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Extracutaneous Involvement (extracutaneou + involvement)

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Medical Sciences	100%

Selected Abstracts

Large B-cell lymphoma of the leg

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2001
Elsa Vasquez-del-Mercado MD
A 74-year-old Mexican man presented with an 18-month history of multiple, violaceous, coalescing, firm, tender nodules with an ulcer in the anterior aspect of the right leg (Fig. 1) and slightly infiltrated, ill-defined erythematous plaques affecting the left leg and both forearms. He had not received any treatment for his condition. Past medical history was relevant for noninsulin-dependent diabetes mellitus and hypertension without formal treatment and a history of heavy alcohol intake in his youth. A biopsy specimen of both plaque-type lesions of the forearm and tumorous lesions of the leg showed a diffuse, nonepidermotropic mononuclear infiltrate throughout the dermis and extending to the subcutis. The infiltrate was composed of pleomorphic, atypical, large mononuclear cells (Fig. 2). Immunostaining with CD20 was positive for the atypical cells while CD3 was positive for normal appearing lymphocytes, characterized as reactive T cells. Additional laboratory and image studies ruled out extracutaneous involvement. The diagnosis of primary cutaneous large B cell lymphoma of the leg (LBCLL) was made. The patient was initiated on radiotherapy localized to the right leg with a very good initial response, nevertheless resolution was not achieved and the plaques in the rest of the limbs remained unchanged. Thus, the patient started chemotherapy with CHOP (Cyclophosphamide, Vincristine, Doxorubicin, Prednisone). He has currently finished his fourth cycle with this chemotherapy regimen. The tumorous lesions involuted leaving only residual hyperpigmentation (Fig. 3) and the plaques in the rest of the limbs disappeared, the area of the ulcer diminished considerably. There is still no evidence of extracutaneous involvement. Figure 1. Nodules and ulcer in the anterior aspect of the right leg Figure 2. Atypical lymphocytes, with large, pleomorphic nuclei and multiple nucleoles. Positivity for CD20 antigen was demonstrated by immunohistochemical analysis (hematoxylin and eosin; X 600) Figure 3. Residual hyperpigmentation and granulation tissue after chemotherapy [source]

Severe Congenital Systemic Juvenile Xanthogranuloma in Monozygotic Twins

PEDIATRIC DERMATOLOGY, Issue 4 2008
Rattanavalai Chantorn M.D.
Juvenile xanthogranuloma with extracutaneous involvement is a rare disease in which significant morbidity and occasional deaths may occur. Monozygotic twins with congenital systemic juvenile xanthogranuloma who presented with multiple skin lesions, hepatosplenomegaly, liver failure, and bone marrow involvement were reported. The diagnosis of systemic juvenile xanthogranuloma was confirmed by histology and immunohistochemical stains of the skin with liver biopsies revealing dense infiltration of lymphohistiocytes with typical Touton giant cells staining positive for CD68 and negative for CD1a and S-100 protein. Both of them received systemic prednisolone 1 mg/kg/day which was gradually tapered off with time according to clinical and investigative responses. At the 17-month follow-up period, both patients showed remarkable regression in all symptoms and laboratory studies. [source]

Acquired Mucosal Indeterminate Cell Histiocytoma

PEDIATRIC DERMATOLOGY, Issue 3 2007
Marta Ferran M.D.
Neoplastic cells express S-100 and CD1a antigens, but lack Birbeck granules. It has been reported in both adults and children, as solitary or multiple cutaneous lesions with rare extracutaneous involvement. We describe a 12-year-old boy with an indeterminate cell histiocytosis manifesting as a solitary verrucous papule on the mucosa of the glans penis. The morphologic features and diagnostic criteria of cutaneous indeterminate cell histiocytic proliferations are reviewed. The possible relationship between indeterminate cell and Langerhans cell histiocytoses is discussed. [source]

Pegylated liposomal doxorubicin in stage IVB mycosis fungoides

BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2005
G. Di Lorenzo
Summary Background, Previous studies have shown that pegylated liposomal doxorubicin (LD) is effective in the treatment of relapsing or recalcitrant cutaneous T-cell lymphoma. Objectives, To evaluate the activity and toxicity of LD in patients with stage IVB mycosis fungoides (MF). Methods, In this retrospective study, we evaluated outcomes and recorded adverse effects in 10 patients with MF (seven men and three women) with extracutaneous involvement. Patients were treated with LD 20 mg m,2 administered intravenously every 4 weeks. Results, All patients received at least two cycles of LD, three patients received four cycles and one patient received six cycles. Three patients (30%) had a partial response and two patients had stable disease. Grade 1,2 leucopenia occurred in three of the 10 patients, and grade 4 leucopenia in one. Three patients had grade 2 palmoplantar erythrodysaesthesia. Conclusions, This study demonstrates that LD is beneficial in terms of activity and toxicity in stage IVB MF. These observations should be verified in larger studies. [source]

Multifocal haemangioma with extracutaneous involvement associated with hypergalactosaemia

CLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 8 2009
Y. Uchida
Summary Neonatal haemangiomatosis, characterized by multiple haemangiomas, is a rare disease that develops during the neonatal period with or without visceral involvement. We report a 1-month-old Japanese boy with multifocal haemangiomas with extracutaneous involvement. A haemangioma on his left lower eyelid, present at birth, increased in size during the first postnatal month and more lesions developed during the same period. Neonatal mass screening showed hypergalactosaemia. Laboratory investigations found raised total bile acid and ammonia. Computed tomography and abdominal ultrasonography studies showed multiple hepatic haemangiomas and intrahepatic portovenous shunts. The child's cutaneous and hepatic haemangiomas disappeared spontaneously with normalization of laboratory data, and galactose accumulation improved with the feeding of lactose-free milk. There were no complications and the child has had no recurrence of the symptoms. Our case implies a possible association of multiple haemangioma and hypergalactosaemia, suggesting the necessity for visceral investigation. [source]