Volume 27 Number 2, Part 1 August 1992
Briefcommunications 259
Woolly hair nevus syndrome Salim A. AI-Harmozi, MSc,a Somaia F. Mahmoud, MD,a and Gershon C. Ejeckam, MRC Path, FRCPCb Doha, Qatar Woolly hair nevus is a rare nonhereditary anomaly of hair growth that usually begins within the first 2 years of life. 1 Although onset in adolescence has been described, it is rare in this age group. The affected hairs are lighter, tightly curled, and occur in a circumscribed area of the scalp. In addition to scalp involvement, ocular abnormalities,2 a pigmented or epidermal nevus on the ipsilateral side of the body, 3 and a verrucous epidermal nevus located in the same area as the primary lesion 4 have been described in association with woolly hair nevus. We present a case of woolly hair nevus with childhood onset associated with multiple hitherto unreported findings.
Fig. 1. Localized area of tightly curled brown hairs in parietal area.
CASE REPORT A lO-year-old Qatari boy had a patch of curly hair of 3 years' duration. Itstarted on the left parietal scalp and gradually spread to the occipital region, but thereafter remained stable. The patient had normal developmental milestones except for delayed speech. He was only able to compose sentences of two or three words at 6 years of age. During the past year his mother had noticed physical growth retardation. There were no past illnesses that would affect physical development. The parents were unrelated and the patient was the third of five healthy children. The patient was underweight (17 kg) and short (112 em). Examination of the scalp showed a well-defined, localized, 5 cm wide band of abnormal tightly curled brown hair in the left parietal region, cephalad and in front ofthe ear and extending to the occipital region (Fig. 1). The hairs were not fragile and there was no erythema or scaling. Underlying the woolly hair was an area of increased pigmentation. This extended down to the face. The rest of the scalp hairs were straight, black, and of normal appearance. Several hairs were manually epilated from the affected area and a nearby normal area. Curly hairs
From the Departments of Dermatology" and Laboratory Medicine & Pathology,b Hamad Medical Corporation. Reprint requests: Salim A. AI-Harmozi, MSc, Dermatology Department, Hamad Medical Corporation, P.O. Box 3050, Doha, Qatar.
16/54/36086
Fig. 2. X-ray of the hands shows marked bone age retardation to 5 to 6 years. Patient is 10 years old. were lighter and appeared smaller in diameter than the normal hairs. Light microscopic examination of the hair revealed no structural abnormalities. Distribution and hair growth on the rest ofthe body were normaL Punch biopsy specimens taken from the affected scalp and face lesions showed a linear epidermal nevus of the scalp and the face. One follicle in the woolly hair nevus appeared curved in the anagen phase. Examination ofthe oral cavity revealed gingivitis and decayed lower canine teeth. No nail or ocular abnormalities were noted. X-ray examination of bones showed marked bone age retardation to the age of 5 to 6 years (Fig. 2). Hormonal studies were normal.
Journal of the American Academy of Dermatology
260 Brief communications DISCUSSION
Association of woolly hair nevus with some ectodermal defects has been documented. However, our patient presented with features that have not been described before. These were bone growth retardation, delayed speech, and a dental anomaly. Also in our patient, the presence of a linear epidermal nevus underneath the woolly hair nevus represents the second report of this in the literature; the first case was reported by Peteiro et a1 4 Because of the additional findings of a linear epidermal nevus underneath the woolly hair nevus, delayed speech, marked bone age retardation, decayed deciduous canine teeth, and childhood onset, we
propose the term woolly hair nevus syndrome, to encompass what is now known as woolly hair nevus in association with any or all other ectodermal defects that may accompany this lesion. REFERENCES 1. Reda AM, Rogers RS III, Peters MS. Woolly hair nevus. J AM ACAD DERMATOL 1990;22:377-80. 2. Jacobsen KU, Lowes M. Woolly hair nevus with ocular involvement: report ofa case. Dermatologica 1975;151:249-52. 3. Lantis SDH, Pepper Me. Woolly hair nevus; two case reports and a discussion of unruly hair forms. Arch DermatolI978;114:233-8. 4. PeteiroC, Oliva NP, ZulaicaA, eta!. Woolly hair nevus: report of a case associated with a verrucous epidermal nevus in the same area. Pediatr DermatoI1989;6(3):188-90.
Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection N. Auffret, MD,a L. Quint, MD,a P. Domart, MD,a L. Dubertret, PhD,b J. Y. Lecam, MD,a and O. Binet, MDa Paris, France Papulosquamous disorders can occur in patients with human immunodeficiency virus (HIV) infection, l most often when the helper T cell count falls below 150jmm 3 • To our knowledge, only two cases of pityriasis rubra pilaris (PRP) have been reported in HIV-positive patients. 2. We report the case of a patient with PRP that was the initial manifestation of her HIV infection. 3-5 CASE REPORT A 28-year-old woman had a 6-month history of a nonpruritic dermatitis. Examination revealed a generalized follicular keratotic papular eruption. Some papules coalesced to form horny plaques, especially on the face (Fig. 1). The palms and soles (Fig. 2) were hyperkeratotic and erythematous; the nails showed subungual hyperkeratosis. Numerous subcutaneous axillary and inguinal nodules and cysts of hidradenitis suppurativa were present. Nodules and pustules were also present on the face. Except for a 12 pound weight loss, the patient appeared relatively well. From the Dermatology Service, Fondation A. de Rothschild"; and the Dermatology Service, H6pital Saint-Louis.b Reprint requests: O. Binet, MD, Fondation A. de Rothschild, 25 rue Manin, 75019 Paris, France.
16/54/36654
Fig. 1. Coalescence of plaques on the face. Filiform hyperkeratosis can be seen.
Briefcommunications 259
Woolly hair nevus syndrome Salim A. AI-Harmozi, MSc,a Somaia F. Mahmoud, MD,a and Gershon C. Ejeckam, MRC Path, FRCPCb Doha, Qatar Woolly hair nevus is a rare nonhereditary anomaly of hair growth that usually begins within the first 2 years of life. 1 Although onset in adolescence has been described, it is rare in this age group. The affected hairs are lighter, tightly curled, and occur in a circumscribed area of the scalp. In addition to scalp involvement, ocular abnormalities,2 a pigmented or epidermal nevus on the ipsilateral side of the body, 3 and a verrucous epidermal nevus located in the same area as the primary lesion 4 have been described in association with woolly hair nevus. We present a case of woolly hair nevus with childhood onset associated with multiple hitherto unreported findings.
Fig. 1. Localized area of tightly curled brown hairs in parietal area.
CASE REPORT A lO-year-old Qatari boy had a patch of curly hair of 3 years' duration. Itstarted on the left parietal scalp and gradually spread to the occipital region, but thereafter remained stable. The patient had normal developmental milestones except for delayed speech. He was only able to compose sentences of two or three words at 6 years of age. During the past year his mother had noticed physical growth retardation. There were no past illnesses that would affect physical development. The parents were unrelated and the patient was the third of five healthy children. The patient was underweight (17 kg) and short (112 em). Examination of the scalp showed a well-defined, localized, 5 cm wide band of abnormal tightly curled brown hair in the left parietal region, cephalad and in front ofthe ear and extending to the occipital region (Fig. 1). The hairs were not fragile and there was no erythema or scaling. Underlying the woolly hair was an area of increased pigmentation. This extended down to the face. The rest of the scalp hairs were straight, black, and of normal appearance. Several hairs were manually epilated from the affected area and a nearby normal area. Curly hairs
From the Departments of Dermatology" and Laboratory Medicine & Pathology,b Hamad Medical Corporation. Reprint requests: Salim A. AI-Harmozi, MSc, Dermatology Department, Hamad Medical Corporation, P.O. Box 3050, Doha, Qatar.
16/54/36086
Fig. 2. X-ray of the hands shows marked bone age retardation to 5 to 6 years. Patient is 10 years old. were lighter and appeared smaller in diameter than the normal hairs. Light microscopic examination of the hair revealed no structural abnormalities. Distribution and hair growth on the rest ofthe body were normaL Punch biopsy specimens taken from the affected scalp and face lesions showed a linear epidermal nevus of the scalp and the face. One follicle in the woolly hair nevus appeared curved in the anagen phase. Examination ofthe oral cavity revealed gingivitis and decayed lower canine teeth. No nail or ocular abnormalities were noted. X-ray examination of bones showed marked bone age retardation to the age of 5 to 6 years (Fig. 2). Hormonal studies were normal.
Journal of the American Academy of Dermatology
260 Brief communications DISCUSSION
Association of woolly hair nevus with some ectodermal defects has been documented. However, our patient presented with features that have not been described before. These were bone growth retardation, delayed speech, and a dental anomaly. Also in our patient, the presence of a linear epidermal nevus underneath the woolly hair nevus represents the second report of this in the literature; the first case was reported by Peteiro et a1 4 Because of the additional findings of a linear epidermal nevus underneath the woolly hair nevus, delayed speech, marked bone age retardation, decayed deciduous canine teeth, and childhood onset, we
propose the term woolly hair nevus syndrome, to encompass what is now known as woolly hair nevus in association with any or all other ectodermal defects that may accompany this lesion. REFERENCES 1. Reda AM, Rogers RS III, Peters MS. Woolly hair nevus. J AM ACAD DERMATOL 1990;22:377-80. 2. Jacobsen KU, Lowes M. Woolly hair nevus with ocular involvement: report ofa case. Dermatologica 1975;151:249-52. 3. Lantis SDH, Pepper Me. Woolly hair nevus; two case reports and a discussion of unruly hair forms. Arch DermatolI978;114:233-8. 4. PeteiroC, Oliva NP, ZulaicaA, eta!. Woolly hair nevus: report of a case associated with a verrucous epidermal nevus in the same area. Pediatr DermatoI1989;6(3):188-90.
Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection N. Auffret, MD,a L. Quint, MD,a P. Domart, MD,a L. Dubertret, PhD,b J. Y. Lecam, MD,a and O. Binet, MDa Paris, France Papulosquamous disorders can occur in patients with human immunodeficiency virus (HIV) infection, l most often when the helper T cell count falls below 150jmm 3 • To our knowledge, only two cases of pityriasis rubra pilaris (PRP) have been reported in HIV-positive patients. 2. We report the case of a patient with PRP that was the initial manifestation of her HIV infection. 3-5 CASE REPORT A 28-year-old woman had a 6-month history of a nonpruritic dermatitis. Examination revealed a generalized follicular keratotic papular eruption. Some papules coalesced to form horny plaques, especially on the face (Fig. 1). The palms and soles (Fig. 2) were hyperkeratotic and erythematous; the nails showed subungual hyperkeratosis. Numerous subcutaneous axillary and inguinal nodules and cysts of hidradenitis suppurativa were present. Nodules and pustules were also present on the face. Except for a 12 pound weight loss, the patient appeared relatively well. From the Dermatology Service, Fondation A. de Rothschild"; and the Dermatology Service, H6pital Saint-Louis.b Reprint requests: O. Binet, MD, Fondation A. de Rothschild, 25 rue Manin, 75019 Paris, France.
16/54/36654
Fig. 1. Coalescence of plaques on the face. Filiform hyperkeratosis can be seen.
