Pretibial dystrophic epidermolysis bullosa with localized cutaneous amyloidosis: coincidental or secondary amyloidosis?

Here, we describe the case of a patient with pretibial dystrophic epidermolysis bullosa (PDEB) with amyloid deposition. The patient was a 40-year-old Japanese woman who presented a blistering eruption in the pretibial area with flat violaceous-brown lichenoid papules. The histology of the blister revealed a subepidermal bulla with antibodies bound to basement membrane antigens on the blister roof by immunoflourescent mapping. Electron microscopy revealed a blister cleavage plane below the lamina densa. The histology of the lichenoid papules showed amyloid deposition in the papillary dermis. Because it was confined to just beneath the bulla base of the blister specimen, the amyloid deposition may have been derived from degenerated keratinocytes induced by damage to the epidermal-dermal junction due to blister formation in PDEB in this case. PDEB, in general, is often misdiagnosed as lichen amyloidosis; however, some PDEB cases could actually be associated with amyloid deposition.

Clinical features of antinuclear antibody-positive patients with atopic dermatitis.

Twenty to thirty percent of patients with atopic dermatitis (AD) are positive for antinuclear antibodies (ANAs). In this study we investigated the prevalence of ANA in 100 patients with AD and examined the difference between ANA-positive (ANA+) and ANA-negative (ANA(-)) patients with AD. ANAs were identified with indirect immunofluorescence on Hep-2 cells. Nineteen patients (19%) with AD were found to be positive for ANAs at titers ranging from 1 : 40 to 1 : 640. The rate of ANA positivity in male patients (20.4%) was higher than that in female patients (17.6%). The rate of ANA positivity differed significantly between patients with AD and healthy control subjects (p=0.0001, odds ratio: 2.8). There was also a relationship between ANA+ AD and photosensitivity in male subjects (p=0.0346). The ANA+ patients with AD showed higher levels of cedar pollen-specific IgE than did ANA(-) patients (p=0.0232). In ANA+ patients disease severity was correlated with basophil counts (r=0.513, p=0.0344) and serum LDH levels (r=0.741, p=0.0056). The results indicate that patients with AD who are positive for ANA are a subpopulation of patients with AD.

Case of anaphylaxis caused by ingestion of royal jelly.

Anaphylaxis is a severe form of allergic disease. Royal jelly is widely consumed in Japan, but a few cases of anaphylaxis caused by royal jelly have been reported. We encountered a 26-year-old Japanese woman who developed anaphylaxis after drinking a beverage of crude royal jelly including honey. She had a history of bronchial asthma, allergic rhinitis, allergic conjunctivitis, atopic dermatitis and food allergy (abalone). Prick tests were performed with the beverage of crude royal jelly including honey and with two other beverages including honey. Only the crude royal jelly beverage showed a positive reaction. An oral challenge test with the crude royal jelly beverage was not performed, but a similar test with a beverage including honey caused no symptoms. A positive response to the beverage of crude royal jelly was not observed in normal volunteers. A positive diagnosis of anaphylaxis due to royal jelly was made based on the positive prick test, systemic clinical symptoms and the negative prick tests in healthy volunteers. Moreover, the patient had no symptoms when taking lemon and orange, which were present as essences in the crude royal jelly beverage, and also had no response to honey after anaphylaxis. Increased consumption of royal jelly in health food supplements may increase the incidence of royal jelly-related allergic reactions. Therefore, royal jelly should be considered as a causative allergen in food-induced anaphylaxis.

Urticarial vasculitis appearing in the progression of systemic sclerosis.

We report a case of urticarial vasculitis that appeared during the course of limited cutaneous systemic sclerosis. The urticarial lesions responded to systemic administration of prednisolone. After the appearance of urticarial vasculitis, the progression of scleroderma in distal sites of her elbow and knee joint became apparent. We consider this case to be consistent with limited cutaneous systemic sclerosis. The patient started treatment with prednisolone and her edema as well as scleroderma softened gradually. We analyzed, by immunohistochemistry, the number of tryptase-positive mast cells of this case in the lesions of urticarial vasculitis as well as systemic sclerosis. The number of tryptase-positive mast cells in the lesions of urticarial vasculitis as well as systemic sclerosis was significantly increased compared to normal skin (P < 0.05 and P < 0.005, respectively). We demonstrate that, in the present case, mast cells might be involved in both courses of urticarial vasculitis and systemic sclerosis as a common factor.

A case of peripheral T-cell lymphoma unspecified involving subcutaneous tissue.

A 36-year-old man presented with a 3-year-old red-brown plaque with subcutaneous nodules on his left thigh. Although a similar lesion was observed on his right thigh 3 years earlier, it spontaneously disappeared 1 year later. However, the lesion on the left thigh was growing larger. Histologically, the lesion showed a diffuse and dense infiltration of atypical lymphocytes extending from the superficial dermis to the subcutaneous tissue. Severe lobular panniculitis, composed of small- and medium-sized atypical lymphocytes and large normal histiocytes, was observed in the subcutaneous adipose tissue. Immunohistochemical studies revealed a post-thymic T-cell phenotype. A genetic analysis demonstrated a rearrangement of the T-cell receptor chain gene. The left skin lesion also gradually disappeared after skin biopsy without therapy, and he continues to be in remission.

Granulocyte adsorptive apheresis for leg ulcers complicated by rheumatoid arthritis: a report on three successfully treated cases.

BACKGROUND: The treatment of inflammatory leg ulcers complicated by rheumatoid arthritis (RA), which are unresponsive to conventional care, can be frustrating. Furthermore, as granulocytes and monocytes (GM) are major sources of inflammatory cytokines, they have the potential to initiate and perpetuate inflammatory skin lesions. Accordingly, a recent study reported the remission of pyoderma gangrenosum following the reduction of activated peripheral blood GM by adsorptive apheresis (GMA). METHODS: In this clinical study, we applied GMA to three cases, each with one leg ulcer below the knee and RA. The ulcers had not responded to conventional therapy, including disinfection, dressing, and antimicrobials, and therefore were thought to represent inflammatory vasculitic lesions. GMA was performed using a column with a capacity of 335 mL, filled with cellulose acetate beads that selectively adsorb granulocytes and monocytes/macrophages (Adacolumn). Each patient received one GMA session/week for five consecutive weeks. The duration of one session was 60 min, with a flow rate of 30 mL/min. RESULTS: The ulcers began to recede after two GMA sessions and, by the end of the fifth session, the ulcers in all three patients had healed. No recurrence has been observed up to the time of this report. The treatment was well tolerated and no severe side-effects were observed. CONCLUSIONS: GMA, which depletes activated neutrophils and monocytes/macrophages, appears to be effective for inflammatory skin ulcers which do not respond to conventional medications.

[The effect of suplatast tosilate on the patients with atopic dermatitis--relationship between clinical symptoms and immunological parameters].

The Th2 cytokine inhibitor, suplatast tosilate (300 mg/day) was administered to 45 cases of patients with atopic dermatitis for 8 weeks. The clinical scores, peripheral blood eosinophil counts, serum LDH levels, total IgE levels, serum eosinophil cationic protein (ECP) levels, and serum IL-5 levels before and after the treatment were observed and comparatively evaluated. The results of this study were summarized as follows. 1) Temporary improvements were found in the severity score, itching score, and sleeplessness score. All evaluated scores decreased significantly for all observation periods at 2, 4, 6 and 8 weeks after administration of suplatast tosilate compared with those before the administration. 2) In severe group, there was a significant improvement of severity score of lower limb. In moderate group there were significant improvements of severity score of head, face, neck and of upper limb. There were significant improvements of severity score of trunk and upper limbs in mild group. 3) The peripheral blood eosinophil counts and serum LDH levels significantly diminished compared with those before administration, but no significant difference was found in total IgE levels and serum ECP levels. 4) The serum IL-5 levels decreased after administration, however, there was no statistical significance. 5) The positive correlations between delta-severity score and delta-peripheral eosinophil count, delta-serum LDH levels, delta-serum ECP levels were found. 6) The positive correlations between delta-peripheral eosinophil count and delta-serum LDH levels, delta-serum ECP levels were observed. 7) There was no sign of adverse effects of the drug. From the above mentioned results, we confirmed the high efficacy of suplatast tosilate in the treatment of atopic dermatitis.

[Clinical analysis of 22 cases with syphilis racenta in Department of Dermatology, Nippon Medical School].

Forty-nine patients with syphilis were seen from January 1996, to June 2000 at the Dermatological Clinic of Nippon Medical School Hospital. The frequency of syphilis among all outpatients was 0.17%, and the number of male syphilis patients was almost twice that of female syphilis patients. Many sexual contacts (especially with female prostitutes) were considered to be the source of the infection in a large proportion of the syphilis patients. Chancres were observed in 50% of the 6 patients with primary syphilis. Macular or papular syphilide and psoriasis syphilitica were the most frequently observed symptoms in the patients with secondary syphilis. The Jarisch-Herxheimer reaction was observed in 18.8%. The titer of IgM-TPHA responded well to the therapy, and decreased or even disappeared after treatment. The titer of TPHA did not change markedly upon treatment. A retrospective study of syphilis from 1980 revealed that the incidence of syphilis, especially early infectious syphilis, in patients at our clinic has decreased markedly since 1991.

Two cases of lymphomatoid papulosis in children.

An 8-year-old Japanese boy had a 4-month history of self-healing crops of violaceous, scaling papules and several small indurated areas of erythema on his limbs, buttocks, and trunk. Histologically there was an infiltration of small lymphocytic cells with scattered large atypical cells expressing CD30. Characterization of T-cell receptor gene rearrangement showed monoclonality of the infiltrating cells. The second patient, a 15-year-old Japanese girl, had a 2-week history of self-healing papulovesicular eruptions on her face and limbs. Large CD30+ atypical cells were also noted in the perivascular lymphocytic infiltration. Immunohistochemical studies revealed CD8 expression on almost all CD30+ cells in the second case. In the literature, there have been two reports of children with large CD30+ atypical cells expressing CD8 and two cases expressing CD4, whereas all adult cases reported have had cells expressing only CD4.

Mast cells in basal cell carcinoma express VEGF, IL-8 and RANTES.

BACKGROUND: Basal cell carcinoma (BCC) is the most common malignant tumor of the skin. Although an increase in mast cell infiltration was observed in BCC lesions, definite evidence of an active role of mast cells in the pathogenesis of BCC is still lacking. BCC is accompanied by cellular infiltrates. Moreover, the stroma in the BCC lesions is characterized by an increased number of mast cells and increased vascularity. The aim of this study was to elucidate the probable role of mast cells in BCC, especially focusing on the relationship between mast cells and lymphocytic infiltration as well as angiogenesis. METHODS: We examined the expression and distribution of VEGF, IL-8 and RANTES in 16 nodular BCC lesions by immunohistochemistry. We also examined the lymphocyte subset, and the correlation between VEGF expression and microvessel density in the BCC lesion. mRNA expression of IL-8 and RANTES was examined by the reverse transcriptase-polymerase chain reaction. RESULTS: T cells, especially CD4+/CD45RO+ memory T cells were the predominant infiltrating lymphocyte population in BCC lesions. An increased number of tryptase+ cells (mast cells) was found in the stroma. VEGF+/IL-8+/RANTES+ cells were also found abundantly in the stroma of all BCC lesions. The number of VEGF+, IL-8+ and RANTES+ cells was significantly higher than that in controls. By immunohistochemistry of serial sections, tryptase+ cells were found to express VEGF, IL-8 or RANTES. Messenger RNA expression of IL-8 and RANTES was also observed in the BCC lesions. CONCLUSION: This study suggests that mast cells may play an active role in the angiogenesis of BCC through the production of VEGF and IL-8. Furthermore, mast cells may also regulate lymphocytic infiltration through the production of IL-8 and RANTES.

Lichen aureus.

Lichen aureus is a localized variant of pigmented purpuric dermatitis (PPD) and is rarely reported. A case of lichen aureus in a 28-year-old woman and a 13-year-old boy are presented. Biopsy results revealed the characteristic pattern of lichen aureus, including diffuse deposition of hemosiderin and dermal infiltrate of lymphocytes and histiocytes, some of which had Birbeck granules in the cytoplasm. The former case is unusual for its zosteriform distribution and localization (lower abdomen).