Reacción granulomatosa en paciente con melanoma metastásico tratado con ipilimumab: primer caso descrito presentando clínica cutánea únicamente / Granulomatous Reaction in a Patient With Metastatic Melanoma Treated With Ipilimumab: First Case Reported With Isolated Cutaneous Findings

Las reacciones granulomatosas "sarcoidosis-like" secundarias a ipilimumab afectarán simultáneamente a múltiples órganos y característicamente se presentarán semanas o meses después de haber iniciado el tratamiento. Paciente varón de 67 años, quien consulta por reacción cutánea granulomatosa a nivel de tronco, extremidades y rostro. Esta se presentó 18 meses después de haber iniciado tratamiento con ipilimumab, medicamento pautado por el diagnóstico de un melanoma metastásico. Reportamos reacción granulomatosa de presentación exclusivamente cutánea, como efecto adverso medicamentoso tardío secundario al tratamiento con ipilimumab. Así mismo resaltamos la necesidad de realizar en los pacientes tratados con ipilimumab un seguimiento prolongado

Granulomatous sarcoidosis-like reactions affecting multiple organ systems at one time have infrequently been described within weeks to months after initiation of Ipilimumab. We present the first case of a 67-year-old man with isolated cutaneous granulomatous reaction involving the trunk, extremities, and face after eighteen months of treatment with ipilimumab for metastatic melanoma. This case documents the eruption of isolated cutaneous granulomatous reaction as a late treatment-related adverse effect of ipilimumab, highlighting the importance of adequate, prolonged follow-up

Granulomatous Reaction in a Patient With Metastatic Melanoma Treated With Ipilimumab: First Case Reported With Isolated Cutaneous Findings. / Reacción granulomatosa en paciente con melanoma metastásico tratado con ipilimumab: primer caso descrito presentando clínica cutánea únicamente.

Granulomatous sarcoidosis-like reactions affecting multiple organ systems at one time have infrequently been described within weeks to months after initiation of Ipilimumab. We present the first case of a 67-year-old man with isolated cutaneous granulomatous reaction involving the trunk, extremities, and face after eighteen months of treatment with ipilimumab for metastatic melanoma. This case documents the eruption of isolated cutaneous granulomatous reaction as a late treatment-related adverse effect of ipilimumab, highlighting the importance of adequate, prolonged follow-up.

Localized pemphigus foliaceus with unilateral facial involvement.

Pemphigus foliaceus is a superficial vesiculobullous disease that typically presents with widespread lesions. Localized presentations are less frequent, and they typically occur in middle-aged patients, following exposure to topical medications, and later on, become more disseminated. We present a case of a 19-year-old female with a localized presentation of pemphigus foliaceus unrelated to previous topical medications, that was a diagnostic and therapeutically challenging case. We also discuss the literature on localized cases, differences in presentations and responses to various treatment modalities.

Pénfigo foliáceo localizado con afectación facial unilateral / Localized Pemphigus Foliaceus with Unilateral Facial Involvement

El pénfigo foliáceo es una enfermedad vesículo-ampollosa superficial caracterizada por la aparición de lesiones generalizadas. Las presentaciones localizadas son menos frecuentes y suelen observarse en pacientes de mediana edad tras la exposición a medicamentos tópicos que posteriormente evolucionan a formas más diseminadas. Presentamos el caso de una mujer de 19 años de edad con pénfigo foliáceo localizado no asociado a medicamentos tópicos previos cuyo diagnóstico y tratamiento han supuesto un reto. También analizamos la literatura existente sobre los casos de pénfigo foliáceo localizado, las diferencias en las presentaciones clínicas y las respuestas a distintos tipos de tratamientos

Pemphigus foliaceus is a superficial vesiculobullous disease that typically presents with widespread lesions. Localized presentations are less frequent, and they typically occur in middle-aged patients, following exposure to topical medications, and later on, become more disseminated. We present a case of a 19-year-old female with a localized presentation of pemphigus foliaceus unrelated to previous topical medications, that was a diagnostic and therapeutically challenging case. We also discuss the literature on localized cases, differences in presentations and responses to various treatment modalities

Púrpura fulminante en relación con septicemia por Streptococcus pneumoniae en un paciente pediátrico asplénico / Purpura Fulminans Associated with Streptococcus pneumoniae Septicemia in an Asplenic Pediatric Patient

La púrpura fulminante es un síndrome rápidamente progresivo de trombosis de pequeños vasos y necrosis hemorrágica de la piel que se acompaña de coagulación intravascular diseminada. Describimos un caso de septicemia por Streptococcus pneumoniae en un niño de 5 años de edad tratado con tacrolimus oral, con una historia médica previa de trasplante de múltiples vísceras y sin bazo, y el desarrollo subsiguiente de púrpura fulminante en su pecho y la parte distal de sus extremidades. La forma aguda infecciosa de púrpura fulminante es debida habitualmente a bacterias gramnegativas. Los casos secundarios a bacterias grampositivas encapsuladas ocurren por lo general cuando los individuos están inmunosuprimidos o presentan asplenia funcional o anatómica. Nuestro paciente presentaba ambas condiciones, lo cual con seguridad aumentó su susceptibilidad, y respondió bien a la terapia antimicrobiana además de a la cobertura profiláctica en el contexto de su inmunosupresión. Revisamos la literatura buscando casos similares debidos a Streptococcus pneumoniae en la población pediátrica y discutimos la etiología y el tratamiento de la púrpura fulminante (AU)

Purpura fulminans is a rapidly progressive syndrome of small-vessel thrombosis and hemorrhagic necrosis of the skin accompanied by disseminated intravascular coagulation. We describe a case of Streptococcus pneumoniae septicemia in an asplenic 5-year-old boy on oral tacrolimus, with a past medical history of multivisceral organ transplantation and subsequent development of purpura fulminans on his chest and distal extremities. The acute infectious form of purpura fulminans is usually caused by gram-negative bacteria. Cases secondary to gram-positive encapsulated bacteria usually occur when individuals are immuno-suppressed or have anatomic or functional asplenia. Our patient had both, which likely increased his susceptibility, and he responded well to antimicrobial therapy in addition to prophylactic coverage in the setting of his immunosuppression. We review the literature for similar cases due to S. pneumoniae in the pediatric population and discuss the etiology and treatment of purpura fulminans (AU)

Purpura fulminans associated with Streptococcus pneumoniae septicemia in an asplenic pediatric patient.

Purpura fulminans is a rapidly progressive syndrome of small-vessel thrombosis and hemorrhagic necrosis of the skin accompanied by disseminated intravascular coagulation. We describe a case of Streptococcus pneumoniae septicemia in an asplenic 5-year-old boy on oral tacrolimus, with a past medical history of multivisceral organ transplantation and subsequent development of purpura fulminans on his chest and distal extremities. The acute infectious form of purpura fulminans is usually caused by gram-negative bacteria. Cases secondary to gram-positive encapsulated bacteria usually occur when individuals are immuno-suppressed or have anatomic or functional asplenia. Our patient had both, which likely increased his susceptibility, and he responded well to antimicrobial therapy in addition to prophylactic coverage in the setting of his immunosuppression. We review the literature for similar cases due to S. pneumoniae in the pediatric population and discuss the etiology and treatment of purpura fulminans.

Tinea faciei inflamatoria que simula un síndrome de Sweet / Inflammatory Tinea Faciei Mimicking Sweet’s Syndrome

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Bullous pemphigoid presenting as exfoliative erythroderma.

Several unusual clinical presentations of bullous pemphigoid have been described such as localized, vesicular, vegetating, nodular, and sine bulla pemphigoid. Patients with erythroderma who fulfill diagnostic criteria for bullous pemphigoid and in whom bullae develop have been reported. We describe a patient with exfoliative erythroderma without any blistering in whom a diagnosis of bullous pemphigoid was based on examination of a biopsy specimen. Circulating antibodies to the basement membrane zone, with an epidermal pattern on salt split skin, and the presence of eosinophilic spongiosis in the skin biopsy specimen supported the diagnosis.

Bullous pemphigoid presenting as generalized pruritus: observations in six patients.

BACKGROUND: Bullous pemphigoid is a chronic immunobullous disease of the elderly. Classically, tense, pruritic blisters develop on normal or erythematous skin. These may be preceded by a prodromal pruritic, urticarial, or eczematous eruption. Occasionally, patients may develop generalized pruritus without blisters as a prodrome of bullous pemphigoid. METHODS: The records of the patients were reviewed. Biopsy specimens were studied by light and immunofluorescence microscopy. Serum specimens were studied by indirect immunofluorescence techniques including the salt-split skin technique. RESULTS: We studied six elderly patients presenting with generalized pruritus as the dominant or single presenting feature of early bullous pemphigoid. Two of the six had rare vesicles at presentation. All had excoriations and one each presented with minimal urticarial or eczematous papules. Routine skin biopsies were largely nonspecific. All patients had confirmation of their diagnosis by either indirect or direct immunofluorescence testing or both. All six patients had their disease completely controlled by their treatment. CONCLUSIONS: The clinical presentation of the six patients in our series and the eight previously reported patients should be regarded as an unusual prodromal manifestation of bullous pemphigoid characterized by generalized pruritus without primary skin lesions. This presentation could be described as "pruritic pemphigoid," because it joins the remarkable clinical finding of generalized pruritus with the underlying diagnosis of bullous pemphigoid. Elderly patients with severe or persistent unexplained generalized pruritus merit immunofluorescence testing to exclude bullous pemphigoid as the cause of the generalized pruritus. Establishing an early diagnosis permits the prompt institution of effective therapy with dapsone or systemic corticosteroids with an excellent prognosis for complete control of the disease.

Cutaneous metastasis from a presumed signet-ring cell carcinoma in a 10-year-old child.

Cutaneous metastases of internal malignancies are very rare in children. In this group, neuroblastoma, leukaemia and lymphoma are the most common malignancies that may develop metastases or neoplastic infiltrates to the skin. Carcinomas have infrequently been reported in children, and cutaneous metastases from carcinoma in this group have not been described. A 10-year-old girl presented with an erythematous plaque on the left hemithorax. Histopathological findings revealed grouped signet-ring cells within the lumina of lymphatic vessels in the dermis. Immunohistochemical examination confirmed the epithelial origin of the tumour. Despite an exhaustive search, the primary site could not be determined. This exceptional observation is, to the best of our knowledge, the first report of cutaneous metastasis from occult carcinoma in a child.

Vitamin K1-induced localized scleroderma (morphea) with linear deposition of IgA in the basement membrane zone.

We describe a 45-year-old white man in whom distinctive clinical and histologic features of localized scleroderma developed at sites of injection of vitamin K1 (phytonadione). A direct immunofluorescence test demonstrated prominent linear deposition of IgA along the basement membrane zone. No circulating antibasement membrane zone IgA antibodies were identified on indirect immunofluorescence testing. We believe that the unusual immunofluorescence finding in our patient is nonspecific and represents an epiphenomenon caused by cutaneous injury.

No evidence for Borrelia burgdorferi infection in lesions of morphea and lichen sclerosus et atrophicus in Spain. A prospective study and literature review.

The possible association of Borrelia burgdorferi with morphea and lichen sclerosus et atrophicus has been the focus of research and discussion in dermatology during the last 10 years. To investigate the etiopathogenic role of B. burgdorferi in morphea and lichen sclerosus et atrophicus lesions in Spain, we studied 14 cases: 8 patients with lichen sclerosus et atrophicus and 6 with morphea. For the whole group, a prospective study was performed, including serologic studies by indirect immunofluorescence, histologic evaluation of skin biopsy specimens, culture studies, and polymerase chain reaction with different primers sensitive for detecting virtually all B. burgdorferi strains tested to date. Although one patient with morphea had positive serologic findings at low titer, we were not able to culture or detect borrelial DNA in any of the specimens. These findings do not confirm an association between B. burgdorferi and morphea and lichen sclerosus et atrophicus.