Description, Causes and Risk Factors:
Atrophoderma vermiculatum is a rare, benign follicular disorder that primarily affects children. It is characterized by symmetric reticular or honeycomb atrophy of the cheeks that may extend to the ears and forehead. The defect is believed to be due to abnormal keratinization in the pilosebaceous follicle.
Atrophoderma vermiculatum was originally described by in 1896. Atrophoderma vermiculatum typically presents in childhood with erythema and follicular keratotic papules that slowly progress to the characteristic reticular atrophy, which has been described as worm-eaten, reticular, or honeycomb, and occurs on the cheeks, preauricular area, and forehead. More rarely, the atrophy may extend to the upper lip; helices; ear lobes; and, in some cases, the limbs. The degree of inflammation, the presence of milia, and the extent of follicular plugs are variable. As seen in our patient, AV tends to be symmetric, but unilateral involvement has been reported. Although AV typically presents before puberty, some patients have developed AV in late adolescence or early adulthood, as seen in our patient. Atrophoderma vermiculatum also is a key feature of Rombo syndrome, an autosomal dominant disorder characterized by AV, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas, and peripheral vasodilation with cyanosis.
The pathogenesis of atrophoderma vermiculatum is unclear. The primary pathologic event seems to be the formation of follicular plugs. Baden and Byers suggest that the keratinocytes in affected individuals mediate release of inflammatory cytokines in response to the plugs. This inflammation then leads to fibrosis and atrophy.
The occurrence of atrophoderma vermiculatum typically is sporadic, though autosomal dominant inheritance has been reported. Atrophoderma vermiculatum usually is an isolated skin finding, but it also has been reported in association with dioxin-induced chloracne, steatocystoma multiplex, epidermal cysts, leukokeratosis oris, leukonychia, congenital heart block, atrial septal defect with associated Eisenmenger complex, neurofibromatosis, and Down syndrome.
Two patients, a father and a daughter, with atrophoderma vermiculatum are presented. The chief characteristics of this rare, benign condition that usually begins in childhood are a symmetric worm-eaten or reticular atrophy of the cheeks that may extend to the ears or forehead. An autosomal dominant mode of inheritance is present in this family. The prominent characteristics, cause, histologic findings, and rare associated medical conditions are reviewed. The prognosis is good, with many instances of spontaneous regression. Primarily a cosmetic problem, therapy for this condition is aimed at reassurance, genetic counseling, and dermabrasion where appropriate.
Atrophoderma vermiculata usually begins in childhood with the onset is between 5 and 12 years old. The cheeks are predominantly involved, with follicular plugs and pit-like depressions and a background of erythema. The depressions merge into each other lead to a striking honeycomb-like atrophy. Scarring alopecia may occur, but rarely. The course is usually one of inexorable worsening. The condition is often sporadic but, in some cases, appears to be inherited as an autosomal dominant trait.
Diagnosis is done by microscopic examination;atrophoderma vermiculatum may display atrophic follicles with sclerosis of dermal collagen, loss of rete ridges, follicular plugs, and variable degrees of perifollicular inflammation.
Management of atrophoderma vermiculatum is challenging. Atrophoderma vermiculatum is mainly a cosmetic problem. However, taking under consideration the emotional stress and the permanent atrophy that it provokes, a therapeutic attempt is considered reasonable. Topical treatment options that have been suggested for this condition include keratolytics, topical and intralesional steroids, and ultraviolet irradiation. When the disease remains stable and well-controlled, dermabrasion, collagen implants, CO2 and Erbium Yag lasers can be also used. The use of topical retinoids was also suggested for keratosis pilaris atrophicans faciei.
There have been previous literature data reporting a beneficial effect of prolonged use of oral retinoids. Among oral retinoids, isotretinoin appears to hold the advantage of a shorter half-life, which minimizes possible adverse events. It has to be considered that, apart from possible common adverse events, this treatment may promote premature epiphyseal plate closure and may stop normal growth in some children.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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