Background The relationship between en coup de sabre morphea and Parry-Romberg syndrome is unclear, and not much is known regarding their demographic and clinical characteristics or the efficacy of the treatments that are used. Objective The purpose of this study was to describe demographic data, clinical features, and medications used in a large cohort of patients with en coup de sabre morphea and Parry-Romberg syndrome. Methods A retrospective review of patients diagnosed with en coup de sabre morphea or Parry-Romberg syndrome at the Mayo Clinic from 1984 to 2004 was conducted. Demographic data, examination findings, and treatments were noted. Results We identified 54 patients who met criteria for inclusion in the study. Twenty-six patients (48%) had en coup de sabre morphea, 13 (24%) had Parry-Romberg syndrome, and 15 (28%) had both. Disease was present bilaterally in 7.4% of patients. Thirteen percent of all patients in the study group had seizures. Of patients who received treatment, most were treated with antimalarial agents (57.1%) or methotrexate (28.6%). Limitations The study design was that of a retrospective review at a tertiary care center with referral bias. There were also limitations present because of the inherent nature of the diseases studied. Conclusion En coup de sabre morphea and Parry-Romberg syndrome frequently coexist and are likely both variants of morphea. Bilateral disease is more common than previously reported. The efficacy of antimalarials and methotrexate in the treatment of these diseases remains unclear.

En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients

As we move into the 21st century, cutaneous tuberculosis has re-emerged in areas with a high incidence of HIV infection and multi-drug resistant pulmonary tuberculosis. Mycobacterium tuberculosis, Mycobacterium bovis, and the BCG vaccine cause tuberculosis involving the skin. True cutaneous tuberculosis lesions can be acquired either exogenously or endogenously, show a wide spectrum of morphology and M. tuberculosis can be diagnosed by acid-fast bacilli (AFB) stains, culture or polymerase chain reaction (PCR). These lesions include tuberculous chancre, tuberculosis verrucosa cutis, lupus vulgaris, scrofuloderma, orificial tuberculosis, miliary tuberculosis, metastatic tuberculosis abscess and most cases of papulonecrotic tuberculid. The tuberculids, like cutaneous tuberculosis, show a wide spectrum of morphology but M. tuberculosis is not identified by AFB stains, culture or PCR. These lesions include lichen scrofulosorum, nodular tuberculid, most cases of nodular granulomatous phlebitis, most cases of erythema induratum of Bazin and some cases of papulonecrotic tuberculid. Diagnosis of cutaneous tuberculosis is challenging and requires the correlation of clinical findings with diagnostic testing; in addition to traditional AFB smears and cultures, there has been increased utilization of PCR because of its rapidity, sensitivity and specificity. Since most cases of cutaneous tuberculosis are a manifestation of systemic involvement, and the bacillary load in cutaneous tuberculosis is usually less than in pulmonary tuberculosis, treatment regimens are similar to that of tuberculosis in general. In the immunocompromised, such as an HIV infected patient with disseminated miliary tuberculosis, rapid diagnosis and prompt initiation of treatment are paramount. Unfortunately, despite even the most aggressive efforts, the prognosis in these individuals is poor when multi-drug resistant mycobacterium are present. An increased awareness of the re-emergence of cutaneous tuberculosis will allow for the proper diagnosis and management of this increasingly common skin disorder.

Cutaneous tuberculosis: diagnosis and treatment.

Clinical manifestations of tuberculosis in children.

Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, muscles, and sometimes extending to the osteocartilaginous structures. It has been associated with various systemic manifestations, particularly neurologic, ophthalmologic and maxillofacial. In this article, we review Parry-Romberg syndrome with its associated findings (neurologic, ophthalmologic, cardiac, rheumatologic, endocrinologic, infectious, orthodontic and maxillofacial, and autoimmune), underlying cause, differential diagnoses (en coup de sabre, scleroderma, and Rasmussen encephalitis), and therapeutic options.

https://www.jaad.org/article/S0190-9622(12)00104-1/pdf

A review of Parry-Romberg syndrome

Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

http://www.scielo.br/pdf/abd/v90n1/0365-0596-abd-90-01-0062.pdf

Localized scleroderma: clinical spectrum and therapeutic update

Aims We undertook this study to analyse the pattern of childhood cutaneous tuberculosis prevailing in northern India over the past 25 years and to highlight differences from and similarities to adult tuberculosis.



Materials and methods  Clinical records of children with cutaneous tuberculosis who attended the Nehru Hospital attached to the Postgraduate Institute of Medical Education and Research, Chandigarh, India, between January 1975 to December 1999 were analysed.



Results  Four hundred and two patients with cutaneous tuberculosis were examined during the 25-year period of observation. These patients formed 0.1% of the total dermatology outpatients. Seventy-five (18.7%) of these 402 cases were children (≤ 16 years). There were 32 (42.7%) boys and 43 (57.3%) girls with a boy : girl ratio of 0.74 : 1.



The majority of the children, 41 (54.7%) were in the 10–14 years age group. There was no significant boy or girl preponderance in any group other than in scrofuloderma (SFD), where girls significantly outnumbered boys at all ages. Of the various patterns of cutaneous tuberculosis seen, 40 (53.3%) had SFD, 30 (40.0%) had lupus vulgaris (LV), 3 (4.0%) had tuberculosis verrucosa cutis (TVC), 1 (1.3%) child each had tuberculids and tubercular gumma. No child had a tubercular chancre or acute miliary cutaneous tuberculosis. The neck was the commonest site afflicted with SFD and the face was the commonest site affected with LV. No child had generalized lymphadenopathy.



Eighteen (60.0%) of the 30 children with LV had regional lymphadenopathy of which 15 (83.3%) had localized disease and 3 (16.7%) had disseminated disease. Of the 16 children with systemic organ involvement, 12 (75.0%) had regional lymphadenopathy.



Of the 62 children in whom the data regarding vaccination status was available, 31 (50.0%) had been vaccinated and 31 (50.0%) had not. Among the vaccinated group no child had disseminated disease. Three (9.7%) children in the nonvaccinated group had disseminated disease.



Information regarding Mantoux reactivity was available in 71 (94.7%) children, 61 (86.0%) with localized disease and 10 (14.1%) with disseminated disease. Of the 61 children with localized disease, 56 (91.8%) were Mantoux positive and of the 10 children with disseminated disease, only 5 (50.0%) were Mantoux positive (> 10 mm). Histopathologic reports were available for evaluation in all 75 children. Out of 30 cases of LV, 24 (80%) showed classical tuberculous histology. In contrast, out of 40 cases with SFD, only 19 (47.5%) showed classical histology. Classical tuberculous histology was noted in all 3 (100%) cases of TVC and 1 (100%) case each with tuberculids and gumma. Tubercle bacilli could be demonstrated in 4/30 (13.3%) cases with LV and 9/40 (22.5%) cases with SFD.



Systemic involvement was seen in 16 (21.3%) children, of whom 3 (18.8%) had LV and 13 (81.3%) SFD. The lungs were the most common organs involved in 8 (50.0%) children followed by bone(s) in 4 (25.0%), abdomen in 2 (12.5%), and both lung and bone in 1 (6.3%) child.



In contrast to adults, girls outnumbered boys in the childhood series; SFD was a common form of presentation in contrast to LV and TVC, tuberculous gumma and tuberculids were noted less often. In both children and adults, Mantoux reactivity did not correlate with the extent of the disease; patients with disseminated disease were found to be less often vaccinated with BCG and regional lymphadenopathy was noted more often in patients with disseminated disease.



Conclusions  In the whole spectrum of cutaneous tuberculosis, there is a proportion of patients with dissemination (systemic involvement) who are of great epidemiological significance as they require a change in the standard therapeutic regimens recommended for cutaneous tuberculosis.

Childhood cutaneous tuberculosis: a study over 25 years from northern India.

Linear scleroderma involving the frontal or frontoparietal region of the scalp (with or without associated facial hemiatrophy) is called "en coup de sabre" (like the stroke of a sabre). We report a case which to the best of our knowledge is only the fifth instance of this subset of localized morphea occurring in a bilateral distribution. We believe that the rarity of this presentation warrants a report.

Bilateral en coup de sabre-a rare entity.

A 40-year-old man with alcoholic liver disease was referred to the dermatology clinic for asymptomatic papulonodular lesions over the face of 15 days’ duration. Cutaneous examination revealed multiple, dusky red to yellow, follicular as well as perifollicular papulonodular lesions, varying in size from 0.5 to 2 cm ( Fig. 1). They were distributed over the forehead, cheeks, eyelids, nose, chin, beard area, retroauricular area, and neck. Careful examination revealed well-defined scaly margins on the back of the pinna ( Fig. 2). KOH examination of a scraping from the neck revealed nonpigmented septate hyphae. Histopathologic examination of the excised nodule revealed epitheloid cell granulomas with neutrophilic microabscesses surrounding the hair follicles. Periodic acid–Schiff stain was noncontributory. Culture of an excised nodule on Sabouraud’s agar showed growth of Trichophyton rubrum. Considering his liver disease, the patient was initially treated with topical terbinafine. Because of a lack of a satisfactory response this was changed to oral terbinafine 250 mg/day. There was marked regression of the lesions by the sixth day. Unfortunately, the patient succumbed to complications relating to his liver disease.




Figure 1. Follicular and perifollicular lesions of Majocchi’s granuloma

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Figure 2. A well-defined scaly margin of tinea on the back of the pina

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Majocchi's granuloma trichophyticum in an immunocompromised patient.

Three hundred leprosy patients were recruited to study the pattern and frequency of nail changes. Nail changes, like longitudinal ridging in finger nails, transverse striations involving both finger and toe nails etc. which occurred with similar frequency in the PB and MB patients in comparison with the control group, were excluded from the analysis. Out of a total number of 150 PB patients, 84 (56%) showed nail changes. Fifty-eight (38.6%) patients showed changes in the finger nails, with an average of 3.2 involved nails per patient. Fifty-three (35.3%) patients showed changes in the toe nails, with an average of 3.0 nails per patient. The most common change observed was longitudinal melanonychia (32.4%) in the finger nails and longitudinal ridging (46.3%) in the toe nails.In comparison, 131/150 (87.3%) MB patients showed nail changes. Finger nail changes were seen in 102 (68%) patients with an average of 5.5 nails affected per patient. Changes in toe nails were seen in 116 (77.3%) patients, with an average of 6.0 nails involved per patient. The most common nail change observed was longitudinal melanonychia in 89/523, (17%) of the total involved finger nails and subungual hyperkeratosis in 164/702, (23.4%) of the total toe nails involvement. Out of a total of 32 colony patients, 31 (96.9%) showed nail changes both in finger and toe nails with an average of 7.9 and 8.4 affected nails per patient, respectively. The most common nail change observed was rudimentary nail(s) on fingers (29%) and toes (21.1%). Among MB patients, a significantly higher number had finger nail involvement in LL group. The frequency of nail involvement for both fingers and toes was significantly greater in LL as compared to BL group of patients. The frequency of nail involvement was significantly more in patients having disease for more than 5 years and in those having trophic changes secondary to loss of sensations and impaired circulation.

Ranju Rai

Papers

Childhood cutaneous tuberculosis: a study over 25 years from northern India.

Bilateral en coup de sabre-a rare entity.

Majocchi's granuloma trichophyticum in an immunocompromised patient.

Nail involvement in leprosy: a study of 300 patients.

Low-dose low-molecular-weight heparin in lichen planus