English Jc Iii

Basaloid follicular hamartoma (BFH) is a benign adnexal tumor consisting of basaloid cells with follicular differentiation. BFH can occur as a solitary lesion, multiple lesions, or an autosomal dominant inherited syndrome—BFH syndrome (BFHS).1 In patients with BFHS, adnexal tumors predominantly occur on the head, neck, and torso. The syndrome often has coexisting hypotrichosis, hypohidrosis, and palmar/plantar pitting.1 A familial presentation of BFHS with palmar pitting is discussed with a review of BFHS, including the differential diagnosis of palmar pitting. 

Case Report
A 67-year-old white woman presented to dermatology with a 20-year history of several lesions on her face that started as flesh-colored papules and progressively darkened. Most lesions were 3 to 6 mm and located symmetrically around the nose, mouth, chin, and eyes. The woman presented with her 39-year-old daughter, who had a 2-year history of similar lesions around her eyes, none in a perioral distribution. The women reported that when the mother's lesions first appeared, they had the same appearance as the daughter's lesions. The persistent lesions were otherwise asymptomatic. Review of systems for both the mother and daughter were negative for other cutaneous lesions and for systemic symptoms such as fever; weight loss; and cardiovascular, respiratory, gastrointestinal, and genitourinary symptoms. The mother's medical history was significant for hypertension, diabetes mellitus, hypothyroidism, and hypercholesterolemia. The daughter's medical history was significant only for diabetes mellitus. Results of a physical examination of the mother revealed hundreds of soft flesh-colored to hyperpigmented papules, most less than 6 mm in diameter. The papules coalesced in the perioral distribution (Figure 1). Results of a physical examination of the daughter revealed similar symmetric distribution of small (most 1–2 mm but all <6 mm in diameter) flesh-colored papules in bilateral nasolabial and periorbital areas. Symmetric palmar pitting was present in both women (Figure 2). The patients had no other clinical manifestations such as frontoparietal bossing, high arched palates, jaw cysts, short fourth metacarpals, ocular hypertelorism, or narrow sloping shoulders to suggest nevoid basal cell carcinoma syndrome (NBCS). Histopathology results of both the mother and daughter revealed adnexal proliferation with follicular differentiation consistent with BFH (Figures 3–5).

Comment

BFH is a rare benign adnexal tumor that exhibits protean clinical manifestations. Despite the polymorphic clinical presentations, lesions exhibit similar histologic appearance, with only small variations.1,2 Lesions exist in both solitary and multiple forms.1-4 Multiple forms generally exhibit autosomal dominant inheritance, while solitary forms usually are not inherited.3 The solitary noninherited forms are more common than the multiple autosomal dominant inherited forms.3,5

Most reported solitary cases of BFH are in women, aged 20 to 88 years (median, 66 years; mean, 63 years).3 Patients with multiple familial disease may exhibit associated systemic diseases, though systemic disease is less likely in patients with solitary lesions.2,3 Systemic diseases that may be associated with BFHS include hypertension, cardiovascular disease, renal disease, obesity, carpal tunnel syndrome, and cancer of the breast and stomach.6 Generalized lesions also have been associated with cystic fibrosis,7 systemic lupus erythematosus,8 and myasthenia gravis.9

BFHS lesions have a predilection for the face and scalp but also have been reported on the neck, axillae, shoulders, and pubic regions.4 Wheeler et al6 examined 18 members in one family and found that dominantly-inherited generalized BFHS exhibits variability of disease. The patients exhibited lesions that ranged from hundreds of pigmented papules with associated hypotrichosis and palmar/ plantar pitting to minimal facial papules and a few milia involving only the face.6 Most papules are 1 to 2 mm.3 In mild forms, the lesions may go unnoticed or may be mistaken as normal.6 Lesions also may be present at birth or may develop in early childhood.6,10-12

Findings on physical examination most commonly associated with BFHS include milialike papules, comedonal-like papules, hypotrichosis, hypohidrosis, and palmar/plantar pitting (Table).6 Less common associated findings include atopic dermatitis, keratosis pilaris, lichen striatus, acrochordons, acanthosis nigricans—like changes, dermatosis papulosa nigra, acne vulgaris, café au lait spots, punctate palmar keratosis, and steatocystoma multiplex.6

The differential diagnoses of multiple facial papules include angiofibromas (tuberous sclerosis), multiple trichilemmomas (Cowden disease), multiple trichoepitheliomas, multiple syringomas, steatocystoma multiplex, seborrheic keratosis, melanocytic nevi, sebaceous hyperplasia, and NBCS or Gorlin syndrome.3 NBCS is believed to be caused by a lack of human homoloque patched (PTCH) tumor suppressor gene function, whereas BFHS is reported to be caused by a decrease in PTCH function.13 Girardi et al10 suggested that familial BFHS possibly is a forme fruste of NBCS (ie, since both entities involve the PTCH gene protein product or some member of its pathway).

Histologically, branching cords and thin strands of undifferentiated and anastomosing basaloid proliferations, which are surrounded by a loosely fibroblastic stroma, identify BFH.1 This histologic pattern may be found in a wide spectrum of clinically nevoid presentations, and individual lesions may be indistinguishable from infundibulocystic basal cell carcinoma (BCC). Occasional peripheral pallisading, papillary mesenchymal bodies, and connections to the epidermis also have been described.1

BFH is categorized within the spectrum of basaloid epithelial neoplasms associated with dysregulation of the PTCH signaling pathway.13 However, in general, BFH lesions show less mitotic figures and necrosis when compared with BCC.6,13 Immunohistochemistry results show CD34+ stromal cells in peritumoral distribution, resembling trichoepithelioma and distinguishing the condition from BCC, which lacks CD34 expression.14 Additional factors that distinguish BFH from BCC include BFH’s low proliferative index (ie, low Ki-67) and lack of bcl-2 expression.13,14

BFHS is a rare entity. It is a mainly histologic diagnosis with varied clinical presentations.6 Prognosis is good and treatment usually is cosmetic with shave, curette, or scissor methods most frequently reported.3 Because of the benign nature of this follicular tumor, observation without treatment is an option. It is important to be able to recognize this disease to avoid overaggressive surgical management and to closely monitor for any new tumors that may develop in these lesions.1 Monitoring and close follow-up of these lesions is necessary because some believe that familial BFHS is possibly a forme fruste of NBCS. Any indications of malignant changes in these lesions (eg, nonhealing and bleeding lesions, biopsy suggestive of BCC or other skin cancer) warrant surgical therapy. If lesions do not indicate malignant change, however, they may be removed for cosmetic reasons, but aggressive surgery, such as Mohs micrographic surgery, may put the patient at risk for unnecessary infection and increased morbidity.1

Basaloid follicular hamartoma (BFH) is a benign adnexal tumor consisting of basaloid cells with follicular differentiation. BFH can occur as a solitary lesion, multiple lesions, or an autosomal dominant inherited syndrome—BFH syndrome (BFHS).1 In patients with BFHS, adnexal tumors predominantly occur on the head, neck, and torso. The syndrome often has coexisting hypotrichosis, hypohidrosis, and palmar/plantar pitting.1 A familial presentation of BFHS with palmar pitting is discussed with a review of BFHS, including the differential diagnosis of palmar pitting. 

Case Report
A 67-year-old white woman presented to dermatology with a 20-year history of several lesions on her face that started as flesh-colored papules and progressively darkened. Most lesions were 3 to 6 mm and located symmetrically around the nose, mouth, chin, and eyes. The woman presented with her 39-year-old daughter, who had a 2-year history of similar lesions around her eyes, none in a perioral distribution. The women reported that when the mother's lesions first appeared, they had the same appearance as the daughter's lesions. The persistent lesions were otherwise asymptomatic. Review of systems for both the mother and daughter were negative for other cutaneous lesions and for systemic symptoms such as fever; weight loss; and cardiovascular, respiratory, gastrointestinal, and genitourinary symptoms. The mother's medical history was significant for hypertension, diabetes mellitus, hypothyroidism, and hypercholesterolemia. The daughter's medical history was significant only for diabetes mellitus. Results of a physical examination of the mother revealed hundreds of soft flesh-colored to hyperpigmented papules, most less than 6 mm in diameter. The papules coalesced in the perioral distribution (Figure 1). Results of a physical examination of the daughter revealed similar symmetric distribution of small (most 1–2 mm but all <6 mm in diameter) flesh-colored papules in bilateral nasolabial and periorbital areas. Symmetric palmar pitting was present in both women (Figure 2). The patients had no other clinical manifestations such as frontoparietal bossing, high arched palates, jaw cysts, short fourth metacarpals, ocular hypertelorism, or narrow sloping shoulders to suggest nevoid basal cell carcinoma syndrome (NBCS). Histopathology results of both the mother and daughter revealed adnexal proliferation with follicular differentiation consistent with BFH (Figures 3–5).

Comment

BFH is a rare benign adnexal tumor that exhibits protean clinical manifestations. Despite the polymorphic clinical presentations, lesions exhibit similar histologic appearance, with only small variations.1,2 Lesions exist in both solitary and multiple forms.1-4 Multiple forms generally exhibit autosomal dominant inheritance, while solitary forms usually are not inherited.3 The solitary noninherited forms are more common than the multiple autosomal dominant inherited forms.3,5

Most reported solitary cases of BFH are in women, aged 20 to 88 years (median, 66 years; mean, 63 years).3 Patients with multiple familial disease may exhibit associated systemic diseases, though systemic disease is less likely in patients with solitary lesions.2,3 Systemic diseases that may be associated with BFHS include hypertension, cardiovascular disease, renal disease, obesity, carpal tunnel syndrome, and cancer of the breast and stomach.6 Generalized lesions also have been associated with cystic fibrosis,7 systemic lupus erythematosus,8 and myasthenia gravis.9

BFHS lesions have a predilection for the face and scalp but also have been reported on the neck, axillae, shoulders, and pubic regions.4 Wheeler et al6 examined 18 members in one family and found that dominantly-inherited generalized BFHS exhibits variability of disease. The patients exhibited lesions that ranged from hundreds of pigmented papules with associated hypotrichosis and palmar/ plantar pitting to minimal facial papules and a few milia involving only the face.6 Most papules are 1 to 2 mm.3 In mild forms, the lesions may go unnoticed or may be mistaken as normal.6 Lesions also may be present at birth or may develop in early childhood.6,10-12

Findings on physical examination most commonly associated with BFHS include milialike papules, comedonal-like papules, hypotrichosis, hypohidrosis, and palmar/plantar pitting (Table).6 Less common associated findings include atopic dermatitis, keratosis pilaris, lichen striatus, acrochordons, acanthosis nigricans—like changes, dermatosis papulosa nigra, acne vulgaris, café au lait spots, punctate palmar keratosis, and steatocystoma multiplex.6

The differential diagnoses of multiple facial papules include angiofibromas (tuberous sclerosis), multiple trichilemmomas (Cowden disease), multiple trichoepitheliomas, multiple syringomas, steatocystoma multiplex, seborrheic keratosis, melanocytic nevi, sebaceous hyperplasia, and NBCS or Gorlin syndrome.3 NBCS is believed to be caused by a lack of human homoloque patched (PTCH) tumor suppressor gene function, whereas BFHS is reported to be caused by a decrease in PTCH function.13 Girardi et al10 suggested that familial BFHS possibly is a forme fruste of NBCS (ie, since both entities involve the PTCH gene protein product or some member of its pathway).

Histologically, branching cords and thin strands of undifferentiated and anastomosing basaloid proliferations, which are surrounded by a loosely fibroblastic stroma, identify BFH.1 This histologic pattern may be found in a wide spectrum of clinically nevoid presentations, and individual lesions may be indistinguishable from infundibulocystic basal cell carcinoma (BCC). Occasional peripheral pallisading, papillary mesenchymal bodies, and connections to the epidermis also have been described.1

BFH is categorized within the spectrum of basaloid epithelial neoplasms associated with dysregulation of the PTCH signaling pathway.13 However, in general, BFH lesions show less mitotic figures and necrosis when compared with BCC.6,13 Immunohistochemistry results show CD34+ stromal cells in peritumoral distribution, resembling trichoepithelioma and distinguishing the condition from BCC, which lacks CD34 expression.14 Additional factors that distinguish BFH from BCC include BFH’s low proliferative index (ie, low Ki-67) and lack of bcl-2 expression.13,14

BFHS is a rare entity. It is a mainly histologic diagnosis with varied clinical presentations.6 Prognosis is good and treatment usually is cosmetic with shave, curette, or scissor methods most frequently reported.3 Because of the benign nature of this follicular tumor, observation without treatment is an option. It is important to be able to recognize this disease to avoid overaggressive surgical management and to closely monitor for any new tumors that may develop in these lesions.1 Monitoring and close follow-up of these lesions is necessary because some believe that familial BFHS is possibly a forme fruste of NBCS. Any indications of malignant changes in these lesions (eg, nonhealing and bleeding lesions, biopsy suggestive of BCC or other skin cancer) warrant surgical therapy. If lesions do not indicate malignant change, however, they may be removed for cosmetic reasons, but aggressive surgery, such as Mohs micrographic surgery, may put the patient at risk for unnecessary infection and increased morbidity.1

Basaloid follicular hamartoma (BFH) is a benign adnexal tumor consisting of basaloid cells with follicular differentiation. BFH can occur as a solitary lesion, multiple lesions, or an autosomal dominant inherited syndrome—BFH syndrome (BFHS).1 In patients with BFHS, adnexal tumors predominantly occur on the head, neck, and torso. The syndrome often has coexisting hypotrichosis, hypohidrosis, and palmar/plantar pitting.1 A familial presentation of BFHS with palmar pitting is discussed with a review of BFHS, including the differential diagnosis of palmar pitting. 

Case Report
A 67-year-old white woman presented to dermatology with a 20-year history of several lesions on her face that started as flesh-colored papules and progressively darkened. Most lesions were 3 to 6 mm and located symmetrically around the nose, mouth, chin, and eyes. The woman presented with her 39-year-old daughter, who had a 2-year history of similar lesions around her eyes, none in a perioral distribution. The women reported that when the mother's lesions first appeared, they had the same appearance as the daughter's lesions. The persistent lesions were otherwise asymptomatic. Review of systems for both the mother and daughter were negative for other cutaneous lesions and for systemic symptoms such as fever; weight loss; and cardiovascular, respiratory, gastrointestinal, and genitourinary symptoms. The mother's medical history was significant for hypertension, diabetes mellitus, hypothyroidism, and hypercholesterolemia. The daughter's medical history was significant only for diabetes mellitus. Results of a physical examination of the mother revealed hundreds of soft flesh-colored to hyperpigmented papules, most less than 6 mm in diameter. The papules coalesced in the perioral distribution (Figure 1). Results of a physical examination of the daughter revealed similar symmetric distribution of small (most 1–2 mm but all <6 mm in diameter) flesh-colored papules in bilateral nasolabial and periorbital areas. Symmetric palmar pitting was present in both women (Figure 2). The patients had no other clinical manifestations such as frontoparietal bossing, high arched palates, jaw cysts, short fourth metacarpals, ocular hypertelorism, or narrow sloping shoulders to suggest nevoid basal cell carcinoma syndrome (NBCS). Histopathology results of both the mother and daughter revealed adnexal proliferation with follicular differentiation consistent with BFH (Figures 3–5).

Comment

BFH is a rare benign adnexal tumor that exhibits protean clinical manifestations. Despite the polymorphic clinical presentations, lesions exhibit similar histologic appearance, with only small variations.1,2 Lesions exist in both solitary and multiple forms.1-4 Multiple forms generally exhibit autosomal dominant inheritance, while solitary forms usually are not inherited.3 The solitary noninherited forms are more common than the multiple autosomal dominant inherited forms.3,5

Most reported solitary cases of BFH are in women, aged 20 to 88 years (median, 66 years; mean, 63 years).3 Patients with multiple familial disease may exhibit associated systemic diseases, though systemic disease is less likely in patients with solitary lesions.2,3 Systemic diseases that may be associated with BFHS include hypertension, cardiovascular disease, renal disease, obesity, carpal tunnel syndrome, and cancer of the breast and stomach.6 Generalized lesions also have been associated with cystic fibrosis,7 systemic lupus erythematosus,8 and myasthenia gravis.9

BFHS lesions have a predilection for the face and scalp but also have been reported on the neck, axillae, shoulders, and pubic regions.4 Wheeler et al6 examined 18 members in one family and found that dominantly-inherited generalized BFHS exhibits variability of disease. The patients exhibited lesions that ranged from hundreds of pigmented papules with associated hypotrichosis and palmar/ plantar pitting to minimal facial papules and a few milia involving only the face.6 Most papules are 1 to 2 mm.3 In mild forms, the lesions may go unnoticed or may be mistaken as normal.6 Lesions also may be present at birth or may develop in early childhood.6,10-12

Findings on physical examination most commonly associated with BFHS include milialike papules, comedonal-like papules, hypotrichosis, hypohidrosis, and palmar/plantar pitting (Table).6 Less common associated findings include atopic dermatitis, keratosis pilaris, lichen striatus, acrochordons, acanthosis nigricans—like changes, dermatosis papulosa nigra, acne vulgaris, café au lait spots, punctate palmar keratosis, and steatocystoma multiplex.6

The differential diagnoses of multiple facial papules include angiofibromas (tuberous sclerosis), multiple trichilemmomas (Cowden disease), multiple trichoepitheliomas, multiple syringomas, steatocystoma multiplex, seborrheic keratosis, melanocytic nevi, sebaceous hyperplasia, and NBCS or Gorlin syndrome.3 NBCS is believed to be caused by a lack of human homoloque patched (PTCH) tumor suppressor gene function, whereas BFHS is reported to be caused by a decrease in PTCH function.13 Girardi et al10 suggested that familial BFHS possibly is a forme fruste of NBCS (ie, since both entities involve the PTCH gene protein product or some member of its pathway).

Histologically, branching cords and thin strands of undifferentiated and anastomosing basaloid proliferations, which are surrounded by a loosely fibroblastic stroma, identify BFH.1 This histologic pattern may be found in a wide spectrum of clinically nevoid presentations, and individual lesions may be indistinguishable from infundibulocystic basal cell carcinoma (BCC). Occasional peripheral pallisading, papillary mesenchymal bodies, and connections to the epidermis also have been described.1

BFH is categorized within the spectrum of basaloid epithelial neoplasms associated with dysregulation of the PTCH signaling pathway.13 However, in general, BFH lesions show less mitotic figures and necrosis when compared with BCC.6,13 Immunohistochemistry results show CD34+ stromal cells in peritumoral distribution, resembling trichoepithelioma and distinguishing the condition from BCC, which lacks CD34 expression.14 Additional factors that distinguish BFH from BCC include BFH’s low proliferative index (ie, low Ki-67) and lack of bcl-2 expression.13,14

BFHS is a rare entity. It is a mainly histologic diagnosis with varied clinical presentations.6 Prognosis is good and treatment usually is cosmetic with shave, curette, or scissor methods most frequently reported.3 Because of the benign nature of this follicular tumor, observation without treatment is an option. It is important to be able to recognize this disease to avoid overaggressive surgical management and to closely monitor for any new tumors that may develop in these lesions.1 Monitoring and close follow-up of these lesions is necessary because some believe that familial BFHS is possibly a forme fruste of NBCS. Any indications of malignant changes in these lesions (eg, nonhealing and bleeding lesions, biopsy suggestive of BCC or other skin cancer) warrant surgical therapy. If lesions do not indicate malignant change, however, they may be removed for cosmetic reasons, but aggressive surgery, such as Mohs micrographic surgery, may put the patient at risk for unnecessary infection and increased morbidity.1

Folliculitis is a common complaint seen in dermatology practice. The differential diagnosis of folliculitis is broad and includes Demodex folliculitis. In humans, the Demodex mite species Demodex folliculorum and Demodex brevis have been found to inhabit the pilosebaceous unit. D folliculorum typically is found in the follicular infundibulum; D brevis exists in the sebaceous and meibomian glands.¹ Although the prevalence of Demodex approaches 100% in middle-aged and elderly adults,¹ mite density normally is low in healthy skin.²Demodex mites are considered pathogenic only when they are found in large numbers or in an intradermal location³; therefore, it has been suggested that D folliculorum may play a role in various papular and pustular eruptions of the head and neck, such as demodicosis and rosacea.³ We examine a case of scalp folliculitis secondary to Demodex infection and the role that this organism plays in the pathogenesis of folliculitis, as well as the available treatment options. 

Case Report

A 57-year-old white man presented to our department in June 2004 with an "infected scalp" and scalp irritation for 2 months. The patient was diagnosed with bacterial folliculitis and treated with clindamycin 1% gel twice daily for 1 month. He presented for follow-up in July 2004 with continued complaint of scalp pruritus and rash (Figure 1). Results of an examination showed a deep pink 10X7-cm plaque on the scalp with hyperkeratosis and pustules. An ectoparasite wet mount prepared from one of the pustules revealed the presence of several Demodex mites (Figure 2). The patient was treated with sulfacetamide 10% plus sulfur 5% cream twice daily, in addition to a 2-week course of selenium sulfide 2.5% shampoo once daily. When the patient was seen for follow-up in September 2004, his entire scalp had cleared (Figure 3). He was instructed to continue the selenium sulfide 2.5% shampoo twice weekly for 6 months to prevent recurrence.

Please refer to the PDF to view the figures

Comment

The Demodex mite is a ubiquitous arthropod measuring approximately 0.1 to 0.4 mm in length. Typically, it infests areas around the eyelids, nose, and ear canals in human hosts.⁴ The life cycle of the mite is 18 to 24 days. The female mite lays 20 to 24 eggs in a hair follicle where the eggs are nourished by the surrounding pilosebaceous unit. The eggs hatch and the nymphs continue to live in the follicle where their main source of food is human glandular secretions.⁵ The mite primarily is an asymptomatic inhabitant of human pilosebaceous follicles and poses no harm to the host.¹

The role of D folliculorum in cutaneous disease in humans remains controversial. The pathogenicity is difficult to establish secondary to the localization of the disease, the widespread prevalence of infection with the D folliculorum mite, and the obligate nature of the parasite; therefore, the detection of the presence of the mite is not, in and of itself, enough evidence to establish pathogenicity.⁶ Results of immunohistochemical staining have shown that helper T lymphocytes predominate in the dermal infiltrate of demodicosis suggesting a possible role of cell-mediated immune response and delayed hypersensitivity.⁷ There also is evidence for a humoral immune response component with increased macrophages and Langerhans cells in the presence of infestation with Demodex.⁷

Demodex mites have been implicated as a causative agent in rosacea and pustular folliculitis.⁶ It is important to consider the possibility that the vascular changes of rosacea create an environment that is favorable to the multiplication of Demodex mites and their penetration into the dermis.⁸ Forton and Seys⁶ reported that Demodex mites are associated with the inflammatory symptoms of rosacea and that the mites are present in greater numbers and higher frequencies in patients with rosacea. Additionally, a study by Georgala et al⁷ evaluated the importance of D folliculorum in the etiology and course of rosacea and showed that D folliculorum was found in 83 (90.2%) of 92 rosacea subjects studied but in only 11 (11.9%) of the 92 controls, thereby concluding that although Demodex mites may not be the cause of rosacea, they may represent an important cofactor. Finally, Vollmer⁹ examined 388 follicles in 24 resections of skin for the presence of histologic folliculitis and Demodex mites. Results showed that Demodex mites were found in 87 (42%) of 208 follicles with inflammation but in just 18 (10%) of 180 follicles without inflammation. Furthermore, 87 (83%) of 105 follicles with Demodex showed inflammation, which demonstrated a nonrandom association between these 2 entities.⁹ A study by Meinking et al¹⁰ supported the rapid clearing of papulopustular dermatosis of the scalp and granulomatous rosacea when treated with scabicidal preparations such as permethrin or ivermectin, thereby supporting the pathogenic role of Demodex in papulopustular eruptions.

On the other hand, a review by Aylesworth and Vance¹¹ found that 117 (10%) of 1124 skin biopsies and 198 (12%) of 1692 follicles incidentally revealed that follicular mites were found in patients with various unrelated skin disorders, thereby suggesting that Demodex is a normal inhabitant of the hair follicle and is not pathogenic.¹¹ Other histologic evidence that failed to show a correlation between Demodex presence and skin disease was an examination of the results of 108 biopsy specimens of rosacea, of which only 20 (19%) contained Demodex.¹² There was no correlation between Demodex mites and skin disease in a study of 29 biopsy samples of the head and neck by Nutting and Green.¹³ We must note that the reported prevalence of Demodex presence is partially determined by the preciseness of the detection method used.

There are several treatment options available for demodicosis. In our case, the patient cleared with a combination of sulfacetamide 10% plus sulfur 5% cream, in addition to selenium sulfide 2.5% shampoo. Other commonly used treatment options include ivermectin,¹⁰ topical antibiotics, and topical retinoids.

The persistence of the patient's folliculitis despite treatment with clindamycin 1% gel; rapid clearance after therapy with sulfacetamide 10% plus sulfur 5% cream twice daily and selenium sulfide 2.5% shampoo once daily is initiated; and positive results of the ectoparasite wet mount suggest a pathogenic role of Demodex in causing the patient's symptoms. Although the link between folliculitis and Demodex infection remains controversial, this case demonstrates the importance of considering the possible role of Demodex in the differential diagnosis of rosacea and papulopustular eruptions of the head and neck. 

Folliculitis is a common complaint seen in dermatology practice. The differential diagnosis of folliculitis is broad and includes Demodex folliculitis. In humans, the Demodex mite species Demodex folliculorum and Demodex brevis have been found to inhabit the pilosebaceous unit. D folliculorum typically is found in the follicular infundibulum; D brevis exists in the sebaceous and meibomian glands.¹ Although the prevalence of Demodex approaches 100% in middle-aged and elderly adults,¹ mite density normally is low in healthy skin.²Demodex mites are considered pathogenic only when they are found in large numbers or in an intradermal location³; therefore, it has been suggested that D folliculorum may play a role in various papular and pustular eruptions of the head and neck, such as demodicosis and rosacea.³ We examine a case of scalp folliculitis secondary to Demodex infection and the role that this organism plays in the pathogenesis of folliculitis, as well as the available treatment options. 

Case Report

A 57-year-old white man presented to our department in June 2004 with an "infected scalp" and scalp irritation for 2 months. The patient was diagnosed with bacterial folliculitis and treated with clindamycin 1% gel twice daily for 1 month. He presented for follow-up in July 2004 with continued complaint of scalp pruritus and rash (Figure 1). Results of an examination showed a deep pink 10X7-cm plaque on the scalp with hyperkeratosis and pustules. An ectoparasite wet mount prepared from one of the pustules revealed the presence of several Demodex mites (Figure 2). The patient was treated with sulfacetamide 10% plus sulfur 5% cream twice daily, in addition to a 2-week course of selenium sulfide 2.5% shampoo once daily. When the patient was seen for follow-up in September 2004, his entire scalp had cleared (Figure 3). He was instructed to continue the selenium sulfide 2.5% shampoo twice weekly for 6 months to prevent recurrence.

Please refer to the PDF to view the figures

Comment

The Demodex mite is a ubiquitous arthropod measuring approximately 0.1 to 0.4 mm in length. Typically, it infests areas around the eyelids, nose, and ear canals in human hosts.⁴ The life cycle of the mite is 18 to 24 days. The female mite lays 20 to 24 eggs in a hair follicle where the eggs are nourished by the surrounding pilosebaceous unit. The eggs hatch and the nymphs continue to live in the follicle where their main source of food is human glandular secretions.⁵ The mite primarily is an asymptomatic inhabitant of human pilosebaceous follicles and poses no harm to the host.¹

The role of D folliculorum in cutaneous disease in humans remains controversial. The pathogenicity is difficult to establish secondary to the localization of the disease, the widespread prevalence of infection with the D folliculorum mite, and the obligate nature of the parasite; therefore, the detection of the presence of the mite is not, in and of itself, enough evidence to establish pathogenicity.⁶ Results of immunohistochemical staining have shown that helper T lymphocytes predominate in the dermal infiltrate of demodicosis suggesting a possible role of cell-mediated immune response and delayed hypersensitivity.⁷ There also is evidence for a humoral immune response component with increased macrophages and Langerhans cells in the presence of infestation with Demodex.⁷

Demodex mites have been implicated as a causative agent in rosacea and pustular folliculitis.⁶ It is important to consider the possibility that the vascular changes of rosacea create an environment that is favorable to the multiplication of Demodex mites and their penetration into the dermis.⁸ Forton and Seys⁶ reported that Demodex mites are associated with the inflammatory symptoms of rosacea and that the mites are present in greater numbers and higher frequencies in patients with rosacea. Additionally, a study by Georgala et al⁷ evaluated the importance of D folliculorum in the etiology and course of rosacea and showed that D folliculorum was found in 83 (90.2%) of 92 rosacea subjects studied but in only 11 (11.9%) of the 92 controls, thereby concluding that although Demodex mites may not be the cause of rosacea, they may represent an important cofactor. Finally, Vollmer⁹ examined 388 follicles in 24 resections of skin for the presence of histologic folliculitis and Demodex mites. Results showed that Demodex mites were found in 87 (42%) of 208 follicles with inflammation but in just 18 (10%) of 180 follicles without inflammation. Furthermore, 87 (83%) of 105 follicles with Demodex showed inflammation, which demonstrated a nonrandom association between these 2 entities.⁹ A study by Meinking et al¹⁰ supported the rapid clearing of papulopustular dermatosis of the scalp and granulomatous rosacea when treated with scabicidal preparations such as permethrin or ivermectin, thereby supporting the pathogenic role of Demodex in papulopustular eruptions.

On the other hand, a review by Aylesworth and Vance¹¹ found that 117 (10%) of 1124 skin biopsies and 198 (12%) of 1692 follicles incidentally revealed that follicular mites were found in patients with various unrelated skin disorders, thereby suggesting that Demodex is a normal inhabitant of the hair follicle and is not pathogenic.¹¹ Other histologic evidence that failed to show a correlation between Demodex presence and skin disease was an examination of the results of 108 biopsy specimens of rosacea, of which only 20 (19%) contained Demodex.¹² There was no correlation between Demodex mites and skin disease in a study of 29 biopsy samples of the head and neck by Nutting and Green.¹³ We must note that the reported prevalence of Demodex presence is partially determined by the preciseness of the detection method used.

There are several treatment options available for demodicosis. In our case, the patient cleared with a combination of sulfacetamide 10% plus sulfur 5% cream, in addition to selenium sulfide 2.5% shampoo. Other commonly used treatment options include ivermectin,¹⁰ topical antibiotics, and topical retinoids.

The persistence of the patient's folliculitis despite treatment with clindamycin 1% gel; rapid clearance after therapy with sulfacetamide 10% plus sulfur 5% cream twice daily and selenium sulfide 2.5% shampoo once daily is initiated; and positive results of the ectoparasite wet mount suggest a pathogenic role of Demodex in causing the patient's symptoms. Although the link between folliculitis and Demodex infection remains controversial, this case demonstrates the importance of considering the possible role of Demodex in the differential diagnosis of rosacea and papulopustular eruptions of the head and neck. 

Folliculitis is a common complaint seen in dermatology practice. The differential diagnosis of folliculitis is broad and includes Demodex folliculitis. In humans, the Demodex mite species Demodex folliculorum and Demodex brevis have been found to inhabit the pilosebaceous unit. D folliculorum typically is found in the follicular infundibulum; D brevis exists in the sebaceous and meibomian glands.¹ Although the prevalence of Demodex approaches 100% in middle-aged and elderly adults,¹ mite density normally is low in healthy skin.²Demodex mites are considered pathogenic only when they are found in large numbers or in an intradermal location³; therefore, it has been suggested that D folliculorum may play a role in various papular and pustular eruptions of the head and neck, such as demodicosis and rosacea.³ We examine a case of scalp folliculitis secondary to Demodex infection and the role that this organism plays in the pathogenesis of folliculitis, as well as the available treatment options. 

Case Report

A 57-year-old white man presented to our department in June 2004 with an "infected scalp" and scalp irritation for 2 months. The patient was diagnosed with bacterial folliculitis and treated with clindamycin 1% gel twice daily for 1 month. He presented for follow-up in July 2004 with continued complaint of scalp pruritus and rash (Figure 1). Results of an examination showed a deep pink 10X7-cm plaque on the scalp with hyperkeratosis and pustules. An ectoparasite wet mount prepared from one of the pustules revealed the presence of several Demodex mites (Figure 2). The patient was treated with sulfacetamide 10% plus sulfur 5% cream twice daily, in addition to a 2-week course of selenium sulfide 2.5% shampoo once daily. When the patient was seen for follow-up in September 2004, his entire scalp had cleared (Figure 3). He was instructed to continue the selenium sulfide 2.5% shampoo twice weekly for 6 months to prevent recurrence.

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Comment

The Demodex mite is a ubiquitous arthropod measuring approximately 0.1 to 0.4 mm in length. Typically, it infests areas around the eyelids, nose, and ear canals in human hosts.⁴ The life cycle of the mite is 18 to 24 days. The female mite lays 20 to 24 eggs in a hair follicle where the eggs are nourished by the surrounding pilosebaceous unit. The eggs hatch and the nymphs continue to live in the follicle where their main source of food is human glandular secretions.⁵ The mite primarily is an asymptomatic inhabitant of human pilosebaceous follicles and poses no harm to the host.¹

The role of D folliculorum in cutaneous disease in humans remains controversial. The pathogenicity is difficult to establish secondary to the localization of the disease, the widespread prevalence of infection with the D folliculorum mite, and the obligate nature of the parasite; therefore, the detection of the presence of the mite is not, in and of itself, enough evidence to establish pathogenicity.⁶ Results of immunohistochemical staining have shown that helper T lymphocytes predominate in the dermal infiltrate of demodicosis suggesting a possible role of cell-mediated immune response and delayed hypersensitivity.⁷ There also is evidence for a humoral immune response component with increased macrophages and Langerhans cells in the presence of infestation with Demodex.⁷

Demodex mites have been implicated as a causative agent in rosacea and pustular folliculitis.⁶ It is important to consider the possibility that the vascular changes of rosacea create an environment that is favorable to the multiplication of Demodex mites and their penetration into the dermis.⁸ Forton and Seys⁶ reported that Demodex mites are associated with the inflammatory symptoms of rosacea and that the mites are present in greater numbers and higher frequencies in patients with rosacea. Additionally, a study by Georgala et al⁷ evaluated the importance of D folliculorum in the etiology and course of rosacea and showed that D folliculorum was found in 83 (90.2%) of 92 rosacea subjects studied but in only 11 (11.9%) of the 92 controls, thereby concluding that although Demodex mites may not be the cause of rosacea, they may represent an important cofactor. Finally, Vollmer⁹ examined 388 follicles in 24 resections of skin for the presence of histologic folliculitis and Demodex mites. Results showed that Demodex mites were found in 87 (42%) of 208 follicles with inflammation but in just 18 (10%) of 180 follicles without inflammation. Furthermore, 87 (83%) of 105 follicles with Demodex showed inflammation, which demonstrated a nonrandom association between these 2 entities.⁹ A study by Meinking et al¹⁰ supported the rapid clearing of papulopustular dermatosis of the scalp and granulomatous rosacea when treated with scabicidal preparations such as permethrin or ivermectin, thereby supporting the pathogenic role of Demodex in papulopustular eruptions.

On the other hand, a review by Aylesworth and Vance¹¹ found that 117 (10%) of 1124 skin biopsies and 198 (12%) of 1692 follicles incidentally revealed that follicular mites were found in patients with various unrelated skin disorders, thereby suggesting that Demodex is a normal inhabitant of the hair follicle and is not pathogenic.¹¹ Other histologic evidence that failed to show a correlation between Demodex presence and skin disease was an examination of the results of 108 biopsy specimens of rosacea, of which only 20 (19%) contained Demodex.¹² There was no correlation between Demodex mites and skin disease in a study of 29 biopsy samples of the head and neck by Nutting and Green.¹³ We must note that the reported prevalence of Demodex presence is partially determined by the preciseness of the detection method used.

There are several treatment options available for demodicosis. In our case, the patient cleared with a combination of sulfacetamide 10% plus sulfur 5% cream, in addition to selenium sulfide 2.5% shampoo. Other commonly used treatment options include ivermectin,¹⁰ topical antibiotics, and topical retinoids.

The persistence of the patient's folliculitis despite treatment with clindamycin 1% gel; rapid clearance after therapy with sulfacetamide 10% plus sulfur 5% cream twice daily and selenium sulfide 2.5% shampoo once daily is initiated; and positive results of the ectoparasite wet mount suggest a pathogenic role of Demodex in causing the patient's symptoms. Although the link between folliculitis and Demodex infection remains controversial, this case demonstrates the importance of considering the possible role of Demodex in the differential diagnosis of rosacea and papulopustular eruptions of the head and neck.