Atrophoderma vermiculata, a form of keratosis pilaris atrophicans, typically presents in childhood with erythema and follicular keratotic papules that slowly progress to characteristic atrophy, which has been described as worm-eaten, reticular, or honeycomb, and occurs on the cheeks, ... Atrophoderma vermiculata, a form of keratosis pilaris atrophicans, typically presents in childhood with erythema and follicular keratotic papules that slowly progress to characteristic 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 (summary by Luria and Conologue, 2009).
MacKee and Parounagian (1918) reported a girl and a boy, aged 16 and 9 years, respectively, with a symmetric eruption over the greater part of both cheeks that consisted of small, pit-like, atrophic areas, irregular in shape and ... MacKee and Parounagian (1918) reported a girl and a boy, aged 16 and 9 years, respectively, with a symmetric eruption over the greater part of both cheeks that consisted of small, pit-like, atrophic areas, irregular in shape and bounded by narrow ridges, which were waxy in appearance and on the same level with unaffected skin, producing a reticulated or honeycomb appearance. Milia were present in the ridges, a few comedones were seen in the depressed areas and in the ridges, and horny plugs were visible. Histologic examination showed a slightly atrophic epidermis with loss of rete pegs. Inflammation in the derma was manifested by vascular and lymphatic dilatation, congestion, edema, and perivascular and perifollicular infiltration of small round cells. There was underdevelopment of the sebaceous glands and a marked overdevelopment of hair follicles. Horn cysts derived from the hair follicles. Little (1920) described a 15-year-old boy who at age 10 years had developed tiny pitted depressions on both cheeks. Scattered within the affected area were a few comedones, filled with hard horny plugs. Little (1920) considered the disorder to be the same as that described by MacKee and Parounagian (1918), but suggested naming the disorder 'folliculitis atrophicans reticulata' because erythema was not a notable feature in his patient. Wertheimer (1932) reported a case of 'folliculitis ulerythematosa reticulata' in a 15-year-old girl with a 5-year history of the condition. Burgess (1932) reported a 14-year-old girl with AVA who at age 9 years had experienced pitting of the skin on her forehead, which later spread to her cheeks. Examination showed innumerable, small, closely set, pit-like areas of atrophy separated by narrow ridges, giving a reticulated appearance. Small follicular horny plugs were present in some places. The affected areas on the cheeks appeared to be erythematous due to fine telangiectases, which were present both on the ridges and on the depressed areas. Histologic examination showed slight atrophy of the epidermis with acanthosis of the follicles. The follicular orifices were dilated and filled with horny plugs. There was general hypoplasia of the sebaceous glands. Although AVA usually occurs alone, Carol et al. (1940) described a family in which atrophodermia reticulata was associated with neurofibromatosis in a mother, son, and daughter. The other daughter had only NF. NF had been inherited from the woman's father, a brother of hers being also affected. Atrophodermia reticulata was seen only in this woman, 1 of 11 sibs, and 2 of her 3 children as mentioned. It affected also the limbs in the daughter and was associated with cardiac defects. A 'mongoloid expression of the face and a slight degree of oligophrenia' were described. Kooij and Venter (1959) reported one instance of atrophodermia reticulata also affecting the limbs and associated with cardiac defects. No consanguinity was known; the parents and 4 other sibs were unaffected. Nico et al. (1998) described a 5-year-old girl with a 6-month history of a rash on her right cheek, which had been diagnosed as 'eczema.' Examination showed multiple tiny, slightly depressed, yellow-pinkish, isolated and confluent lesions arranged in a band-like fashion from the right cheek to the upper lip. Although there was no gross macroscopic atrophy, the histopathologic specimen exhibited pilosebaceous compromise with atrophy and fibrosis. Luria and Conologue (2009) reported a 20-year-old man who presented with progressive atrophic scarring of both cheeks of 3 years' duration, associated with numerous follicular keratotic papules on the extensor surface of the arms and back. He was asymptomatic and otherwise healthy. He denied any history of acne or alopecia. A punch biopsy specimen of an atrophic keratotic papule on the upper back showed follicular hyperkeratosis with mild perifollicular fibrosis as well as perifollicular and perivascular chronic inflammation.