bzw. Etretinattherapie bei jeweils einem Patienten – sind in der Literatur nicht bekannt. Bei unseren Patienten konnte am Ende der durchschnittlich 3jährigen Therapie keine Rückbildung der Haut- und Schleimhautveränderungen und insbesondere der Heiserkeit erzielt werden. Bei der Patientin 1 nahm die Krankheit einen progressiven Verlauf, wobei die neu aufgetretene Luftnot und die zunehmende Heiserkeit eine Stimmbandoperation erforderlich machte. Somit erwies sich die 3jährige orale DMSO-Therapie bei 3 Patienten mit Lipoidproteinose als unwirksam.SummaryLipoid proteinosis is a rare autosomal recessive disorder with a chronic, benign course. There is no generally accepted systemic therapy apart from the experimental oral use of dimethyl sulphoxide (DMSO) and etretinate in two single cases. We treated two sisters and an unrelated man with lipoid proteinosis with longterm oral DMSO (60xa0mg/kg/d). At the end of an average treatment time of 3 years, DMSO was withdrawn because it produced no beneficial effects with regard to their skin, mucosal lesions or hoarseness. Additionally, one patient showed progression of her disease with worsening hoarseness and onset of dyspnea, requiring surgical removal of vocal cord infiltrates. Three patients with lipoid proteinosis failed to show any beneficial response to long term treatment with DMSO.

Lokale DMSO-Behandlung der makulösen und papulösen Amyloidose

ZusammenfassungMakulöse Amyloidose (MA) und Lichen amyloidosus (LA) sind die zwei am häufigsten vorkommenden Varianten der primär kutanen Amyloidosen, bei denen ein heftiger Juckreiz im Vordergrund der klinischen Symptomatik steht. Leider zeigen aber die verschiedenen Therapiemaßnahmen wie Antihistaminika, Kortikosteroide, UVB, Etretinat, Dermabrasion usw. wenig oder keinen Erfolg. In den letzten Jahren wurde in der Literatur über die erfolgreiche Therapie mit lokalem Dimethylsulphoxid (DMSO) berichtet. In unserer Studie haben wir Patienten mit MA, LA und biphasischer Amyloidose mit 50%iger DMSO-Lösung lokal behandelt. Während einer Behandlungsdauer von 6–20 Wochen wurde bei 9 von insgesamt 10 Patienten klinische Besserung erzielt. Das schnelle Ansprechen des Juckreizes auf die Therapie innerhalb der ersten Woche ist auf den die Mastzelldegranulation bzw. -depletion fördernden Effekt von DMSO zurückzuführen. Mittels DMSO-Therapie wurde auch eine vollständige Rückbildung der lichenoiden papulösen Effloreszenzen innerhalb von durchschnittlich 11 Wochen erzielt, welche am ehesten durch das Abklingen des Juckreizes und des damit verbundenen Kratzeffektes erklärt werden kann. Trotz der guten klinischen Ergebnisse war histopathologisch kein Verschwinden der Amyloidmassen festzustellen. In den weiteren Verlaufskontrollen nach Ende der Therapie wurden Rezidive der klinischen Symptomatik beobachtet, wobei weitere prospektive Studien zur Bestimmung der optimalen Behandlungsdauer notwendig erscheinen.SummaryMacular amyloidosis (MA) and lichen amyloidosus (LA) are the two major variants of the primary cutaneous amyloidoses which present with severe and therapy resistent itching. Various therapeutic modalities such as antihistamines, intralesional injection or topical application of corticosteroids, etretinate, UVB irradiation and dermoabrasion have been employed with variable success. Recently, in a few case reports authors have observed encouraging beneficial clinical effects by using topical dimethyl sulphoxide (DMSO). In our study 10 patients with either MA or LA or biphasic amyloidosis were treated with a 50% solution of DMSO in water. 9 of them showed marked clinical improvement at the end of 6–20 weeks of treatment. Degranulation and depletion of the mast cells by DMSO is the most probable explanation for the rapid improvement of itching beginning within the first week of therapy. Remarkable flattening of the lichenoid papules which was obtained within 11 weeks of treatment is interpreted as a result of the improvement of itching and the related scratch effect. Histological examination after treatment revealed no disappearance of amyloid deposits in the papillary dermis. In the follow-up period relapses of itching and papules were observed. Therefore further studies are needed to find out the optimal procedure of therapy.

Osteoporosis and Osteopathy Markers in Patients with Mastocytosis

Objective: Osteoporosis, osteosclerosis, and lytic bone lesions have been observed in patients with systemic mastocytosis (SM). We examined bone mineral density (BMD) biochemical turnover markers and serum tryptase levels in SM, which is considered a rare disease. Materials and Methods: Seventeen adult patients (5 females, 12 males; median age: 33 years, range: 20-64) with mastocytosis were included in this study. We investigated the value of quantitative ultrasound (QUS) of the calcaneus in the assessment of BMD in SM patients, as well as BMD of the lumbar spine (L1-L4), femoral neck, and distal radius using dual energy x-ray absorptiometry (DXA) and plasma tryptase levels, biochemical markers of bone turnover. Results: At lumbar spine L1-L4, the femoral neck, and the distal radius or as calcaneus stiffness, 12 of 17 patients had T-scores of less than -1 at least at 1 site, reflecting osteopenia. Three of 17 patients had T-scores showing osteoporosis (T-score <-2.5). There was no relationship between DXA and bone lesion severity. We also found a significant positive correlation between tryptase levels and disease severity, as well as between disease severity and pyridinoline (p<0.01 by Spearman’s test). Conclusion: DXA and calcaneal QUS may not be appropriate techniques to assess bone involvement in SM patients because of the effects of osteosclerosis. This study further shows that the osteoclastic marker pyridinoline is helpful in patients with severe disease activity and sclerotic bone lesions to show bone demineralization.

Annular Erythematous Patches as the Presenting Sign of Extranodal Natural Killer/T-Cell Lymphoma

Extranodal natural killer/T-cell lymphoma (ENKTL) is a distinct type of lymphoma strongly associated with Epstein-Barr virus (EBV) infection and showing an aggressive course [1]. It usually presents as a localized disease in the upper aerodigestive tract, from the nasal cavity to the hypopharynx [2,3], but it may rapidly extend to the neighboring tissues and disseminate to various organs such as the small intestine, epiglottis, testes, adrenal gland, kidneys, and breasts [4,5]. As nasal/upper aerodigestive tract involvement may only cause nonspecific symptoms in the early period, diagnosis may be initially established based upon skin lesions [6]. We present two ENKTL patients with unusual dermatological findings.

The Dilemma of Coexisting Nevoid Hyperkeratosis of the Nipple and Areola in Mycosis Fungoides: A Report of Three Cases.

Nevoid hyperkeratosis of the nipple and areola (NHNA) is a rare clinicopathological entity showing persistent and strictly localized hyperkeratotic lesions of the nipple, areola or both with unknown etiopathogenesis. A similar clinical appearance may also be seen in different diseases with specific histopathological features. There are a few anecdotal reports on the association of NHNA with mycosis fungoides (MF), but they do not describe a uniform condition. In this report, we present 3 patients with hyperkeratotic lesions of the nipple and areola associated with MF but showing different histopathological features. We also review similar cases in the literature and discuss possibilities concerning this association. Two of our cases represent the association between MF and NHNA without histopathological features of MF on the nipple-areola complex. The other case represents hyperkeratosis of the nipple and areola with specific histological and immunohistochemical features of MF. Hence, we would like to hypothesize that MF may involve the nipple and areola and have an appearance similar to NHNA. Intriguingly, however, NHNA may occasionally also be seen in association with MF. However, this peculiar association requires further explanation.

Clinical atlas of skin tumors

BENIGN SKIN TUMORS.- Epidermal Benign Tumors.- Epidermal Precancerous Lesions and in Situ Malignancies of Skin.- Nevo-Melanocytic Benign Tumors.- Cutaneous Cysts.- Cutaneous Adnexial Tumors.- Benign Vascular Tumors Of The Skin.- Benign Neural Tissue Tumors.- Benign Fibrohistiocytic Tumors.- Mastocytosis.- Subcutaneous Fat Tissue Tumors.- MALIGNANT SKIN TUMORS.- Malignant Epithelial Tumors.- Malignant Melanoma.- Sarcomatous Tumors of Skin.- Cutaneous Lymphomas.- Histiocytosis.- Cutaneous Metastasis.- Predilection sites of skin tumors.

Periungual pyogenic granuloma-like lesions following plaster cast immobilization: a case managed with symptomatic therapy

Pyogenic granuloma (PG) is an eruptive capillary hemangioma not uncommonly seen in the nail folds. The most common causes of periungual PG include acute and chronic trauma, infection, drugs, and high levels of sex hormones associated with pregnancy [ 1 ] . Some patients develop more than one lesion. PG-like lesions of the nail folds, accompanied by onychomadesis, have also been reported as a rare complication of plaster cast immobilization and attributed to mild peripheral nerve injury [ 1–4 ] . While neurological symptoms observed in most of the cases previously reported support this hypothesis, we recently treated a patient with multiple PG-like lesions following plaster cast immobilization without any associated neurological fi ndings. A 25-year-old male sustained a closed traumatic fracture of the middle phalanx of the left third fi nger. The hand was placed in a plaster cast that extended from halfway up the arm down to the proximal nail folds of the second to fi fth fi ngers. Two weeks after removal of the plaster cast, initial periungual edema was noted on the second to fourth fi ngers of the same hand (Figure 1 a). Subsequently, the lesions turned into erosive erythematous papules and nodules (Figure 1 b). The patient reported no associated neurological symptoms such as paresthesia, pain, or increased sweating, and the neurological exam, too, was unremarkable. Clinically, the papulonodular lesions were considered to be PG. Initial treatment included wet dressings, topical silver nitrate, and systemic antibiotics (amoxicillin/clavulanic acid for ten days). Due to lack of clinical improvement, the patient was started on oral methylprednisolone (0.3 mg/kg/ day) – tapered over the course of twelve days – leading to a decrease in the size of the tumors (Figure 1 c). The lesions were then treated with a topical corticosteroid/antibiotic preparation (fusidic acid combined with betamethasone valerate), resulting in the regression of edema and nearly complete healing of the PG-like lesions after six weeks (Figure 1 d). All three affected fi ngers showed onychomadesis (Figure 1 e) after the lesions had regressed but this sequela, too, resolved without any nail deformity within six months (Figure 1 f). No recurrence was observed during the one year follow-up period. Although retronychia was also considered in the differential diagnosis, factors that argued against this condition

Spiradenocylindroma: Rapid increase in size attributed to hemorrhage

rhage was not observed histologically in the biopsy specimens obtained from the expanding area 1 month after the first consultation. In addition, platelet counts, PT and APTT were within normal range through the clinical course. Thus, it is likely that expansion of the lesion was mainly caused by tumor growth itself. The size of the lesion in our patient was too large to be treated by surgical excision or intralesional IFN-a injection. Kimura et al. had demonstrated the efficacy of soft X-ray for treating this disease. Hence, we decided to use radiation therapy. Further, Ishikawa et al. reported disease recurrence 2 months after electron beam therapy with a 20-Gy dose. Therefore, the radiation dose was set at 30 Gy each for the front and lateral sides. Although, in our patient, recurrence was not observed for 20 months after radiation therapy, the condition may recur in the future. It is thus necessary that regular follow-up examinations are conducted.