Patient Presentation A 45-year-old Caucasian man presented with a 3-year history of asymptomatic whitish pebbly papules on the lobule of the right ear. Upon presentation, he was otherwise in a good health and was not taking any oral medications. He denied any use of topical medications, cosmetics or soaps to the affected area. He has no history of surgery or ear piercing. He had no family history of similar lesions. On physical examination there was a well-defined crescent-shaped plaque on the lobule of the right ear, measuring 1 cm by 3 cm. The plaque was composed of numerous whitish-yellow papules measuring 1 mm to 3 mm in diameter overlying erythematous skin. No lymphadenopathy was present. WHAT IS YOUR DIAGNOSIS? (Turn to page 74 for an answer and for more details about the condition and this case.) Diagnosis: Milia en Plaque (MEP) MEP, a rare clinical variant of milia (singular: milium), was first described by Baizer and Fouquet1 in 1903 as a group of milia forming on an erythematous plaque in the retroauricular area. In 1978, Hubler et al,2 reported two patients with milia on an erythematous edematous base, and first introduced the name “milia en plaque.” MEP is a benign entity that is usually asymptomatic, although mild pruritus and burning have been reported, but it is cosmetically concerning to the patients. MEP typically occurs in the retroauricular area on an erythematous base, but can occur in different locations and age-groups1-4 including preauricular areas, ear lobes, the bridge of the nose, inner canthus, upper and lower eyelids, submandibular area, and supraclavicular area. It occurs in males and females between the ages of 6 and 845-7 and has no racial predilection.8 Milia: Types and Associated Diseases Milia represent benign epidermal tumors. They are white to yellowish papules ranging from 1 mm to 5 mm in diameter and occur below the epidermis. Primary milia, which represent benign, keratinizing tumors derived from vellus hairs, arise spontaneously without predisposing conditions. It has been speculated that primary milia arise in sebaceous glands not fully developed, which explains the high prevalence in newborn and infants. Primary milia in infants commonly occur on the face, especially the nose and mucosa and palate. Palatal lesions are known as Epstein pearls. Primary milia in older children and adults develop on the face, particularly around the eyes. Secondary milia are retention cysts that develop from eccrine sweat ducts or epidermis of hair follicles. They arise following blistering or trauma due to destruction of the eccrine sweat duct, and are found anywhere on the body at the sites affected by the predisposing condition. This type of milia has been associated with bullous pemphigoid, inherited and acquired epidermolysis bullosa, bullous lichen planus, porphyria cutanea tarda, and burns. They may also occur as a result of trauma such as dermabrasion, chronic topical steroid use, 5-fluorouracil therapy (Carac, Efudex), oral NSAIDs (benoxaprofen), and radiotherapy..3,4,8,9 A rare subtype of secondary milia, “rosacea like” with marked inflammatory reaction was also described. Genodermatoses Associated with Milia Bazex-Dupré-Christol syndrome and X-linked dominant syndrome , which includes follicular atrophoderma on the extremities, “ice pick marks,” localized or generalized hypohidrosis, hypotrichosis, the appearance of basal cell carcinomas of the face at an early age, milia and hair shaft abnormalities (pili torti, trichorrhexis nodosa). Nicolau-Balus syndrome. Includes micropapular eruptive syringomas, milia and atrophoderma vermiculata. Rasmussen’s syndrome. This autosomal dominant syndrome includes milia, trichoepitheliomas and cylindromas. Other genodermatoses include congenital ectodermal defects, reticular pigmented genodermatosis with milia (Naegeli-Franceschetti-Jadassohn syndrome); and congenital absence of dermal ridges, syndactyly and facial milia. The exact pathogenesis of MEP isn’t clear but one case was reported in a patient with pseudoxanthoma elasticum;9 another was described in a patient with discoid lupus erythematosus.10 Elastin degeneration in the first case and damage to the adnexal structures in the second case have been considered as etiologic factors responsible for milia formation. Other causes of trauma such as wearing spectacles, earrings and perfumes have been reported as possible etiologic factors for MEP. It has been proposed that MEP may be a sequela of previous lichen planus tumidus folliculans.11 Histopathology MEP is similar to epidermoid cysts but the cysts are smaller. Milia are found in the superficial dermis and have a complete epithelial lining. The cysts contain keratin and have an intense surrounding lymphocytic infiltrate. One case of MEP presented histologically as a hybrid cyst with epidermal and trichilemmal keratinization. The common primary milia in infants and children are found in the undifferentiated sebaceous hair collar surrounding vellus hair follicles. Milia secondary to blistering often are found in eccrine sweat ducts. Differential Diagnosis Histologically, a number of diseases need to be differentiated from MEP. MEP may mimic follicular mucinosis or follicular mycosis fungoides (MF). The presence of mucinous follicular deposits is characteristic of follicular mucinosis. Follicular mycosis fungoides is a rare variant of MF, which presents as folliculotropic lichenoid infiltrate associated with cysts and comedones grouped in plaques that may mimic MEP. Xanthelasma palpebrarum is distinguished from MEP by the presence of foamy cells and absence of small epidermoid cysts.12 Trichoadenoma occurs as a nodular tumor and is histologically distinct by the presence of horn cysts surrounded by eosinophilic cells.12 Comedone nevus occurs at birth and is characterized histologically by spacious infundibular dilations full of keratin with bulbous proliferation of basaloid cells at the base. Nodular elastosis of Favre-Racouchot occurs lateral to the eyes and is differentiated by the presence of giant comedones, follicular cysts and actinic damage. Lichen planus tumidus folliculans (LPTF) is a plaque variety of lichen planus pilaris and is characterized by reddish plaque composed of small cysts and comedones and may mimic MEP. Histologically, LPTF appears as follicles and cysts surrounded by a lichenoid infiltrate. Epithelioma adenoids cysticum appears at puberty and occurs in patients with a positive family history and occurs as multiple tumors on the face. Managing this Condition MEP usually persists without treatment, although spontaneous regression has been reported. Treatment options include incision with a scalpel and expression with a comedo extractor. Or, a sterile 24- to 27-gauge straight needle with a cutting beveled tip is bent in the middle at a right angle as described by Thami et al.13 Topical tretinoin has been used with moderate results. A sub-group of patients with rosacea like MEP benefited from minocycline 100 mg/day for 2 to 3 months. A combination of tretinoin and minocycline was also used.4 Electrodessication, photodynamic therapy, laser ablation, and surgical excision have been used with varying results.3 Noto and Dawber reported using a single freeze-thaw cycle of 75 seconds of open-spray liquid nitrogen cryosurgery with success. Their Patient had complete healing with good aesthetic result and remained milia-free at 2 years of follow-up.14 Our patient declined treatment. We need to be aware of this peculiar condition in order to introduce more efficacious treatment modalities.
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What Are These Whitish Papules?
Patient Presentation A 45-year-old Caucasian man presented with a 3-year history of asymptomatic whitish pebbly papules on the lobule of the right ear. Upon presentation, he was otherwise in a good health and was not taking any oral medications. He denied any use of topical medications, cosmetics or soaps to the affected area. He has no history of surgery or ear piercing. He had no family history of similar lesions. On physical examination there was a well-defined crescent-shaped plaque on the lobule of the right ear, measuring 1 cm by 3 cm. The plaque was composed of numerous whitish-yellow papules measuring 1 mm to 3 mm in diameter overlying erythematous skin. No lymphadenopathy was present. WHAT IS YOUR DIAGNOSIS? (Turn to page 74 for an answer and for more details about the condition and this case.) Diagnosis: Milia en Plaque (MEP) MEP, a rare clinical variant of milia (singular: milium), was first described by Baizer and Fouquet1 in 1903 as a group of milia forming on an erythematous plaque in the retroauricular area. In 1978, Hubler et al,2 reported two patients with milia on an erythematous edematous base, and first introduced the name “milia en plaque.” MEP is a benign entity that is usually asymptomatic, although mild pruritus and burning have been reported, but it is cosmetically concerning to the patients. MEP typically occurs in the retroauricular area on an erythematous base, but can occur in different locations and age-groups1-4 including preauricular areas, ear lobes, the bridge of the nose, inner canthus, upper and lower eyelids, submandibular area, and supraclavicular area. It occurs in males and females between the ages of 6 and 845-7 and has no racial predilection.8 Milia: Types and Associated Diseases Milia represent benign epidermal tumors. They are white to yellowish papules ranging from 1 mm to 5 mm in diameter and occur below the epidermis. Primary milia, which represent benign, keratinizing tumors derived from vellus hairs, arise spontaneously without predisposing conditions. It has been speculated that primary milia arise in sebaceous glands not fully developed, which explains the high prevalence in newborn and infants. Primary milia in infants commonly occur on the face, especially the nose and mucosa and palate. Palatal lesions are known as Epstein pearls. Primary milia in older children and adults develop on the face, particularly around the eyes. Secondary milia are retention cysts that develop from eccrine sweat ducts or epidermis of hair follicles. They arise following blistering or trauma due to destruction of the eccrine sweat duct, and are found anywhere on the body at the sites affected by the predisposing condition. This type of milia has been associated with bullous pemphigoid, inherited and acquired epidermolysis bullosa, bullous lichen planus, porphyria cutanea tarda, and burns. They may also occur as a result of trauma such as dermabrasion, chronic topical steroid use, 5-fluorouracil therapy (Carac, Efudex), oral NSAIDs (benoxaprofen), and radiotherapy..3,4,8,9 A rare subtype of secondary milia, “rosacea like” with marked inflammatory reaction was also described. Genodermatoses Associated with Milia Bazex-Dupré-Christol syndrome and X-linked dominant syndrome , which includes follicular atrophoderma on the extremities, “ice pick marks,” localized or generalized hypohidrosis, hypotrichosis, the appearance of basal cell carcinomas of the face at an early age, milia and hair shaft abnormalities (pili torti, trichorrhexis nodosa). Nicolau-Balus syndrome. Includes micropapular eruptive syringomas, milia and atrophoderma vermiculata. Rasmussen’s syndrome. This autosomal dominant syndrome includes milia, trichoepitheliomas and cylindromas. Other genodermatoses include congenital ectodermal defects, reticular pigmented genodermatosis with milia (Naegeli-Franceschetti-Jadassohn syndrome); and congenital absence of dermal ridges, syndactyly and facial milia. The exact pathogenesis of MEP isn’t clear but one case was reported in a patient with pseudoxanthoma elasticum;9 another was described in a patient with discoid lupus erythematosus.10 Elastin degeneration in the first case and damage to the adnexal structures in the second case have been considered as etiologic factors responsible for milia formation. Other causes of trauma such as wearing spectacles, earrings and perfumes have been reported as possible etiologic factors for MEP. It has been proposed that MEP may be a sequela of previous lichen planus tumidus folliculans.11 Histopathology MEP is similar to epidermoid cysts but the cysts are smaller. Milia are found in the superficial dermis and have a complete epithelial lining. The cysts contain keratin and have an intense surrounding lymphocytic infiltrate. One case of MEP presented histologically as a hybrid cyst with epidermal and trichilemmal keratinization. The common primary milia in infants and children are found in the undifferentiated sebaceous hair collar surrounding vellus hair follicles. Milia secondary to blistering often are found in eccrine sweat ducts. Differential Diagnosis Histologically, a number of diseases need to be differentiated from MEP. MEP may mimic follicular mucinosis or follicular mycosis fungoides (MF). The presence of mucinous follicular deposits is characteristic of follicular mucinosis. Follicular mycosis fungoides is a rare variant of MF, which presents as folliculotropic lichenoid infiltrate associated with cysts and comedones grouped in plaques that may mimic MEP. Xanthelasma palpebrarum is distinguished from MEP by the presence of foamy cells and absence of small epidermoid cysts.12 Trichoadenoma occurs as a nodular tumor and is histologically distinct by the presence of horn cysts surrounded by eosinophilic cells.12 Comedone nevus occurs at birth and is characterized histologically by spacious infundibular dilations full of keratin with bulbous proliferation of basaloid cells at the base. Nodular elastosis of Favre-Racouchot occurs lateral to the eyes and is differentiated by the presence of giant comedones, follicular cysts and actinic damage. Lichen planus tumidus folliculans (LPTF) is a plaque variety of lichen planus pilaris and is characterized by reddish plaque composed of small cysts and comedones and may mimic MEP. Histologically, LPTF appears as follicles and cysts surrounded by a lichenoid infiltrate. Epithelioma adenoids cysticum appears at puberty and occurs in patients with a positive family history and occurs as multiple tumors on the face. Managing this Condition MEP usually persists without treatment, although spontaneous regression has been reported. Treatment options include incision with a scalpel and expression with a comedo extractor. Or, a sterile 24- to 27-gauge straight needle with a cutting beveled tip is bent in the middle at a right angle as described by Thami et al.13 Topical tretinoin has been used with moderate results. A sub-group of patients with rosacea like MEP benefited from minocycline 100 mg/day for 2 to 3 months. A combination of tretinoin and minocycline was also used.4 Electrodessication, photodynamic therapy, laser ablation, and surgical excision have been used with varying results.3 Noto and Dawber reported using a single freeze-thaw cycle of 75 seconds of open-spray liquid nitrogen cryosurgery with success. Their Patient had complete healing with good aesthetic result and remained milia-free at 2 years of follow-up.14 Our patient declined treatment. We need to be aware of this peculiar condition in order to introduce more efficacious treatment modalities.
Patient Presentation A 45-year-old Caucasian man presented with a 3-year history of asymptomatic whitish pebbly papules on the lobule of the right ear. Upon presentation, he was otherwise in a good health and was not taking any oral medications. He denied any use of topical medications, cosmetics or soaps to the affected area. He has no history of surgery or ear piercing. He had no family history of similar lesions. On physical examination there was a well-defined crescent-shaped plaque on the lobule of the right ear, measuring 1 cm by 3 cm. The plaque was composed of numerous whitish-yellow papules measuring 1 mm to 3 mm in diameter overlying erythematous skin. No lymphadenopathy was present. WHAT IS YOUR DIAGNOSIS? (Turn to page 74 for an answer and for more details about the condition and this case.) Diagnosis: Milia en Plaque (MEP) MEP, a rare clinical variant of milia (singular: milium), was first described by Baizer and Fouquet1 in 1903 as a group of milia forming on an erythematous plaque in the retroauricular area. In 1978, Hubler et al,2 reported two patients with milia on an erythematous edematous base, and first introduced the name “milia en plaque.” MEP is a benign entity that is usually asymptomatic, although mild pruritus and burning have been reported, but it is cosmetically concerning to the patients. MEP typically occurs in the retroauricular area on an erythematous base, but can occur in different locations and age-groups1-4 including preauricular areas, ear lobes, the bridge of the nose, inner canthus, upper and lower eyelids, submandibular area, and supraclavicular area. It occurs in males and females between the ages of 6 and 845-7 and has no racial predilection.8 Milia: Types and Associated Diseases Milia represent benign epidermal tumors. They are white to yellowish papules ranging from 1 mm to 5 mm in diameter and occur below the epidermis. Primary milia, which represent benign, keratinizing tumors derived from vellus hairs, arise spontaneously without predisposing conditions. It has been speculated that primary milia arise in sebaceous glands not fully developed, which explains the high prevalence in newborn and infants. Primary milia in infants commonly occur on the face, especially the nose and mucosa and palate. Palatal lesions are known as Epstein pearls. Primary milia in older children and adults develop on the face, particularly around the eyes. Secondary milia are retention cysts that develop from eccrine sweat ducts or epidermis of hair follicles. They arise following blistering or trauma due to destruction of the eccrine sweat duct, and are found anywhere on the body at the sites affected by the predisposing condition. This type of milia has been associated with bullous pemphigoid, inherited and acquired epidermolysis bullosa, bullous lichen planus, porphyria cutanea tarda, and burns. They may also occur as a result of trauma such as dermabrasion, chronic topical steroid use, 5-fluorouracil therapy (Carac, Efudex), oral NSAIDs (benoxaprofen), and radiotherapy..3,4,8,9 A rare subtype of secondary milia, “rosacea like” with marked inflammatory reaction was also described. Genodermatoses Associated with Milia Bazex-Dupré-Christol syndrome and X-linked dominant syndrome , which includes follicular atrophoderma on the extremities, “ice pick marks,” localized or generalized hypohidrosis, hypotrichosis, the appearance of basal cell carcinomas of the face at an early age, milia and hair shaft abnormalities (pili torti, trichorrhexis nodosa). Nicolau-Balus syndrome. Includes micropapular eruptive syringomas, milia and atrophoderma vermiculata. Rasmussen’s syndrome. This autosomal dominant syndrome includes milia, trichoepitheliomas and cylindromas. Other genodermatoses include congenital ectodermal defects, reticular pigmented genodermatosis with milia (Naegeli-Franceschetti-Jadassohn syndrome); and congenital absence of dermal ridges, syndactyly and facial milia. The exact pathogenesis of MEP isn’t clear but one case was reported in a patient with pseudoxanthoma elasticum;9 another was described in a patient with discoid lupus erythematosus.10 Elastin degeneration in the first case and damage to the adnexal structures in the second case have been considered as etiologic factors responsible for milia formation. Other causes of trauma such as wearing spectacles, earrings and perfumes have been reported as possible etiologic factors for MEP. It has been proposed that MEP may be a sequela of previous lichen planus tumidus folliculans.11 Histopathology MEP is similar to epidermoid cysts but the cysts are smaller. Milia are found in the superficial dermis and have a complete epithelial lining. The cysts contain keratin and have an intense surrounding lymphocytic infiltrate. One case of MEP presented histologically as a hybrid cyst with epidermal and trichilemmal keratinization. The common primary milia in infants and children are found in the undifferentiated sebaceous hair collar surrounding vellus hair follicles. Milia secondary to blistering often are found in eccrine sweat ducts. Differential Diagnosis Histologically, a number of diseases need to be differentiated from MEP. MEP may mimic follicular mucinosis or follicular mycosis fungoides (MF). The presence of mucinous follicular deposits is characteristic of follicular mucinosis. Follicular mycosis fungoides is a rare variant of MF, which presents as folliculotropic lichenoid infiltrate associated with cysts and comedones grouped in plaques that may mimic MEP. Xanthelasma palpebrarum is distinguished from MEP by the presence of foamy cells and absence of small epidermoid cysts.12 Trichoadenoma occurs as a nodular tumor and is histologically distinct by the presence of horn cysts surrounded by eosinophilic cells.12 Comedone nevus occurs at birth and is characterized histologically by spacious infundibular dilations full of keratin with bulbous proliferation of basaloid cells at the base. Nodular elastosis of Favre-Racouchot occurs lateral to the eyes and is differentiated by the presence of giant comedones, follicular cysts and actinic damage. Lichen planus tumidus folliculans (LPTF) is a plaque variety of lichen planus pilaris and is characterized by reddish plaque composed of small cysts and comedones and may mimic MEP. Histologically, LPTF appears as follicles and cysts surrounded by a lichenoid infiltrate. Epithelioma adenoids cysticum appears at puberty and occurs in patients with a positive family history and occurs as multiple tumors on the face. Managing this Condition MEP usually persists without treatment, although spontaneous regression has been reported. Treatment options include incision with a scalpel and expression with a comedo extractor. Or, a sterile 24- to 27-gauge straight needle with a cutting beveled tip is bent in the middle at a right angle as described by Thami et al.13 Topical tretinoin has been used with moderate results. A sub-group of patients with rosacea like MEP benefited from minocycline 100 mg/day for 2 to 3 months. A combination of tretinoin and minocycline was also used.4 Electrodessication, photodynamic therapy, laser ablation, and surgical excision have been used with varying results.3 Noto and Dawber reported using a single freeze-thaw cycle of 75 seconds of open-spray liquid nitrogen cryosurgery with success. Their Patient had complete healing with good aesthetic result and remained milia-free at 2 years of follow-up.14 Our patient declined treatment. We need to be aware of this peculiar condition in order to introduce more efficacious treatment modalities.
