ABSTRACT
Pseudocarcinomatous hyperplasia (PCH) is a benign reactive epithelial proliferation that may be associated to lymphomas of the skin or external mucous membranes. We present a case of single lesion mycosis fungoides (Woringer-Kollop's reticulosis pagetoid) associated with PCH that was initially misdiagnosed as squamous cell carcinoma (SCC) and review all PubMed-indexed previously reported cases on lymphomas of the skin or external mucous membranes associated to PCH, SCC, and keratoacanthomas. Including our own case, we collected data of 114 cases of cutaneous or mucosal lymphoproliferative disorders associated to PCH, 3 cases associated to SCC, and other 3 cases associated to keratoacanthomas. All cases were tabulated to the following parameters whenever data was available: sex, age, previous medical conditions, number of lesions (single × multiple), site of involvement (mucosa, skin or both), clinical impression, initial equivocal histopathologic diagnosis, final diagnosis, keratinocytic atypia (presence × absence), lymphocytic atypia (presence × absence), CD30-status, and treatment.
Subject(s)
Carcinoma, Squamous Cell/pathology , Keratoacanthoma/pathology , Mucous Membrane/pathology , Pagetoid Reticulosis/pathology , Skin Neoplasms/pathology , Skin/pathology , Adolescent , Adult , Aged , Aged, 80 and over , Biomarkers, Tumor/analysis , Biopsy , Carcinoma, Squamous Cell/chemistry , Carcinoma, Squamous Cell/therapy , Child , Diagnostic Errors , Female , Humans , Hyperplasia , Immunohistochemistry , Keratoacanthoma/metabolism , Keratoacanthoma/therapy , Male , Middle Aged , Mucous Membrane/chemistry , Pagetoid Reticulosis/chemistry , Pagetoid Reticulosis/therapy , Predictive Value of Tests , Skin/chemistry , Skin Neoplasms/chemistry , Skin Neoplasms/therapy , Young AdultABSTRACT
A cutaneous lymphoproliferative disorder, localized pagetoid reticulosis (LPR), is rare but distinctive in its clinical and histopathological manifestations. Without an evidence-based medicine treatment guideline because of extremely low incidence, LPR poses a therapeutic challenge. We describe a 67-year-old Chinese woman with LRP who showed complete response to narrow-band ultraviolet B combined with intramuscular interferon alpha-2b.
Subject(s)
Interferon-alpha/therapeutic use , Pagetoid Reticulosis/therapy , Skin Neoplasms/therapy , Ultraviolet Therapy/methods , Aged , Combined Modality Therapy , Female , Humans , Interferon alpha-2 , Interferon-alpha/administration & dosage , Pagetoid Reticulosis/pathology , Recombinant Proteins/administration & dosage , Recombinant Proteins/therapeutic use , Skin Neoplasms/pathology , Treatment OutcomeABSTRACT
Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded as aggressive epidermotropic CD8 T-cutaneous lymphoma, cutaneous gamma/delta T-lymphoma, or tumor-stage mycosis fungoides, depending on the clinical-histological picture. This case highlights a rare and challenging presentation of Ketron-Goodman disease with an indolent presentation and evolution and good response to a low-grade treatment regimen, not fitting well into the new classification criteria.
Subject(s)
Pagetoid Reticulosis/pathology , Skin Neoplasms/pathology , Biopsy , Humans , Lymphoma, T-Cell, Cutaneous/pathology , Male , Middle Aged , Pagetoid Reticulosis/therapy , Skin Neoplasms/therapy , Time Factors , Treatment OutcomeABSTRACT
Ketron-Goodman disease was formerly considered a disseminated type of pagetoid reticulosis. However, according to the new classification consensus, it should be regarded as aggressive epidermotropic CD8 T-cutaneous lymphoma, cutaneous gamma/delta T-lymphoma, or tumor-stage mycosis fungoides, depending on the clinical-histological picture. This case highlights a rare and challenging presentation of Ketron-Goodman disease with an indolent presentation and evolution and good response to a low-grade treatment regimen, not fitting well into the new classification criteria.
A doença de Ketron-Goodman foi inicialmente considerada uma forma disseminada de reticulose pagetóide. Mas, de acordo com o atual sistema de classificação e dependendo do quadro clínico-patológico deve ser antes vista como um linfoma T CD8 agressivo epidermotrópico, linfoma T gama/delta ou micose fungóide, estadio tumoral. Pretendemos realçar esta doença rara que pode suscitar dúvidas no diagnóstico. Neste caso, a apresentação e evolução foram indolentes com boa resposta a um tratamento pouco agressivo, não se enquadrando bem nas novas propostas de classificação da doença.
Subject(s)
Humans , Male , Middle Aged , Pagetoid Reticulosis/pathology , Skin Neoplasms/pathology , Biopsy , Lymphoma, T-Cell, Cutaneous/pathology , Pagetoid Reticulosis/therapy , Skin Neoplasms/therapy , Time Factors , Treatment OutcomeABSTRACT
Childhood Granulomatous Periorificial Dermatitis is an acneiform facial rash that affects the periorificial area in children. The clinical aspectare asymptomatic 1-3 mm papules of, monomorphic, erythematous or hypopigmented in periorificial areas - mouth, nose and eyes. It's a benign and self-limited disease that heals spontaneously without scarring and specific therapy. Differential diagnoses include perioral dermatitis, granulomatous-rosacea, sarcoidosis, and lupus miliaris disseminatus faciei. We present the case of a 4-year-old boy, presenting papules in periorificials areas. Due to its low incidence and low number of publications we report the present case.
A Dermatite Periorificial Granulomatosa da Infância é erupção facial acneiforme que afeta área periorificial do segmento cefálico de pré-puberes. Consiste em pápulas assintomáticas de 1 a 3 mm, monomorfas, eritematosas ou hipopigmentadas em áreas periorificiais - boca, nariz e olhos. A doença é benigna e auto-limitada, curando sem deixar cicatriz e por regra sem terapia específica. Diagnósticos diferenciais incluem a dermatite perioral, rosácea granulomatosa, sarcoidose e lúpus miliar da face. Relata-se paciente de 4 anos, masculino, com erupção papulosa há 2 anos em áreas periorificais. Devido à sua baixa incidência e o reduzido número de publicações relata-se o presente caso.
Subject(s)
Humans , Male , Middle Aged , Pagetoid Reticulosis/pathology , Skin Neoplasms/pathology , Biopsy , Lymphoma, T-Cell, Cutaneous/pathology , Pagetoid Reticulosis/therapy , Skin Neoplasms/therapy , Time Factors , Treatment OutcomeABSTRACT
We present a case of bullosis diabeticorum. It is a rare disorder, probably underdiagnosed, associated with long-term diabetes mellitus. Its etiology remains unclear. It is characterized by tense blisters, with serous content, recurrent and spontaneous on normal skin especially in the acral regions. Displays self-limiting course. No specific laboratory tests for diagnosis of this bullous disease exist. Clinical and conservative management to prevent secondary infection reduces morbidity in diabetic patients.
Apresenta-se um caso de bulose diabeticorum, que consiste em uma desordem rara de etiologia ainda incerta, provavelmente subdiagnosticada, associada ao diabetes mellitus de longa evolução. Caracteriza-se por bolhas tensas, recorrentes, de conteúdo seroso e aparecimento espontâneo sobre pele pouco inflamada, especialmente nas regiões acrais, que evolui com curso autolimitado. Não há testes laboratoriais específicos para o diagnóstico desta bulose. O reconhecimento clínico e o manejo conservador para evitar infecção secundária reduz a morbidade nos pacientes diabéticos.
