1/19. Invisible mycosis fungoides: A diagnostic challenge.We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/19. Generalized pruritus without primary lesions. Differential diagnosis and approach to treatment.A 65-year-old man presented with recurrent generalized pruritus and excoriations of many years' duration. He had been treated with antihistamines, topical corticosteroids, and antibiotics for secondary wound infections, but improvement was only temporary. He had also been hospitalized for leg ulcers complicated by cellulitis. Examination revealed multiple oval and linear red papules and nodules measuring 0.5 to 2 cm in diameter. Some of the lesions were eroded and had a central crater and yellowish crust. The patient also had hypopigmented linear scars localized to the posterior scalp, neck, upper back, chest, abdomen, arms, and legs with sparing of the middle and lower back (figures 1 and 2). An ulcer measuring 1.5 x 2 cm that was surrounded by indurated skin was present on the medial aspect of his right ankle. The ulcer was partially covered by yellow exudate. There was no evidence of cellulitis. liver enzyme, serum creatinine, and thyrotropin levels, as well as a chest roentgenogram, were normal. Wound cultures for bacteria and fungi were nonsignificant. A punch biopsy from a representative lesion showed an abrupt epidermal defect with sparse superficial lymphocytic infiltrate in the dermis. The patient was admitted to the hospital to isolate him from his home environment. He received a 10-day course of systemic cephalexin, topical clobetasol propionate ointment for the affected skin areas, and oral hydroxyzine for pruritus. Ultraviolet light therapy was instituted once daily and was to continue for 2 months. His lesions had improved moderately by the time he was discharged from the hospital. On follow-up 2 weeks later, his lesions were flat and had resulted in hypopigmented scars. Three months later, however, he had persistent, intense pruritus, and new excoriations had developed on his forearms and back. He improved after receiving treatment with oral doxepin hydrochloride.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
3/19. Secondary cutaneous amyloidosis in disseminated superficial porokeratosis: a case report.Disseminated superficial porokeratosis (DSP) is a rare cause of secondary cutaneous amyloidosis. An 83-year-old male patient showed an increase in both size and number of DSP lesions after contracting pulmonary tuberculosis. The DSP lesions of the patient consisted of numerous annular eruptions on both sun-exposed and sun-protected areas, which occurred over a period of 20 years. Multiple skin biopsies were taken from normal or lesional/sun-exposed or sun-protected skin samples. Histopathologic examination included routine H&E stains, congo red stains, thioflavin-T stains and anticytokeratin antibodies (AE1, AE3). And the results were as follows; 1) Positive staining with congo red and thioflavin-T indicated an amyloid nature for the deposits, 2) confinement of the amyloid deposition just below the lesional epidermis (while sparing the neighboring uninvolved or distant normal skin) indicated some role of the lesional epidermis, and 3) positive staining with AE3 further indicated an epidermal origin-type II epithelial keratin-of the amyloid. We present a case of DSP with a local amyloid deposit, characterized by association of positive familial background, severe pruritus and pulmonary tuberculosis.- - - - - - - - - - ranking = 2keywords = dermis (Clic here for more details about this article) |
4/19. Invisible mycosis fungoides: a diagnostic challenge.We describe a 76-year-old woman who had persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. skin biopsy specimens obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/19. Normolipemic plane xanthoma associated with adenocarcinoma and severe itch.Normolipemic plane xanthomas are yellow-red-colored flat patches or plaques with barely palpable borders, under normolipemic conditions usually involving the eyelids, the lateral sides of the neck, the upper aspect of the trunk, or the flexural folds. Histologically the lesions are characterized by an infiltrate consisting of foamy macrophages in the papillary and middermis with a distinct perivascular localization. Plane xanthoma has been associated with monoclonal gammopathy, cryoglobulinemia, and myeloproliferative disorders. We present a patient in whom plane xanthoma developed on the upper aspect of the back, which was accompanied by severe itch in the affected area. These symptoms started 1 month after resection of an adenocarcinoma of the rectum that was complicated by recurrent abdominal abscesses and, currently, by ongoing inflammatory bowel disease. A hypothetic pathophysiologic scheme of events leading to xanthoma formation in this patient is presented.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
6/19. Cutaneous larva migrans.Cutaneous larva migrans is occasionally seen in pediatric emergency outpatient care settings. It results from infestation of hookworm larvae into the epidermis. The infestation is self-limited but may produce severe discomfort. The diagnosis relies entirely on clinical findings. Laboratory findings only support the clinical diagnosis but do not confirm it. Treatment is typically with topical thiabendazole, but oral thiabendazole may be indicated in severe cases.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
7/19. An itching and excoriated dermatosis during intrahepatic cholestasis of pregnancy.A 35-year-old woman at 30th gestation week of her second pregnancy presented to our department with a 2-month history of intense and generalized pruritus. She had a spontaneous abortion 1 year earlier. Itching initially presented during nighttime and localized on lower limbs and after became continuous, diffuse, and associated with excoriations due to scratching. The patient was previously treated with oral corticosteroids (25 mg/d) in a gynecological department with temporary response. On our examination, she presented linear excoriations with hemorrhagic crusts localized on the trunk, buttocks, and upper and lower limbs. biopsy specimen from the lesional area of the right buttock submitted for routine histology documented a mild perivascular and interstitial infiltrate of lymphocytes and monocytes with rare eosinophils on superficial dermis. Indirect and direct immunofluorescence (performed on perilesional skin) were negative. Laboratory investigations revealed microcytic anemia (hemoglobin 7.5 g/dL; medium corpuscular volume 61.7 fl), erythrocyte sedimentation rate (21 mm) and serum bile acid levels (18.3 nmol/L; normal values 1.00-8.90) increase. On the basis of clinical, serological, and histological findings, we diagnosed an itching dermatosis during an intrahepatic cholestasis of pregnancy. We treated the patient with ursodeoxycholic acid (600 mg) and topical corticosteroids with gradual resolution of itching. Furthermore, she delivered a healthy boy at 39th gestation week with normal birth weight and normal apgar score.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
8/19. Inflammatory stage of disseminated superficial porokeratosis.Disseminated superficial porokeratosis (DSP) is a keratinization disorder characterized by multiple small lesions with a slightly elevated, sharply defined ridge over the whole body. Unusual DSP cases with acute exacerbation of their lesions accompanied by severe pruritus have been reported and designated as "eruptive pruritic papular porokeratosis" or "inflammatory DSP". Histologically, the pruritic lesions in the majority of these unusual DSP cases had a dense infiltration of eosinophils and lymphocytes in the vicinity of blood vessels in the upper dermis. In this report, we describe an additional case of DSP with a similar clinical course and histopathological findings. A review of the literature showed that the pruritic condition in these unusual DSP cases can be transient and is not necessarily related to spontaneous regression. We propose the term "Inflammatory stage of DSP" for describing this unusual variant of DSP.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
9/19. Successful treatment of recalcitrant necrotizing eosinophilic folliculitis using indomethacin and cephalexin.A 56-year-old man presented with a 4-month history of a painful and pruritic eruption consisting of crusted plaques and blisters on his face, scalp and chest. The patient suffered from headaches and malaise but was afebrile. Two skin biopsies revealed an epidermis which was eroded and covered by locules of serum and neutrophils. In the underlying dermis, there was a marked mixed inflammatory reaction including lymphocytes, neutrophils and numerous eosinophils. There was exocytosis of eosinophils into several follicles with areas of follicular mucinosis. A diagnosis of necrotizing eosinophilic folliculitis was made based upon the clinical and histopathological findings. The diagnosis was supported by the rapid response to a combination of indomethacin and cephalexin. The patient has taken continuous indomethacin (with rabeprazole and misoprostol cover) and cephalexin for 2 years. If treatment is withdrawn he experiences a flare of his disease within 2 weeks. This case highlights the potentially chronic nature of this disease.- - - - - - - - - - ranking = 2keywords = dermis (Clic here for more details about this article) |
10/19. tryptophan-induced eosinophilia-myalgia syndrome.Eight patients who became ill while taking tryptophan had myalgia, fatigue, rash, fever, edema, alopecia, arthralgias, diminished joint motion, skin tightening, muscle cramping, and distal paresthesias. Three had shortness of breath, and one had pulmonary hypertension. Laboratory abnormalities included peripheral eosinophilia, leukocytosis, thrombocytosis, raised erythrocyte sedimentation rate, and elevated serum levels of aldolase, lactate dehydrogenase, and liver enzymes. Of 4 chest radiographs, 3 were abnormal. Of 5 skin and muscle biopsies, 4 showed sclerosis or mixed inflammatory cell infiltration of the dermis, subcutis, and fascia. eosinophils were often present, but vasculitis was absent. Muscle inflammation was minimal. We conclude that the "eosinophilia-myalgia syndrome" is related to the ingestion of tryptophan and that abnormalities in the secretion of lymphokines may be important in its pathogenesis.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
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