Cutaneous Mucinosis of Infancy

Abstract: Cutaneous mucinosis of infancy has been reported only rarely in the literature. We describe a case occurring in a black infant giri. Although no associated abnormalities have been described previously, our patient had a history of developmental delay, congenital cataracts, bilateral inguinal hernias, and an accessory tragus. The significance of these features is unclear.     

An Uniisual Case of Infantile Dermal Mucinosis

Abstract: Dermal mucinosis occurred in a 3-month-old and persisted for six years. The features suggest is represents a novel type of childhood mucinosis.     

皮肤局灶性黏蛋白病1例

患者男,53岁。颈部丘疹20年余,无自觉症状,也无甲状腺功能异常。皮损组织病理示:真皮中上部胶原纤维稀疏,其内充满均质性无定形物质。阿新蓝染色阳性。诊断:皮肤局灶性黏蛋白病。     

Cutaneous Mucinosis in a Child with Systemic Lupus Erythematosus

Cutaneous mucinosis is rarely associated with systemic lupus erythematosus. A 13-year-old girl had a two-month history of numerous cutaneous mucinous papules and the new onset of systemic lupus erythematosus. The differential diagnosis and pathogenesis of this variant of cutaneous mucinosis are discussed. This is the first report of a child with this unusual entity.     

Nodular Lesions of Self-healing Juvenile Cutaneous Mucinosis: A Pitfall!

ABSTRACT:: Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder of unknown origin, which occurs in children in good health. It is characterized by the multiplication of transient cutaneous papules and nodules, mainly located on the head and periarticular areas that spontaneously resolve. Histological features of SHJCM have been well described; therefore, the diagnosis is usually made easily when papules are biopsied. We report a series of 3 new cases of SHJCM presenting mainly with nodular lesions. Histological examination of these nodules showed either lesions consistent with nodular or proliferative fasciitis or nonspecific panniculitis. Mucinous deposits were present but often inconspicuous, so could be disregarded. We wanted to emphasize this misleading presentation because a biopsy for histological examination is always mandatory in cases of proliferating nodules to rule out malignant tumors. Therefore, the diagnosis always requires discussion between pathologists and clinicians to rapidly reassure the parents and avoid inappropriate therapy.     

Mucinosis eritematosa reticular

—Reticular erythematous mucinosis (REM) is a rare entity characterized by infiltrated erythematous plaques on exposed areas of the thorax and back. It has been related to sun exposure, hormonal alterations, rheumatological processes and breast cancer.A 28-year-old woman presented with an infiltrated erythematous plaque on the left breast, which had been developing for 4 years, and which had spread to both breasts over time. The histopathological study showed a perivascular and periadnexal lymphocytic infiltrate, which was accompanied by evident mucin deposits in the upper and mid-dermis. Treatment with colchicine and hydroxychloroquine was administered, with partial regression of the lesions.     

Ultrastructure of Follicular Mucinosis

Electron microscopic study of follicular mucinosis revealed, in the affected hair follicles, disappearance of cell organelles, granular agglutination of the cytoplasmic matrices and the nuclear chromatin usually connected with tonofilaments. Myelin figures and onion-like aggregations of low-electron-density lamellar structures were found in the areas of severe cytoplasmic degeneration. Findings suggesting a release of mucin by a degeneration or a degradation process were not detected, although granular substances and collagen fibrils apparently coated with an amorphous material were observed.     

毛囊粘蛋白病(附2例报告)

1957年Pinkus等^①以“粘蛋白性秃发”(Alopecia mucinosa)的命名报告了本病6例.1959年Jablonska等^②提议改名为“毛囊粘蛋白病”(Follicular mucinosis),因为并非每个病例都具有秃发.     

毛囊粘蛋白病1例

患者男,21岁。头部浸润性斑块伴脱发8年。组织病理示毛囊周围可见粘蛋白样物质沉积,甲苯胺蓝染色呈紫红色。诊断:毛囊粘蛋白病。     

毛囊黏蛋白病1例

患者男,13岁。头皮起红疹伴脱发半年。皮肤科情况:后枕部见一1.5cm×1.5cm红色圆形斑块,其上有细小脱屑,头发大部分脱落。皮损组织病理毛囊口角栓形成,真皮内毛囊皮脂腺细胞中大量淡兰色物质沉积,血管周围不等量炎细胞浸润;阿西蓝染色阳性。诊断:毛囊黏蛋白病。     

Cutaneous Mucinosis of Infancy: A Rare Congenital Case with Coexisting Progressive,Eruptive, and Spontaneously Involuting Lesions

Cutaneous mucinosis in infancy is rare. We report an infant with multiple congenital papules distributed over the trunk, neck, and extremities. These papules were mainly dispersed, but they also coalesced into plaques. Histopathologic findings showed features of cutaneous mucinosis of infancy (CMI). Over 2 years of follow‐up, we observed that the preexisting lesions on the lower back and left trunk progressively increased in size, and a few new scattered papules continued to appear, mainly on the trunk; several lesions spontaneously resolved with no further complications. CMI is considered to be a persistent cutaneous disorder, even though spontaneously regressing cases have rarely been reported. This case demonstrates the broad clinical spectrum of CMI, with progressive, eruptive, and spontaneously involuting lesions all present in the same patient. This condition should be considered in the differential diagnosis of congenital or infantile‐onset papules and plaques, especially those yellowish in color.     

Benign Idiopathic versus Mycosis-Fungoides-Associated Follicular Mucinosis

A study was undertaken in an attempt to identify useful histologic criteria that may allow differentiation between benign idiopathic and mycosis-fungoides-associated follicular mucinosis. We chose young patients because no person under 20 years of age with coexisting follicular mucinosis and mycosis fungoides disease has ever been reported. Our three most important observations in benign juvenile idiopathic follicular mucinosis were as follows: The lymphocytic infiltrate was generally confined to follicular, perifollicular, or perivascular zones with no extension of either normal or atypical mononuclear cells into the epidermis or into papillary/reticular dermis. Within follicular epithelium there were dense collections of lymphocytes with occasionally atypical-appearing nuclei in three of the eight patients, but never as Pautrier microabscesses. There was absence of a significant associated plasma cell or eosinophil-containing inflammatory dermal infiltrate. These findings are in contrast to those of older patients with follicular mucinosis and mycosis fungoides.     

Multiple Familial Pilomatricomas: A Cutaneous Marker for Gardner Syndrome?

Abstract: A 40-year-old man and his 6-year-old only son had numerous, firm papulonodular lesions on their faces. Their medical histories were unremarkable and no family consanguinity was recorded. Surgical excision of several lesions was performed on each patient. All the lesions were solid tumors with the characteristic histopathologic features of pilomatricoma. A gastrointestinal radiologic and fibroscopic survey disclosed numerous adenomatous colonic polyps in the father. Additional studies revealed that he also had minor dental abnormalities, a small osteoma on the right mandible, and unilateral, ocular, pigmented retinal macules. The diagnosis of multiple adenomatous colonic polyposis was established only after the well-known association of pilomatricoma-like changes in epidermal cysts in patients with Gardner syndrome was considered. Possibly, multiple familial pilomatricomas could be considered a cutaneous marker of Gardner syndrome.