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A 51-year-old, previously healthy white man presented with a 1-year history of tightening of the skin about his hands, fingers, upper trunk area, and perioral region, first noted following an influenzalike illness. The skin tightening progressed to involve his ears, neck, back, arms, and thighs, and he began noting decreased grip strength and hand coordination as well as difficulty in swallowing. A review of the systems demonstrated dysphagia and weight loss. There was no history of tryptophan use or significant industrial chemical exposure. The physical examination revealed a thin man in no distress. The skin examination revealed a generalized erythematous to violaceous hue with induration of the anterior aspect of the upper trunk area and arms (Figure 1), hands, thighs, neck, ears, and face. The maximal oral aperture was greatly decreased. Multiple flesh-colored to slightly yellow, 1to 2-mm-diameter papules were present bilaterally on the dorsal aspect of the hands and fingers (Figure 2) and the anterior aspect of the neck. Grip strength, finger flexion, and neck rotation were all decreased. There was no notable proximal nail fold capillary dilatation, cutaneous calcinosis, or telangiectases. The results of the following laboratory tests or determinations were normal or negative: liver and thyroid function; complete blood cell count with differential; electrolyte, aldolase, creatine kinase, and complement levels (C3, C4, and CH50); erythrocyte sedimentation rate (5 mm/h); fluorescent antinuclear antibody (.1:40 and ,1:160 with nucleolar staining); anti–SCL-70 and anticentromere antibodies; rheumatoid factor; urine protein electrophoresis; barium swallow; 12-lead electrocardiography; and posterior-anterior and lateral chest radiography. Serum protein electrophoresis revealed a monoclonal IgG-l peak of 0.1 g/dL. A punch biopsy specimen was obtained from 1 of the papules on the anterior lateral aspect of the left side of the neck and revealed a hypercellular dermal infiltrate of spindle-shaped fibroblasts (Figure3) with increased mucin deposition on colloidal iron stain (Figure 4). What is your diagnosis?

REPORT OF A CASE A 68-year-old West African man presented with a 4-day history of a tender eruption on the face, ears, trunk, and upper thighs. There was no associated fever, weakness, or arthralgias. A similar eruption had been recurrent for several years, with each episode responding to oral prednisone prescribed by a local physician in the patient's native country. The patient had a medical history notable for treated syphilis and poliomyelitis. He worked as a grocer and had recently arrived in Boston, Mass, to visit family members. Physical examination revealed an afebrile man with several erythematous, tender, infiltrated plaques and nodules, ranging in size between 2 cm and 6 cm, distributed over the right infraorbital skin, the pinnae of the ears with sparing of the lobules, the upper trunk, arms, and proximal thighs. The mucous membranes, sclerae, nose, nails, and joints appeared normal. There were no palpable nerves and

REPORT OF A CASE A 44-year-old white man was seen for a minor dermatitis. He noted no changes in his skin. His recent history was remarkable for chronic renal failure (requiring peritoneal dialysis), hypertension, mild hypothyroidism, chronic sinusitis, and trigeminal neuralgia. As a young adult, he experienced three spontaneous pneumothoraces necessitating cautery. During childhood, he suffered from heat intolerance associated with hypohidrosis. He had no history of bleeding disorders. Family history was noncontributory and family members were not examined. His medications were carpamazepine, nifedipine, levothyroxine, and calcitriol. Complete skin examination revealed linear and macular telangiectases of the lips (Figure 1), buccal mucosa, and upper trunk. Several 1- to 2-mm red papules were present on the thighs, scrotum, and umbilicus (Figure 2). A punch biopsy specimen from an abdominal lesion is shown inFigure 3andFigure 4. On slit lamp examination of the eyes, faint radial lens and corneal

REPORT OF A CASE For over 20 years, a 37-year-old man noted numerous lesions over his upper trunk and shoulders ( Figure 1 ) that were associated with mild irritation. The lesions were more distinct in the summer as they failed to tan. The lesions consisted of peculiar linear and angulated erythematous pale macules with an adherent scale ( Figure 2 ). These tended to be confluent and gave the skin a reticulated appearance. The distribution of the lesions had remained stable for many years. The family had no known history of similar skin findings. Multiple skin biopsy specimens showed identical features ( Figure 3 ), and these were accentuated with the orcein and Giemsa stains, highlighting the elastic fiber network in the dermis ( Figure 4 ). What is your diagnosis? Figure 1. Figure 2. Figure 3. Figure 4. SECTION EDITOR: ANTOINETTE F. HOOD, MD Peculiar Linear and Angulated Lesions in a Mantle Distribution Over the Upper

We found the case of ''Multiple Facial, Neck, and Upper Trunk Papules in a Black Child'' reported by Hansen et al 1 in a recent ''Off-Center Fold'' section of theArchivesmost interesting, as we believe it is the first to document extrafacial involvement of this condition. We were disappointed, however, that like the report by Frieden et al 2 of granulomatous perioral dermatitis, the authors have missed the earlier British literature describing this condition. 3,4 The condition was probably first described in detail in 1974 by our senior colleagues at King's College Hospital, London, England, as ''An Unusual Papular and Acneiform Facial Eruption in the Negro Child.'' 3 They reported 22 cases with clinical features identical to those described later by Frieden et al, 2 although granulomas were not seen on histologic examination. At the December 1987 meeting of the St John's Hospital Dermatological Society, we presented two children

REPORT OF A CASE A 67-year-old white man with metastatic melanoma began therapy with continuous intravenous suramin infusion. On the third day of treatment, an asymptomatic eruption developed over his upper trunk and arms. Numerous nonconfluent 2- to 3-mm papules, pustules, and vesicles on an erythematous base were present (Fig 1). They were randomly distributed. The patient otherwise felt well, and the findings from his physical examination were normal, except for the cutaneous changes. A punch biopsy specimen of one of the papules is shown in Figs 2 through 4. What is your diagnosis? DIAGNOSIS: Squamous syringometaplasia (squamous metaplasia) of the eccrine glands. HISTOPATHOLOGIC FINDINGS An acanthotic squamous epithelium with overlying parakeratosis was seen. Moderate dysplastic features were present in the epidermal keratinocytes and the acrosyringium. A mild chronic inflammatory infiltrate was seen in the upper dermis. The dermal eccrine coil and glands were uninvolved

REPORT OF A CASE A 4.5-year-old black boy was referred to the pediatric dermatology clinic for evaluation of numerous, slightly pruritic papules distributed on the face and neck and the upper aspects of the chest and back. The papules erupted initially on the face approximately 1 year before our evaluation; over the ensuing year, they had developed on the neck and the upper aspect of the trunk. The child had a history of infantile atopic dermatitis but was otherwise healthy. Family history was notable for eczema and asthma in several maternal family members. The papular eruption had been treated with various low- to high-potency steroid ointments, antifungal creams, systemic corticosteroids, and antibiotics. With the exception of transient resolution during a course of prednisone therapy, the eruption was refractory to treatment. Examination showed numerous infiltrated papules, skintone to erythematous, ranging in size from 1 to 4 mm, located in the infraorbital

IN 1964, Steele et al 1 focused attention upon "a distinct clinicopathological syndrome" which they termed progressive supranuclear palsy. The disorder was characterized clinically by ophthalmoplegia, mainly affecting vertical, and particularly downward gaze, pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and upper trunk, and by other less constant cerebellar and pyramidal signs. It was emphasized that intellectual impairment was mild. The pathologic picture was one of neuronal loss, gliosis, neurofibrillary tangles, granulovacuolar degeneration, and demyelination which they believed to represent a degenerative disease, affecting chiefly the brain stem, basal ganglia, and cerebellum. There have been subsequent reports of this disorder, some with minor variation from the complete picture described by Steele et al, 1 but always with striking ophthalmoplegia and tonic rigidity as the outstanding clinical features. The purpose of this report is to present the second case of a woman studied pathologically as well as clinically, and

To the Editor.— We read with interest the report on electrophysiologic evaluation in brachial plexus neuropathy by Flaggman and Kelly (Archives37:160-164, 1980). We recently reported a similar study that involved ten patients with idiopathic and three with familial brachial plexopathy. 1 There were no distinguishing features between the groups. Five patients had denervation in predominantly upper trunk distribution, four had diffuse plexus involvement, three had very patchy denervation not easily explained by circumscribed lesions of the brachial plexus, and one had denervation in muscles supplied by the posterior cord. Nerve conduction studies (NCSs) in six patients gave abnormal results, comprising low amplitude of evoked sensory or compound muscle action potentials, together with normal conduction velocities in the elbow to wrist segments. In one of our patients, sensory nerve action potentials were intact when muscles sharing their segmental and peripheral innervation were severely denervated. Some patients showed unusual patterns of

Three patients suffered injury to the upper trunk of the right brachial plexus from firearm recoil. The proposed mechanism is entrapment of the upper trunk of the plexus between the forcefully retracting clavicle and the underlying scalene muscles. Two patients had only temporary disability, but the third continued shooting and his deficit is permanent.

REPORT OF A CASE A 63-year-old man presented with a one-year history of cutaneous lesions that had progressively increased in size and number. They initially appeared on his abdomen and then spread to his upper trunk, neck, face, and arms. The lesions, beginning as small papules, enlarged over several months to form plaques or nodules, some of which ulcerated and slowly healed with atrophy and scarring. The patient had noted easy fatigability and a 9-kg weight loss over the preceding four months. Physical examination revealed a rather thin, pale man with approximately 100 papules, plaques, and nodules on his abdomen, chest, back, neck, arms, and face. The lesions were yellow-brown and most had a faint rim of erythema. A few were ulcerated, while others were so deep that they were more palpable than visible (Figs 1 through 3). The remaining physical examination was remarkable only for a massively enlarged spleen.

To the Editor. —I read with great interest the excellent review by Kline et al1in which the diagnosis and treatment of brachial plexus lesions was discussed. I would dispute the statement that "there are no good sensory stimulation and recording sites to test the C-5 root." The lateral cutaneous nerve of the forearm is a terminal sensory branch of the musculocutaneous nerve, and supplies the lateral volar forearm to the wrist. It has fibers originating primarily from the C-5 root. The techniques of stimulation and recording its sensory nerve action potential are well established.2,3It is an excellent study for differentiating a preganglionic lesion, such as a C-5 root avulsion, from a postganglionic lesion, such as an upper trunk and/or lateral cord lesion. I think that this study should be done in all patients with a suspected brachial plexus lesion, especially in stretchcontusion injuries in which the

To the Editor.— We describe two patients in whom, while receiving isotretinoin for severe cystic acne previously uncontrolled by conventional therapy, severe vasculitis developed, one patient with Wegener's granulomatosis and the other patient with small-vessel angiitis. Report of Cases.—Case 1 (Wegener's Granulomatosis).— Acne of the face and upper trunk developed in this patient at age 15 years in 1980. In November 1983, he began therapy with isotretinoin orally administered at 80 mg (1.1 mg/kg)/d. For the previous year, he had had a "stuffed-up nose," epistaxis, and a serous otitis media severe enough to require lancing of the tympanic membrane. After six weeks, he had transient migratory joint pains and more severe epistaxis. After 11 weeks, the patient had areas of exuberant, crusted, painful granulation tissue on the chest and shoulders, decreased hearing, and increased nasal congestion. By 14 weeks, he had

REPORT OF A CASE A 59-year-old woman presented with a 20-year history of extensive hyperpigmentation of the upper trunk, neck, and extremities. The pigmentary changes began on the arms and legs, and then spread to involve other areas. Initially, the cutaneous changes were asymptomatic; pruritus developed in a generalized distribution several months prior to her presentation. She had a history of atopic dermatitis during childhood but no history of asthma or allergic rhinitis. On physical examination, the patient appeared to be in good general health. Extensive bilaterally symmetric reticulated macular hyperpigmentation was present on the extremities, the posterior and lateral aspects of the neck, and the upper chest and back, sparing the interscapular areas (Figs 1 and 2). Involvement was most prominent over the extensor surfaces of the arms and legs, where a few small papules were scattered over the pigmented areas. Diffuse xerosis of the extremities was present.

REPORT OF A CASE A 32-year-old woman was referred for evaluation of a rapidly progressive, tender, crusted eruption of the face and upper trunk of 5 days' duration. She had a history of atopic dermatitis and was treated for 3 days prior to referral with prednisone (10 mg/d orally) for possible contact dermatitis. The lesions began periorbitally and spread rapidly over the face and upper trunk despite the systemic steroid treatment. The patient described a similar eruption occurring twice as a child; once at age 8 years after being in a swimming pool near other children who had recently been vaccinated against smallpox, and a second time at age 12 years after she was vaccinated against smallpox. Both outbreaks were limited to the arms. On physical examination, she was afebrile and had a widespread eruption of crusted and umbilicated papules and vesicles on erythematous bases over the entire face, anterior

• Two infants, younger than 2 months of age, had brachial plexus involvement and true paralysis secondary to osteomyelitis of the proximal humerus. To date, no previous cases of this association have been reported. Electromyography (EMG) demonstrated significant denervation in muscles innervated by the upper trunk, with scattered involvement of other muscles. Nerve conduction velocities were normal in both patients. Both patients had significant residual weakness after appropriate antibiotic therapy. One patient, 12 months after therapy for osteomyelitis, continued to demonstrate both clinical and EMG evidence of a lower motor neuron neuropathy. The pathophysiologic cause was thought to be ischemic nerve damage secondary to occlusion of vasa nervorum. (Am J Dis Child1982;136:1054-1056)

To the Editor: In two papers 1,2 presented in 1965, we reported results which support the findings recently recorded in theArchives of Dermatologyby Epstein and Pinski. 3 In our series of 12 cases of porphyria cutanea tarda, three had hemoglobin values exceeding 17.5 gm/100 cc and six had hemoglobin values exceeding 16 gm/100 cc. The most outstanding case was that of a white Australian aged 64 who was seen in October 1962. He had a 12 month history of symptoms of cutaneous porphyria and had had two episodes of acute abdominal discomfort. In addition to fragility and blistering of the light exposed areas he was developing sclerodermiform changes of the head, neck, and upper trunk which were rapidly extending when first seen. Following intravenous chelation therapy with sodium calciumedetate in the Queen Elizabeth Hospital, Adelaide, his condition improved steadily with objective softening of all the sclerodermiform areas. When

CUTANEOUS manifestations of the carcinoid syndrome commonly include transient flushing of the face and upper trunk, patchy cyanosis, and telangiectasia. 1 There have been occasional reports of severe pruritus, "orange blotches," 2 pellagra-like dermatitis, 3 acropachyderma, pachyperiostitis, 4 and sclerodermatous changes. 5 The occurrence of cutaneous metastatic nodules is infrequent. 4,6-9 After recent observation of a patient with the carcinoid syndrome with nodular cutaneous metastases as well as two unusual necrotic areas involving the skin, we reviewed the literature and were unable to find a report concerned with the latter lesions. Case Report A 62-year-old white man was first admitted to the University of Minnesota Hospitals in March 1967 with a diagnosis of carcinoid syndrome which had been confirmed elsewhere by abdominal exploration. Because of hepatic metastasis in the right lobe of the liver, it was removed in April 1965. A metastatic lesion in the mediastinum was removed in

A distinctive pruritic syndrome associated with systemic cancer is described. Prolonged, variably severe pruritus with a tendency to localization on the legs, upper trunk, and extensor surfaces of the upper extremities is the presenting symptom. Patients with unexplained persistent itching as herein described should be investigated for a hidden malignancy. The persistence or recurrence of itching, months or even years after apparent cure of a systemic carcinoma, suggests that destruction or removal of the primary tumor was incomplete, or that metastasis has supervened. A small carcinomatous focus may exist for many months and at times for years before cinical recognition is possible with diagnostic methods currently available.