Follicular occlusion triad: an isotopic response or adverse effect of rituximab?

Follicular occlusion triad is a symptom complex of three conditions with a similar pathophysiology including hidradenitis suppurativa (HS), dissecting cellulitis of the scalp, and acne conglobata. Although the exact pathogenesis of the triad is unknown, it appears to be related to follicular occlusion in areas with apocrine glands. Wolf isotopic response refers to the occurrence of a new dermatosis at the site of another, unrelated, previously healed dermatosis. We present a 26-year-old man with a history of pemphigus foliaceus (PF) who developed large draining nodules with scarring and sinus tracts, compatible with follicular occlusion triad, preferentially at areas previously affected by PF thirteen months after treatment with rituximab. To the authors' knowledge there are no reported cases of follicular occlusion triad or HS manifesting as an isotopic response. However, one member of the triad, HS, has been reported to occur infrequently following the use of biologic agents such as adalimumab, infliximab, tocilizumab, and rituximab for chronic immune-mediated inflammatory diseases (psoriasis, Crohn disease, rheumatoid arthritis, and ankylosing spondylitis).

Cutaneous hemophagocytosis: Clinicopathologic features of 21 cases.

BACKGROUND: Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. OBJECTIVE: To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD. METHODS: Case series analyzing clinicopathologic features and follow-up data on patients presenting with histopathologic signs of CH. RESULTS: Biopsy specimens from 21 patients were included. None of the patients had HLHS. The majority (n = 11) presented with leukocytoclastic vasculitis. Other associated diseases were lupus erythematous (n = 2), arthropod bite reaction (n = 2), erysipelas (n = 1), acne conglobata (n = 1), and Sweet syndrome (n = 1). Three patients had a nonspecific rash concomitant with Chlamydia pneumonia, middle ear infection, and pharyngitis, respectively. LIMITATIONS: This was a single-center, retrospective study. CONCLUSION: Isolated CH in conditions other than CTCL and cRDD is a histopathologic finding related mostly to leukocytoclastic vasculitis. Extensive investigations should be performed only if patients have other signs or symptoms of HLHS.

Caracterización clínico epidemiológica del acné conglobata en las provincias de Holguín y Granma / Clinical Epidemiological Description of Conglobate Acne in Holguin and Granma Provinces

Introducción: el acné es una de las afecciones dermatológicas más frecuentes en la práctica médica, de ellas, el acné conglobata se caracteriza por ser poco común. La génesis del acné conglobata es compleja y depende de la interacción de varios factores, entre ellos, los genéticos.Objetivo: caracterizar de forma clínica, epidemiológica e histopatológica el acné conglobata en familiares de la región Holguín –Granma.Método: se realizó un estudio de serie de casos en el período comprendido de enero 2000 a diciembre 2014. Se describió el contexto medioambiental donde se desarrollaron los enfermos. Los enfermos fueron examinados para confeccionar el árbol genealógico, se le realizó seguimiento clínico de las lesiones y biopsia para estudio histopatológico.Resultados: la enfermedad afectó a mujeres y hombres en edad antes de 21 años. Las primeras lesiones generalmente fueron noduloquísticas. Los quistes, los nódulos, los macrocomedones, los conglomerados fistulizados, las bridas cicatriciales tuvieron poca capacidad de resolución con el tratamiento convencional y alcanzaron grandes tamaños a medida que avanzó el tiempo de evolución. Las lesiones se distribuyeron con predilección en la espalda, las axilas y los glúteos. Los cambios histopatológicos fueron la hiperqueratosis con tapones córneos, las alteraciones foliculares y la presencia de los quistes de inclusión epidérmica con trayectos fistulosos. La herencia se comportó autonómico dominante. Las zonas con mayor número de casos fueron las dispuestas en las márgenes del río Cauto y en lugares aledaños.Conclusiones: se definieron los elementos diagnósticos de la enfermedad, tanto clínico y epidemiológicos, como histopatológicos.(AU)

Introduction: acne is one of the most frequent dermatology affections in medical practices, and conglobate acne is characterized as uncommon. The genesis of this illness is complicated and depends on the interaction of many factors, for example the genetic factors.Objective: to describe histopatological, epidemiological and clinically the conglobate acne incidence in some families from Holguín Granma regions.Method: a case series study was carried out for the period from January 2000 to December 2014. The environmental context where the patients were developed was described. The patients were examined to make the family tree, clinical follow-up of lesions and biopsy for histopathological study.Results: the disease affected women and men before the age of 21. The nodule cystic lesions were the first ones. Nodules, macrocomedones, fistulized conglomerates, scar flanges had little resolution capacity with conventional treatment and reached large sizes as the evolution time advanced. The lesions were distributed with a preference in the back, underarms and glutes. Histopathological changes were hyperkeratosis with corneal plugs, follicular alterations and the presence of epidermal inclusion cysts with fistulous pathways. The inheritance behaved autonomously dominant. The areas with the highest number of cases were those located on the banks of the Cauto River and in surrounding areas.Conclusions: the diagnostic elements of the disease, both clinical and epidemiological, as well as histopathological, were defined.(AU)