Idiopathic intracranial hypertension (IIH), sometimes called benign intracranial hypertension (BIH) or pseudotumor cerebri (PTC) is a neurological disorder that is characterized by increased intracranial pressure (ICP), in the absence of a tumor or other intracranial pathology.

Explanation of terms

The terms "benign" and "pseudotumor" have often been used for this disorder to make clear that the increased ICP is not caused by a tumor or malignancy. However, these terms belie the significance and potential morbidity of the disorder - Thus, it is most appropriately referred to as IIH, and not by its other names. Cases of increased ICP with a known cause can be called secondary intracranial hypertension (SIH), but for the purposes of this article the distinction is not entirely vital.

Diagnosis

The diagnosis of IIH is one of exclusion. The principle sign of IIH, papilledema, can occur because of brain tumors (hence the term "pseudotumor cerebri," which literally means "false brain tumor"), or in other conditions involving increased ICP. Thus, a thorough evaluation is essential to the diagnosis of IIH. Radiologic imaging scans are, as a rule, normal in IIH save for the finding of small or slit-like cerebral ventricles, and what may appear to be an 'empty' sella turcica (caused by flattening of the pituitary under pressure). Cerebrospinal fluid (CSF) is a clear fluid which surrounds and circulates through the brain and spinal cord - it is to the fluid pressure of the CSF that the concept of 'intracranial pressure' refers. The chronic pressure increase in IIH is, as the word "idiopathic" indicates, of uncertain etiology; Most researchers believe that the body's ability to absorb CSF is somehow impaired in those individuals with IIH. A less likely possibility (one that is now generally dismissed) is that of CSF overproduction. Many scientists and doctors believe that there is also some degree of brain swelling or engorgement.

Signs and symptoms

IIH most commonly affects women, particularly overweight women between ages 15 and 45. However, the disorder is not limited to women, and can affect people of all ages and races, both male and female, of all shapes and sizes. The 'cardinal sign' of IIH is papilledema (swelling of the optic nerves), although some atypical patients may not have papilledema. Occasionally patients may present with abducens or other cranial nerve palsies. Symptoms can include severe headache, pulsatile tinnitus, visual disturbances (e.g. diplopia), nausea/vomiting, etc. Most serious is the potential for permanent loss of vision or even blindness. Risk factors include female gender, obesity, excess or deficiency of vitamin A, certain medications, and some other disorders. In cases linked to medication or other clear causes, the line between truly idiopathic IH and secondary IH (as mentioned above) can become quite murky.

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Scrotal ulceration as a consequence of all-trans-retinoic acid for the treatment of acute promyelocytic leukemia
From Journal of Drugs in Dermatology, 3/1/05 by Soheil Simzar

Abstract

Induction therapy with all-trans-retinoic acid (ATRA), an oral vitamin A derivative, has been shown to improve the short and long-term outcome of patients with acute promyelocytic leukemia (APML). Common side effects include headache, fever, dry skin, and bone pain, and approximately 25% of treated patients experience ATRA syndrome, which includes fever, dyspnea, weight gain, pulmonary infiltrates, and pleural or pericardial effusions. Scrotal ulcerations due to ATRA are rare with 16 previously documented cases, most of whom were Asian. We report a Caucasian male with APML who developed scrotal ulceration during ATRA induction therapy and review the previously reported cases. Physicians and patients should be aware of this disturbing, but self-limited, dermatologic complication of ATRA.

**********

Introduction

Acute promyelocytic leukemia (APML, M3) is characterized by a severe hemorrhagic syndrome, hypergranular leukemic cells, and a t(15; 17) translocation. (1) All-transretinoic acid (ATRA, Vasanoid[R], Roche Laboratories) significantly improves the outcome of patients with APML by inducing differentiation of leukemic cells. (1,2) We report a case of scrotal ulceration due to ATRA and a review of similar cases.

Case Report

A 34-year-old white man presented with a 3-week history of progressively worsening epistaxis. Work-up revealed pancytopenia, coagulopathy, and a t(15; 17) translocation. The patient was diagnosed with APML and initiated on idarubicin and all-trans-retinoic acid (ATRA), 50 mg/[m.sup.2] twice a day, as induction therapy.

By day 16 of ATRA therapy, the patient developed a lowgrade fever and a slightly pruritic scrotal rash that became painful and ulcerated by the third week. On day 23, exam of the right scrotum revealed a well-defined, tender 2.3 cm necrotic ulcer with a central black eschar and surrounding superficial scale and erythema (Figure 1). Review of systems was negative and the rest of the physical exam was unremarkable. Blood cultures were negative as were viral, bacterial, and fungal cultures from the scrotum. ATRA was continued and the ulceration was treated with 1% hydrocortisone ointment in the morning and mupirocin ointment in the evening. One week later, fever, pain, and erythema subsided, but the ulcer was unchanged. ATRA was continued without additional complications and the ulcer healed within 2 weeks.

[FIGURE 1 OMITTED]

Discussion

ATRA is generally well-tolerated, and 90% of treated patients achieve complete remission of APML. (3) Reversible adverse effects reported include headaches, fevers, xerosis and mucosal dryness, liver abnormalities, muscle and bone pain, hyperlipidemia, and pseudotumor cerebri. One reported complication is ATRA syndrome, affecting approximately 25% of treated patients, and characterized by fever, dyspnea, weight gain, radiographic pulmonary infiltrates, and pleural or pericardial effusions. Rarely, scrotal involvement such as ulceration, (3-8) exfoliative dermatitis, (8) or Fournier's gangrene (4) has been reported.

Based on previously reported cases, ATRA-induced genital ulceration occurs between the first and fourth week of initiating therapy. Except in one case of progression, the lesions heal within 2 months, an outcome that appears to be unrelated to treatment or whether ATRA is discontinued. (3-5) One reported patient had a relapse of APML after 2 years and redeveloped scrotal lesions upon re-administration of ATRA, suggesting a strong association between the lesions and the medication. (7) Our patient developed scrotal ulceration 3 weeks after therapy and had relief with topical medications despite continuing ATRA therapy.

The pathogenesis of scrotal ulceration remains unclear. Neutrophilic superoxide and cytokine release due to ATRA are hypothesized to cause tissue damage. (5,7) Biopsies of ulcers have not been reported, but based on negative cultures, infectious agents seem to be unlikely causes. (5) Some patients had fever and/or other characteristics of ATRA syndrome concurrent with the ulceration, suggesting it may be part of an ATRA syndrome spectrum.

Meticulous wound care, topical steroids, and monitoring for secondary infection may be the most suitable management. Physicians and patients should be aware of this disturbing, but self-limited, dermatologic complication of ATRA.

References

1. Mandelli F, Avvisati G, Lo CF. Advances in the understanding and management of acute promyelocytic leukemia. Rev Clin Exp Hematol. 2002;6:60-71.

2. Huang ME, Ye YC, Chen SR, et al. Use of all-trans retinoic acid in the treatment of acute promyelocytic leukemia. Blood. 1988;72:567-72.

3. Esser AC, Nossa R, Shoji T, Sapadin AN. All-transretinoic acid-induced scrotal ulcerations in a patient with acute promyelocytic leukemia. J Am Acad Dermatol. 2000;43:316-7.

4. Fukuno K, Tsurumi H, Goto H, Oyama M, Tanabashi S, Moriwaki H. Genital ulcers during treatment with ALL-trans retinoic acid for acute promyelocytic leukemia. Leuk Lymphoma. 2003;44:2009-13.

5. Charles KS, Kanaa M, Winfield DA, Reilly JT. Scrotal ulceration during all-trans retinoic (ATRA) therapy for acute promyelocytic leukaemia. Clin Lab Haematol. 2000;22:171-4.

6. Pavithran K, Arjun R, Aruna R, Thomas M. Scrotal ulceration during induction therapy of acute promyelocytic leukemia with ATRA. Am J Hematol. 2004;75:260-1.

7. Mori A, Tamura S, Katsuno T, et al. Scrotal ulcer occurring in patients with acute promyelocytic leukemia during treatment with all-trans retinoic acid. Oncol Rep. 1999;6:55-8.

8. Sun GL, Ouyang RR, Chen SJ, et al. Treatment of acute promyelocytic leukemia with all-trans retinoic acid. A five-year experience. Chin Med J (Engl). 1993;106:743-8.

Soheil Simzar BS, Adam M. Rotunda MD, Noah Craft MD PhD

Division of Dermatology, Department of Medicine, David Geffen School of Medicine at University of CA, Los Angeles

Address for Correspondence

Noah Craft, MD

Division of Dermatology, Department of Medicine, David Geffen School of Medicine

University of California at Los Angeles (UCLA)

Suite 450

Box 956957

Los Angeles, CA 90095-6957

Tel: 310-206-6371

Fax: 310-794-7005

e-mail: ncraft@ucla.edu

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