Manual of Histoid Leprosy Govind Srivastava, Virendra N Sehgal
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IntroductionChapter 1

Histoid Leprosy is a well-recognized clinical entity, characterized by cutaneous and/or subcutaneous nodules and plaques present over an apparently normal skin with unique histopathology and characteristic bacterial morphology.1, 2 It usually manifests itself in patients on diaminodiphenyl sulfone (DDS) for a long time, reflecting initial improvement followed by a relapse.3–9 Irregular10–12 and inadequate13 therapy may further compound its occurrence. Some workers5, 10,14–17 believe that it is due to the development of drug resistance to DDS, while others9, 10,14 postulate that mutant organisms, that emerge from a predominantly sulfone susceptible bacterial population, may be responsible. Treatment with DDS creates a selective environment by killing or at least inhibiting, multiplication of drug sensitive organisms, thus allowing the growth of the drug resistant, ‘histoid’ bacilli.6, 10, 12, 18–24 Its de novo occurrence may make it difficult to diagnose. So, a high degree of suspicion is imperative in such cases.
Apart from its occurrence in lepromatous leprosy, histoid may occasionally be seen in borderline,7, 10, 18, 25–28 and indeterminate leprosy.29
Ever since the description of histoid leprosy by Wade,30, 31 an emphasis was laid on its retrospective histological diagnosis,31 which was subsequently reaffirmed.5, 6, 19 However, in Sehgal and Srivastava's experience,1, 32 it can be diagnosed with certainly, relying on its prospective clinical features, a fact appreciated and duly reiterated by Professor Samuel L Moschella, while reviewing a scintillating report on the entity in the Year Book of Dermatology33 1988.
 
Historical
The term histoid was originally coined in the year 1960 by Wade,30 as a historical concept of bacillary rich leproma 2composed of spindle shaped cells, along with an absence of globus formation, so conspicuous, in the ordinary leproma, and which exhibits, a fibromatoid, tendency in chronic form. Since then many reports,3–6, 10–14, 25, 31, 32, 34 have documented this entity. Although some authors,1, 4, 26 regard it as a distinct clinicopathological entity, yet, others3–6, 10, 12–15, 18, 19, 25 consider it as a variant of lepromatous leprosy. The entity initially was recorded in multibacillary patients, on irregular, and inadequate dapsone (mono) therapy, but later de novo cases were also encountered.1, 32
The ultrastructure of these lesions, was studied, for the first time, by Job et al,34 describing its prime cell type as a histiocytes, behaving both as a macrophage and fibroblast. Later, Ridley and Liu et al,26, 35 also documented its ultra-structure.
Despite sporadic reporting, histoid leprosy was not given a thought, until Sehgal et al36 in the year 1985 documented for the first time the immunological profile of this intriguing and exquisite expression of multibacillary leprosy. In the subsequent years, Sehgal and Srivastava,32 advanced dialogs, stressing prospective clinical diagnosis, in their two authoritative, investigative reports.1, 32 The latter, in particular, brought to light certain important revelations, which led to more lucid understanding of this enigmatic disease.
After these revelations, the research workers across the globe developed interest in this enigmatic, unique expression, the histoid leprosy, of a very common entity of the developing countries, and several reports37–49 started pouring from different parts of the world. A high degree of suspicion brought to book several more cases of histoid leprosy hither to undiagnosed.50–54
Sehgal and Srivastava32, 36, 55 based on these extensive studies on the entity postulated that histoid should occupy a separate position on the continuous leprosy spectrum besides lepromatous leprosy.
 
Definition
Histoid leprosy is an expression of multibacillary leprosy, characterized by the occurrence of nodules and/or plaques 3in the skin or the subcutaneous tissues. Well-demarcated, cutaneous nodule is its cardinal feature. They are present over an apparently normal skin.1, 18 The reactional episodes are seldom recorded in this entity.30, 31 However, they are recognized by their distinct bacteriology and histopathology.31
Cytodiagnosis using fine needle aspiration of such nodules or plaques can be an instant tool for diagnosis of histoid leprosy.37
 
Nomenclature
Histoid is a universally accepted nomenclature, the credit for which goes to Wade.31 Some workers,12 however, prefer the name ‘histoid nodules’ to histoid leprosy, while others use Mycobacterium leprae histiocytoma (cutis)2 or leprous histiocytoma.56 Prior to Wade's description,30, 31 the entity was relatively unknown, although, Mitsuda57 had commented on a similar histological picture,5 earlier.
Jonquire27 called such nodules as young lepromas or pseudofibromatous lepromas before Wade31 assigned the name ‘histoid lepromatous leprosy’. Such cases were not so uncommon in the pre-sulfone era, and had been designated as ‘florida lepromatous leprosy’, ‘advanced nodular lepromatous leprosy’, and formed a part of the ever-obliging group of nodular lepromatous leprosy.18
An interesting record of a 70 years old Italian man with a nodular skin eruption which was diagnosed after much of futile academic efforts, prompted the authors to label it as ‘pseudotumour leprosy’.58
While coining the term ‘histoid leprosy’, Wade31 had considered, only its histopathological features for making a retrospective diagnosis. However, after an extensive experience of these patients, Sehgal and Srivastava,32 advanced several prospective guidelines for delineating this as a distinct clinical entity. They stressed that it should occupy a separate position besides lepromatous leprosy on the leprosy spectrum.
4References
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