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Childhood sarcoidosis presenting with extensive cutaneous lesions, lymphadenopathy, and hypercalcemia
P2407
P2409
AUTOIMMUNE ABNORMALITIES ASSOCIATED WITH ALOPECIA AREATA AND VITILIGO IN CHILDREN Lucy Martin, MD, Laura Black, MPH, Andrea Trowers, MD, Lawrence Schachner, MD, University of Miami School of Medicine, Department of Dermatology and Cutaneous Surgery, Miami, FL, United States
CHILDHOOD SARCOIDOSIS PRESENTING WITH EXTENSIVE CUTANEOUS LESIONS, LYMPHADENOPATHY, AND HYPERCALCEMIA Neil Mortimer, MBChB, Toby Chave, MBChB, Department of Dermatology, Leicester, England; Alan Elias-Jones, MBChB, Department of Paediatrics, Leicester, England; Robin Graham-Brown, MBChB, Department of Dermatology, Leicester, England Childhood sarcoidosis is rare. We describe the case of a 9-year-old boy of African Caribbean descent who presented with numerous, waxy, yellow/orange papules predominantly in a perioral distribution. There were less dense lesions scattered over his trunk and limbs. He had large-volume axillary and inguinal lymphadenopathy. Clinically he appeared healthy, although his parents reported polyuria and polydipsia of several months’ duration. Serum angiotensin-converting enzyme levels were raised, and his calcium level was markedly elevated (3.77 mmol/L). Chest x-ray showed bilateral hilar lymphadenopathy. Histology of a skin and lymph node biopsy revealed naked noncaseating granulomas. Initial management of the hypercalcemia with rehydration and forced diuresis was unsuccessful. Subsequently he was treated with oral prednisolone (2 mg/kg per day). This led to rapid normalization of the serum calcium and resolution of the cutaneous lesions and lymphadenopathy. The dosage of systemic corticosteroids continues to be tapered. We discuss the clinical features and management of this unusual condition in children.
Background: Alopecia areata (AA) and vitiligo have been associated with autoimmune abnormalities, most commonly pernicious anemia, thyroid diseases, lupus erythematosus, and diabetes mellitus. Objective: To determine the prevalence of autoimmune and thyroid abnormalities associated with AA and vitiligo in the University of Miami Pediatric Dermatology Clinic. Methods: We conducted a retrospective chart review of patients with diagnoses of AA and/or vitiligo between 1998 and 2002. Only those patients younger than 18 years with laboratory results were included in the analyses. Laboratory results (thyroid-stimulating hormone [TSH], T3, T4, antinuclear antibodies [ANA], antimicrosomal antibodies, and anti-thyroid antibodies) were reviewed. Results: Of 275 charts reviewed, 107 met the criteria for this study. Forty-four patients (41.1%) were diagnosed with AA, 61 patients (57%) were diagnosed with vitiligo, and 2 patients (1.9%) were diagnosed with both AA and vitiligo. There were an equal number of female and male patients diagnosed with AA in this study sample. There were more females diagnosed with vitiligo (54%) than males (46%). ANAs and TSH were among the laboratory results most commonly found to be positive.
Nothing to disclose.
Conclusions: Because of a small sample size that was tested for both thyroid and autoimmune functions, we recommend conducting a prospective study in which laboratory data are collected in a systematic manner to test the association between AA and/or vitiligo diagnoses and abnormal thyroid and autoimmune functions. Nothing to disclose.
P2410 CLASSIC PACHYDERMODACTYLY: STUDY OF 5 CASES IN YOUNG BOYS ˜ uls, MD, Mar Blanes, MD, Isabel Betlloch, MD, Natalia Pastor, MD, Jose Ban Hospital General de Alicante, Alicante, Spain Introduction: Pachydermodactyly is a superficial benign digital fibromatosis that usually involves the proximal portion of the fingers. Five types of this entity have been proposed. We report 5 new cases of classic pachydermodactyly.
Xanthelasma palpebrarum, or simply xanthelasma, is the most common type of cutaneous xanthoma and typically involves eyelids. It has been reported that 50% of patients with xanthelasma have underlying hyperlipidemia. Its prevalence is higher in women than in men, and it tends to be increased with age; however, xanthelasma in child is very rare. We report a case of xanthelasma in a 9-year-old boy who had bilateral skin lesions on both upper and lower eyelids without hyperlipidemia and family history. Our case is unique in several aspects; the skin lesion developed during the first 5 years of age, occurred first in the lower eyelid, started and involved lateral canthal area, and the skin lesions in the upper eyelids currently had longer than those in the lower eyelid.
Case report: Five patients with pachydermodactyly, ranging in age from 14 to 30 years, were studied. All patients were male without previous pathologic conditions. They have noticed a progressive growing of their fingers. X-ray examination of the affected fingers was performed in 3 cases showing soft tissue hyperplasia without bone involvement. Four patients underwent cutaneous biopsy, which showed the characteristic epidermal hyperplasia, with increase of dermal collagen bundles and extension of collagenous fibers into the subcutaneous tissue. Hormonal studies were carried out to rule out acromegaly and hypothyroidism. The personal history of all patients revealed a compulsive habit of interlacing or rubbing the fingers. The treatment included protective bandages and gloves, avoiding the mechanical injury. Improvement of the lesions was observed in all cases. Discussion: Pachydermodactyly is characterized by symmetrical, diffuse swelling of the skin around the dorsal aspect and sides of the proximal phalanges of the index, ring, and middle fingers. Classification of this entity into 5 types has been proposed. Classic pachydermodactyly typically affects more than one digit, and frequently mechanical trauma plays a role in the development of this condition. The classification also includes a localized type, a more extensive type or transgradiens pachydermodactyly, a familial type, and pachydermodactyly associated with tuberous sclerosis. All our patients fit in the classic type of this entity. Differential diagnosis of pachydermodactyly includes occupational callosities, ‘‘obsessive chewing pads,’’ and knuckle pads. According to our series, there is a clear male predominance. Classic pachydermodactyly could be the expression of compulsive habits. Control over these habits would improve the lesions.
Nothing to disclose.
Nothing to disclose.
P2408 BILATERAL XANTHELASMA PALPEBRARUM ON BOTH EYELIDS IN A 9-YEAR-OLD BOY Hyung-Seok Park, MD, Min-Gu Kim, MD, Won-Serk Kim, MD, Eil-Soo Lee, MD, PhD, Department of Dermatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
MARCH 2005
J AM ACAD DERMATOL
P153
P2409
AUTOIMMUNE ABNORMALITIES ASSOCIATED WITH ALOPECIA AREATA AND VITILIGO IN CHILDREN Lucy Martin, MD, Laura Black, MPH, Andrea Trowers, MD, Lawrence Schachner, MD, University of Miami School of Medicine, Department of Dermatology and Cutaneous Surgery, Miami, FL, United States
CHILDHOOD SARCOIDOSIS PRESENTING WITH EXTENSIVE CUTANEOUS LESIONS, LYMPHADENOPATHY, AND HYPERCALCEMIA Neil Mortimer, MBChB, Toby Chave, MBChB, Department of Dermatology, Leicester, England; Alan Elias-Jones, MBChB, Department of Paediatrics, Leicester, England; Robin Graham-Brown, MBChB, Department of Dermatology, Leicester, England Childhood sarcoidosis is rare. We describe the case of a 9-year-old boy of African Caribbean descent who presented with numerous, waxy, yellow/orange papules predominantly in a perioral distribution. There were less dense lesions scattered over his trunk and limbs. He had large-volume axillary and inguinal lymphadenopathy. Clinically he appeared healthy, although his parents reported polyuria and polydipsia of several months’ duration. Serum angiotensin-converting enzyme levels were raised, and his calcium level was markedly elevated (3.77 mmol/L). Chest x-ray showed bilateral hilar lymphadenopathy. Histology of a skin and lymph node biopsy revealed naked noncaseating granulomas. Initial management of the hypercalcemia with rehydration and forced diuresis was unsuccessful. Subsequently he was treated with oral prednisolone (2 mg/kg per day). This led to rapid normalization of the serum calcium and resolution of the cutaneous lesions and lymphadenopathy. The dosage of systemic corticosteroids continues to be tapered. We discuss the clinical features and management of this unusual condition in children.
Background: Alopecia areata (AA) and vitiligo have been associated with autoimmune abnormalities, most commonly pernicious anemia, thyroid diseases, lupus erythematosus, and diabetes mellitus. Objective: To determine the prevalence of autoimmune and thyroid abnormalities associated with AA and vitiligo in the University of Miami Pediatric Dermatology Clinic. Methods: We conducted a retrospective chart review of patients with diagnoses of AA and/or vitiligo between 1998 and 2002. Only those patients younger than 18 years with laboratory results were included in the analyses. Laboratory results (thyroid-stimulating hormone [TSH], T3, T4, antinuclear antibodies [ANA], antimicrosomal antibodies, and anti-thyroid antibodies) were reviewed. Results: Of 275 charts reviewed, 107 met the criteria for this study. Forty-four patients (41.1%) were diagnosed with AA, 61 patients (57%) were diagnosed with vitiligo, and 2 patients (1.9%) were diagnosed with both AA and vitiligo. There were an equal number of female and male patients diagnosed with AA in this study sample. There were more females diagnosed with vitiligo (54%) than males (46%). ANAs and TSH were among the laboratory results most commonly found to be positive.
Nothing to disclose.
Conclusions: Because of a small sample size that was tested for both thyroid and autoimmune functions, we recommend conducting a prospective study in which laboratory data are collected in a systematic manner to test the association between AA and/or vitiligo diagnoses and abnormal thyroid and autoimmune functions. Nothing to disclose.
P2410 CLASSIC PACHYDERMODACTYLY: STUDY OF 5 CASES IN YOUNG BOYS ˜ uls, MD, Mar Blanes, MD, Isabel Betlloch, MD, Natalia Pastor, MD, Jose Ban Hospital General de Alicante, Alicante, Spain Introduction: Pachydermodactyly is a superficial benign digital fibromatosis that usually involves the proximal portion of the fingers. Five types of this entity have been proposed. We report 5 new cases of classic pachydermodactyly.
Xanthelasma palpebrarum, or simply xanthelasma, is the most common type of cutaneous xanthoma and typically involves eyelids. It has been reported that 50% of patients with xanthelasma have underlying hyperlipidemia. Its prevalence is higher in women than in men, and it tends to be increased with age; however, xanthelasma in child is very rare. We report a case of xanthelasma in a 9-year-old boy who had bilateral skin lesions on both upper and lower eyelids without hyperlipidemia and family history. Our case is unique in several aspects; the skin lesion developed during the first 5 years of age, occurred first in the lower eyelid, started and involved lateral canthal area, and the skin lesions in the upper eyelids currently had longer than those in the lower eyelid.
Case report: Five patients with pachydermodactyly, ranging in age from 14 to 30 years, were studied. All patients were male without previous pathologic conditions. They have noticed a progressive growing of their fingers. X-ray examination of the affected fingers was performed in 3 cases showing soft tissue hyperplasia without bone involvement. Four patients underwent cutaneous biopsy, which showed the characteristic epidermal hyperplasia, with increase of dermal collagen bundles and extension of collagenous fibers into the subcutaneous tissue. Hormonal studies were carried out to rule out acromegaly and hypothyroidism. The personal history of all patients revealed a compulsive habit of interlacing or rubbing the fingers. The treatment included protective bandages and gloves, avoiding the mechanical injury. Improvement of the lesions was observed in all cases. Discussion: Pachydermodactyly is characterized by symmetrical, diffuse swelling of the skin around the dorsal aspect and sides of the proximal phalanges of the index, ring, and middle fingers. Classification of this entity into 5 types has been proposed. Classic pachydermodactyly typically affects more than one digit, and frequently mechanical trauma plays a role in the development of this condition. The classification also includes a localized type, a more extensive type or transgradiens pachydermodactyly, a familial type, and pachydermodactyly associated with tuberous sclerosis. All our patients fit in the classic type of this entity. Differential diagnosis of pachydermodactyly includes occupational callosities, ‘‘obsessive chewing pads,’’ and knuckle pads. According to our series, there is a clear male predominance. Classic pachydermodactyly could be the expression of compulsive habits. Control over these habits would improve the lesions.
Nothing to disclose.
Nothing to disclose.
P2408 BILATERAL XANTHELASMA PALPEBRARUM ON BOTH EYELIDS IN A 9-YEAR-OLD BOY Hyung-Seok Park, MD, Min-Gu Kim, MD, Won-Serk Kim, MD, Eil-Soo Lee, MD, PhD, Department of Dermatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
MARCH 2005
J AM ACAD DERMATOL
P153