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The clinical findings of systemic amyloidosis are often variable and nonspecific. The early recognition of this disorder relies upon clinical suspicion, and definite diagnosis requires the confirmation of amyloid deposits in tissue. We reviewed 31 skin biopsy specimens from 20 patients, including 17 with amyloid light chain (AL) protein amyloidosis and 3 with serum amyloid A (AA) protein amyloidosis. Histologically, amyloid can be present in the papillary dermis, reticular dermis, subcutis, around the appendages, and in or around blood vessels. In our series, all 20 patients had at least one positive skin biopsy. Amyloid was found in 30 of 31 specimens (97%), a higher rate than in most series. Skin biopsy is a simple, safe procedure with high yield and may be used to obtain a firm diagnosis of systemic amyloidosis.

We report a case of Wells' syndrome (eosinophilic cellulitis) with acute cutaneous swelling followed by indolent infiltration. The histopathology is characterized by a dense infiltrate of eosinophils and "flame figures" in the dermis. The electron microscopic findings are peculiar. This case responded well to oral antihistamines. The skin signs and symptoms disappeared completely two weeks later.

We report a case of systemic amyloidosis with the initial sign of polyneuropathy. The patient eventually developed heart failure, macroglossia, and ecchymoses 6 years later. Biopsies from the sural nerve and normal-looking skin both proved the existence of amyloid.

We reviewed 86 cases of bullous pemphigoid and the results were compared with those reported in the literature. Seventy-eight per cent of the patients developed generalized blisters and 22% had localized blisters, including three cases of dyshidrosiform pemphigoid and one case of pretibial pemphigoid. Oral mucosal involvement was noticed in 12.8% of the patients. Fifteen per cent of the patients had internal malignancies but the incidence was not significantly different from the control group. Direct immunofluorescence in our series showed a high positive rate of 98.8%. Indirect immunofluorescence was positive in 48.1% of the 54 patients in whom this was carried out. Peripheral blood eosinophilia was observed in 22.1% of the patients. Prednisolone alone or in combination with immunosuppressive agents was the mainstay of treatment. Treatment side-effects was observed in 33% of the patients. Thirty per cent of the patients had a complete remission after a mean follow-up period of 26.9 months. Bullous pemphigoid (BP) is a chronic debilitating autoimmune blistering disease. It is characterized clinically by generalized tense bullae and histologically by subepidermal blisters. Immunofluorescence is crucial in the diagnosis and shows linear deposits of C3 and/or IgG at the basement membrane zone (BMZ). Information regarding this disease in Chinese patients is quite limited. In this study, 86 patients with BP were reviewed. The clinical and histological features, immunofluorescence, modes of therapy and outcome were studied.

Extramammary Paget's disease (EMPD) is more frequently seen in Caucasian females than in males (3.2:1 female: male ratio). During the past 14 year period, we have collected 22 patients, all Chinese males, with EMPD. They presented with eczema-like lesions in the early stages in the genital or perianal regions. Histological sections showed Paget cells within the epidermis or skin appendages and even within the dermis. No underlying adnexal carcinoma or adjacent internal carcinoma could be detected after thorough examinations. Mode of therapy and outcome are presented. EMPD seems to affect more males than females in Orientals. The incidence of concomitant malignancy in Chinese male patients with genital Paget's disease seems to be much lower than that in Caucasians. However, if EMPD involves the glans penis or perianal area, a search for internal malignancy is still warranted.

A 10-year-old Chinese boy with the characteristic skin manifestations of dyschromatosis universalis is described. In addition, the patient had congenital nystagmus with poor visual acuity, and ophthalmological examination revealed foveal hypoplasia and albino-like fundi. Histopathology showed giant pigment granules in the skin. Based upon the finding of giant pigment granules in clinically normal skin of the patient's mother, the patient was diagnosed as a case of dyschromatosis universalis with X-linked ocular albinism. To the best of our knowledge, this is the first documented case of this combination.

It has long been recognized that certain characteristic mucocutaneous symptoms and signs may act as important clues in diagnosing the early stage of systemic amyloidosis. Since the first description of clinical cutaneous lesions of primary amyloidosis by Koenigstein 1 in 1925, the spectrum of mucocutaneous manifestations in this disorder has been well documented. 2–7 There is no truly satisfactory classification of systemic amyloidosis; however, systemic amyloidosis could be simply classified as follows: 1.1. Primary systemic amyloidosis: usually no evidence of preceding or coexisting disease but paraproteinemia or plasma-cell dyscrasia can be found. 2.2. Amyloidosis associated with multiple myeloma. 3.3. Secondary systemic amyloidosis: evidence for coexistence of previous chronic inflammatory or infectious conditions. 4.4. Heredofamilial amyloidosis. Based on the result of amyloid protein analysis, Glenner 8 has proposed a more detailed clinical-chemical-pathological classification for human amyloidoses. It appears, however, that this classification is overly complex for clinicians, thus its clinical usage seems rather limited. In primary systemic and myeloma-associated amyloidosis, deposits consist mainly of light-chain (AL) amyloid protein produced as a result of plasma-cell dyscrasia. In a large series of 236 cases of systemic amyloidosis, 4 56% were of primary type and 26% were multiple-myeloma patients. During the period 1978–1987, we have found 14 cases of systemic amyloidosis in our hospital; primary systemic amyloidosis with a type of paraprotein and amyloidosis secondary to multiple myeloma occurred in nine cases (64%) and five cases (36%), respectively. Multiple myeloma associated with amyloidosis has been widely studied in medicine. It has been pointed out that amyloidosis develops in 6–15% of patients with myeloma. 9 In our hospital, five cases (4%) out of 159 myeloma patients showed an association with systemic amyloidosis.

A mass outbreak of poisoning occurred in central Taiwan in 1979 due to the ingestion of rice-bran oil contaminated with polychlorinated biphenyls (PCBs). In addition to PCBs, polychlorinated dibenzofurans (PCDFs) and polychlorinated quaterphenyls (PCQs) were also found in the contaminated oil. The levels of toxic agents in the rice-oil samples collected from factory and school cafeterias and the families of the intoxicated patients were in the range of 53-99 ppm, 0.18-0.40 ppm, and 25-53 ppm for PCBs, PCDFs, and PCQs, respectively. Major components of PCBs and PCDFs in the toxic oil were separated and identified by gas chromatography (GC) and gas chromatography/mass spectrometry (GC/MS) using glass capillary columns. The most toxic PCB reported in commercial PCB preparations, 3,4,3',4'-tetrachlorobiphenyl, was found in the toxic oil as well as in one of the patients' blood and adipose tissue at an early stage of poisoning. The blood samples of 165 patients collected 9 to 18 months after the onset of poisoning contained 10 to 720 ppb of PCBs with a mean value of 38 ppb. One of the most toxic PCDFs, 2,3,4,7,8-pentachlorodibenzofuran, was retained in the liver of a deceased patient. This compound could play an important role in the etiology of PCB poisoning in Taiwan.

A 65-year-old women with systemic amyloidosis was given dimethyl sulfoxide orally for 4 years without significant side effects. Her cutaneous lesions improved markedly after the treatment, and she still survives in satisfactory condition.

One Chinese construction worker and a Chinese cook experienced unknown insect bites during their stay in Abha of Saudi Arabia and then developed skin ulcers. After returning to Taiwan, Republic of China, they were diagnosed in our hospital as having cutaneous leishmaniasis on clinical and dermatopathologic grounds. We were successful in culturing the Leishmania organism with Tobie medium and liquid metacyclic stage culture medium from the skin ulcers of these two patients. The electron microscopic findings of the parasites, Leishmania tropica, both in the tissue (amastigote) and in the cultured medium (promastigote), were also reported.

A 41-year-old patient with classical manifestations of hyalinosis cutis et mucosae was presented. This rare genodermatosis has mainly been described in Caucasian patients. Our case is the first reported case of hyalinosis cutis et mucosae in a Chinese patient. The patient was given dimethyl sulfoxide orally. The possible mechanism of this therapy is discussed.

We report the successful treatment of lipoid proteinosis in a 41-year-old man using oral dimethyl sulphoxide. The initial dosage was 40 mg/kg/day, which was then increased progressively to 60 mg/kg/day. After 3 years of treatment, the patient's skin lesions, hoarseness of voice and abnormal oesophageal function improved remarkably. No side-effects were noted except for a garlic-like smell on the patient's breath.

To the Editor.— Calcinosis cutis may be the most troublesome and debilitating complication in juvenile dermatomyositis. The pathophysiology of this peculiar state is unknown. Treatment, so far, remains unsatisfactory. We report a case of juvenile dermatomyositis with calcinosis cutis that showed remarkable response to orally administered aluminum hydroxide. Report of a Case.— A 13-year-old girl was admitted to our department in August 1986. The patient suffered from multiple erythematous, slightly elevated, papular eruptions over the cheek, trunk, both arms, and both legs, associated with proximal muscle weakness and fever, at the age of 2 years. The diagnosis of juvenile dermatomyositis was made in another hospital, and the patient was treated with low-dose orally administered prednisolone thereafter. In 1982, a flare-up of skin lesions occurred and was followed one year later by multiple yellow-white nodules and plaques protruding over the forehead, back, and upper extremities. On examination, the child appeared with

• Seven patients with lichen amyloidosus were treated with dermabrasion during the last seven years. All patients have had follow-up examinations for at least five years (one patient for seven years). The main symptom, itching, disappeared in all cases, and the clinical results were satisfactory, except for mild hyperpigmentary and depigmentary changes in the treated areas. ( Arch Dermatol 1982;118:302-304)

Thirty cases of lichen amyloidosus were found in a skin clinic during one year. The peak incidence occurred in patients between 40 and 60 years of age. Most cases had long durations, ranging from 5 to 20 years. The extensor surfaces of the limbs were most commonly involved. No evidence of systemic involvement was found.