Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma.

Subcutaneous panniculitis-like T cell lymphoma is a rare subtype of cutaneous lymphomas with distinct clinical, histological and immunophenotypic characteristics, as well as an indolent clinical course. Rarely, it may be complicated with hemophagocytic lymphohistiocytosis: a hyperinflammatory syndrome which, if not diagnosed early, carries a dismal outcome. In this article, we describe a case of subcutaneous panniculitis-like T cell lymphoma in a middle-aged female patient which was complicated with secondary hemophagocytic lymphohistiocytosis with a favorable outcome following etoposide-based therapy. The various histological mimics of subcutaneous panniculitis-like T cell lymphoma and the management options are also briefly discussed.

Eritema Nodoso Leproso Forunculoide: Presentación de un caso / No disponible

La Lepra es una enfermedad infectocontagiosa crónica de larga evolución que puede presentar episodios sintomáticos agudos, en ocasiones muy severos, que obligan a buscar atención médica y que se conocen con el nombre de lepra reaccional o leprorreacciones. Diagnosticar lepra reaccional no es dificultoso frente a un paciente con diagnóstico previo de la enfermedad. Aún así, se deberá estar alerta ante la aparición de síntomas generales y/o manifestaciones viscerales, en ausencia de otros signos cutáneos de reacción. Por el contrario, su reconocimiento como primer motivo de consulta puede ser, en ocasiones, un desafío diagnóstico tanto para el dermatólogo como para el médico clínico. Presentamos un raro caso de un paciente de 84 años de edad, procedente de medio rural del Paraguay, diabética, que debutó con lesiones compatibles con forúnculos, tratada previamente como tal por internista y ante falta de mejoría remitida a nuestro servicio, llegando al diagnóstico clínico, histopatológico y baciloscópico de lepra reaccional: eritema nodoso leproso atípico, de tipo forunculoide

Leprosy is a chronic infectious disease that can have longstanding acute symptomatic episodes, in severe cases, requiring them to seek medical attention and are known by the name of leproreactions of leprosy reactions. Diagnosing leprosy reaction is not difficult compared to a patient with a previous diagnosis of the disease, Still, should be alert to the appearance of general symptoms and/or visceral manifestations, in the absence of other signs o skin reaction. On the contrary its recognition as a first reason for consultation can sometimes be a diagnostic challenge for the dermatologist to the clinician. We present rare case a 84 year old female from rural area of Paraguay, diabetic, which debuted with furunculoid lesions and consistent with that was previously treated by an Internist and because the lack of improvement she was referred to our department, arriving at clinical and histopathological diagnosis of atypical erythema nodosum of furunculoid type

Tender cutaneous nodules of the legs: diagnosis and clinical clues to diagnosis.

BACKGROUND: "Tender cutaneous nodules of the legs" is a common manifestation in dermatology. Histopathological investigation is usually required for this condition, because clinical data are frequently insufficient to make a definite diagnosis. OBJECTIVE: To identify and analyze the causes of patients presenting with tender leg nodules and to reveal clinical clues that could help to differentiate causes. MATERIALS AND METHODS: The medical records and histopathological slides of patients presenting with tender cutaneous nodules of the legs between January 2005 and December 2007 were retrospectively reviewed. RESULTS: Of the total of 154 patients, 122 (79.2%) were female. Definite diagnoses were categorized into four groups: inflammation (84.4%); infection (5.8%); tumor (6.5%); and nonspecific (3.2%). The most common cause in the inflammation group was erythema nodosum. The infections found were Acremonium spp., Penicillium sp., Mycobacterium abscessus, Mycobacterium fortuitum and Mycobacterium leprae. The tumors included leiomyoma, leukemia cutis, and lymphomas. Clinical data that correlated with and could be used as clues for the inflammation group were female sex (P = 0.03, OR 6.43) and lower leg involvement (P = 0.03, OR 7.14). LIMITATIONS: The retrospective manner of this study is a limitation. CONCLUSION: Various inflammatory conditions, infections, and tumors can present as tender cutaneous nodules of the legs. Female sex and lower leg involvement were clinical data that could be used as clues for the diagnoses in the inflammation group. However, histopathological investigation is still crucial to determine a definite diagnosis in patients presenting with tender cutaneous nodules of the legs.

Panniculitis: clinical overlap and the significance of biopsy findings.

BACKGROUND: Panniculitides are well-recognized clinicopathologic entities but the non-specificity of their clinical and pathological features often troubles the diagnostician. METHODS: This study retrospectively evaluates the clinical overlaps and the significance of histological findings among various panniculitides. RESULTS: The clinical evaluation in 55 panniculitides cases suggested the diagnosis of typical erythema nodosum (EN) in 26 cases, atypical EN in 17 cases, atypical nodular vasculitis (NV) in two cases, soft tissue infection in five cases and five cases remained unclassified. Skin biopsy evaluation provided definite panniculitis diagnosis in 53 cases including EN (28 cases), leukocytoclastic vasculitis (seven cases), NV (four cases), superficial thrombophlebitis (ST) (two cases), eosinophillic panniculitis (EP) (three cases), infection-related panniculitis (five cases), and one case each of erythema nodosum leprosum (ENL), lupus panniculitis (LP), pancreatic fat necrosis and acne conglobata with two cases remaining unclassified. Histologically, 'predominantly septal' and 'mixed panniculitis' were the chief inflammatory patterns in EN cases, while mixed panniculitis was seen in most LCV cases and predominantly lobular and mixed panniculitis in NV cases. CONCLUSIONS: Biopsy evaluation of a panniculitis lesion is usually significant, and the application of a combination of histologic features rather than of a single biopsy finding or an inflammatory pattern is helpful in the diagnosis of panniculitis.

Subcutaneous panniculitis-like T-cell cutaneous lymphoma.

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma classified in the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification as a unique extranodal lymphoma with characteristic by T cell receptor (TCR) gene rearrangement. We report here a case of SPTCL in a 22 year-old woman who had presented with variably sized multiple nodules on both her legs. Initial differential diagnoses considered were panniculitis and lupus panniculitis. The histopathology showed a predominantly subcutaneous lobular infiltrate with atypical lymphocytes, karyorrhexis and rimming of adipocytes by lymphoid cells. Immunohistochemistry showed CD4-, CD8+, CD56- T-cell phenotype. Although TCR rearrangement studies were not done, the above T-cell phenotype and sparing of epidermis and dermis suggested the possibility of an SPTCL alpha/beta type. The patient received five cycles of a cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) regimen which resulted in the regression in her skin lesions and constitutional symptoms.

Vasculitis nodular: forma clínica tipo hipodermitis en banda a nivel de brazo

Se presenta un caso de paniculitis en forma de banda subcutánea, infiltrada y con formaciones nodulares, localizada exclusivamente a nivel de brazo en una mujer de 34 años, con historia familar de tuberculosis e hiperergia tuberculínica. El estudio histológico correspondía a una vasculitis nodular, y la respuesta al tratamiento tuberculostático fue satisfactoria. Los autores destacan la excepcional forma de manifestación clínica y acepatan la unificación de la denominación clínica e histopatológica de la vasculitis nodular para las formas asociadas o no a hiperergia frente al bacilo tuberculoso.