LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY. / HISTIOCITOSE DE CÉLULAS DE LANGERHANS AUTOLIMITADA E DE INÍCIO TARDIO: RELATO DE UMA ENTIDADE RARÍSSIMA.

OBJECTIVE: To report a case of late-onset self-healing Langerhans cell histiocytosis. CASE DESCRIPTION: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later. COMMENTS: Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy.

OBJETIVO: Relatar um caso de histiocitose de células de Langerhans autolimitada e de início tardio. DESCRIÇÃO DO CASO: Paciente com 4 meses e meio de idade do sexo feminino, apresentando uma erupção cutânea eritematosa purpúrea, foi submetida a uma biópsia de pele, sendo diagnosticada com histiocitose de células de Langerhans cutânea isolada. As lesões regrediram em poucos meses e ela foi diagnosticada, retrospectivamente, com histiocitose de células de Langerhans autolimitada e de início tardio, após não apresentar nenhum envolvimento cutâneo ou sistêmico durante um seguimento de quatro anos. COMENTÁRIOS: A histiocitose de células de Langerhans autolimitada caracteriza-se pela proliferação clonal das células de Langerhans e apresenta-se com lesões cutâneas, sendo uma variante autolimitada rara de histiocitose. A doença só pode ser diagnosticada de forma retrospectiva, após o paciente não apresentar nenhum envolvimento sistêmico durante vários anos. Embora existam casos de manifestações ao nascimento ou durante o período neonatal, apenas alguns casos de histiocitose de células de Langerhans de idade tardia foram relatados. Lesões purpúreas que aparecem após o período neonatal podem sugerir histiocitose de células de Langerhans autolimitada e de início tardio. Uma vez confirmado o diagnóstico por biópsia cutânea, tais pacientes devem ser acompanhados regularmente, pois pode haver comprometimento sistêmico.

HISTIOCITOSE DE CÉLULAS DE LANGERHANS AUTOLIMITADA E DE INÍCIO TARDIO: RELATO DE UMA ENTIDADE RARÍSSIMA / LATE-ONSET SELF-HEALING LANGERHANS CELL HISTIOCYTOSIS: REPORT OF A VERY RARE ENTITY

RESUMO Objetivo: Relatar um caso de histiocitose de células de Langerhans autolimitada e de início tardio. Descrição do caso: Paciente com 4 meses e meio de idade do sexo feminino, apresentando uma erupção cutânea eritematosa purpúrea, foi submetida a uma biópsia de pele, sendo diagnosticada com histiocitose de células de Langerhans cutânea isolada. As lesões regrediram em poucos meses e ela foi diagnosticada, retrospectivamente, com histiocitose de células de Langerhans autolimitada e de início tardio, após não apresentar nenhum envolvimento cutâneo ou sistêmico durante um seguimento de quatro anos. Comentários: A histiocitose de células de Langerhans autolimitada caracteriza-se pela proliferação clonal das células de Langerhans e apresenta-se com lesões cutâneas, sendo uma variante autolimitada rara de histiocitose. A doença só pode ser diagnosticada de forma retrospectiva, após o paciente não apresentar nenhum envolvimento sistêmico durante vários anos. Embora existam casos de manifestações ao nascimento ou durante o período neonatal, apenas alguns casos de histiocitose de células de Langerhans de idade tardia foram relatados. Lesões purpúreas que aparecem após o período neonatal podem sugerir histiocitose de células de Langerhans autolimitada e de início tardio. Uma vez confirmado o diagnóstico por biópsia cutânea, tais pacientes devem ser acompanhados regularmente, pois pode haver comprometimento sistêmico.

ABSTRACT Objective: To report a case of late-onset self-healing Langerhans cell histiocytosis. Case description: A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later. Comments: Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy.

The Impact of Inflammatory and Infectious Diseases of Vulvar on Quality of Life.

OBJECTIVES: This study aimed to evaluate the effects of vulvar dermatoses (VD) and vulvar infections (VE) on anxiety, depression scores, general, and dermatological quality of life (QOL) of women. METHODS: We conducted a cross-sectional survey on patients diagnosed with VD and VE. All patients were assessed using a sociodemographic form, Skindex-29 scale, World Health Organization QOL (WHOQOL) BREF scale, Hamilton anxiety rating scale (HAM-A), Hamilton depression rating scale (HAM-D). RESULTS: HAM-A score was significantly higher in the VD group than in the control group. Based on age groups, all subscales of Skindex-29 were found to be higher in women > 50 years. For women with severe pruritus, the symptom subscale was higher in patients with skin involvement and the function and emotion subscales were higher in patients with and emotion subscales were higher in patients with mucosal involvement. CONCLUSIONS: We found that VD was particularly significantly associated with decreased QOL.

Total congenital anonychia.

Total or partial absence of nails at birth is rare. Anonychia can be inherited as an autosomal dominant or recessive trait. An otherwise healthy 4-year-old boy whose parents were first-degree relatives was diagnosed with total congenital anonychia.

Vitamin D status in patients with Behcet's Disease.

OBJECTIVES: This study investigated the serum 25-hydroxyvitamin D levels of patients with Behcet's Disease. DESIGN AND METHODS: Thirty-two patients with Behcet's Disease and 31 matched healthy controls were enrolled in this study. The erythrocyte sedimentation rate (ESR) and the levels of C-reactive protein (CRP), serum 25-hydroxyvitamin D, calcium (Ca), phosphate (P), and total alkaline phosphatase (ALP) were measured in both groups. RESULTS: There were no significant differences between the two groups regarding demographic data. The serum 25-hydroxyvitamin D levels of patients and controls were 13.76 (range: 4.00-35.79) and 18.97 (range: 12.05-36.94) ng/ml, respectively. In patients with Behcet's Disease, 25-hydroxyvitamin D values were significantly lower than those of the healthy controls (p<0.001). Serum Ca, P, and ALP levels were similar in both groups. Serum ESR and CRP levels were significantly higher in patients than controls (p<0.05). There was no correlation between 25-hydroxyvitamin D levels and age, body mass index (BMI), disease duration, ESR, or CRP levels. Multivariate regression analysis parameters showed that smoking, alcohol intake, and use of colchicine were the main predictors of 25-hydroxyvitamin D levels. Of the parameters studied, the largest impact was due to colchicine therapy (p<0.001). We did not find a significant relationship between the use of corticosteroids and 25-hydroxyvitamin D levels. CONCLUSION: Our results suggest that serum 25-hydroxyvitamin D levels are decreased in patients with Behcet's Disease. Smoking, alcohol intake, and use of colchicine appear to affect vitamin D levels.

Antistreptolysin O Levels in Patients with Behcet's Disease.

OBJECTIVE: Behcet's disease is a multisystem inflammatory disorder, and its etiology has not been defined clearly yet. In this study, we aimed to investigate the antistreptolysin O (ASO) levels of patients with Behcet's disease. MATERIALS AND METHODS: Thirty patients with Behcet's disease and 30 healthy controls were enrolled in this study. We measured erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP), and ASO levels in both groups. RESULTS: There was no statistically significant difference between the two groups with respect to demographic data (p>0.05). The ASO levels of the patients and the controls were 288.4±145.7 and 170.6±142.4 ng/ml, respectively. In the patients with Behcet's disease, ASO (p<0.01) and ESR (p<0.05) values were significantly higher than in the healthy controls. There was no other significant difference in serum CRP levels between the patients and the controls. We could not find any correlation among ASO, CRP, and ESR values. CONCLUSION: Our results suggest that serum ASO levels may increase in patients with Behcet's disease. Further studies are needed in order to define the relationship between ASO levels and inflammation status in Behcet's disease.

Vitamin D status in patients with Behcet's Disease

OBJECTIVES: This study investigated the serum 25-hydroxyvitamin D levels of patients with Behcet's Disease. DESIGN AND METHODS: Thirty-two patients with Behcet's Disease and 31 matched healthy controls were enrolled in this study. The erythrocyte sedimentation rate (ESR) and the levels of C-reactive protein (CRP), serum 25-hydroxyvitamin D, calcium (Ca), phosphate (P), and total alkaline phosphatase (ALP) were measured in both groups. RESULTS: There were no significant differences between the two groups regarding demographic data. The serum 25-hydroxyvitamin D levels of patients and controls were 13.76 (range: 4.00-35.79) and 18.97 (range: 12.05-36.94) ng/ml, respectively. In patients with Behcet's Disease, 25-hydroxyvitamin D values were significantly lower than those of the healthy controls (p<0.001). Serum Ca, P, and ALP levels were similar in both groups. Serum ESR and CRP levels were significantly higher in patients than controls (p<0.05). There was no correlation between 25-hydroxyvitamin D levels and age, body mass index (BMI), disease duration, ESR, or CRP levels. Multivariate regression analysis parameters showed that smoking, alcohol intake, and use of colchicine were the main predictors of 25-hydroxyvitamin D levels. Of the parameters studied, the largest impact was due to colchicine therapy (p<0.001). We did not find a significant relationship between the use of corticosteroids and 25-hydroxyvitamin D levels. CONCLUSION: Our results suggest that serum 25-hydroxyvitamin D levels are decreased in patients with Behcet's Disease. Smoking, alcohol intake, and use of colchicine appear to affect vitamin D levels.

Multiple cutaneous metastases from male breast carcinoma.

Skin involvement as a symptom, or even the presenting sign, of this breast cancer in men is not well known by dermatologists. In fact, a review of the English-language literature reveals few references about this subject. We report a case of breast carcinoma in a 58-year-old man with rapidly progressive cutaneous metastases that did not respond to any treatment schedules.

Rapidly developing giant sized lupus vulgaris on the chest associated with bilateral scrofuloderma on the neck.

Lupus vulgaris and scrofuloderma are the opposite poles of cutaneous tuberculosis. Lupus vulgaris of a giant size and scrofuloderma in the vicinity of this lesion were both present in a 70-year-old female patient. The purified protein derivative of tuberculin (PPD) skin test was strongly positive. In histopathological examination, granulomatous infiltration without caseation necrosis was seen in the dermis. The patient was treated with a four-drug therapy consisting of pyrazinamide (25 mg/kg), isoniazid (5 mg/kg), rifampin (10 mg/kg) and ethambutol (15 mg/kg) daily for 2 months, followed by dual therapy with isoniazid and rifampin for 6 months. Her cutaneous lesions significantly regressed after 4 months, leaving keloid scars.

Chromosome and sister chromatid exchange studies in Behcet's patients.

Behcet's disease is a chronic multisystemic disease of unknown pathogenesis characterized by four major symptoms: oral aphthous ulcers, skin lesions, ocular symptoms and genital ulcerations. The disease is spread throughout the world, but it is most frequent in Turkey, Japan, Korea and China. Although HLA-Bw51 has been found to predominate in Behcet's cases, the genetic etiology has not yet been clarified. In this study, we investigated the chromosomal abnormalities and sister chromatid exchange rates in patients with Behcet's diseases. Thirty-eight patients with Behcet's disease (diagnosed for the first time) and 30 healthy subjects (as controls) were included in this study. Although numerical and structural chromosomal abnormalities were not detected in our patients, we found an increased rate of sister chromatid exchange in patients over the control groups (P < 0.01). On the basis of these results, we discuss the genetic etiology of Behcet's disease.

Androgen receptor levels of oral and genital ulcers and skin pathergy test in patients with Behçet's disease.

BACKGROUND: Hormonal factors have long been proposed to play a role in Behçet's disease (BD). Male sex, systemic onset, HLA-B51 positivity and a younger age of onset in BD are associated with severer disease, and the disease generally runs a milder course in women. Vascular involvement is more common, and the skin pathergy test (SPT) is more strongly positive in men. BD rarely develops before puberty or after the age of 50 years. Clinical manifestations of the disease, with the exception of eye symptoms, tend to improve with time. Therefore, BD may be androgen driven to some degree. OBJECTIVES: We aimed to investigate androgen receptor (AR) levels of oral ulcers (OU), genital ulcers (GU) and SPT areas and compared them with those of adjacent normal-appearing skin/mucosa from patients with BD. METHODS: Thirty-eight patients with BD (16 female, 22 male; mean +/- SD age, 36.45 +/- 10.2 years), diagnosed according to the criteria of the International Study Group for Behçet's Disease, were included in the study with blind histological examination. Biopsies from OU of 10 patients, GU of 11 patients, SPT areas of 17 patients and adjacent (approximately 2 cm distant) normal-appearing skin/mucosa in patients with BD were performed. Nuclear AR levels were studied by an immunohistochemical technique, using monoclonal antibodies. The percentage of positively staining cells was recorded as the AR index (ARI). In addition, the prevalence and the positivity rate of SPT has also been evaluated. RESULTS: ARI values in the lesional and control (non-lesional adjacent) skin/mucosa were found to be 14.5 versus 18% for OU, 28.7 versus 25.5% for GU and 36.3 versus 21.8% (p = 0.068) for SPT areas. The positive SPT areas in male patients showed a higher ARI than those of female patients (43.36 and 23.33%; p = 0.078). The ARI values of SPT areas in male patients but not in female patients were found to be significantly higher as compared with non-lesional skin (21.63%; p = 0.039). The SPT positivity was also more common in male patients compared with female patients (86.4% and 62.5%), although the difference was not significant (p = 0.88). SPT have been found to be more strongly positive among the males (4.63 +/- 3.3) compared with female patients (3.18 +/- 1.9), and the difference was statistically significant (p = 0.022). CONCLUSIONS: Our findings indicate that androgens seem to play a role both in the formation and increased positivity of the SPT areas in male patients with BD.

Treatment of confluent and reticulated papillomatosis with azithromycin.

Confluent and reticulated papillomatosis (CRP) is a relatively rare disorder of unknown origin, mostly affecting young female adults. We here present the case of a 21-year-old male patient with confluent and reticulated papillomatosis. Skin examination revealed brownish, verrucous, hyperkeratotic, 2 to 5 mm papules, which formed confluent patches and plaques with a reticulate network on the interscapular area. The patient was initially treated with ketoconazole cream for two weeks without improvement. The disease can be rather persistent and resistant to topical therapy. Our case showed a satisfactory response to treatment with azithromycin. Although this treatment is known to be effective in some cases, the action mechanism of azithromycin on CRP is not fully understood.

Scrofuloderma after BCG vaccination.

A 7-month-old boy with scrofuloderma following BCG vaccination is presented. He had a 2 cm x 1 cm erythematous lesion with a sinus tract in the right cervical area. In addition, he had ulcerated erythematous lesions 0.5 cm x 0.5 cm in size on the left deltoid area and left forearm where BCG vaccination and a PPD test were performed, respectively. Histopathologic findings were consistent with the clinical diagnosis.