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Lymphomatoid papulosis (LyP) is a recurrent papulonodular eruption, characterized by self-healing skin lesions. While this disorder usually has a benign course, about 10% of the patients develop lymphoproliferative disorders such as CD30+ T cell lymphoma. We report 2 patients with LyP type C. In one case progress to a cutaneous CD30+ large T cell lymphoma could not be excluded. Histologic differentiation between LyP and malignant lymphoma causes considerable problems. In this regard, the evaluation of the clinical course is essential. A long-term follow-up including diagnostic imaging studies is required.

Lupus erythematosus (LE) is a rare disorder in childhood. Most patients reported in the literature present with systemic manifestations, and, to date, fewer than 20 children with chronic cutaneous LE have been documented. In this article, we describe 3 patients with childhood LE tumidus, an uncommon but distinct subtype of chronic cutaneous LE. The lesions are characterized by erythematous, urticaria-like, nonscarring plaques in sun-exposed areas and, unlike in other variants of chronic cutaneous LE, there is no epidermal involvement. The clinical, photobiological, and histologic features as well as the differential diagnoses of childhood LE tumidus are discussed and compared with the adult form of this disease., (Copyright 2003 S. Karger AG, Basel)

Mucin deposition is a common histopathologic finding in lupus erythematosus (LE) but is rarely present in sufficient quantity to produce clinically apparent skin lesions. Until now, fewer than 40 cases of papulonodular mucinosis associated with LE have been reported in the literature, and it was associated with either systemic LE or discoid LE. For the first time, to our knowledge, we describe 2 patients with subacute cutaneous LE who presented with papulonodular mucinosis as a major clinical manifestation of their disease., (Copyright 2003 S. Karger AG, Basel)

Cutaneous leiomyomas are rare benign tumours of the skin, which present in multiple disseminated, segmental or solitary forms. The pathogenesis of segmental cutaneous leiomyomatosis is not yet fully known. Most recently two types of segmental manifestation of autosomal dominant inherited diseases were postulated. Type 1 reflects heterozygosity for the underlying mutation with a clinical picture similar to that in a non-mosaic phenotype. In type 2, loss of heterozygosity leads to homo- or hemizygosity with a pronounced segmental manifestation of lesions in the affected segment. In our patient the lesions were restricted to one segment and therefore she most probably has a type 1 segmental cutaneous leiomyomatosis.

Early and correct diagnosis of malignant melanoma is of utmost importance to ensure adequate treatment and the best outcome. Prompted by the death of a patient with an apparent metastasising melanoma in situ, we reassessed 104 people with this malignant disorder, whose diagnosis had been histopathologically verified. We did immunohistochemical analysis of cells with the melanocytic marker melan-A/MART-1, and results of this analysis showed that 30 (29%) of 104 patients had invasive melanomas. One patient died of distant metastases, and the tumour recurred in another. Our finding could be relevant for diagnosis and treatment of melanoma in situ.

Lupus erythematosus tumidus (LET) is a disease with characteristic clinical and histopathologic features that has not always been considered a subset of cutaneous lupus erythematosus (CLE). Although LET was first mentioned in the literature in 1930, it has rarely been documented, and immunohistochemical studies have never been performed. The aim of the present study was to characterize the inflammatory infiltrate and to analyze the expression of endothelial cell adhesion molecules in skin specimens from patients with LET and to compare the results with those from patients with other variants of CLE, such as discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). Cryostat sections of lesional skin specimens from ten patients with LET demonstrated an infiltrate composed of more than 75% CD4+, CD8+, and HLA-DR+ cells. Interestingly, CD45RO+ cells, in contrast to CD45RA+ cells, were the prevailing inflammatory cell population. Compared with skin specimens from patients with DLE and SCLE, the mean expression of CD4+ and CD8+ cells was higher (but not significantly so) in LET, and no differences were observed with the other three antibodies. Furthermore, in contrast to controls, intercellular adhesion molecule-1, vascular adhesion molecule-1, E-selectin, and P-selectin showed the same expression pattern in skin specimens from patients with DLE, SCLE, and LET. In conclusion, the inflammatory infiltrate of LET primarily consists of CD4+/CD8+ lymphocytes. Furthermore, expression of endothelial cell adhesion molecules was equally upregulated in LET compared with the expression in DLE and SCLE, suggesting a similar immunopathomechanism of these subtypes of CLE.

Purpose: The novel immunosuppressant RAD, 40-0-(2-hydroxy-ethyl)-rapamycin, has synergistic effects with cyclosporin A. The aim of this study was to evaluate the combined effect of RAD and cyclosporin A in the prevention of acute allograft rejection after murine corneal transplantation., Methods: Fisher donor corneas were implanted into Lewis recipients. Postoperative evaluation included slit-lamp biomicroscopy and immunohistology. Treatment groups were comprised of rats treated orally with RAD 2.5 mg/kg/day, cyclosporin A 10 mg/kg/day, RAD 1.5 mg/kg/day plus cyclosporin A 5 mg/kg/day., Results: Therapy with RAD 2.5 mg/kg and cyclosporin A 10 mg/kg led to a statistically significant and comparable prolongation of transplant survival. However. combination therapy was significantly superior. There was a significant reduction in the number of infiltrating cells in the animals treated with RAD and cyclosporin A., Conclusions: This is the first study on the efficacy of a double drug regimen with RAD and cyclosporin A for the control of acute corneal allograft rejection. Combination therapy resulted in superior graft survival.

Background: RAD is a novel macrolide immunosuppressant with effects on growth factor signalling. We investigated the potency of RAD in inhibiting allograft rejection in the rat model of orthotopic allogeneic penetrating keratoplasty., Methods: Fifty-four allogeneic orthotopic penetrating keratoplasties were performed using Fisher rats as donors and Lewis rats as recipients. The animals were divided into five groups: syngeneic control, allogeneic control, RAD 1.5 mg/kg bw per day, RAD 2.5 mg/kg bw per day, cyclosporin A (CSA) 10 mg/kg bw per day. Medication started on the day of operation and continued daily for the duration of 18 days. Each animal was examined by slit-lamp microscopy every 3rd day. For immunohistological evaluation rats were killed on day 14. Immunohistology was performed using monoclonal mouse anti-rat antibodies against CD4, CD8, CD25, CD45 and CD54., Results: The average transplant survival time in the allogeneic combination was 12.3 days (+/- 0.3). Therapy with RAD 1.5 mg/kg and 2.5 mg/kg led to a statistically significant prolongation of transplant survival to 32.3 days (+/- 11.3, P<0.05) and 37.7 days (+/- 12.5), respectively. This efficacy was similar to that of CSA 10 mg/kg (39.7 +/- 12.5 days). There was a statistically significant reduction in the number of CD4+, CD8+ as well as CD45+ cells in both the RAD- and the CSA-treated animals compared with the allogeneic control., Conclusions: The results show that oral immunosuppression with RAD significantly prolongs corneal allograft survival. Further investigation of RAD in preclinical and clinical high-risk keratoplasty is warranted.

Background: Cicatricial pemphigoid (CP) is a rare autoimmune bullous disease that affects the skin and mucous membranes. It commonly ends by serious complications such as blindness, stenosis, and stricture formation and is difficult to treat. Mycophenolate mofetil has been reported to be effective in the treatment of pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid either as monotherapy or as a steroid-sparing agent., Objective: Our purpose was to evaluate the effectiveness of mycophenolate mofetil as a steroid-sparing agent in treating patients with CP., Methods: Three patients with CP were treated with mycophenolate mofetil and prednisolone., Results: All 3 patients responded very well to the therapy. None of them showed relapse of the disease for a follow-up period of 6 to 14 months after complete cessation of mycophenolate mofetil and prednisolone. No side effects were seen., Conclusion: Mycophenolate mofetil appears to be a safe and effective steroid-sparing agent in the treatment of CP.

It has long been observed that sun exposure can induce or exacerbate skin lesions in patients with certain forms of lupus erythematosus. Despite the frequency of photosensitivity in these patients, the mechanism by which ultraviolet radiation alters the pathogenic course of this disease remains poorly understood. After development of standardized test methods, our group demonstrated in 1986 that skin lesions in patients with lupus erythematosus can be experimentally reproduced by UVA and UVB irradiation. In the following years, phototesting has received much attention as a valid model to study photosensitivity of different forms of lupus erythematosus and the pathogenetic mechanism of this disease. Further investigations have also made it possible to find genetic and immunologic factors associated with photosensitivity and have helped to identify the pathophysiologic steps involved in the induction of such skin lesions. We present phototesting results and clinical correlations of more than 400 patients with different forms of lupus erythematosus and discuss the recent advances in provocative phototesting.

Photosensitivity is an important characteristic feature of several forms of lupus erythematosus (LE), and induction of skin lesions by UV-A and UV-B irradiation has been proved to be an optimal model for evaluating light sensitivity in patients with this disease. Because lupus erythematosus tumidus (LET) has rarely been documented in the literature and is often difficult to differentiate from other photodermatoses such as polymorphous light eruption, we performed photoprovocation tests in 60 patients with LET according to a standardized protocol. Areas of uninvolved skin on the upper back were irradiated with single doses of UV-A (100 J/cm2) and/or UV-B (1.5 minimal erythema dose) daily for three consecutive days. Interestingly, patients with LET are more photosensitive than those with subacute cutaneous lupus erythematosus, and in our study experimental phototesting revealed characteristic skin lesions in 43 patients (72%). Because of the latency period in developing positive phototest reactions, it might be difficult for these patients to link sun exposure with their skin lesions. Furthermore, our data revealed a positive correlation of antinuclear antibodies and positive provocative phototest reactions in these patients as seen for other forms of LE. In conclusion, the high incidence of positive phototest reactions in correlation with the clinical findings, history of photosensitivity and antinuclear antibodies enable the classification of LET as the most photosensitive type of LE.

Purpose: We reviewed the records of patients with chronic cicatricial disease of the conjunctiva for differences in prognosis between clinical and histopathological subgroups of the disease and in therapeutic options., Patients: In 30 patients (58 eyes) with an average age of 69 years (52-86) chronic cicatricial disease of the conjunctiva was diagnosed clinically. Only in 22/30 patients conjunctival biopsies could be performed. The correlation of histopathological and immunohistochemical diagnoses with clinical course under systemic immunosuppression was studied., Results: 15/22 biopsies led to a classification into different subgroups. Under systemic immunosuppression disease ceased to progress for a mean time of 15 months in 13 of 15 patients with positive biopsies and in 9 of 15 without classification. The results after cyclosporine A therapy (4 of 5 patients stabilized after a mean of 27.5 months) and mycophenolate mofetil (8 of 11 patients stabilized at a mean of 7.8 months) were better than those after therapy with dapsone, azathioprine and cyclophosphamide., Conclusions: Histopathological and immunohistochemical examinations led to a classification in two-thirds of the patients with clinical aspects of chronic cicatricial disease of the conjunctiva. There was no correlation between different histopathological subgroups, success of therapy and prognosis of the disease. There is little hope in using new systemic immunosuppression such as cyclosporine A and mycophenolate mofetil.

Scarring alopecia is a rare complication of scalp psoriasis which in most cases occurs in areas of long-standing lesions. A HIV-positive 35 year-old Moroccan patient (CDC C3) developed scalp psoriasis with scarring alopecia within five weeks. Topical antipsoriatic therapy resulted in alleviation of psoriatic lesions and stopped the progression of the alopecia. This case report suggests potential pathogenic links between scalp psoriasis, scarring alopecia, and HIV-infection. In addition, early and efficient antipsoriatic treatment is recommended to prevent the disfiguring complication of scarring alopecia.

Introduction: The effect of RAD, a new macrolide immunosuppressant, was examined as mono- and combination therapy with mycophenolate mofetil (MMF) in prevention of acute allograft rejection in murine corneal transplantation., Methods: Both drugs were administered orally for 18 days beginning at the day of transplantation. The inbred strains Fisher and Lewis were used as donors and recipients, respectively. Five groups were involved: syngeneic control, allogeneic control, 2.5 mg/kg RAD, 40 mg/kg MMF, and double drug therapy with 1.5 mg/kg RAD and 20 mg/kg MMF., Results: The median transplant survival time in the allogeneic combination was 12 (+/-0.3) days. Monotherapy with 2.5 mg/kg RAD and 40 mg/kg MMF led to a statistically significant prolongation of transplant survival to 25.5 (+/-12.5, P=0.0001) days and 19.5 (+/-13.9, P=0.0053) days, respectively. Combination therapy was superior to both monotherapies (100+/-15.8 days, P=0.03). There was a significant reduction in the number of CD4+, CD8+, as well as CD45RA+ cells in the RAD- and double drug-treated animals when compared with the allogeneic control. This significant reduction in graft-infiltrating lymphocytes has not been found in the MMF monotherapy., Conclusions: The unique finding of this first study on the combination of RAD and MMF in murine corneal transplantation is that double drug therapy produces a highly synergistic effect in prevention of acute allograft rejection without a higher incidence of complications related to drug toxicity or overimmunosuppression.

Verrucous carcinomas represent rare, highly differentiated variants of squamous cell carcinoma. They preferentially develop on the oropharyngeal mucosa (Ackerman tumor), the urogenital mucosa (Buschke-Loewenstein tumors), and the soles of the feet (epitheliomata cuniculata). Various synonyma have been coined for these tumors. We report the uncommon occurrence of a large verrucous carcinoma on apparently uninvolved chest skin of a 79-year old patient. The tumor was excised radically. Complete extirpation was confirmed histologically and there was no sign of recurrence during a 3 month observation period. The clinical appearance, histomorphological features, epidemiological aspects, differential diagnosis, therapy, and nomenclature of verrucous carcinomas are discussed.

Hyperpigmentation of the skin is often refractory to conventional therapies, but has significant cosmetic implications if located on visible areas. Because laser systems are capable of removing pigment deposits caused by selective photothermolysis, we addressed the issue of whether the Q-switched ruby laser could be a useful alternative in the treatment of nonmelanotic hyperpigmented skin lesions. We report the successful treatment of a patient with hyperpigmentation caused by iatrogenic human herpesvirus 8-associated Kaposi's sarcoma and a patient with hyperpigmentation caused by long-term antimalarial therapy for cutaneous lupus erythematosus. In both patients, clinical lightening of the darkly pigmented lesions was seen after a single treatment, and a significant improvement was observed after 3 laser applications. The patients tolerated the laser therapy well without any short-term side effects and did not experience either scarring or considerable textural skin changes. Histologic examination was performed before and after laser treatment to confirm the reduction of the pigment deposits. Our data indicate that treatment of nonmelanotic skin hyperpigmentation with the Q-switched ruby laser might be a safe and powerful therapeutic method.

Background: Lupus erythematosus tumidus (LET) is characterized clinically by erythematous, succulent, edematous, nonscarring plaques in sun-exposed areas. Results of histological examination show perivascular and periadnexal lymphocytic infiltration and interstitial mucin deposition. The main differential diagnoses are polymorphous light eruption, Jessner's lymphocytic infiltration of the skin, reticular erythematous mucinosis, and pseudolymphoma. Since its first description in 1930, LET has been documented rarely in the literature, and its clinical importance has not been fully appreciated., Observations: We characterized 40 patients with clinical and histological features of LET observed at our department from 1984 through 1998. The onset of the disease clustered in summer because of sun exposure, and 28 (70%) of the patients showed a remarkable photosensitivity confirmed by results of provocative phototesting. A complete resolution of the skin lesions was seen after systemic therapy with antimalarials and, in some cases, with local corticosteroids or spontaneously without any treatment. In 4 (10%) of the patients, antinuclear antibodies were detected; however, there was no evidence of underlying systemic involvement in any of the patients., Conclusions: Our data constitute the largest number of patients with LET collected until now. The clinical picture, extreme photosensitivity, histological findings, and effective treatment with antimalarials are so characteristic that LET should be considered as a separate entity and differentiated from other variants of cutaneous LE. Arch Dermatol. 2000;136:1033-1041

Lichen myxoedematosus is an uncommon and distinct disease entity characterized by cutaneous mucin deposition which, depending on the distribution and overall skin involvement, can be classified into several subtypes. We now describe the case of a discrete papular type of lichen myxoedematosus in a patient without any conspicious laboratory findings including normal thyroid function and the absence of any abnormal immunglobulins.

Malignant melanomas frequently show loss of alleles on the long arm of chromosome 10. The PTEN (MMAC1) gene has been identified as a tumour suppressor gene at 10q23.3 that is mutated in various types of advanced human cancers. We have investigated a series of 40 sporadic melanomas from 37 patients (15 primary cutaneous melanomas and 25 melanoma metastases) for allelic losses on chromosome 10, as well as for deletion and mutation of the PTEN gene. Microsatellite analysis revealed loss of heterozygosity at loci located on 10q in tumours from 15 of 34 patients investigated (44%). Somatic PTEN mutations were identified in melanomas from 4 of 37 patients (11%), all of whom had metastatic disease. In two of these patients, the tumours had additionally lost one PTEN allele, indicating complete loss of wild-type PTEN in the tumour cells. Our findings corroborate that loss of heterozygosity on chromosome 10 is a frequent aberration in malignant melanomas and implicate PTEN as a tumour suppressor gene inactivated by somatic mutation in a fraction of these tumours.

Periorbital edema may occur initially or in the course of a wide variety of diseases. One of these diseases is lupus panniculitis, a variant of lupus erythematosus, characterized by firm subcutaneous nodules that may ulcerate with subsequent scar formation. We present a case of lupus panniculitis in which the periorbital edema was the initial manifestation. Knowledge about the differential diagnostic possibilities of periorbital edema as well as the different variants of cutaneous lupus erythematosus is important to reach the correct diagnosis.

Background: The results of a recent study suggested that ultraviolet A1 radiation (UVA1R; 340-400 nm) phototherapy for atopic dermatitis works through induction of apoptosis in skin-infiltrating helper T cells, indicating the possibility that other helper T cell-mediated skin diseases may respond to UVA1R as well., Objective: The purpose of this open pilot study was to assess the therapeutic effectiveness of UVA1 phototherapy for cutaneous T-cell lymphoma (CTCL)., Methods: UVA1 phototherapy was used as monotherapy in patients (n = 3) with histologically proven CTCL (stages IA and IB). For daily whole body UVA1 irradiations, either a high-dose (n = 2; 130 J/cm2 UVA1 per exposure) or medium-dose (n = 1; 60 J/cm2 UVA1) regimen was used. Therapeutic effectiveness was assessed clinically and histologically., Results: In each of the 3 patients, skin lesions began to resolve after only a few UVA1 radiation exposures. Complete clearance was observed between 16 and 20 exposures, regardless of whether the high- or medium-dose regimen had been employed., Conclusion: These studies suggest that patients with CTCL stages IA and IB can be treated effectively with UVA1 phototherapy.

Basal cell carcinoma (BCC), the most common malignant neoplasm of the skin, has many different clinical and histological appearances. Four patients with a new distinctive form, polypoid BCC, are described. Polypoid BCC is clinically distinguished from other types of BCC by being pedunculated and having a stalk that connects it to the skin surface. Histologically, it is distinctive in that the tumour aggregations are restricted to the exophytic polypoid zone.

We report the case of a caucasian woman who, between the ages of 49 and 51 years, developed multiple (> 20) basal cell carcinomas (BCC). There was no family history of BCC. No abnormalities in the human homologue of the Drosophila segment polarity gene patched (PTCH), glutathione S-transferases T1 and M1, or cytochrome P450 1A1 were detected by polymerase chain reaction (PCR)-based molecular analysis. There was, however, actinic damage of the skin in sun-exposed areas. The patient was diagnosed as having hairy cell leukaemia (HCL) at the age of 51 years, based upon leucocyte morphology as assessed by light and electron microscopy, tartrate-resistant acid leucocyte phosphatase (TRAP) staining, fluorescence activated cell scanning of peripheral blood leucocytes and bone marrow histology. As the leukaemia slowly progressed over a 3-month period, the patient developed four further BCCs. Given that HCL is characterized by a profound defect in T-cell function, it is conceivable that T-cell immune dysregulation can contribute to the pathogenesis of BCC, possibly enhancing the aetiological effect of ultraviolet irradiation.

About one-third of sporadic basal cell carcinomas (BCCs) of the skin and 10-15% of primitive neuroectodermal tumors (PNETs) of the central nervous system show mutations in the PTCH tumor suppressor gene. The PTCH gene product (Ptch) functions as a transmembrane receptor for the Sonic hedgehog protein (Shh) and interacts with another transmembrane protein called Smoh. To further elucidate the significance of alterations in the Shh signaling pathway, we investigated 31 sporadic BCCs and 15 PNETs for the mutation and/or expression of SMOH, PTCH, SHH, and GL11. In addition, we fine-mapped the SMOH gene locus by fluorescence in situ hybridization to chromosomal band 7q32. Mutational analysis identified four BCCs with somatic missense mutations in SMOH affecting codon 535 (TGG==>TTG: Trp==>Leu) in three tumors and codon 199 (CGG==>TGG: Arg==>Trp) in one tumor. A missense mutation at codon 533 (AGC==>AAC: Ser==>Asn) was found in one PNET. PTCH mutations were detected in eight BCCs and one PNET. Two BCCs demonstrated mutations in both SMOH and PTCH. The majority of tumors showed an increased expression of SMOH, PTCH, and GL11 transcripts as compared with that of normal skin and nonneoplastic brain tissue, respectively. In contrast, only one BCC and one PNET expressed SHH mRNA at levels detectable by reverse transcription-PCR, and no SHH gene mutations were found. In summary, our results indicate that both PTCH and SMOH represent important targets for genetic alterations in sporadic BCCs and PNETs.

Extramammary Paget's disease (EPD) is a rare malignancy occurring mainly in apocrine gland-bearing regions. Surgical excision is the treatment of choice. This may be very difficult or even impossible if the disease is widespread or located in a critical anatomical site. We report on the successful treatment of a 71-year-old man with EPD in the suprapubic region with CO2 laser guided by photodynamic diagnosis.

We report on 4 elderly patients aged 54-85 years with histopathologically diagnosed pilomatricoma who had been seen in our department from 1993 to 1997. Pilomatricoma is a rather uncommon neoplasm in middle-aged and old patients. It is much better known in children. Here, we clinically studied these 4 cases of pilomatricoma in elderly patients (above 50 years of age) to show the variety of clinical differential diagnosis and discuss the clinicopathological features. The results of the present study indicate that the differential diagnosis of pilomatricoma should also be considered in elderly individuals.

Linear IgA disease is characterized by circulating IgA autoantibodies recognizing basement membrane zone components, including an anchoring filament protein, ladinin. In this study, we have cloned the mouse ladinin cDNA, elucidated the intron-exon organization of the corresponding gene (Lad1), and determined its chromosomal assignment. We have also characterized the promoter region of Lad1 and examined its tissue-specific expression. The mouse Lad1 gene consists of 10 exons spanning approximately 13.4 kb of the mouse genome on chromosome 1, and Southern analysis suggested that Lad1 is a single-copy gene. The coding region comprises 1584 nucleotides and encodes a 528-amino-acid polypeptide with a calculated molecular mass of 59 kDa. The deduced polypeptide contained two putative N-glycosylation and two O-glycosylation sites, and sequence analysis predicted a 15-amino-acid signal peptide. The 5' upstream region demonstrated the presence of consensus cis-elements for AP2 and SP1 and was GC rich, consistent with eukaryotic promoter. Northern analysis revealed expression in cultured keratinocytes, but not in fibroblasts, with the mRNA transcript being approximately 2.5 kb in size. A significant level of expression was also noted in the kidney and lung, and to a lesser degree in the liver, spleen, and brain. Ladinin is a novel component of the basement membranes and may function in contributing to the stability of the association of the epithelial layers with the underlying mesenchyme.

Purpura fulminans must be treated as an emergency in internal medicine and dermatology. Its characteristic features are the sudden development of progressively enlarging haemorrhagic skin necrosis, severe disseminated intravascular coagulation with consumption of anticoagulant factors, and signs of shock. Purpura fulminans can be classified into a neonatal form with inherited protein C deficiency and an acquired type for which multiple causes are known. Clinically it is characterized by massive ecchymosis with haemorrhagic blebs and acral necrosis. Histologically the lesions show widespread extravasation of erythrocytes and thrombosis of small vessels. Thrombocytopenia, decrease of coagulation factors, the presence of fibrinogen split products and fragmented erythrocytes in the blood smear help to confirm the diagnosis. The therapy includes fresh-frozen plasma, heparin, antibiotics and surgical debridement of necrotic areas. It is important to recognize the disease promptly because the mortality rate is about 30-40% and only quick intervention helps to save the life of the patient.

CHILD syndrome (congenital hemidysplasia with ichthyosiform nevus and limb defects) occurs, as a rule, exclusively in girls because the underlying X-linked gene exerts a lethal effect on male embryos. In this report the characteristic manifestations of CHILD syndrome are described in a 2-year-old boy with a normal chromosome constitution 46,XY. This exceptional case is best explained by the assumption of an early somatic mutation and thus compatible with the concept of X-linked dominant male-lethal inheritance of this trait.

Subepidermal bullous dermatoses are a heterogenous group of disorders characterized by loss of tissue adhesion in the dermoepidermal junction and formation of a vesicle or bulla in the lamina lucida or under the lamina densa. These diseases can be classified into the following: 1. Subepidermal autoimmune bullous dermatoses. These are characterized by circulating autoantibodies against normal constituents of the basement membrane zone and include the following: Epidermolysis bullosa acquisita, bullous pemphigoid, dermatitis herpetiformis Duhring, linear IgA disease, herpes gestationis, cicatricial pemphigoid and bullous systemic lupus erythematosis (SLE). II. Subepidermal bullous dermatoses, due to mutation of the basement membrane zone proteins. These are epidermolysis bullosa junctionalis and epidermolysis bullosa dystrophicans. III. Subepidermal bullous dermatoses due to metabolic disorders: Porphyria cutanea tarda (PCT). IV. Drug induced Lyell-Syndrome Due to space limitation it is not possible to discuss the histology of all these dermatoses. I will therefore choose the most important of them and display their histology in detail.

The unusual case of an 8-year-old girl with a hair disorder is presented, characterized by brittle, short and sparse hair. On polarizing microscopy the latter reveals a "tiger tail" pattern, whereas severe cuticular defects are detected on scanning electron microscopy. The patient's hair has a normal cystine content but is completely devoid of methionine and reveals distinct changes of its visoelastic parameters. It is presently unknown whether the lack of methionine may be implicated in the pathogenesis of this hair disorder, which to the best of our knowledge has not been previously described.

We describe a 55-year-old woman with an amelanotic nodular malignant melanoma of the vulva. The tumour had developed within 3 months. Physical examination showed a solid, erythematous, centrally depressed nodular tumour with a waxy appearance. It was 1.3 cm across and located on the right labium majus. The surface was eroded and showed teleangiectasias. Histopathological examination revealed a nodular malignant melanoma with predominantly spindle-shaped tumour cells, stage IV-V, and 7.2 mm thick. The primary tumour was excised but the patient died of generalized metastases 19 months later. There are very few reports on melanoma of the vulva in the dermatological literature. In addition to this case report we review the literature and discuss the clinical picture, histopathology and prognosis of this melanoma.

Schönlein-Henoch purpura is characterized by palpable purpura, colicky abdominal pain, gastrointestinal hemorrhage, arthralgias, and renal involvement. Bacterial and viral infections, as well as drugs and diseases associated with immune complexes, are thought to be responsible. We describe the case of a 21-year-old woman with Schönlein-Henoch purpura and chronic active gastritis with erosions. Helicobacter pylori was found in gastric mucosa using the newly introduced, nontoxic, noninvasive 13C-urea breath test; infection was confirmed by gastric mucosal biopsy. After eradication of H. pylori with omeprazole and amoxicillin, the skin changes, gastric complaints, and proteinuria disappeared. Ten months later, Schönlein-Henoch purpura recurred. H. pylori was again detected. After therapy, H. pylori was eradicated and the clinical manifestations faded. To our knowledge, H. pylori has not previously been described as a cause of Schönlein-Henoch purpura.

We report a 39-year-old woman with agminated segmental naevi on the right side of her face. The lesions were present at birth, and she had no other congenital abnormalities.

X-linked dominant chondrodysplasia punctata is a rare genodermatosis characterized by transient punctate epiphyseal calcifications and ichthyotic skin changes, usually resolving during early infancy. We describe the case of a 32-year-old woman with ichthyotic skin lesions that developed during early childhood and persisted into adulthood. Psoriasiform skin changes became evident for the first time during adulthood. Both the ichthyotic and psoriasiform skin lesions followed Blaschko's lines. This case is unique because of the coexistence of ichthyotic and psoriasiform skin changes in an adult with X-linked dominant chondrodysplasia punctata.

Therapy with khellin and UVA irradiation (KUVA) is a therapeutic approach to vitiligo. Little is known about the photobiological properties of khellin and its long-term side-effects after prolonged administration. A 47-year-old woman suffering from acral vitiligo since the age of 3 is reported, who developed blisters on hands and fingers during KUVA-therapy. Laboratory findings were consistent with hereditary porphyria cutanea tarda. Electron microscopic examination of a blister revealed clefting below the basement membrane. It is proposed that khellin in some way provoked the porphyria cutanea tarda in this patient.

We describe a 59-year-old woman with amelanotic polypoid balloon cell melanoma. Physical examination showed exophytic erythematous tumour simulating a basal cell carcinoma or an adnexal tumour. Histopathology revealed a polypoid tumour that was composed mainly of balloon cells in sheets and nests. Examination 37 months after excision of the tumour with 3 cm safety margins disclosed no evidence of enlarged lymph nodes or cutaneous metastases. Metastases to internal organs could not be assessed because the patient repeatedly refused further examination for staging. To our knowledge our patient is the first case of amelanotic polypoid balloon cell melanoma to be reported in the German literature.

We describe a 44-year-old woman who had a unilateral relapsing linear dermatosis for 12 years. The lesions consisted of erythematous, discrete and grouped papules and papulovesicles that were localized to the left side of the upper and lower limbs, chest, abdomen, and back. They were distributed along Blaschko's lines. There was also a diffuse erythematous scaly hyperkeratosis of both palms. The lesions healed spontaneously. Examination of biopsy specimens from the back and the left palm revealed acute and subacute spongiotic dermatitis, respectively. This dermatosis was first described by Grosshans and Marot in 1990 and termed "Blaschkite de l'adulte." Because the disease is acquired, relapsing, heals spontaneously, follows Blaschko's lines, and is characterized histopathologically by a spongiotic dermatitis, we propose the term "acquired relapsing self-healing Blaschko dermatitis."

Although neurofibroma is a relatively common tumor, some histopathologic variants are so rare that they are not well known. In a study of 130 neural cutaneous tumors seen between 1986 and 1991 in the department of dermatology at the University of Heinrich-Heine, we identified 114 neurofibromas of different types. We present herein the histopathological features of these tumors. The differentiating features from other neural tumors--melanocytic or mesenchymal tumors that display "neuroid" features--are also discussed. also discussed.

A 62-year-old female patient presented with bullous, intensely itching cutaneous lesions, which clinically and histopathologically resembled dermatitis herpetiformis (Duhring's disease). Therapy with DADPS was unsuccessful. Because of associated cervical lymph node enlargement and splenomegaly, a lymph node biopsy was taken. Histopathology of a lymph node and of the bone marrow confirmed the diagnosis: angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma. Intensely pruritic associated skin eruptions are typical for this peculiar kind of lymphoma. These skin lesions are due to inflammatory cells and not to neoplastic infiltrations. This case report is the first report of AILD with bullous skin lesions to appear in the literature.

We report on the clinical, histopathological, and immunohistochemical features of a case of cellular schwannoma. This rare benign nerve sheath tumor merits recognition because it frequently shows moderate nuclear atypia and mitotic activity leading in some instances to the false diagnosis of sarcoma.

We report the clinical, histopathologic, and immunohistopathologic features of two cases of plexiform schwannoma. This rare benign tumor is worthy of recognition because it can be misdiagnosed as plexiform neurofibroma. Differentiation from plexiform neurofibroma is important because the latter is pathognomonic of von Recklinghausen disease and carries a significant risk of malignant transformation. Plexiform schwannoma is, in contrast, not necessarily associated with von Recklinghausen disease and malignant transformation has never been observed.

Although palisaded encapsulated neuroma was first described over 20 years ago, it has received little attention. We present the clinical, histopathologic, and immunohistochemical features of 10 cases of this entity. The cases were studied by routine light-microscopic examination and immunohistochemistry using the avidin-biotin-peroxidase technique. Clinical information and follow-up data were obtained from the hospital records. The lesions were solitary, asymptomatic, skin-colored papules that were located on the faces of patients who ranged in aged from 37 to 66 years (average, 52 years). The female to male ratio was 1:1. None of the patients had von Recklinghausen's disease, and none of the lesions recurred after excision. Histopathologically, the tumors were well circumscribed and situated predominantly in the dermis. They were encapsulated and composed of spindle cells arranged in interlacing fascicles. The tumor cells were positive for S-100 protein. The capsule was composed of flattened, elongated cells that showed positivity for epithelial membrane antigen (EMA).

The report deals with the clinical features of a 26-year-old man with the extraabdominal form of deep fibromatosis, which became manifest at the age of 18 years as acute ileus caused by diffuse intraabdominal fibromatous tumours. In the later course of the disease extensive fibromatous lesions developed, mainly at the skin. In addition, multiple keloids occurred after excision or after negligible traumas. The pathogenetic events of the clinical picture described may be linked to the activation of mesenchymal cells leading to the generation of either fibromatous tumours or posttraumatic keloids.

We report on a 78-year-old woman with a 47-year-history of lichen sclerosus et atrophicus of the vulva on which squamous cell carcinoma developed. The lesion started as a small nodule that increased gradually in size and became eroded. The tumour and the inguinal lymph nodes on both sides were excised. Physical examination, chest X-ray and sonography of the abdomen 15 months later showed no local recurrence or distant metastases. Lichen sclerosus et atrophicus of the vulva should be considered a possible precancerous condition.

The treatment of epidermolysis bullosa acquisita (EBA) is difficult and often disappointing. We report on the successful treatment of two EBA patients with colchicine. The drug was administered orally at an initial dose of 2 mg/day. After 2 weeks of therapy a dramatic improvement was observed. Most of the cutaneous and buccal mucosal lesions had healed and both of the patients were able to go about their normal daily activities. In the first patient the disease was refractory to dapsone alone or combined with steroids. In the second patient no other treatment was tried. After 6 months a maintenance dose of 1 mg/day colchicine was given. The disease had remained stable in both patients at the time of writing for more than 8 months. No side effects were observed. We suggest that colchicine may be a helpful and safe drug for patients with EBA.

One of the most important tools for the development of Diesel engines is the numerical simulation of the combustion processes. One of the key variables for such simulations is the liquid-phase temperature. The potential study of a fluorescence thermometer for Diesel spray investigations is discussed. Nonintrusive measurements of the liquid-phase temperature in a Diesel spray have been carried out for the first time to the author's knowledge. The results are presented as two-dimensional temperature maps.