Louise Barnes

Suppression of the cutaneous immune response following topical application of the prostaglandin PGE2

UVB irradiation (290-320 nm) and topical applications of arachidonic acid (AA) in mice decrease the number of identifiable Langerhans cells and alter the cutaneous immune response. Application of contact allergens such as dinitrofluorobenzene (DNFB) to irradiated or AA-treated skin induces antigen-specific tolerance. Indomethacin (IM), a cyclooxygenase inhibitor, administered orally to mice prior to UVB irradiation or prior to the topical application of arachidonic acid, abrogates suppression of contact hypersensitivity (CHS) to DNFB. This suggests a byproduct of arachidonic acid generated through the cyclooxygenase pathway may be involved in the immune suppression. Topical application of various prostaglandins (PGE2, PGD2, PGF2 alpha, and CTXA2) did not cause alterations in the population density of the identifiable Ia+ dendritic Langerhans cells. PGE2, but no other tested agent, produced a suppression of the CHS response to DNFB. These observations suggests that of the various prostaglandins, PGE2 might be one of several biochemical signals which mediate the suppression of contact hypersensitivity reactions following ultraviolet radiation exposure. However, the mechanisms by which PGE2 produces its suppressive effects have not been identified.

Multiple Hamartomas Associated with Intracranial Malformation

Abstract: We examined a newborn infant with multiple hamartomas, including an epidermal nevus syndrome and a giant pigmented congenital nevocellular nevus, associated with other structural developmental abnormalities such as nevus flammeus, vascular malformation, cutis aplasia congenita of the scalp, cartilage hamartoma, and a lipodermoid of the conjunctiva. This child had a significant brain malformation, diagnosed by sonography and computerized tomography, consisting of a significant enlargement of the left hemisphere not associated with asymmetry of the skull or facial bones. We suggest a careful investigation of the intracranial structures by computerized tomography and/or ultrasonography in case of either extensive linear nevus sebaceous sequence and/or giant pigmented nevocellular nevus.

Pustular pyoderma gangrenosum associated with ulcerative colitis in childhood: Report of two cases and review of the literature

Pyoderma gangrenosum is a cutaneous disorder associated with systemic diseases such as ulcerative colitis, Crohns disease, rheumatoid arthritis, and blood dyscrasias. We are reporting two cases of pustular pyoderma gangrenosum associated with ulcerative colitis. One patient had inactive bowel disease when she developed her third episode of pustules, erosions, and nodules on the left leg. The other patient exhibited a widespread painful vesiculopustular eruption that coincided with the onset of her colitis. Both patients presented with pustules as the primary manifestation of their pyoderma gangrenosum. Histologic examination of skin from both patients revealed an acute perifollicular inflammation. Pyoderma gangrenosum should be considered in the differential diagnosis of pustular disorders in children with underlying conditions such as ulcerative colitis.

Childhood Subacute Cutaneous Lupus Erythematosus Associated with Homozygous Complement 2 Deficiency

Abstract: We describe a 9‐year‐old boy with an Ro‐positive, subacute, cutaneous lupus rash associated with homozygous C2 deficiency. His response to a mild topical steroid and sunscreen was excellent. Hereditary complement deficiency and its association with childhood lupus erythematosus are discussed.

Dermatomyositis associated with angiotropic lymphoma

We describe a 64‐year‐old man with dermatomyositis sine myositis who presented with unusual cutaneous plaques and nodules. Initial investigations failed to reveal evidence of malignancy. Eighteen months later he became systemically unwell and repeat biopsies from the nodules confirmed angiotropic T‐cell lymphoma. Cutaneous lymphoma is rarely reported with dermatomyositis and in the previously reported cases, mycosis fungoides was the variant seen. This is the first report of angiotropic T‐cell lymphoma associated with dermatomyositis.

Poikiloderma Congenitale: Case Report and Review of the Literature

Abstract: Since birth an 8‐year‐old girl had a rash on both cheeks, consistent clinically and histologically with poikiloderma. She also had visual impairment to the extent of light perception only, due to microphthalmia and dense scleralization of the corneas. These features were considered consistent with a diagnosis of Rothmund‐Thomson syndrome.

Depigmentation: Its significance in patients with melanoma

Abstract Malignant melanoma is the leading cause of death from tumors of the skin, and its incidence is rising at an alarming rate. 1,2 The American Cancer Society estimated that melanomas account for 1–2% of all cancer deaths. 3 The prognosis for a malignant melanoma is directly related to the thickness of the tumor as measured by the Breslow or Clark technique. 4 Although the incidence of melanoma has increased, fortunately there also has been an improved survival rate. Patients with clinical stage 1 disease (no clinical evidence of tumor in regional lymph nodes) and a tumor depth of less than 0.75 mm have a 95–100% 5-year survival rate 5 if the malignancy is properly excised. The improvement in cure rates or longer survivals probably results from earlier diagnosis made possible by public awareness campaigns, rather than from advances in medical or surgical therapy. 62,73 Those patients with a deep tumor and/or advanced disease (stage 2, regional lymph node metastases; stage 3, distal metastases) have a dismal prognosis. Treatment of patients with metastatic melanoma is disappointing. Surgical ablation of tumor in regional lymph nodes (clinical stage 2) gives a 20% 5-year survival rate, but even these patients cannot be assured of a cure. Melanomas can recur as long as 20 years after excision. There have been numerous isolated reports of depigmentation occurring in association with cutaneous or ocular melanomas. 8–18 This is a rather paradoxical association. Melanoma is an uncontrolled proliferation of malignant melanocytes. In contrast, depigmentation is an abnormal destruction of benign melanocytes. That the two phenomena occur together has attracted attention in recent years. 20–23 It has been suggested that patients with metastatic disease who develop a vitiligo-like depigmentation survive for longer periods than might ordinarily be expected. This observation led to speculation that induction of vitiligo might be considered after resection of the primary melanoma to prevent metastases and to enhance survival. 24 In this chapter, the literature on the subject of melanoma and depigmentation is reviewed. Also, the question of whether their joint occurrence influences the longevity of survival is examined. Various examples of similar observed phenomena within the animal kingdom are reviewed. Lastly, some attempt is made to explain the pathogenesis of this interesting phenomenon.

The spectrum of psoriasis in an Irish HIV population

Object Report of the pattern of psoriasis and response to therapy in psoriatic patients infected with HIV‐1.

Granulomatous leg ulcers : an unusual presentation of Crohn's disease in a young man

Crohns disease is a granulomatous disease of the bowel in which several extraintestinal manifestations have been described. Some cutaneous manifestations bear a close histologic resemblance to the bowel disease as noncaseating granulomas are present within the skin. When found in skin distant from the bowel, this has been termed metastatic Crohns disease. We report the case of a young man with Crohns disease who came to us for treatment of fungating leg ulcers.

Pancreatic panniculitis preceding acute pancreatitis and subsequent detection of an intraductal papillary mucinous neoplasm: A case report

Pancreatic panniculitis is a rare skin manifestation of pancreatic disease. It is believed to result from seepage of pancreatic enzymes into the systemic circulation, which leads to skin and occasionally joint manifestations. It is reported to occur in up to 3% of patients with pancreatic disease.1 Pancreatic panniculitis can develop before, concomitantly with, or after the diagnosis of clinically detectable pancreatic conditions. The condition is most frequently associated with acute pancreatitis, chronic pancreatitis, and pancreatic carcinoma. The most common carcinoma is acinar cell type. Here we report an unusual association of pancreatic panniculitis preceding a single episode of acute pancreatitis and subsequent development of an intraductal papillary mucinous neoplasm (IPMN).