Biomedical subjects
Publications and source records attributed to Dean S Morrell.
We describe a female infant with an acrodermatitis enteropathica-like eruption as the presenting sign of rapidly fatal cystic fibrosis. The patient had growth retardation, developed an erythematous eruption unresponsive to oral zinc, and finally a generalized erosive dermatitis with associated edema, anemia and hypoproteinemia.
Chromobacterium violaceum is a gram-negative bacterium of soil and water in tropical and subtropical environments. Typically, it is considered a bacterium of low virulence although, uncommonly, it causes human infection, particularly in persons with defects in host defenses. Infection generally follows exposure of broken skin to contaminated water and soil, and is often characterized by pustules, lymphadenitis, fever, and vomiting, as well as rapid dissemination and a high mortality rate. Unfortunately, because C violaceum is ubiquitous, it is often dismissed as a contaminant when cultured. Because rapid diagnosis (by taking appropriate specimens) and treatment are vital to a good prognosis, it is imperative that physicians be aware of this organism. In addition, patients with chromobacterial infections should have an immunologic workup because infections in immunocompetent individuals are rare. Here we report an aggressive yet nonfatal case of C violaceum septicemia in an adolescent male, diagnosed through a punch biopsy of a skin lesion, and resulting in a new diagnosis of chronic granulomatous disease.
BACKGROUND: Self-healing juvenile cutaneous mucinosis is a rare disease affecting young people characterized by transient cutaneous lesions and sometimes mild inflammatory symptoms. The deep dermal and subcutaneous features of this disorder have not yet been well described. OBJECTIVE: The purpose of our study was to present 3 cases of self-healing juvenile cutaneous mucinosis in which the histopathologic features caused diagnostic confusion between this disorder and proliferative fasciitis. METHODS: The study includes clinical and histologic findings of 3 patients, complemented by a literature review. RESULTS: The histologic descriptions of nodular lesions in self-healing juvenile cutaneous mucinosis reveal features of proliferative fasciitis, including a myxoid stroma and gangliocyte-like giant cells. LIMITATIONS: Self-healing juvenile cutaneous mucinosis is a rare condition and has not been frequently reported in medical literature. Our findings are based on the pathologic features of 3 patients. CONCLUSIONS: Our findings further elucidate the histologic features of self-healing juvenile cutaneous mucinosis and expand the differential diagnosis for entities in which gangliocyte-like giant cells are noted.
AGEP (acute generalized exanthematous pustulosis) is a relatively rare exfoliative skin syndrome consisting of generalized eruption of pustules in response to medication or infection. Because AGEP may have other systemic manifestations, such as renal failure, hyperthermia, lab abnormalities, and/or hemodynamic instability, it is important to make the distinction between AGEP and other life-threatening generalized skin diseases, such as toxic epidermal necrolysis (TEN). Here we present a case of AGEP in response to valdecoxib, which has not previously been described in the literature. The patient presented with profound hypotension requiring fluid and vasopressor support and was referred to the burn service for treatment of TEN, but her skin lesions were inconsistent with this diagnosis. A dermatology consult was obtained and suggested a diagnosis of AGEP, which a biopsy confirmed. TEN and AGEP present with similar history, types of associated drugs, and immunology. Both can be associated with antibiotics, non-steroidals, and anticonvulsants, but AGEP is more frequent with aminopenicillins, while TEN is associated more often with sulfonamide antibiotics. Both disorders have a proposed T cell-mediated immune response, but they differ in the mechanism. A description of valdecoxib and its role as a sulfonamide in producing cutaneous reactions is also provided.
BACKGROUND: Hemophagocytic lymphohistiocytosis is a rare, rapidly progressive, and potentially fatal disorder of activated histiocytes. The initial clinical presentation commonly includes fever, hepatosplenomegaly, and pancytopenia. Skin eruptions are described in up to 65% of patients. Information regarding the morphological features, configuration, and distribution of these eruptions is lacking and is typically reported as nonspecific and "maculopapular." The aim of this report is to better delineate the cutaneous manifestations of the disorder to assist in differentiating the process from other systemic diseases. OBSERVATION: A case report of a neonate with hemophagocytic lymphohistiocytosis with generalized purpuric macules is described. The clinical features of 5 other patients with hemophagocytic lymphohistiocytosis at Children's Hospital of Wisconsin, Milwaukee, are summarized. Clinical images of 1 additional neonatal patient with hemophagocytic lymphohistiocytosis are presented as well. These observations demonstrate the varied cutaneous manifestations of hemophagocytic lymphohistiocytosis: erythroderma, generalized purpuric macules and papules, and morbilliform eruptions. CONCLUSION: Awareness of cutaneous involvement can assist in the initial diagnosis of hemophagocytic lymphohistiocytosis and potentially signify recurrences.
Two children with chronic and asymmetric skin indurations are presented. The clinical and pathologic features are suggestive of asymmetric childhood scleredema and stiff skin syndrome. The key features of scleredema and stiff skin syndrome are discussed.
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Nickel is the most ubiquitous contact allergen among children and adolescents. Metal blue jeans buttons and belts have been noted to cause nickel dermatitis around the umbilicus. For these children, traditional teaching is strict avoidance of all pants with metal snaps/buttons, particularly blue jeans. In this study we tested 90 pairs of blue jeans and 47 belts for nickel using the dimethylglyoxime spot test. Only 10% of blue jeans tested positive, while 53% of belts tested positive. Furthermore, 10 pairs of nickel-negative blue jeans remained negative after 10 washings. Overall we found no resistance to testing in clothing stores. From these results, we recommend that patients with allergic contact dermatitis secondary to nickel need not strictly avoid blue jeans and metal belt buckles. Rather, families should be encouraged to use the dimethylglyoxime spot test to test these items for nickel prior to purchase.
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Fixed drug eruption is an infrequent but well-known adverse event most commonly associated with antibiotics and nonsteroidal anti-inflammatory medications. We herein describe a second reported instance of vancomycin-induced fixed drug eruption involving an extensive area of the body surface.
Painful erythematous plaques and a pathognomonic, dense, dermal infiltrate of neutrophils characterize Sweet syndrome. Although numerous instances of this disease have been reported in children, only four have had an associated immunodeficiency to date. Sweet syndrome has been traditionally treated with systemic corticosteroids despite an unknown pathogenesis. Prior reports suggest that immunodeficient children with Sweet syndrome may have a persistent course and fail to respond to standard treatment. We describe a child with debilitating Sweet syndrome and a primary immunodeficiency whose symptoms were controlled with intravenous immunoglobulin and dapsone.