Wells' syndrome. Recurrent granulomatous dermatitis with eosinophilia.

Two cases of granulomatous dermatitis with eosinophilla (Wells' syndrome) are reported. With Wells' original four cases, these two cases define a distinctive dermatosis with onset as cellulitis and formation of solid edema and either final spontaneous resolution or resolution with steroid therapy. Microscopic study showed diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. Biopsy of muscle and fascia showed comparable fasciitis and eosinophilic myositis. Immunofluorescence in one case disclosed fibrin in the dermis and lgM, lgA, and C3 in the blood vessels of the muscle. Recurrences of the lesions often appeared to be related to drug administration or surgery.

Cicatricial pemphigoid and rheumatoid arthritis.

A review of our experience with cicatricial pemphigoid revealed three patients with cicatrical pemphigoid and rheumatoid arthritis and one patient with ankylosing spondylitis who had a high titer of rheumatoid factor. A comparison of these four patients with patients who had bullous pemphigoid and rheumatoid arthritis shows similarities between cicatricial pemphigoid and bullous pemphigoid in relation to the development of rheumatoid arthritis.

American experience with low-dose thalidomide therapy for severe cutaneous lupus erythematosus.

BACKGROUND: There is a renewed interest in thalidomide therapy after its surprising effectiveness in treating erythema nodosum leprosum was first published. Thalidomide has subsequently been reported to be effective in treating a number of dermatoses, including cutaneous lupus erythematosus. We examined the efficacy and adverse effects of low-dose, long-term thalidomide monotherapy in 7 patients with various forms of cutaneous lupus erythematosus that were unresponsive to traditional systemic treatments. OBSERVATIONS: Six of the 7 patients treated with thalidomide after discontinuation of other oral agents had complete or marked resolution of their previously treatment-resistant cutaneous lesions, with an average response time of 2.2+/-0.8 months. Our cohort of 7 patients with cutaneous lupus erythematosus was treated with thalidomide therapy for an average of 2.4+/-3.1 years (range, 1 month to 9 years). The most common adverse effects were sedation, constipation, and weight gain. Two patients reported experiencing intermittent shaking episodes, an adverse effect not previously reported in the literature. Four patients reported symptoms of paresthesia, but none was found to be caused by thalidomide-induced peripheral neuropathy. CONCLUSIONS: A low starting dose of thalidomide as a monotherapy with continued sun avoidance is a safe and effective treatment for the various cutaneous manifestations of lupus erythematosus after traditional therapeutic options have failed to control disease. Our experience with low-dose, long-term thalidomide therapy suggests that peripheral neuropathy is not as common as suggested by other studies (up to 50% of patients treated with thalidomide in some series).

Limited Wegener's granulomatosis. Unusual cutaneous, radiographic, and pathologic manifestations.

A case of limited Wegener's granulomatosis is presented with unusual black linear and circular digital infarcts, unilateral bursitis and subcutaneous nodules, interstitial pneumonitis, and unusual pulmonary pathology. Response to cyclophosphamide was curative.

Striate palmoplantar keratoderma: a clinical and ultrastructural study.

Palmoplantar keratodermas are poorly understood heterogeneous disorders of cornification. Autosomal dominant and autosomal recessive keratodermas with a varied clinical presentation are recognized. Striate keratoderma is uncommon. We report the case of a 15-year-old girl with keratoderma that began at 5 months of age. Her clinical and ultrastructural findings are reviewed. Routine microscopic examination revealed eosinophilic inclusions in the granular cell layer as well as prominence of the granular cell layer. Electron microscopic examination revealed corneocytes with an imbricated pattern. Ultrastructural findings in this unique case are contrasted with those of other palmoplantar keratodermas.

Erythema ab igne caused by a car heater.

Erythema ab igne is a reticulated erythematous hyperpigmented eruption that occurs after chronic exposure to heat. In the past, the shins were the most common area of involvement, but with the widespread availability of central climate control in most buildings the incidence has decreased dramatically. New causes of erythema ab igne have been noted that have been caused by such heat sources as therapeutic chairs with built-in heaters. We present a case in which prolonged and close contact of the legs to a car heater led to erythema ab igne. Practitioners must be aware of the changing causes of erythema ab igne so that an appropriate history can be obtained.

Skin manifestations of pentazocine abuse.

Seventeen patients who had cutaneous complications following pentazocine injections are presented. The mean age was 50 years; total daily pentazocine dose ranged from 60 to 2,400 mg; evidence of psychiatric illness was present in 94%, and previous drug or alcohol abuse was noted in 65%. Fifty-three percent of our series of patients had a medical or paramedical background. Eighteen percent had diabetes mellitus, and 65% had a personal or family history (or both) of diabetes. A hypothesis is presented for this association. Characteristic histologic findings included fibrosis of the dermis and panniculus, with vascular alterations, fat necrosis with granulomatous inflammation, and vascular thrombosis with occasional endarteritis. We emphasize that medical and paramedical personnel and patients with a personal or family history of diabetes should be added to the group of patients considered to be at special risk for cutaneous complications of pentazocine injections.

Squamous cell carcinoma secondary to recessive dystrophic epidermolysis bullosa. A report of 4 patients with 17 primary cutaneous malignancies.

Four patients with recessive dystrophic epidermolysis bullosa and 17 invasive primary cutaneous squamous cell carcinomas (SCCs) are presented. All skin cancers arose at the site of scarring due to recessive dystrophic epidermolysis bullosa. Three of 17 (18%) primary cutaneous SCCs recurred locally following initial treatment with either surgical excision or wide surgical excision. Patients with recessive dystrophic epidermolysis bullosa are more likely to develop SCCs of the extremities than nonimmunocompromised patients or patients with epidermodysplasia verruciformis. Like patients with epidermodysplasia verruciformis, patients with recessive dystrophic epidermolysis bullosa are likely to suffer from invasive and metastatic SCCs at a much younger age than nonimmunocompromised patients.

Cat-scratch disease. Bacteria in skin at the primary inoculation site.

Cat-scratch disease is a zoonotic infection characterized by a skin papule at the site of the scratch followed by regional lymphadenitis. Recently, small gram-negative pleomorphic bacilli were demonstrated in sections of lymph node from patients with the disease. We now report identical bacteria in the primary inoculation site of three patients with cat-scratch disease. Lymph nodes from two of these patients also contained the same bacilli. Identical bacteria in both skin and lymph nodes from these patients are further evidence that the bacilli are the cause of cat-scratch disease. In early infections, biopsy of the primary site of inoculation and demonstration of bacilli may replace excision and histologic examination of lymph node in establishing the diagnosis of cat-scratch disease.