Centrifugal lipodystrophy of the scalp presenting with an arch-form alopecia: A 10-year follow-up observation

We describe a 10-year follow-up observation of progressive arch-form alopecia caused by centrifugal lipodystrophy (CLD) in a Japanese boy. A 2.5-year-old boy developed a slightly depressed lesion demarcated by a horseshoe-shaped erythematous border on his right neck, which then extended to the scalp. Four years later, arch-form alopecia became apparent in the right temporal region along with an erythematous border. The arch-form alopecia gradually expanded centrifugally, leaving a slight residual depression, but hair regrowth was seen within the area of alopecia. Histological examination of the erythematous border revealed non-specific inflammatory changes in the subcutaneous fat. Magnetic resonance imaging findings revealed a loss of subcutaneous fat inside the lesion. The alopecia continuously extended until he was 12 years old, but, thereafter, expansion ceased and hair regrowth gradually occurred in the arch-form alopecia. A linear non-hairy lesion 5 cm in length still remained when he was 13 years old. CLD might involve the scalp and cause linear, arch-form alopecia.     

Erythema nodosum induced by kerion celsi of the scalp

A 5-year-old girl presented with a 2-week history of a sharply demarcated, inflammatory, granulomatous lesion on the right side of her scalp. Shortly afterward, painful, subcutaneous nodules developed on her shins and thighs. Trichophyton mentagrophytes was isolated from the scalp lesion and a diagnosis of erythema nodosum induced by kerion of the scalp was made. The patient was started on oral therapy with 18 mg/kg/day griseofulvin, associated with topical crystal violet. Her erythema nodosum regressed in 10 days, while the kerion healed 6 weeks later, leaving residual scarring alopecia. Erythema nodosum represents a reaction pattern to a wide variety of inflammatory stimuli. The interest of this case lies in the unusual association of kerion erythema nodosum, of which only nine cases have been reported in the international literature.     

Mediation of alopecia areata by cooperation between CD4+ and CD8+ T lymphocytes: transfer to human scalp explants on Prkdc(scid) mice

OBJECTIVE: To determine the role of CD4+ and CD8+ T lymphocytes in the pathogenesis of alopecia areata. DESIGN: Relapse of alopecia areata was induced in autologous human scalp grafts on Prkdc(scid) mice by injection of activated T lymphocytes derived from lesional skin. CD4+ and CD8+ T cells were separated by magnetic beads before injection. SETTING: University-based dermatology practice. PARTICIPANTS: Eleven patients with either alopecia totalis or severe alopecia areata. MAIN OUTCOME MEASURES: Hair regrowth, hair loss, and immunohistochemical findings of scalp explants. INTERVENTION: Transfer of scalp T cells to autologous lesional scalp explants on Prkdc(scid) mice. RESULTS: Injection of unseparated T cells and mixed CD4+ plus CD8+ T cells resulted in significant hair loss (P<.01) in 5 of 5 experiments. However, injection of purified CD4+ or CD8+ T cells alone did not result in reproducible hair loss. CD4+ and CD8+ T cells induced follicular expression of intercellular adhesion molecule 1 (CD54), HLA-DR, and HLA-A, HLA-B, and HLA-C after injection into scalp grafts. CONCLUSIONS: CD4+ and CD8+ T cells have a role in the pathogenesis of alopecia areata. It is hypothesized that CD8+ T cells act as the effector cells, with CD4+ T cell help. It is now necessary to look for HLA-A, HLA-B, and HLA-C associations with alopecia areata. Therapeutic manipulations that interfere with CD8+ activity should be examined.     

Cutaneous alternariosis with chronic granulomatous disease

We report here a case of dermal cutaneous alternariosis in a 69-year-old man with X-linked chronic granulomatous disease (CGD). The lesion on the back of the right hand spread and became indurated, even though oral itraconazole 100 mg daily for 12 weeks was administered. After 28 weeks of treatment with oral fluconazole at 200 mg daily, the lesion disappeared and left only slight pigmentation. Alternaria species are common saprophytes that are not usually pathogenic in humans. However, there are some reports of cutaneous alternariosis in immunocompromised patients. To our knowledge, this is the first case of cutaneous alternariosis in CGD and the response to fluconazole, a drug not usually used for this mycosis.     

Pyoderma gangrenosum and cranial osteolysis: case report and review of the paediatric literature

Pyoderma gangrenosum is a poorly understood, ulcerating cutaneous disorder which is rarely seen in the paediatric age-group. We report a 3-year-old boy who developed an ulcer over the left frontoparietal scalp at the age of l year. A 9-cm area of'underlying cranial bone was destroyed. The appearance on radiographs and CT scun was suggestive of eosinophilic granuloma, osteomyelitis. or other destructive processes. Biopsics of the scalp lesion and calvaria showed granulation tissue and degenerating bone. After the biopsies the scalp lesion increascd in size, and wound dehiscencc occurred, Ulceration developed al the site of a PPD skin test, which on biopsy was consistent with the diagnosis of pyoderma gangrenosum. Pyoderma gangrenosum should be added to the differcntial diagnosis of cutaneous disorders which can result in osleolylic/osteonecrotic defects.     

婴儿头皮前B淋巴母细胞性淋巴瘤

报道1例婴儿头皮前B淋巴母细胞性淋巴瘤。患儿男,9个月,因头皮肿块,脱发6个月余就诊。皮损表现为7．5cm×10．5cm浸润性肿块,呈暗红色,形态不规则,皮损区头发稀疏伴斑状脱发。皮肤活检示真皮及皮下组织弥漫性分布肿瘤细胞,瘤细胞中等大小,胞质少,核呈圆或卵圆形,核仁小,可见核分裂相,表真皮之间有细胞浸润带将表真皮分开。免疫组化CD20、BCL-2、Ki-67和PAX5均呈阳性表达,CD3、TdT、CD30、AI。K、CK5／6、CD56、TIA-1、CD43、CD117、穿孔素和颗粒酶B为阴性。     

Concurrence of alopecia areata and vitiligo at the same anatomical site

Both alopecia areata and vitiligo are common skin disorders that are considered to be caused by an autoimmune response targeted to hair follicle and melanocyte antigens, respectively. The association of these two diseases in the same patient is well known, however, coexistence of alopecia areata and vitiligo within the same lesion is very rare. Herein, we report an 8-year-old boy who had colocalization of alopecia areata and vitiligo on the frontal portion of his scalp.     

Centrifugal lipodystrophy presenting with serpiginous erythema and alopecia

We describe serpiginous erythema with alopecia developing on the scalp of a 10-year-old boy during follow-up of centrifugal lipodystrophy. Because the clinical and histopathologic features of these lesions were identical to those of centrifugal lipodystrophy, we conclude that involvement of a hairy region by this disorder could cause alopecia and that the hair loss might be an indirect effect of interstitial inflammatory infiltrates around the hair follicles and in the subcutaneous fat.     

Alopecia Universalis in a Patient with Common Variable Immunodeficiency

A 12-year-old boy with common variable immunodeficiency (CVI) who developed severe alopecia is presented. His sister also had alopecia and recurrent infections and died of lung infection at the age of 7 years. The loss of hair in both children was total; the pathology of a scalp skin biopsy specimen was typical for alopecia areata. The boy was subjected to clinical and immunologic evaluation and the results were compatible with common variable immunodeficiency.     

Alopecia totalis incognito

We report an 11-year-old boy with a strong family history of alopecia areata who initially developed alopecia areata with a single circular patch of hair loss on the scalp with exclamation mark hairs at the periphery, which evolved over time into alopecia totalis and then into a novel pattern of hair growth with fine depigmented hair, uniformly 5 mm in length. The hair has not grown any longer over a 48-month follow-up period. Scalp biopsies from the occipital scalp demonstrate dense peribulbar lymphocytic infiltrate and uniform miniaturized secondary vellus hairs. This previously undescribed pattern of alopecia areata is remarkable for total lack of alopecia.     

Allergic and irritant contact dermatitis compared in the treatment of alopecia totalis and universalis. A comparison of the value of topical diphencyprone and tretinoin gel

Diphencyprone is a potent topical sensitizer, but is non-mutagenic in the Ames test (unlike dinitroclorobenzene) and remains relatively stable in solution (unlike squaric acid dibutyl ester). Seventeen patients with total loss of scalp hair (eight alopecia totalis, nine alopecia universalis) were treated by maintaining on one side of the scalp an allergic contact dermatitis induced by 2,3 diphenylcyclopropenone-I ('diphencyprone'), and on the other side an irritant contact dermatitis using tretinoin gel (Retin A). After 20 weeks, treatment with tretinoin was stopped and diphencyprone was applied bilaterally for a further 10 weeks. Satisfactory regrowth of terminal hair on the scalp was achieved in only one patient. Eyebrow, eyelash and beard regrowth was achieved in one individual whilst in another, moderate, but not cosmetically satisfactory, scalp regrowth took place. In no patient did regrowth take place at tretinoin treated sites until after diphencyprone was substituted.     

Erosive pustular dermatosis of the scalp successfully treated with oral prednisone and topical tacrolimus

Erosive pustular dermatosis of the scalp is a rare inflammatory disorder of the scalp, affecting elderly patients after local trauma and leading to scarring or cicatricial alopecia. Case Report: An elderly female patient complained of painful pustules on the parietal region bilaterally with progressive enlargement and ulceration. A biopsy suggested erosive pustular dermatosis of the scalp and the patient was treated with prednisone 40 mg/day and 0.1% topical tacrolimus. After 10 weeks complete closure of the eroded areas was observed and a stable scarring alopecia developed.     

Ulcerated infantile hemangioma treated with imiquimod

A 5-month-old boy was observed in our department presenting with an ulcerated infantile hemangioma on the right buttock. This lesion appeared during the first week of life and had been growing progressively, showing ulceration for 3 weeks. We started treatment with corticosteroids, first with the association of betametasone and fusidic acid topically, and then systemically. After 6 weeks of oral treatment as there was no significant improvement, corticosteroid therapy was slowly tapered and local application of imiquimod 5 percent cream, on alternate days, was started. After 12 weeks of therapy with imiquimod there was complete resolution of the ulceration. There were no side effects.     

Dramatic effect of hydroxychloroquine on lupus alopecia

Lupus alopecia is usually difficult to treat. We report a case of a 40‐year‐old woman with Sjögren's syndrome and atopic dermatitis who presented with discoid lupus erythematosus on the forearms and lupus erythematosus profundus with alopecia involving the scalp. A biopsy specimen taken from the discoid lupus erythematosus lesion on the forearm further exhibited a xanthomatous reaction, which however was not detected in another specimen from the lupus erythematosus profundus on the scalp. Treatment with oral hydroxychloroquine showed dramatic effects and complete hair regrowth was obtained 3 months later.     

The PUVA-turban as a new option of applying a dilute psoralen solution selectively to the scalp of patients with alopecia areata

BACKGROUND: Alopecia areata is a burden for many patients and often resistant, even to extensive therapy. Orally administered PUVA therapy has been shown among numerous systemic and topical treatment modalities to be a therapeutic alternative. However, the clinical use of oral PUVA is often limited by systemic side effects. Bath-PUVA therapy offers an alternative solution because of the negligible systemic absorption of psoralen with this technique. Through use of a "PUVA-turban" it is now possible to administer a dilute bathwater solution containing 8-methoxypsoralen (8-MOP) to the scalp. OBJECTIVE: The purpose of this study was to determine whether PUVA turban therapy is effective in treating alopecia areata in different clinical stages. METHODS: We treated 9 patients with severe, rapidly progressing, treatment-resistant alopecia areata with PUVA-turban treatment as a modification of bath-PUVA therapy. At each treatment session a cotton towel was soaked with a 0.0001% 8-MOP solution (1 mg/L) at 37 degrees C, wrung gently to remove excess water, and wrapped around the patient's head in a turban fashion for 20 minutes. This was directly followed by UVA radiation. Treatment sessions were initially performed 3 to 4 times per week. RESULTS: The cumulative UVA doses given over treatment periods of up to 24 weeks were 60.9 to 178.2 J/cm(2), with single doses ranging from 0.3 to 8.0 J/cm(2). After up to 10 weeks of treatment, hair regrowth could be noticed in 6 of 9 patients. Two patients did not respond to the treatment, and one patient showed only vellus hair regrowth. CONCLUSION: PUVA-turban therapy can be considered a useful method of administering a dilute psoralen solution selectively to the scalp of patients. It has been shown to be a well-tolerated and, in some patients, efficient therapeutic alternative in the treatment of alopecia areata.     

Syphilitic alopecia

A case of alopecia as the only symptom of secondary syphilis in a 32-year old Indian man is described. The man presented with patchy hair loss on the scalp, eyebrows, chest and legs, and generalized nontender lymphadenopathy. Laboratory tests were positive for RPR (rapid plasma reagin test) at 1:64, FTA Ab (fluorescent treponema antibody absorption test). He had a history of heterosexual contact 9 months previously. He was treated with procaine penicillin 600,000 units im daily for 10 days, and hair growth resumed within 6 weeks. Hair loss described as "moth eaten alopecia" is common in secondary syphilis. Other patterns of hair loss related to treponema infection include diffuse, extensive alopecia as a penicillin reaction, and peripheral scalp[ alopecia in infants with congenital syphilis.     

Melanocyte-associated T cell epitopes can function as autoantigens for transfer of alopecia areata to human scalp explants on Prkdc(scid) mice

Alopecia areata is a tissue restricted autoimmune condition affecting the hair follicle, resulting in hair loss. The goal of this study was to test the hypothesis that the autoantigen of alopecia areata is melanocyte associated. Potential autoantigens were tested in the human scalp explant/Prkd(scid) CB-17 mouse transfer system. Scalp T cells from lesional (bald) alopecia areata scalp were cultured with antigen-presenting cells, and antigen, along with interleukin-2. The T cells were then injected into autologous lesional scalp grafts on SCID mice, and hair regrowth was measured. Hair follicle homogenate was used as an autoantigen control. T cells cultured with melanoma homogenate induced statistically significant reduction in hair growth (p <0.01 by ANOVA). HLA-A2-restricted melanocyte peptide epitopes were then tested with lesional scalp T cells from HLA-A2-positive alopecia areata patients. Melanocyte-peptide-activated T cells significantly reduced the number of hairs regrowing in two experiments with six patients (p <0.001 by ANOVA). Injected scalp grafts showed histologic and immunochemical changes of alopecia areata. The most consistent peptide autoantigens were the Gp100-derived G9-209 and G9-280 peptides, as well as MART-1 (27-35). Melanocyte peptide epitopes can function as autoantigens for alopecia areata. Multiple peptides were recognized, suggesting epitope spreading.     

Alopecia Areata Associated with Myasthenia Gravis and Thymoma: A Case of Alopecia with Marked Improvement Following Thymectomy and High Level Prednisolone Administration

A 57-year-old Japanese woman who suffered from alopecia areata associated with myasthenia gravis (MG) and thymoma responded well to thymectomy and high doses of glucocorticosteroid administration. Several treatments for alopecia areata including administration of systemic prednisolone were attempted, but loss of hair on the scalp progressed. After thymectomy and high level glucocorticosteroid administration for MG, the lesions on the scalp improved within four weeks. Consequently, we suggest that this thymectomy and high level glucocorticosteroid administration assisted in improving the immune dysfunction causing the alopecic lesions in this patient.     

Alopecia areata is a T-lymphocyte mediated autoimmune disease: lesional human T-lymphocytes transfer alopecia areata to human skin grafts on SCID mice

Much evidence suggests that alopecia areata is a tissue restricted autoimmune disease. Alopecia areata responds to immunosuppressive agents, and is associated with other tissue restricted autoimmune diseases, including autoimmune thyroiditis and vitiligo. Furthermore, hair regrows when involved scalp is transplanted to nude mice. This study was undertaken to determine whether alopecia areata is mediated by T lymphocytes. Involved scalp from alopecia areata patients was grafted onto SCID mice. Additional biopsies from lesional scalp of the same patients were used to isolate T lymphocytes. These T lymphocytes were cultured with hair follicle homogenate, as well as autologous antigen presenting cells. The T lymphocytes were then injected into autologous scalp grafts on the SCID mice, which had regrown hair. Injection of scalp T lymphocytes resulted in hair loss. Hair loss was associated with the histologic and immunochemical changes of alopecia areata, including perifollicular infiltrates of T cells, along with HLA-DR and ICAM-1 expression by the follicular epithelium. Scalp T lymphocytes that had not been cultured with hair follicle homogenate did not have this effect. Preliminary data suggests hair loss requires a collaboration between CD8+ and CD4+T cells. These studies have demonstrated that alopecia areata can be induced by the transfer of T cells that recognize a hair follicle autoantigen.     

Alopecia areata associated with regression of cutaneous melanoma

A 41-year-old white female with a past medical history of hypothyroidism and alopecia universalis presented on January 24, 2002 with a recently changing mole. She indicated changes in size and color of the superior aspect of a mole that had been present for more than 8 years. She had approximately 20 lifetime peeling sunburns due to being a lifeguard. No family or previous personal history of skin cancers, including melanoma or atypical nevi, was reported. Her history of alopecia universalis began 12 years previously and has partially resolved with remaining patchy alopecia of the scalp and eyebrows. On diagnosis of alopecia universalis, she was initially treated with oral prednisone for 1 year and topical minoxidil for 3 months. Currently, she is not being treated for this condition. She denied other previous skin conditions. She had a surgical history of tonsillectomy at the age of 7 years. Her current medication includes levothyroxine (0.015 microg) for hypothyroidism diagnosed 12 years previously. She reported no known drug allergies. During the initial physical examination, she presented with phototype II skin with two adjacent pigmented lesions on her left foot within a 1.3 cm square. The first lesion on the left posterior distal heel was an irregular, brown-black, 0.5 x 0.6 cm macule. The second lesion, on the left posterior proximal heel, was an irregular, brown, speckled, 0.3 x 0.4 cm macule (Fig. 1). The patient had ophiasis of the scalp and total alopecia of the bilateral eyebrows. In keeping with the patient's wishes, alopecia lesions were not biopsied and clinical photographs of the alopecia are not included in this article. Two 3 mm punch biopsies were performed within each lesion. The left posterior proximal lesion showed malignant melanoma, with a Breslow depth of 0.4 mm, anatomic level II, marked lymphocytic response and partial regression (Fig. 2). The left posterior distal lesion showed malignant melanoma in situ, arising in a lentiginous compound nevus, with architectural disorder and cytological atypia. These two lesions were concluded to be one lesion with clinical regression. She underwent local excision with 1-cm margins and sentinel lymph node biopsy owing to the presence of regression, which showed no evidence of metastatic melanoma. Lactate dehydrogenase and chest X-ray were within normal limits. The alopecia areas were not biopsied previously or at that time.