Marcelo H. Grunwald

Stewart-Treves syndrome in a patient with elephantiasis

Angiosarcoma that develops in areas of chronic lymphedema is also called Stewart-Treves syndrome. It usually appears in areas of lymphedema several years after mastectomy for breast carcinoma. Only 10% of these angiosarcomas occur in areas of chronic lymphedema as a result of another cause. We present a patient with epithelioid angiosarcoma as a rare complication of elephantiasis.

Association of Pemphigus Vulgaris and Herpes Simplex Virus Infection

A 39‐year‐old man developed sore throat with swallowing difficullies in late September 1983. A suspicion of foreign body in the esophagus was ruled out by esophagosc opy. At that time, the patient was in good general health, with normal body temperature. The only pertinent physical finding was a wide erosion over the right side of the palate, fie was admitted 3 weeks later to the otorhinolaryngology department at Rambam Medical Center because of a sudden worsening of his oral complaints and rise in body temperature to 38C with general malaise. On examination, there were wide erosions on the palate; and on laryngoscopy, the epiglottis showed edema, redness, and fibrin deposits. Total peripheral blood count showed hemoglobin 16 mg/dL and leukocytes 10.500/mm3, with a differential count of 75% polymorpho‐nuclears, 1% stabs, 11% lymphocytes, l% monocytes, 5% eosinophils, and 7% atypical cells. The results of the following routine blood examinations were normal: glucose, urea, electrolytes, calcium, phosphorus, alkaline phosphatase, creatinine, uric acid, albumin, globulin, and complement C3 and C4. Electrophoresisand immunoelectrophoresis of serum proteins and urinalysis results were within normal values. Antinuclear factor was negative. The diagnosis of acute epiglottitis was made, and the patient was treated by intravenous crystalline penicillin with only slight improvement. A week later, a blistering eruption appeared on the skin, and the patient was transferred to the dermatologic department for further investigation. On examination, the eruption consisted of flaccid vesicles, bullae, and erosions on the back, dorsa of hands, and inguinal folds; erosions of the oral mucosa were still present. Nikoisky sign was positive. A biopsy specimen from the skin showed a suprabasal blister, and the basal cells remain attached to the dermis, giving a “row of tombstones” appearance (Fig. 1). The diagnosisof pemphigus vulgaris was made, and the patient was put on an initial dose of daily 100 mg fluocortolone. After 2 weeks, the dosage was elevated to 150 mg per day, and 10 days later, improvement was achieved. The dosage of oral corticosteroids was cautiously tapered, and the patient was discharged at the end of November 1983.

Nail sampling in onychomycosis: comparative study of curettage from three sites of the infected nail

Background: Onychomycosis is a common problem.Obtaining accurate laboratory test results before treatment is important in clinical practice since the treatment of onychomycosis requires expensive oral antifungal therapy with potentially serious side‐effects. The purpose of this study was to compare results of curettage technique of nail sampling in the diagnosis of onychomycosis from three different sites of the affected nail to establish the best location of sampling.

Bullous lichen sclerosus et atrophicus

A 47-year-old man suffered from a widespread pruritic eruption for 3 months. Examination revealed lesions on the arms, elbows, knees, and trunk. Some consisted of erythematous, discrete papules and plaques covered with a slight scale, whereas others were macular and atrophic, with a porcelain white color (Fig 1). On the lower back, a 6-cm hemorrhagic bulla was present on top of an atrophic erythematous plaque (Fig 2). The genital area was not involved. A biopsy specimen of an atrophic plaque showed hyperkeratosis, atrophy of the stratum malpighii, hydropic degeneration of the basal cells, pronounced edema and homogenization of the collagen in the papillary dermis, and a perivascular inflammatory infiltrate in the middermis. A biopsy specimen of the bulla showed similar findings with more pronounced edema of the papillary dermis, resulting in a subepidermal split (Fig 3). Direct immunofluorescence was negative. Treatment with a moderate-strength topical steroid cream caused clearing of the bulla, but the other lesions responded only partially.

Nikolsky's or Pseudo-Nikolsky's sign in bullous pemphigoid.

Nikolsky’s sign is important in the clinical diagnosis of bullous diseases. Slight pressure or rubbing the skin causes separation of the upper layers, leaving a raw, moist abrasion. It i s classically present in pemphigus vulgaris, especially near an active and widespread lesion, and represents lack of cohesion between epidermal cells. It is caused by acantholysis that eventually produces vesicle formation. Nikolsky‘s sign has been demonstrated “through rarely, if ever, in uninvolved skin in bullous pemphigoid,”’ and it also could be elicited in polydysplastic epidermolysis bullosa, Toxic epidermal necrolysis, and Porphyria variegata.’ During the years 1982-1 983, 23 patients with bullous pemphigoid were admitted to the Department of Dermatology of the Beilinson Medical Center. In all cases, the diagnosis was confirmed by histologic and immunofluorescent criteria. Three of the patients (1 3%) had positive Nikolsky’s sign even in areas distant from active lesions. Histologic examination of the site where the skin was rubbed in one patient showed separation of the entire epidermis from dermis, leaving the basal cell layer attached to the epidermis (Fig. 1 ). This phenomenon seems not to be as uncommon in bullous pemphigoid as reported in the literature. It is preferable to use the term “pseudo-Nikolsky’s sign” in cases in which subepidermal separation of skin occurs, as in bullous pemphigoid, polydysplastic epidermolysis bullous, and toxic epidermal necrolysis and porphyria variegata3 and to reserve the term “Nikolsky’s sign” for those cases in which the lack of cohesion i s between epidermal cells, as in pemphigus.

Solitary glomus tumour

A 42‐year‐old woman developed a purplish, tender nodule on her wrist of 6 months duration. Histological examination showed a well‐circumscribed lesion situated in the dermis and subcutis characterized by numerous, dilated thin‐walled vascular spaces surrounded by sheets of glomus cells. The histological findings are characteristic of the diagnosis of an uncommon type of glomus tumour, known as glomangioma.

Adenoid (Pseudoglandular) Squamous Cell Carcinoma at the Base of Cutaneous Horn

13, Billings JK, Ellis CN, Milgraum SS. Cutaneous granuloma formations in Crohns disease. JAMA. 1986,255:2661. Burgdorf W, Orkin M. Cranulomatous perivasculitis in Crohns disease. Arch Dermatol. 1981,-117:674-675. McPoland PR, Moss RL. Cutaneous Crohns disease and progressive vitiligo. J Am Acad Dermatol. 1988;19:421-425. Duhra P, Paul CJ. Metastatic Crohns disease responding to metronidazole. BrJ Dermatol. 1988;119:87-91.