Juvenile gangrenous vasculitis of the scrotum was described by Piňol et al in 1974 as a unique variant of scrotal gangrene of unknown origin, occurring exclusively in young individuals. It was characterized by an acu...Juvenile gangrenous vasculitis of the scrotum was described by Piňol et al in 1974 as a unique variant of scrotal gangrene of unknown origin, occurring exclusively in young individuals. It was characterized by an acute onset of skin ulcers undergoing complete resolution after appropriate therapy, with no relapses. We present a typical case of this extremely rare disease affecting a 16-year-old boy in whom the scrotal ulcerations were preceded by an episode of pharyngitis with fever. The condition promptly regressed after administration of intramuscular betamethasone in combination with oral ciprofloxacin. According to Piňol et al, juvenile gangrenous vasculitis of the scrotum, although poorly known to dermatologists, should be regarded as a distinctive entity within the wide group of scrotal gangrenes. On the other hand, the hypothesis that this condition may well represent a variant of pyoderma gangrenosum is discussed.展开更多
We describe a 72-year-old woman with a 13-year history of a lichenoid dermatitis, who developed multiple, papular keratoacanthoma (KA)-like lesions and few crater-like nodules on the extremities over a period of 6 mon...We describe a 72-year-old woman with a 13-year history of a lichenoid dermatitis, who developed multiple, papular keratoacanthoma (KA)-like lesions and few crater-like nodules on the extremities over a period of 6 months before our observation. Her medical history also recorded multiple myeloma diagnosed a few years before. The long-standing dermatosis was diagnosed, clinically, as keratosis lichenoides chronica (KLC), although, histologically, a lichenoid tissue reaction pattern was not evident. On the other hand, histology from papular and nodular lesions of recent onset was consistent with a possible early phase of KA and spinocellular carcinoma, respectively. Oral acitretin induced regression of KA-like lesions and improvement of KLC but had no effects on crater-like nodules, which required surgical excision, KLC is a chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhoea-like dermatitis. We emphasize in our case the association between KLC and multiple possible KAs, never previously reported, and speculate that these two rare conditions may represent here a ’ continuum’ from a pathogenetic point of view.展开更多
Reticular erythematous mucinosis(REM)is a rare,primary cutaneous mucinosis clinically characterized by a persistent reticular erythema on the mid chest and mid-upper back,and histologically by a mononuclear cell infil...Reticular erythematous mucinosis(REM)is a rare,primary cutaneous mucinosis clinically characterized by a persistent reticular erythema on the mid chest and mid-upper back,and histologically by a mononuclear cell infiltrate and deposits of mucin in the dermis.To our knowledge,the present report of REM occurring in a Caucasian man and his sister is the first reported case of familial REM.Since a host-specific immune response to unknown antigens may be involved in the pathogenesis of this entity,human leukocyte antigen typing was determined and compared to those reported in autoimmune diseases.展开更多
We describe a male patient with rosacea who had a 2-year history of persistent bilateral oedema of the eyelids, leading to an elephantoid condition with blepharoptosis.An upper eyelid blepharoplasty was performed, but...We describe a male patient with rosacea who had a 2-year history of persistent bilateral oedema of the eyelids, leading to an elephantoid condition with blepharoptosis.An upper eyelid blepharoplasty was performed, but swelling progressively recurred over a few months.Based on the case history, clinical appearance and histological findings, rosaceous lymphoedema was considered to be the diagnosis.The latter is a bilateral, solid oedema of the mid-third of the face, regarded as a rare complication of rosacea.It is thought to occur as a result of chronic inflammation and lymphatic stasis, but its exact aethiopathogenesis remains elusive.Predominant eyelid involvement, causing severe visual impairment as in our patient, is unique.展开更多
Anetoderma is a rare cutaneous disease characterized by a loss of normal elast ic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunologi...Anetoderma is a rare cutaneous disease characterized by a loss of normal elast ic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, mos t notably lupus erythematosus and antiphospholipid antibodies with or without cl inical manifestations of the antiphospholipid syndrome. A retrospective study wa s conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetoderma-associated lupus erythemato sus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 pat ients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus pannic ulitis may be regarded as an uncommon variant of cutaneous lupus erythematosus m ainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholi pid syndrome. Antiphospholipid antibodies could play a role in the elastolytic p rocess, leading to anetoderma.展开更多
Background: Wells syndrome, an uncommon inflammatory dermatosis, is characterized by protean cutaneous manifestations, suggestive but not specific histopathologic findings, and usually a recurrent course. Because of i...Background: Wells syndrome, an uncommon inflammatory dermatosis, is characterized by protean cutaneous manifestations, suggestive but not specific histopathologic findings, and usually a recurrent course. Because of its original description as a distinct entity, it has come to be regarded as an abnormal eosinophilic response to a number of causative agents. Observations: The medical records of 19 patients (12 adults and 7 children) with Wells syndrome referred to the Institute of Dermatological Sciences from 1990 to 2005 were evaluated for the type and prevalence of skin lesions, clinical course and response to treatment, and possibly associated systemic symptoms, as well as histologic, laboratory, and immunofluorescence findings. The classic plaque-type variant proved to be the most common presentation in children but not in adults, who more frequently had the annular granuloma-like variant. Unilesional forms were found to occur more frequently in children. The course was recurrent, although slowly progressing, with amean duration of disease of 5 years for adults and 3 years for children. Conclusions: We emphasize the concept that the diagnosis of Wells syndrome is a clinicopathologic diagnosis. Although it should be classified within a spectrum that includes multisystem eosinophilic disorders, such as Churg-Strauss and hypereosinophilic syndromes, Wells syndrome, which has 7 variants, is a distinct cutaneous disease lacking systemic involvement.展开更多
文摘Juvenile gangrenous vasculitis of the scrotum was described by Piňol et al in 1974 as a unique variant of scrotal gangrene of unknown origin, occurring exclusively in young individuals. It was characterized by an acute onset of skin ulcers undergoing complete resolution after appropriate therapy, with no relapses. We present a typical case of this extremely rare disease affecting a 16-year-old boy in whom the scrotal ulcerations were preceded by an episode of pharyngitis with fever. The condition promptly regressed after administration of intramuscular betamethasone in combination with oral ciprofloxacin. According to Piňol et al, juvenile gangrenous vasculitis of the scrotum, although poorly known to dermatologists, should be regarded as a distinctive entity within the wide group of scrotal gangrenes. On the other hand, the hypothesis that this condition may well represent a variant of pyoderma gangrenosum is discussed.
文摘We describe a 72-year-old woman with a 13-year history of a lichenoid dermatitis, who developed multiple, papular keratoacanthoma (KA)-like lesions and few crater-like nodules on the extremities over a period of 6 months before our observation. Her medical history also recorded multiple myeloma diagnosed a few years before. The long-standing dermatosis was diagnosed, clinically, as keratosis lichenoides chronica (KLC), although, histologically, a lichenoid tissue reaction pattern was not evident. On the other hand, histology from papular and nodular lesions of recent onset was consistent with a possible early phase of KA and spinocellular carcinoma, respectively. Oral acitretin induced regression of KA-like lesions and improvement of KLC but had no effects on crater-like nodules, which required surgical excision, KLC is a chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhoea-like dermatitis. We emphasize in our case the association between KLC and multiple possible KAs, never previously reported, and speculate that these two rare conditions may represent here a ’ continuum’ from a pathogenetic point of view.
文摘Reticular erythematous mucinosis(REM)is a rare,primary cutaneous mucinosis clinically characterized by a persistent reticular erythema on the mid chest and mid-upper back,and histologically by a mononuclear cell infiltrate and deposits of mucin in the dermis.To our knowledge,the present report of REM occurring in a Caucasian man and his sister is the first reported case of familial REM.Since a host-specific immune response to unknown antigens may be involved in the pathogenesis of this entity,human leukocyte antigen typing was determined and compared to those reported in autoimmune diseases.
文摘We describe a male patient with rosacea who had a 2-year history of persistent bilateral oedema of the eyelids, leading to an elephantoid condition with blepharoptosis.An upper eyelid blepharoplasty was performed, but swelling progressively recurred over a few months.Based on the case history, clinical appearance and histological findings, rosaceous lymphoedema was considered to be the diagnosis.The latter is a bilateral, solid oedema of the mid-third of the face, regarded as a rare complication of rosacea.It is thought to occur as a result of chronic inflammation and lymphatic stasis, but its exact aethiopathogenesis remains elusive.Predominant eyelid involvement, causing severe visual impairment as in our patient, is unique.
文摘Anetoderma is a rare cutaneous disease characterized by a loss of normal elast ic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, mos t notably lupus erythematosus and antiphospholipid antibodies with or without cl inical manifestations of the antiphospholipid syndrome. A retrospective study wa s conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetoderma-associated lupus erythemato sus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 pat ients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus pannic ulitis may be regarded as an uncommon variant of cutaneous lupus erythematosus m ainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholi pid syndrome. Antiphospholipid antibodies could play a role in the elastolytic p rocess, leading to anetoderma.
文摘Background: Wells syndrome, an uncommon inflammatory dermatosis, is characterized by protean cutaneous manifestations, suggestive but not specific histopathologic findings, and usually a recurrent course. Because of its original description as a distinct entity, it has come to be regarded as an abnormal eosinophilic response to a number of causative agents. Observations: The medical records of 19 patients (12 adults and 7 children) with Wells syndrome referred to the Institute of Dermatological Sciences from 1990 to 2005 were evaluated for the type and prevalence of skin lesions, clinical course and response to treatment, and possibly associated systemic symptoms, as well as histologic, laboratory, and immunofluorescence findings. The classic plaque-type variant proved to be the most common presentation in children but not in adults, who more frequently had the annular granuloma-like variant. Unilesional forms were found to occur more frequently in children. The course was recurrent, although slowly progressing, with amean duration of disease of 5 years for adults and 3 years for children. Conclusions: We emphasize the concept that the diagnosis of Wells syndrome is a clinicopathologic diagnosis. Although it should be classified within a spectrum that includes multisystem eosinophilic disorders, such as Churg-Strauss and hypereosinophilic syndromes, Wells syndrome, which has 7 variants, is a distinct cutaneous disease lacking systemic involvement.
