Alopecia areata (AA) is a nonscaring type of hair loss, which is considered an autoimmune disease. The patchy loss of hair occurring in alopecia areata may involve not only the scalp but also any hair bearing area. This condition almost always involves the scalp, so alopecia areata only involving other body sites without involving the scalp is rare. To the best of our knowledge, there is no previously reported case of alopecia areata limited to the pubic area in the Korean dermatological literature. Herein, we report the first case of alopecia areata limited to the pubic area without involving other sites.
Alopecia ; Alopecia Areata ; Autoimmune Diseases ; Hair ; Scalp ; Ursidae

Alopecia areata is a common skin disease with diverse modalities of therapy. The eyebrows are one of the target sites of alopecia areata and may be the only affected site. We report our recent experiences of superficial cryotherapy with liquid nitrogen to promote eyebrow hair growth due to alopecia areata. According to our results, superficial cryotherapy of alopecia areata in eyebrows can be an effective mode of treatment.
Alopecia Areata* ; Alopecia* ; Cryotherapy* ; Eyebrows* ; Hair ; Nitrogen ; Skin Diseases

A two-year-old spayed female pug presented with symmetrical hyperpigmented alopecic lesions on her axillary and inguinal regions. There were no remarkable findings in dermatologic examinations and hormonal assays. Histological examination of biopsied tissues revealed prominent lymphocytic perifolliculitis along with shrunk hair follicles. Immunohistochemistry for CD3, CD79a, CD4, and CD8 showed a positive stain for CD4 antigen around hair bulbs, suggesting CD4 positive T lymphocyte infiltration. This case suggests the possibility that CD4 T lymphocyte-mediated inflammatory reaction could be a main mechanism in canine alopecia areata. Additional studies are warranted to investigate the immunological mechanism in canine species.
Alopecia Areata* ; Alopecia* ; Antigens, CD4 ; Autoimmune Diseases ; Female ; Hair ; Hair Follicle ; Humans ; Immunohistochemistry ; Lymphocytes

BACKGROUND: Alopecia areata(AA) is believed to be an autoimmune disease in which a mononuclear cell infiltrate develops in and around anagen hair follicles. There is no clearly superior therapy in the treatment of AA, especially AA with atopic dermatitis and alopecia universalis. The theory of autoimmune pathogenesis of alopecia areata suggests a potential therapeutic effect of cyclosporin-A(CsA). OBJECTIVE: The purpose of this study is to evaluate the effectiveness of CsA in the treatment of AA. METHOD: 12 patients with severe or refractory AA were treated with DPCP for at least 12 months. They showed resistance to treatment using DPCP. CsA was made up as a 0.01M, 0.005M solution in an ethanol preparation. 1cc of 0.01M CsA solution was applied on the Lt. side scalp and 1cc of 0.005M CsA solution was applied on the Rt. side scalp. The drug was applied once per week. Response to treatment was evaluated as follows: complete recovery, more than a 80% extent of hair regrowth; marked recovery, hair regrowth of 60% to 80%, moderate recovery, hair regrowth of 40% to 60%; slight recovery, hair regrowth of 20% to 40%; no response, hair regrowth of 0% to 20%. RESULT: The Six patients with focal type AA showed a moderate recovery. Of the six patients with alopecia totalis, 4 patients showed a moderate recovery, two patients showed no response. CONCLUSION: Topical CsA therapy is recommended in severe and refractory AA.
Alopecia Areata* ; Alopecia* ; Autoimmune Diseases ; Dermatitis, Atopic ; Ethanol ; Hair ; Hair Follicle ; Humans ; Scalp

Menkes disease, so called kinky-hair syndrome, is a rare fatal X-linked recessive disorder, which is caused by a mutation in the ATP7A gene encoding the copper transporting ATPase. Dysfunction of copper-dependent enzymes results in various clinical features, including skin and hair hypopigmentation, progressive neurologic degeneration, bone and connective tissue alterations with soft doughy skin and joint laxity, and vascular abnormalities, including aneurysms and bladder diverticula. Patients have the characteristic hair, which is kinky, colorless or steel-colored, and brittle with cutis laxa. Early diagnosis and treatments are perquisites for improving the clinical outcomes. Herein, we describe a rare case of Menkes disease accompanied by hair abnormality and cutis laxa in a 35-days-old boy.
Adenosine Triphosphatases ; Aneurysm ; Connective Tissue ; Copper ; Cutis Laxa ; Diverticulum ; Early Diagnosis ; Hair ; Hair Diseases ; Humans ; Hypopigmentation ; Joint Instability ; Menkes Kinky Hair Syndrome ; Skin ; Urinary Bladder

Menkes disease, so called kinky-hair syndrome, is a rare fatal X-linked recessive disorder, which is caused by a mutation in the ATP7A gene encoding the copper transporting ATPase. Dysfunction of copper-dependent enzymes results in various clinical features, including skin and hair hypopigmentation, progressive neurologic degeneration, bone and connective tissue alterations with soft doughy skin and joint laxity, and vascular abnormalities, including aneurysms and bladder diverticula. Patients have the characteristic hair, which is kinky, colorless or steel-colored, and brittle with cutis laxa. Early diagnosis and treatments are perquisites for improving the clinical outcomes. Herein, we describe a rare case of Menkes disease accompanied by hair abnormality and cutis laxa in a 35-days-old boy.
Adenosine Triphosphatases ; Aneurysm ; Connective Tissue ; Copper ; Cutis Laxa ; Diverticulum ; Early Diagnosis ; Hair ; Hair Diseases ; Humans ; Hypopigmentation ; Joint Instability ; Menkes Kinky Hair Syndrome ; Skin ; Urinary Bladder

We report here on the diffusion-weighted imaging of unusual white matter lesions in a case of Menkes disease. On the initial MR imaging, the white matter lesions were localized in the deep periventricular white matter in the absence of diffuse cortical atrophy. The lesion showed diffuse high signal on the diffusion-weighted images and diffuse progression and persistent hyperintensity on the follow up imaging. Our case suggests that the white matter lesion may precede diffuse cortical atrophy in a patient with Menkes disease.
Menkes Kinky Hair Syndrome/*diagnosis ; Male ; Infant ; Humans ; *Diffusion Magnetic Resonance Imaging ; Diagnosis, Differential ; Brain Diseases/*diagnosis ; Atrophy

A 19 year old female patient presented with diffuse alopecia as her chief medical complaint. A clinical examination revealed hirsutism limited only to the on midline lower abdomen with elevated DHEA-S(dehydroepiandrosterone sulfate) and total testosterone levels. Polycystic ovarian disease (PCOD) was diagnosed during the treatment with dexamethasone and spironolactone, which was effective to improve her alopecia. We believe that, with increasing, concerns about hair conditions of teen-age girls there should be increasing chances for dermatologists to care for patients of PCOD first before other specialities in medicine.
Abdomen ; Alopecia* ; Dexamethasone ; Female* ; Hair ; Hirsutism ; Humans ; Ovarian Diseases* ; Spironolactone ; Testosterone ; Young Adult

Menkes syndrome is a rare fatal neurodegenerative disease with X-linked recessive inheritance. The clinical characteristic features are progressive neurological disturbances, arterial degeneration, connective tissue and hair abnormalities. This syndrome is a disorder of copper transport caused by mutations in a gene for a copper-transporting P-type ATPase. We have experienced a case of Menkes syndrome in an eight-month old male patient, who manifested seizure, developmental delay and characteristic pili torti. He was diagnosed by clinical features, laboratory tests such as low serum copper and ceruloplasmin level, cerebral atropy in brain magnetic resonance(MR) image and arterial abnormality in MR angiography. We review literatures and describe the distinctive features of this rare disorder.
Adenosine Triphosphatases ; Angiography ; Brain ; Ceruloplasmin ; Connective Tissue ; Copper ; Genes, vif ; Hair ; Humans ; Male ; Menkes Kinky Hair Syndrome* ; Neurodegenerative Diseases ; Seizures ; Wills

Menkes disease, so called kinky-hair syndrome, is a rare, genetic and progressive neurodegenerative disorder. It is caused by a mutation in the ATP7A gene, which codes for the copper transporting ATPase in the cell organelles. The dysfunction of many copper-dependent enzymes results in low concentration of copper in some tissues and accumulation of copper in others. We report a boy presented with kinky hairs, developmental delay, hypotonia and connective tissue abnormalities at the age of 4 months. Despite the treatment with various antiepileptic drugs, atonic seizures still persisted. At the age of 7 months, his atonic seizures was changed into extensor spasms with modified hypsarrhythmia for some years. The seizure were controlled by topiramate and vigabatrin. At the age of 22 months, serum copper and ceruloplasmin rechecked as 17 ug/dL(80-150 ug/dL) and 7.3 mg/dL(20-46 mg/ dL) respectively. The gene study showed ATP7A mutation and the patient was diagnosed as Menkes disease so that copper-histidine was daily injected. We experienced a case of a 4-month-old boy with Menkes disease and infantile spasm, confirmed by ATP7A mutation.
Adenosine Triphosphatases ; Anticonvulsants ; Ceruloplasmin ; Connective Tissue ; Copper ; Hair ; Humans ; Infant ; Infant, Newborn ; Male ; Menkes Kinky Hair Syndrome* ; Muscle Hypotonia ; Neurodegenerative Diseases ; Organelles ; Seizures ; Spasm ; Spasms, Infantile* ; Vigabatrin