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An Update on Chronic Prurigo. Wallengren, Joanna An update on chronic prurigo. Wallengren, Joanna Published in: Current Medical Litterature: Dermatology 2011 Document Version: Publisher's PDF, also known as Version of record Link to publication Citation for published version (APA): Wallengren, J. (2011). An update on chronic prurigo. In Current Medical Litterature: Dermatology (Vol. 16:4, pp. 89-95). Remedica Publishing. 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LUND UNIVERSITY PO Box 117 221 00 Lund +46 46-222 00 00 89 Leading Article An Update on Chronic Prurigo Joanna Wallengren, MD, PhD University Hospital, Lund, Sweden CML – Dermatology 2011;16(4):89–95. Submit comments or questions for the authors at www.currentmedicalliterature.com The term prurigo, originating from the being more affected than men. The lesions Latin word pruire (to itch), was first coined by are hemispherical, often irregular nodes Ferdinand von Hebra in the mid-19th century with a horny, rhagadiform, or crateriform to characterize intensely itchy papules and depressed surface. They may be as large nodules that occur mainly on the arms and as several centimeters in diameter and are legs. At that time, prurigo was one of the most mainly located on extensor surfaces of the frequent forms of skin disease in Europe, being extremities, although the trunk, face, and closely associated with the stings of parasites even the palms can be affected [1]. New (such as fleas and mites) that commonly nodules can develop over time, and existing afflicted humans. Today, prurigo strophulus – nodules can remain pruritic indefinitely, which is generally provoked by stings of fleas, although some regress spontaneously and mosquitos, ticks, or dog parasites – has come to leave scars. The cardinal symptom is itch. designate an acute form of prurigo. There has been much confusion in The genuine chronic form of prurigo is the literature concerning PN. “Prurigo prurigo nodularis (PN) of Hyde, after the man nodularis” is often used erroneously for who coined the term in 1909 [1]. This review all forms of chronic prurigo. True PN is will deal with this and other forms of chronic an endogenous form of chronic pruigo prurigo secondary to underlying systemic that is often associated with atopy [2]. The disease or external provoking factors. In lesions are large nodules (up to 3 cm in addition, it will focus on the preponderance of diameter) compared with smaller nodules of prurigo in certain ethnic groups. secondary prurigo. The diagnosis of prurigo is a clinical one During the 19th century there was and histopathology confirms what is seen by much discussion among dermatologists the naked eye, including hyperkerathosis, about whether the PN lesion appears acanthosis, and occasionally epidermal first and produces an urge to scratch or necrosis due to picking. whether its appearance is brought about as a consequence of scratching [1]. According PN of Hyde to Hebra, the prurigo papule appears first. PN can occur at any age, although mainly In 1899, Johnston wrote in the Journal in those aged 20–60 years, with women of Cutaneous Diseases that the number of 90 Joanna Wallengren hypertrophic nerve fibers is increased Internal diseases in prurigo lesions [2]. These nerve fibers Itch is a common symptom in patients with show immunoreactivity for sensory chronic renal failure who are receiving neuropeptides, such as calcitonin gene- maintenance hemodialysis. Perforating related peptide, and substance P, which act folliculitis with superimposed PN in as transmitters in both the peripheral and these patients was first described in 1982 in central nervous systems [3]. This finding [6]. Interestingly, in one patient, who had supports the theory of itch being elicited been completely unresponsive to various from the prurigo lesion. treatments, the skin lesions cleared up The central neuronal pathways that are rapidly 1 week after cessation of hemodialysis involved in itch transmission terminate in and renal allograft transplantation [6]. the somatosensory cortex and the motor Acquired reactive perforating collagenosis, areas that cause scratching [4]. Tissue a rare disease that is usually associated with injury at different signal transmission diabetes mellitus or renal failure, may involve levels or any alterations in neurotransmitter chronic prurigo [7]. In addition, prurigo- concentrations (such as in various like lesions are associated with various psychogenic disorders) may contribute to other collagen diseases such as discoid lupus both itch perception and the desire to scratch erythematosus, systemic scleroderma, and [4]. Occlusion with elastic bandages for adult-onset Still’s disease [8]. 4 weeks will improve the clinical picture of prurigo, with no enlarged nerve fibers or Malabsorption neurinoma-like structures remaining visible. The association between prurigo and Such an involution of prurigo nodules intestinal malabsorption was first observed would favor the theory that scratching is the by Wells in 1962, who described a patient triggering factor of prurigo. with gluten enteropathy [9]. Since then, As shown in a clinical study of 46 patients seven additional patients with celiac disease with chronic prurigo, 72% of patients felt and therapy-resistant prurigo have been that psychosocial problems were of relevance described in the literature. After several to their skin disease and 50% were found months of treatment involving a gluten-free to be suffering from depression, anxiety, or diet supplemented with vitamins and iron, another psychological disorder that might the intestinal symptoms improved and the require medical intervention [5]. A possible typical clinical prurigo nodules disappeared explanation for this association is that or improved. mood neurotransmitters such as dopamine, Itching and prurigo are also clinical serotonin, or opioid peptides modulate features that are associated with anorexia sensor perception. nervosa [10]. The symptoms disappear with the restoration of weight in these patients. Secondary prurigo in association with systemic disease Malignancy Secondary prurigo is characterized by Prurigo has been associated with T cell disseminated, chronic, severely pruritic lymphoma, leukemia, and visceral neoplasia reddened, flattened, slightly keratotic in the esophagus, ventricles, rectum, papules, which are usually 0.4–1.0 cm in liver, and the bile duct [11,12]. Prurigo in diameter. The lesions occur mainly on lymphoma may precede the symptoms of the extremities and upper back secondary malignancy. Its occurrence can also be a to prolonged and severe scratching in warning signal for malignant transformation patients with no underlying dermatological in patients with tumors that were previously condition. Several internal diseases diagnosed as benign [12]. Squamous cell associated with itching may involve the carcinoma may occasionally complicate formation of PN-like lesions. chronic prurigo nodules. An Update on Chronic Prurigo 91 Investigation of an The erythematous papular eruption affects associated disease light-exposed areas, most commonly the face Screening is limited to a complete blood cell [17]. This disease is rarely seen in Europe count as a marker of hematological disease and and appears to be identical to the actinic liver enzymes as markers of hepatic and renal prurigo found predominantly in native disease. In case of any differing pathologies North- and South-American Indians [18]. a specific investigation is recommended. Actinic prurigo is a chronic Furthermore, gastrointestinal symptoms are photodermatitis characterized by intense investigated by specific examination. pruritus, prurigo-like papules on light- In reluctant, longstanding cases of exposed areas, cheilitis, conjunctivitis, chronic prurigo a biopsy is advised to scars, and alopecia of the eyebrows [18]. ensure that a squamous cell carcinoma It generally appears at an early age, (that may occasionally complicate usually according to a family history and longstanding chronic prurigo nodules) is predominantly in females. Furthermore, it not overlooked. Furthermore, biopsy with is more frequently observed in subjects who immunofluorescence may be useful to exclude live at high altitudes (>2000 m) who have the diagnosis of pemphigoid nodularis. outdoor occupations and a predominance of human leukocyte antigen-DR4. Prurigo in association Sutton summer prurigo of the elbows with external factors appears as a papular eruption, usually Primary forms of prurigo elicited by external limited to the elbows, although it may also factors – including insect stings, parasites, affect the knees, hands, or chest [19].
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