1/10. vitamin a deficiency phrynoderma: due to malabsorption and inadequate diet. We describe a patient with vitamin a deficiency phrynoderma caused by a combination of inadequate dietary intake of vitamin A and beta-carotene and malabsorption secondary to primary visceral myopathy and total colectomy. ( info) |
2/10. Calcified nodule on the heel of a child following a single heel stick in the neonatal period. Dystrophic cutaneous calcification may arise at sites of local trauma or in association with various disorders. Calcified nodules of the heel have been reported in high-risk neonates following repeated heel sticks to draw blood. We present a healthy 2-year-old boy with a calcified nodule on the heel secondary to a single heel stick in the neonatal period. The patient was born full-term at 38 weeks' gestation, with a birth weight appropriate for gestational age. A firm nodule was noticed at the age of 8 months; this became tender. histology revealed epidermal and subepidermal deposition of calcium. serum calcium and phosphate levels were normal. Although calcified heel nodules occur mostly in high-risk neonates, this case suggests that this condition also can occur in healthy children after only a single heel stick. Dermatologists should include this entity in the differential diagnosis of warty papules on the heels of children. ( info) |
3/10. Acquired reactive perforating collagenosis in a nondiabetic hemodialysis patient: successful treatment with allopurinol. The authors present a case of acquired reactive perforating collagenosis developed in a nondiabetic hemodialysis patient, who was treated successfully with allopurinol. Treatment of acquired reactive perforating collagenosis is difficult and often ineffective. The patient had been unresponsive to conventional treatments, but the pruritus was controlled, and skin lesions subsequently resolved after the treatment with allopurinol. Possible mechanisms of allopurinol treatment for acquired reactive perforating collagenosis are discussed. ( info) |
| necrolytic migratory erythema was first described in 1942 in a patient with pancreatic islet cell carcinoma. The disease can, however, have other etiologies including nutritional dermatoses. Here, we describe the clinicopathological picture of a 7-year-old female patient who presented with necrolytic migratory erythema which we believe is secondary to a rare combination of zinc deficiency and propionic acidemia. ( info) |
5/10. Thermosensitive lichen amyloidosis. BACKGROUND: A 26-year-old male presented with a 3-year history of lichen amyloidosis. On examination, there was a pigmented papular eruption with a ripple pattern affecting the limbs and trunk but sparing the axillae, antecubital and popliteal fossae, central chest, neck and face. There was also prominent sparing of the skin overlying the superficial veins of the limbs. The sparing of the superficial veins of the limbs by lichen amyloidosis raised the possible role of cutaneous temperature in governing the distribution of amyloid deposits in our patient. OBSERVATIONS: Total body infrared thermography demonstrated consistent sparing of the amyloid deposits in areas with higher cutaneous temperatures such as the neck and axillae as well as the course of the superficial veins. The cooler areas such as the extensor surfaces of the arms and legs corresponded to areas of amyloid deposition. Narrow band ultraviolet B (NBUVB) phototherapy over a 5-month period resulted in a marked improvement of pruritus and clearing of the amyloid deposits. CONCLUSIONS: Our patient clearly demonstrated lichen amyloidosis in a thermosensitive distribution. This may be a gross manifestation of previous reports of in vitro thermosensitivity of amyloid fibril formation and may have potential implications in treatment at least in a subset of patients demonstrating this clinical feature. ( info) |
| We report a 53-year-old Japanese woman with multiple, red, and elastic soft nodules on the left waist, left thigh, and right lower leg. She had had polyclonal hyperglobulinemia for one year, rheumatoid arthritis for 13 years, and sjogren's syndrome (SjS) for 18 years. Histochemical examination of the nodule on the left thigh revealed a deposition of amyloid by congo red staining. It was also positively stained with both anti-kappa and -lambda light chain antibodies. Moreover, the cytoplasm of the infiltrating plasma cells also positively reacted to both antibodies. The major amyloid proteins of primary localized cutaneous nodular amyloidosis (PLCNA) generally consist of monoclonal immunoglobulin light chains. A review of literature demonstrates 13 cases of PLCNA with SjS, in which immunoglobulin light chains were demonstrated in the amyloid in 5 cases. Amyloid in the 3 cases was composed of a single class immunoglobulin light chain and that in the 2 cases was composed of both kappa and lambda light chains. Polyclonal immunoglobulin amyloid has been reported only in PLCNA with SjS, which may be related to the fact that a certain population of SjS develops polyclonal B cell proliferation and hyperglobulinemia. ( info) |
| A 71-year-old woman with a history of sjogren's syndrome presented for evaluation of a waxy nodule present on the scalp for 6 months. Histopathologic examination revealed deposition of homogenous eosinophilic material throughout the reticular dermis consistent with amyloidosis. Primary cutaneous nodular amyloidosis is a rare phenomenon characterized by the deposition of immunoglobin light chains by a clonal plasma cell population. patients need to be monitored for progression to systemic amyloidosis or plasma cell dyscrasias. ( info) |
8/10. calciphylaxis in the absence of end-stage renal disease. OBJECTIVE: To report a case of calciphylaxis in the absence of renal failure in a patient with secondary hyperparathyroidism and low calcium/phosphorus product, in whom total parathyroidectomy resulted in relief of pain and healing of ulcerations. methods: We present the clinical, laboratory, and pathologic findings in a 62-year-old woman with calciphylaxis in the absence of end-stage renal disease. RESULTS: A 62-year-old woman presented with painful nonhealing bilateral calf ulcerations. pathology examination of tissue specimens from surgical debridement revealed intravascular calcification, consistent with calciphylaxis. Laboratory investigation revealed normal renal function; however, hypocalcemia and hypophosphatemia were present--a corrected serum calcium level of 7.5 mg/dL (normal, 8.5 to 10.2) and a serum phosphorus value of 1.0 mg/dL (normal, 2.5 to 4.5). These abnormalities were likely due to vitamin d deficiency, evidenced by a 25-hydroxyvitamin D level of 14 ng/mL, which provoked an elevation of the serum parathyroid hormone (PTH) concentration, documented by an intact PTH of 213 pg/mL (normal, 15 to 65) and a whole PTH (1-84 PTH) of 70.6 pg/mL (normal, 7 to 36). Her quality of life was severely impaired, not only by the ulcerations but also by intractable pain that necessitated epidural analgesia during the hospitalization. The patient underwent total parathyroidectomy and transcervical thymectomy, with cryopreservation of parathyroid tissue. One year after the parathyroidectomy, the patient had no recurrence of calciphylaxis. CONCLUSION: This case suggests that despite the potential complex pathophysiologic aspects of calciphylaxis, even in the absence of both renal failure and an elevated calcium/phosphorus product, early parathyroidectomy in patients with appreciably increased PTH levels may improve wound healing and diminish pain. ( info) |
9/10. Methylmalonic acidemia, cobalamin C type, presenting with cutaneous manifestations. BACKGROUND: Erosive dermatitis resembling the skin lesions of acrodermatitis enteropathica has been described in a number of aminoacidopathies and organic acidemias. In some, the dermatitis is a manifestation of untreated disease, while in others, including methylmalonic acidemia, skin lesions have been ascribed to nutritional deficiency due to therapeutic amino acid restrictions. OBSERVATIONS: We report 2 cases of methylmalonic acidemia presenting with cutaneous manifestations in the perinatal period before restrictive nutritional interventions. The cutaneous involvement consisted of cheilitis and diffuse erythema with erosions and desquamation. Methylmalonic acidemia, cobalamin C type, was subsequently diagnosed in both cases. CONCLUSIONS: An erosive, desquamating dermatitis with histopathologic characteristics resembling acrodermatitis enteropathica may be a presenting sign in cobalamin C methylmalonic acidemia, even in the absence of long-standing nutritional restrictions or deficiency. ( info) |
10/10. Diabetic pigmented spots on the extrapretibial region: immunohistochemical study of type IV collagen in dermal vessel wall. A case of diabetes mellitus associated with multiple pigment spots is reported. The patient had multiple pigmented atrophic patches on the abdominal area and thighs. There were no pigmented spots on the pretibial area. Histologically, dermal vessels showed intimal thickening and deposition of periodic acid-Schiff (PAS)-positive fibrillar material in vessel walls. Clinical and histological features indicated that these pigment spots were diabetic pigmented pretibial patches occurring in the extrapretibial area. Immunohistochemical studies of lesional and non-lesional skin using the antibodies for alpha1-alpha6 chains of type IV collagen revealed that PAS-positive material of vessel walls in both lesional and non-lesional skin was stained with alpha1 and alpha2 chain antibodies but not with alpha3-alpha6 chain antibodies, indicating that alpha1 and alpha2 chains of type IV collagen, which are normal components of the dermal vessel basement membrane, accumulate in the vessel walls in diabetes mellitus. ( info) |