Porphyria cutanea tarda and human immunodeficiency virus: two cases associated with hepatitis C.

Hepatitis C virus (HCV) was recently recognized as a probable etiologic factor for porphyria cutanea tarda (PCT). A review of the literature revealed 40 cases of PCT associated with the human immunodeficiency virus (HIV). In most of these cases, hepatitis C status was unknown. We describe two patients with PCT who were coinfected with HIV and HCV and discuss the interaction of these two viruses. A diagnosis of PCT, especially in a young patient, should prompt investigation for underlying HIV and HCV infection. Porphyrin studies should be performed in any patient with HIV and photosensitivity. Clinicians should be aware of the infectious risk associated with the vesicles and erosions in these patients.

Recognizing cutaneous drug eruptions. Reaction patterns provide clues to causes.

Cutaneous drug eruptions caused by use of various medications are becoming more and more common. Fortunately, most such eruptions resolve when the offending medication is withdrawn. Nonetheless, it is vital that physicians can recognize drug-related eruptions and ascertain the degree of severity, since some reactions are potentially fatal. A carefully taken drug history and a thorough physical examination are crucial in this effort.

Trigeminal trophic syndrome.

Seven cases of trigeminal trophic syndrome are reported. In this rare condition, neurotrophic ulcers occur on the face, especially in the ala nasi area in a dermatome of the trigeminal nerve that has been rendered anesthetic, usually as a complication of trigeminal ablation by surgical means or injection for treatment of trigeminal neuralgia. The period from time of trigeminal nerve injury to onset of the ulcer varies from weeks to several years, with a mean of 1 to 2 years. Self-induced trauma is believed to produce the tissue destruction. Once the ulcers appear, they are extremely persistent.

Indeterminate cell histiocytosis treated successfully with 2-chlorodeoxyadenosine.

We report the case of a 54-year-old man with a ten-year history of a generalized papular eruption consistent with the diagnosis of indeterminate cell histiocytosis. The patient responded favorably to a course of treatment with 2-chlorodeoxyadenosine.

Pancreatic panniculitis.

BACKGROUND: Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic disease. OBJECTIVE: Our purpose was to study the clinical and histopathologic features of this disease. METHODS: We conducted a retrospective review of 11 patients. RESULTS: In five patients subcutaneous fat necrosis preceded the diagnosis of pancreatic disease by an average of 13 weeks. Five of our patients had underlying pancreatitis, and the remainder had carcinoma of the pancreas. Joint manifestations were prominent in six patients. Intestinal submucosal fat necrosis developed in two cases and caused massive gastrointestinal bleeding in one. Acute lesions demonstrated focal fat necrosis with "ghost cells," pathognomonic of this condition. Chronic lesions showed granulomatous and lipophagic panniculitis, with rare areas of basophilic fat necrosis. Cholecystectomy for gallstone pancreatitis was curative in two patients. CONCLUSION: Pancreatic panniculitis has distinctive clinical and pathognomonic histopathologic findings that can be the presenting features of pancreatic disease.

Scleromyxedema.

BACKGROUND: Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality. OBJECTIVE: Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents. METHODS: A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted. RESULTS: Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-lambda (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients. CONCLUSION: The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.

Treatment of classic pityriasis rubra pilaris.

BACKGROUND: Treatment of classic pityriasis rubra pilaris, which almost always progresses to a generalized erythroderma with marked, often disabling keratoderma of the palms and soles, remains problematic. OBJECTIVE: Our purpose was to evaluate the results of treatment in a recent period during which the retinoid group of drugs has been available. METHODS: The clinical course of 75 patients with classic pityriasis rubra pilaris seen from 1982 to 1992 was reviewed. RESULTS: Of 15 patients treated with isotretinoin, 10 had complete and 2 had partial clearing. Of six treated with etretinate, four had clearing. All eight patients treated with methotrexate had a favorable response. Other forms of treatment, including Goeckerman regimen, corticosteroids, vitamin A, and cyclosporine, were ineffective. CONCLUSION: Early diagnosis and early treatment with retinoids appear to offer the best chance for clearing of pityriasis rubra pilaris. If retinoids fail or cannot be used, methotrexate should be considered.

Lasers in dermatology. A review.

The use of lasers in dermatology has become widespread over the past decade. This article reviews the types of lasers in current use and the skin problems that could benefit from their use.

A clinical and histopathologic study of granulomatous rosacea.

A retrospective clinical and histopathologic study of 53 patients with granulomatous rosacea was undertaken. The patients had a broad clinical spectrum of lesions that ranged from primarily erythema to papulonodular lesions. Extrafacial lesions occurred in 15% of patients. Histologic examination showed mixed lymphohistiocytic inflammation (primarily lymphocytic inflammation in 40% of patients and primarily histiocytic with a few giant cells in 34%), epithelioid granulomas in 11% of patients, and epithelioid granulomas with caseation necrosis in 11%. Most patients had a good response to oral antibiotic therapy. Granulomatous rosacea is not a distinct disease but can be regarded and treated as a subtype of rosacea.

Periorbital edema: an important physical finding in dermatomyositis.

Periorbital edema is frequently present in dermatomyositis and can be seen in the absence of other skin lesions. Sometimes, as in the two cases reported here, it can be the dominant physical sign.

Argon laser treatment of the red nose.

Ten patients with red nose due to telangiectasias were treated with the argon laser. Seven had telangiectasias due to rosacea, two had had rhinoplasty, and one had experienced trauma. All of the patients had a good clinical result.

Treatment of psoriasis with etretin: a preliminary report.

Eight patients with psoriasis (seven with plaque-type and one with palmoplantar pustular psoriasis) were treated with the synthetic retinoid etretin, the active metabolite of etretinate. An initial 8-week double-blind phase of the study with dosages of 0, 25, or 50 mg/day was followed by an open phase in which variable dosages of 25, 50, or 75 mg/day were used to achieve an optimal clinical response. All patients completed a minimum of 6 months of therapy. A good or an excellent response (at least 50% clearing) after 8 months of treatment was noted in six of the eight patients. One patient had a poor response (less than 50% clearing), and one patient continued to have worsening of psoriatic involvement during treatment. The best response occurred in those patients with the most extensive initial plaque involvement or palmoplantar pustulosis. The clinical side effects were similar to those reported with use of etretinate and seemed to be related to the dose. The laboratory abnormalities--primarily mild intermittent elevations of liver enzymes and elevations in serum lipids--were similar to those described in previous reports about etretinate. Both etretin and etretinate are potent teratogens. Because of its shorter half-life, etretin will likely be preferred, especially in female patients of childbearing potential.

Isotretinoin treatment of pityriasis rubra pilaris.

Five patients with pityriasis rubra pilaris were treated with isotretinoin from September 1982 through 1985. Isotretinoin at an average dose of 1.16 mg/kg/day for 16 to 24 weeks caused complete or almost complete clearing in four of the five patients.

Treatment of the red nose with the argon laser.

Nasal telangiectasias, a common condition, can be caused by chronic exposure to ultraviolet light, rosacea, topically applied corticosteroids, or rhinoplasty. Use of the argon laser for 15 to 45 minutes in a continuous mode over the entire involved area can produce considerable lightening of the nasal redness and improvement in the cosmetic appearance, as illustrated in the two cases described in this report.

Bowel-associated dermatosis-arthritis syndrome: bowel bypass syndrome without bowel bypass.

The bowel bypass syndrome is a well-recognized complication in patients who have had jejunoileal bypass for morbid obesity. It consists of an influenza-like illness with increased temperature, chills, polyarthralgia, myalgia, and inflammatory papules and pustules that are 2 to 4 mm in diameter and that usually appear on the extremities and the upper part of the trunk. A patient is reported who had an identical bowel-associated dermatosis-arthritis syndrome but who had not undergone a jejunoileal bypass operation. Seven previously reported similar cases are reviewed.

Malignant pyoderma.

Malignant pyoderma is a destructive, ulcerating skin disease that occurs chiefly in young adults. Only eight cases of this rare disease have been reported. The head and neck have been involved in all cases, and a predilection for the periauricular region has been noted in several of the cases. Although the disease is progressive and chronic, responses to high-dose systemic corticosteroids have been noted, but usually a flare is associated with a reduction in dose. An additional case is described in which therapy with isotretinoin and sulfapyridine led to complete remission.