[Linear tuberculoid leprosy along the lines of Blaschko: a very rare presentation]. / Lèpre tuberculoïde de disposition linéaire blaschkoïde: une présentation exceptionnelle.

BACKGROUND: Leprosy continues to be present in certain regions throughout the world, and the dermatologist plays a central role in its diagnosis. Herein we report a case of tuberculoid leprosy that is atypical in terms of its linear presentation which appears to follow the lines of Blaschko. PATIENTS AND METHODS: A patient from Mayotte was referred to the neurological department for suspected tuberculoid leprosy. He was presenting a deficiency of the ulnar nerve together with neuronal hypertrophy and cutaneous involvement. Dermatological examination revealed linear hypo-aesthetic hypopigmented lesions on the arm and forearm. The atypical clinical presentation also suggested to us pigmented mosaicism or post-inflammatory pigmentation. The biopsy showed granulomatous epithelial dermatitis with perinervous involvement. Imaging examinations confirmed the presence of neuronal hypertrophy. A diagnosis of linear tuberculoid leprosy was made. DISCUSSION: This case illustrates the need to bear in mind a diagnosis of leprosy in the event of hypopigmented lesions, even where they are linear and of Blaschkoid appearance. Several potential hypotheses may account for this particular topographical pattern.

A practical approach to enlargement of nerves, plexuses and roots.

Detecting enlargement of accessible nerves is very helpful in assessing patients with peripheral nerve disorders, as only a few types of neuropathy lead to nerve thickening. The three leading causes are leprosy, hereditary motor and sensory neuropathies (types 1 and 3) and chronic inflammatory demyelinating neuropathies. MRI, neurography and ultrasonography allow assessment of clinically inaccessible portions of deep-seated nerves, plexuses and roots. As a result, isolated proximal segment thickenings, as found in chronic inflammatory sensory polyradiculopathy, can now be better evaluated and managed. Similarly, focal nerve enlargements due to infection, inflammation, infiltration and neoplasm are being identified and treated effectively. We present a practical approach to the diagnosis and management of patients with enlarged peripheral nerves, plexuses and roots, including cranial nerves.

Syringomyelia, limb hypertrophy and sympathetic overactivity: a rare association.

A 32-year-old man presented with uniform enlargement of right upper limb for 6 years. Examination revealed painful disorganised elbow joint along with sensory impairment in the affected limb with weakness of small muscles. The patient was given empirical antileprotic therapy from outside without any benefit. Ultrasonography showed pan-hypertrophic nature of local tissues. Although axonal type of sensorimotor neuropathy involving right ulnar and median nerve was detected in nerve conduction velocity study, biopsy of the same failed to confirm any axonal degeneration or evidence of leprosy. Considering the nature of sensory abnormality MRI of cervical spine was done which revealed a syrinx extending from C3 till D2. Tests for vasomotor tone showed positive results on the affected limb. Syringomyelia can rarely give rise to pan-hypertrophy of limb due to sympathetic overactivity, which is infrequently reported in literatures and deficient in logical grounds.

[Histoid leprosy in Morocco: retrospective study of 18 cases]. / Lèpre histoïde au Maroc: etude rétrospective de 18 cas.

INTRODUCTION: Histoid leprosy is a special and rare anatomoclinical form of multibacillary leprosy. Most of the few large series describing this entity In literature have been reported from India. The purpose of this study was to analyze the epidemiological, clinical and therapeutic characteristics of patients with histoid leprosy in Morocco. MATERIAL AND METHODS: This retrospective study was conducted at the National Centre of Leprology (CNL) in Casablanca from January 1991 to December 2006. Only histologially confirmed cases of histoid leprosy were included. Epidemiological, clinical, histological and therapeutic characteristics were compiled from records and analyzed using EPI-Info version 6. RESULTS: Confirmed histoid leprosy accounted for 18 of the new cases of leprosy recorded at CNL recorded during the 16-year study period. There were 13 men and 5 women with a mean age of 35.2 years. Family exposure was noted in 9 patients (50%) including two who had more than two relatives with leprosy. Nodules/subcutaneous nodules were the most common morphological pattern (88.8%). Neurological involvement was found in 10 patients. Seven patients had grade 1 deformities. Slit skin smears from histoid lesions revealed abundant bacilli with a high bacteriological index ranging from 3 + to 5 + according to the Ridley index. All patients received antibiotic treatment according to the Moroccan protocol. Outcome was favorable in all patients. Six patients (33.3%) developed erythema nodosum leprosum during the course of disease. With a average follow-up of 9 years, no recurrence has been observed. DISCUSSION: Because of its multibacillary character and despite its rarity, histoid leprosy poses a challenge to the leprosy eradication in Morocco. Like other forms of multibacillary leprosy, histoid leprosy requires early detection and prompt multidrug therapy. These requirments should be made a priority in the national program against leprosy.

[Pathology of the heart and blood vessels in Hansen's disease].

There are few reports on the cardiovascular changes accompanying Hansen's disease, and many aspects remain unclear. We performed macroscopic and histologic examinations on the cardiovascular system in autopsy (47 cases of L type, 2 cases of B type and 14 cases of T type) and biopsy cases of Hansen's disease. The changes in small blood vessels and capillaries were also observed using electron microscopy. Cardiac changes such as atrophy, hypertrophy, fibrosis and endocarditis, epicarditis and coronary sclerosis, were observed, and severe fibrosis was significantly more common in the T type cases. The heart weight tended to decrease with age in the T type cases, but such tendency was not observed in the L type cases, with some cases showing an increased heart weight. Marked atrophy, fibrosis, swelling and hyperplasia were observed in the peripheral nerves around the arteries in the epicardial adipose tissue and arterioles in the myocardium. These changes were caused by ageing of the vessels feeding the peripheral nerves, together with changes due to Hansen's disease, such as circulatory disturbance in vessels feeding the peripheral nerves, augmentation of vascular permeability and ischemic changes due to over-stratification of the basement membrane. Because these peripheral nerves were distributed around the arteries in the epicardial adipose tissue and they were probably vasomotor nerves. These results suggest that paralytic arterial changes may promote the ischemic cardiac disease or fibrosis.

GM-CSF activates regenerative epidermal growth and stimulates keratinocyte proliferation in human skin in vivo.

Granulocyte/macrophage-colony-stimulating factor (GM-CSF), an immunomodulator of hematopoietic cells, has also been shown to stimulate human keratinocyte proliferation in vitro and speed healing of wounds in the skin of lepromatous leprosy patients. In this study we have examined the in vivo effects of recombinant human GM-CSF on epidermal keratinocyte proliferation and on expression of proteins marking regenerative epidermal growth. Skin biopsies from GM-CSF injected cutaneous sites were obtained between 1 and 6 d following administration of 7.5 or 15 micrograms of the growth factor. Activation of keratinocyte proliferation, quantified as the expression of the Ki67+ nuclear antigen, was noted 1 d following GM-CSF administration. A regenerative epidermal phenotype, demonstrated by immunohistochemical staining of cellular proteins involucrin, filaggrin, and keratin 16, was similarly noted as early as 1 d following GM-CSF injection. This phenotype persisted as late as 6 d post-injection. These results suggest that GM-CSF injection into human skin induces keratinocyte proliferation as well as regenerative differentiation of the epidermis. To date no other cytokine has been shown to be mitogenic for human keratinocytes both in vivo and in vitro or to alter keratinocyte differentiation along the "alternate" or regenerative pathway.

[Treatment of leprotic neuritis. Exclusive medical treatment or combined with decompression]. / Evolution du traitement des nevrites hanséniennes. Traitement médical exclusif ou associé à la décompression.

The authors consider the evolution of the treatment of leprotic neuritis based on homogenous series of numerous observations (a minimum of 250-300). These observations are well registered and documented with some results dimensioned by the classical A. L. E. R. T. tests carried out at intervals of time and verified by comparison between series only medical treatment and series of medico-surgical treatment. Considering some remote results and some comparative series, the indications of the exclusive medical treatment have been well defined, and the ones of the surgical decompression have been reduced. The early case finding of a leprotic neuritis by the mean of easy methods as well as its monitoring during its early specific treatment is essential. By this way we get an important proportion of functional results: satisfactory (50 p.c.) or interesting (20 p.c.). In the case of painful hypertrophia, the complementary decompression secure not only the functional recovery of mortricity, but also the epicritic sensibility.

Asymptomatic nerve hypertrophy in lepromatous leprosy: a clinical, electrophysiological and morphological study.

In order to learn more about early nerve lesions observed in leprosy, we performed a clinical, electrophysiological and morphological study in seven patients with untreated lepromatous leprosy, palpably enlarged radial cutaneous nerve and preserved sensation in the corresponding territory. The conduction velocity of the cutaneous radial nerve, which was decreased in all patients, did not significantly differ from that of a group of patients with lepromatous leprosy, hypertrophy of the radial cutaneous nerve and sensory loss. In contrast, the sensory action potential was significantly lower in patients with sensory loss, which demonstrates that axon loss is more important than demyelination in producing sensory loss. In all patients nerve enlargement was due to thickening of the epineurium and of the perineurium subsequent to inflammatory infiltrates and proliferation of fibroblasts and perineurial cells. In several fascicles, the inflammatory infiltrates and the infected cells infiltrated endoneurial connective tissue septa and blood vessels. Mycobacteria leprae were abundant in perineurial cells, fibroblasts, macrophages, Schwann cells and endothelial cells, and lymphocytic vasculitis present in all cases. The average density of myelinated fibres was 2600 SD 880 fibres/mm2 (control: 7700 fibres/mm2), with marked differences between individual fascicles, versus 420 fibres/mm2 in patients with nerve hypertrophy and sensory loss (range 0-2080 fibres/mm2). Single fibre preparations showed that segmental demyelination predominated in two patients, axonal degeneration in one, while inflammatory infiltrates and proliferation of connective tissue adhering to individual fibres were prominent in the others.(ABSTRACT TRUNCATED AT 250 WORDS)