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Multiple neurofibromas and plexiform neurofibromas with overlying well to ill-hypopigmented macules.
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A case of Neurofibromatosis I (NFI) occurring in association with symmetrical peripheral nerve enlargement and multiple hypopigmented macules strikingly limited to the neurofibromas, with normal to minimally reduced sensations, evoking a strong clinical suspicion of co-existent lepromatous leprosy, is being reported. Leprosy was ruled out by microb...
Context in source publication
Context 1
... was no history of seizures or sensory loss. Family history was not significant. On examination, multiple, soft, ill-defined papules, plaques and nodules were present over the face, trunk and extremities with overlying hypopigmentation but no atrophy or sparsening of appendages. The hypopigmentation also extended to adjacent apparently normal skin (Fig. ...
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Leprosy, known since antiquity, is a world infectious disease due to Mycobacterium leprae. The transmission is probably via nasal droplets. The clinical range, from tuberculoid to lepromatous leprosy is a result of variation in the cell-mediated immune response, with a chronic inflammation in skin and peripheral nerves. Diagnosis of leprosy is clin...
Citations
... There are no conflicts of interest. [3] Scalp Two Leukotrichia Ettl A et al., 1996 [6] Face Two Hyperpigmentation and hypertrichosis Khandpur et al. [7] Back Multiple Hypopigmented macules Choudhary SV et al., 2017 [8] Right shoulder to upper three fourth of arm Single Café-au-lait macule Singh et al., 2018 [9] Back One Becker's nevus Schaffer JV et al. [10] Trunk One Hyperpigmentation and hypertrichosis Khachemoune A et al. [11] Thigh Single Café-au-lait macule ...
... Mittal et al. 9 reported a case of NF with gross enlargement of all peripheral nerve trunk simulating leprosy. 9 On the other hand, Khandpur et al. 10 reported a case of NF where an unusual hypopigmentation with symmetrical nerve enlargement raised the strong clinical suspicion of coexistent lepromatous leprosy. But leprosy was ruled out by microbiological, histopathological and electrophysiological studies. ...
Leprosy and neurofibromatosis are different entities. Both diseases present with nerve enlargement and skin lesions but the coexistence is rare. Nodules of neurofibromatosis may be mistaken for that of lepromatous leprosy and vice versa. Their simultaneous occurrence in same person may lead to diagnostic dilemma. We report a case which was at first mistaken as lepromatous leprosy. But proper history and examination aided by slit skin smear and skin biopsy for histopathological examination helped to reach the appropriate diagnosis of borderline tuberculoid leprosy associated with neurofibromatosis.
... In our case, more than six CALM were present on the thigh and back, with regular borders and of varying size (1.5-2.5 cm), further emphasizing that these are manifestation of NF1. 11 The central ossifying fibroma was excluded, as it is radiographically well defined with varying degrees of calcification, but rarely radiopaque. It has a symmetric growth pattern, which is equal in all directions, and occasionally has a soft tissue capsule, unlike FD, which blends with surrounding bone. ...
Neurofibromatosis and fibrous dysplasia show the presence of café-au-lait spots, bone lesions, and endocrinopathies. There has been speculation whether neurofibromatosis and fibrous dysplasia are different manifestations of the same disease or if these conditions are in some way related. We provide a case of whether neurofibromatosis and fibrous dysplasia complicated by hyperparathyroidism and osteoporosis.
LEPROMATOUS LEPROSY WITH COEXISTENT NEUROFIBROMATOSIS TYPE 1 – A CASE REPORT
Mallawaarachchi KV1; Gunasekera CN.2
Neurofibromatosis type 1 (NF 1) and leprosy are two different disease entities as the former is a genodermatosis while the latter is an infectious disease mostly confined to tropical countries. Interestingly, among their many clinical manifestations, both conditions share the common features of involvement of the skin and Schwann cells of the peripheral nerves. The presence of lepromatous leprosy in a patient with NF 1 is relatively rare and may pose certain diagnostic dilemmas. In fact, a pathogenetic relationship between two conditions has been suggested since abundance of perineural tissue containing Schwann cells in NF 1 patients might make them susceptible to leprosy.
In this case report we describe manifestation of lepromatous leprosy in a 31 years old patient with pre existing NF 1. He was referred from a medical ward with painful swelling of both lower limbs for which he was treated unsuccessfully with a course of intravenous antibiotics. Clinical examination revealed multiple cutaneous neurofibromas, a plexiform neurofibroma, more than 6 café au lait macules each more than 15 mm in diameter and axillary freckling suggesting the diagnosis of NF 1. The features for NF 1 were obvious and dominated the clinical picture. However, careful examination revealed that patient’s ear lobes and facial skin were infiltrated with supracilliary madarosis. In addition to neurofibromas which were soft nodules with positive buttonhole sign, there were multiple firm infiltrated nodules without “buttonholing”. Some of them, which were tender, could have been otherwise missed due to the dominant nature of the neurofibromata. He had uniformly thickened symmetrical non tender ulnar and common peronial nerves.
His ear lobe smear was positive for acid fast bacilli with bacterial index of 6+. Biopsy from an infiltrated firm nodule showed a diffuse foamy histiocytic infiltration admixed with lymphocytes of the dermis with a Grenz zone, suggestive of lepromatous leprosy.
1Senior registrar, Dermatology, Colombo North Teaching Hospital, Ragama, Sri Lanka ,2 Consultant Dermatologist, National Hospital of Sri Lanka
PGIM/AS/13/516
The coexistence of leprosy with neurofibromatosis is rare both the diseases present with nerve thickening and skin lesions (patches and nodules). The coexistence of neurofibroma with borderline tuberculoid, lepromatous, histoid, and neuritic leprosy has been reported in the past. We report here a case of borderline lepromatous leprosy coexisting with neurofibromatosis in a 60 year-old male, who presented with neurofibromata and nerve thickening. Histopathology of skin biopsy from the leprosy and neurofibroma nodules confirmed the diagnosis of leprosy and neurofibroma.
