Jeffrey P. Callen

Neutrophilic dermatosis-associated sterile chronic multifocal osteomyelitis in pediatric patients: case report and review.

Atypical pyoderma gangrenosum (PG) and Sweet syndrome are neutrophilic dermatoses that share some common features. Sterile chronic recurrent multifocal osteomyelitis is a rare association of these neutrophilic dermatoses that has only been reported in children. We report a 3‐year‐old girl who initially presented with pain in her left hand and right leg. Roentgenograms and bone scan revealed findings of multifocal osteomyelitis affecting both femurs, the right tibia, left clavicle, right eighth costochondral junction, and left ulna. She was treated with antibiotics without improvement. Bone biopsy of the left ulna revealed histologic changes consistent with osteomyelitis, however, all cultures for bacteria, mycobacteria, and fungi were negative. She subsequently developed an ulcer surrounded by a violaceous, undermined border at the site of the bone biopsy, which also did not improve during antibiotic treatment. A biopsy specimen from this lesion demonstrated a dense perivascular and periappendageal infiltrate of neutrophils within the dermis and edema of the papillary dermis compatible with a neutrophilic dermatosis. She was treated with oral prednisone which resulted in resolution of skin lesions, bone pain, and soft tissue swelling. This case further documents the association between PG or Sweet syndrome and multifocal sterile osteomyelitis.

Multicentric reticulohistiocytosis in a 14-year-old girl.

Abstract: Multicentric reticulohistiocytosis is a rare multisystem disorder in which an infiltration of histiocytic cells causes papulonodular skin lesions and potentially a destructive polyarthritis. The active disease typically resolves spontaneously after 5–8 years, but the articular destruction can lead to permanent joint deformities. We present a case of multicentric reticulohistiocytosis in a 14‐year‐old girl. The number of papules on her hands decreased in number and her arthritic symptoms improved after 4 months of oral naproxen. Thirty months later her joint symptoms remained inactive and only six very small papules remained on her hands. Small flexor deformities were present in the distal interphalangeal joints of both index fingers. This case is an example of how multicentric reticulohistiocytosis can be a relatively stable and self‐limited disease but still cause permanent joint deformities.

Recurrent Photosensitive Dermatitis Preceding Juvenile Dermatomyositis

Abstract: The classic features of childhood dermatomyositis include muscle weakness, elevated muscle enzymes, and characteristic abnormal muscle biopsy and electromyography. Paramount to the diagnosis are cutaneous dermatoses that include a heliotrope rash and Gottrons papules. Rarely, a photosensitive dermatosis may occur. A recurrent photoexacerbated dermatoses can be an initial sign of occult childhood dermatomyositis.

Rothmund‐Thomson Syndrome: A Case Report

Abstract: We present a 4‐year‐old girl with polklloderma, radiai aplasia, short stature, facial dysmorphism, and sparse hair. We believe these findings to be consistent with a diagnosis of Rothmund‐Thomson syndrome.

Dapsone-induced peripheral neuropathy

ABSTRACT: A young man with dermatitis herpetiformis developed fatigue and neurologic complaints 4 years after he began oral dapsone therapy. Neurologic examination and nerve conduction studies confirmed the presence of a combined motor and sensory peripheral neuropathy. The symptomatic improvement reported by the patient was supported by improvement in the nerve conduction studies after cessation of dapsone therapy. Substitution of sulfapyridine did not adversely affect the resolution of his neuropathy.

Prevalence of Cutaneous Findings in Hospitalized Pediatric Patients

Abstract: Cutaneous findings can be useful in establishing the diagnosis and treatment of hospitalized patients. Observation and identification of cutaneous abnormalities can improve the accuracy of diagnosis and result in improved patient care. We set out to determine the prevalence of cutaneous abnormalities in hospitalized pediatric patients in a hospital and how often these findings were noted and properly diagnosed by the admitting team of physicians. Children with medical problems admitted to Kosair Childrens Hospital during the month of January 1995 were randomly selected for a skin examination, which was performed within 24 to 28 hours of admission. Parental consent was required prior to admission into the study. Of 117 patients offered participation, 110 accepted. Physical findings were noted and in addition the completeness of charting by the admitting physician and the relationship of any cutaneous findings to admitting diagnosis were noted. One hundred five of the 110 patients (95%) had cutaneous findings consisting of either a “rash” or a “congenital lesion.” Fifty‐one had more than one cutaneous finding noted on examination. Dermatitis was the most common diagnosis made, followed by pigmented lesions and congenital vascular malformations of all types. In 35 of the 105 patients with a dermatologic diagnosis, the dermatologic diagnosis related directly to the admitting diagnosis. In only 22 of these 35 (63%) was the cutaneous involvement noted by the admitting physician. In 9 of the 110 cases (8%), the findings on dermatologic examination altered the primary diagnosis and/or treatment. Cutaneous findings are very common in the hospitalized pediatric patient. The diagnosis, charting, and treatment of dermatologic conditions by the primary pediatric team were often incomplete, although an expert cutaneous examination can be critical to patient care.

Cutaneous Lymphoproliferative Disorder Complicating Infectious Mononucleosis in an Immunosuppressed Patient

Abstract:  Infectious mononucleosis is the syndrome produced by primary infection with Epstein‐Barr virus during adolescence or early adulthood. In immunosuppressed individuals, depressed T‐cell function allows the Epstein‐Barr virus‐driven B‐cell proliferation to continue unabated, potentially leading to a lymphoproliferative disorder. A 15‐year‐old girl with a history of ulcerative colitis treated with 6‐mercaptopurine and mesalamine presented with the acute onset of a rapidly enlarging, ulcerative nodule on her left lower eyelid 4 weeks following recovery from infectious mononucleosis. The biopsy revealed an Epstein‐Barr virus‐positive lymphoproliferative disorder. Systemic disease was absent. Following discontinuation of 6‐mercaptopurine, the patient was treated with two courses of intravenous cyclophosphamide. The lesion resolved completely and she remains disease free at 14 months following diagnosis. We report a solitary cutaneous lesion of an immunosuppression‐related lymphoproliferative disorder (IR‐LPD) occurring as a complication of infectious mononucleosis, and review the pathogenesis and reported cases of Epstein‐Barr virus‐related immunosuppression‐related lymphoproliferative disorder arising in the setting of inflammatory bowel disease. It is important for dermatologists and dermatopathologists to be aware of the occurrence of IR‐LPD in patients being treated for inflammatory conditions, including inflammatory bowel disease. Given the role of primary infection with Epstein‐Barr virus in the development of IR‐LPD, consideration may be given to assessing Epstein‐Barr virus status prior to initiating immunosuppressive therapy in young patients.

Immunologic Testing in the Dermatologic Patient

F requently, dermatologists become involved in the care of patients witb diseases that are possibly immunologically mediated. Commonly, we order tests or are asked to interpret tests that aid in the diagnosis. Some tests may even allow the physician to prognosticate. It then becomes extremely important to have a full knowledge ofthe testing methods and, in particular, know how to interpret the test results based on their sensitivity and specificity. In this commentary these terms will be defined and then applications to several clinical situations will be discussed. Sensitivity refers to how often the test result is positive in a population with a given disease. Sensitivity can be altered through defining a disease process by a positive test result, however, in general, if a test result is positive in 80 of 100 patients with a disease, the test is 80% sensitive. Thus, there would be 20% false-negative results. Specificity refers to how often the test result is positive in a control population without a disease process. If, for exmaple, 10% of controls had a positive test result, there is a 10% false-positive rate and the test is 90% specific. Ideally, a test should be very sensitive and yet specific. In the real world, there are few, if any, tests that are both sensitive and specific. The following examples will detail some problems I have encountered in the practice of dermatology.