Cutaneous Markers of Internal Disease SUMMARY SOMMAIRE Cutaneous Markers of Internal Disease Are Les Indices Cutanes Des Maladies Internes Sont Nombreux

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R. R. Forsey P. Michael Reardon Cutaneous Markers of Internal Disease SUMMARY SOMMAIRE Cutaneous markers of internal disease are Les indices cutanes des maladies internes sont nombreux. Cet article discute des desordres legion. This artide discusses the pigmentaires, de l'acanthosis nigricans, du prurit, pigmentary disorders, acanthosis nigricans, des xanthomes et du probleme de photosensibilit6, pruritus, the xanthomas and problems of soulignant les procedures appropriees afin d'etablir photosensitivity, outlinng the appropriate un diagnostic d6finitif et, dans certains cas, le procedures to establish a definite diagnosis, traitement de tels patients. and in some cases the management of such patients. (Can Fam Physician 1982; 28:1415-1421). ..I- a'

Dr. Forsey is a consultant seen the patient for a long time. The to stimulate overlying melanocytes to dermatologist in the Department of following classification illustrates increase their activity. Dermatology at the Montreal many of the causes of generalized Gastrointestinal. Malabsorption General Hospital, and Dr. Reardon hyperpigmentation. from a variety of causes and biliary is chief resident in the same Endocrine. Addison's disease re- cirrhosis are associated with increased department. Reprint requests to: sults in hyperpigmentation secondary pigmentation.2 Dr. R. R. Forsey, 1414 Drummond to the effect of unsuppressed pituitary Connective Tissue Disease. Sclero- St., Suite 1005, Montreal, PQ. beta melanocyte stimulating hormone derma is occasionally associated with H3G lWl. (B-MSH)1 or cutaneous melanocytes. Addisonian-like pigmentation.2 Asso- Clinically, the pigmentation is diffuse ciated cutaneous signs usually leave but accentuated on exposed areas of little doubt as to the primary diag- T HE SKIN manifestations of inter- the body. Areolae, genitalia, palmar nosis. nal disease are myriad.1 2 The creases, scars and mucus membranes Drugs. Systemically administered usual custom is to present the cutan- are also areas of predilection. Sixteen psoralen in combination with long- eous findings of specific diseases; our percent of patients with Cushing's syn- wave ultraviolet light produces a pho- plan is to present some cutaneous drome, 40% of acromegalics and some totoxic reaction, with the subsequent symptoms and signs which might give patients with adrenogenital syndrome appearance of tremendously increased clues to the diagnosis of systemic dis- and Graves disease display similar pig- pigmentation. Some medications, or ease. mentary findings.2 Control of the un- their metabolic products, may accu- derlying disease usually results in a re- mulate in the dermis and produce pig- Assorted Disorders turn to normal pigmentation. mentation by themselves or by increas- Metabolic. Hemochromatosis is as- ing melanin production. Classic Of Pigmentation sociated with deposition of iron in var- examples include chlorpromazine and Generalized Hyperpigmentation ious parenchymal- organs with secon- heavy metals such as silver (argyria).3 A generalized increase in pigmenta- dary deficiencies of function resulting Malignancy. Some tumors, particu- tion may be quite gradual and not read- in clinical disease. Deposits of iron are larly oat cell carcinoma of the lung, ily apparent to a patient or a close rela- present in the dermis but are not re- have been found to produce MSH-like tive. It may be easily recognized from sponsible for the increase in pigmenta- peptides. Other malignancies, such as old photos, or by a friend who has not tion.1 The presence of iron is believed Hodgkin's disease, will cause hyper-

CAN. FAM. PHYSICIAN Vol. 28: AUGUST 1982 1415 pigmentation without an apparent hor- such as pruritus, and even syncope or sive inheritance, absent or decreased monal influence. The very rare gener- death, may occur with widespread re- melanin, nystagmus, photophobia and alized melanosis of metastatic lease of histamine. Although serious decreased visual acuity. The overall malignant melanoma is a grave sign side effects are rare and presumably incidence is approximately 1:20,000. associated with 100% mortality. related to mast cell load, it is advisable All albinos have an increased suscepti- to avoid drugs known to release hista- bility to carcinogenic ultraviolet rays. Localized Hyperpigmentation mine from mast cells (aspirin, co- They should use sunscreens and be ex- Cafe-au-lait spots. These are hyper- deine, opiates and polymyxin B). amined at least yearly for the presence pigmented patches, usually 1.5-20 cm Physical stimuli, such as bathing in hot of premalignant lesions (actinic kera- in diameter. They are frequently pres- water or vigorous rubbing, may also toses) and malignant ones, including ent at birth and have been found to precipitate symptoms. Diagnosis can basal cell carcinoma, squamous cell occur in 10-20% of normal patients. be easily confirmed by biopsy. The carcinoma and malignant melanoma. They often increase in size and number pathologist should be advised of the Two of the rare variants have systemic with age. In children below age five, suspected diagnosis so that stains high- manifestations. In Chediak-Higashi the presence of five or more cafe-au- lighting mast cells will be performed. syndrome, besides the pigmentary ab- lait spots of greater than 0.5 cm is Regular antihistamines by themselves, normality, there is a leucocyte chemo- diagnostic of neurofibromatosis.4 In or in combination with H2 receptor an- tactic defect, increased susceptibility adults or older children, six or more le- tagonists (Cimetidine), are useful in to infection, convulsions, neuropathy sions greater than 1.5 cm are diagnos- treatment. Recently, the use of oral and a propensity to develop pancyto- tic.5 At least 90% of the patients with disodium cromoglycate has been penia and lymphoreticular 'malig- this disease have 1-4 mm spots in the found useful in systemic disease.7 nancy. In Hermansky Pudlak syn- axillae (Crowe's sign).6 The impor- drome, a hemorrhagic diathesis secon- tance of these cutaneous markers is Generalized Hypopigmentation dary to platelet abnormality may that they occur before the development Complete. Congenital generalized occur. of cutaneous neurofibromas. hypopigmentation of the skin,9 hair Partial. Localized hypopigmenta- The clinical presentation of neurofi- and eyes is usually related to a defi- tion occurs due to a variety of causes. bromatosis may be extremely variable. ciency in the synthesis of melanin (al- It is frequently seen as a response to It may be asymptomatic or grossly de- binism). Other conditions including various cutaneous and systemic dis- bilitating, resulting in retardation, sei- homocystinuria, phenylketonuria, eases. It is seen in localized sclero- zures, or a variety of benign and even Menkes kinky hair syndrome and histi- derma (morphea), lichen sclerosis at malignant tumors. The patient and dinemia may also produce such hypo- atrophicus, ash leaf macule of tu- available relatives should be assessed pigmentation and can be ruled out by berous sclerosis, post-inflammatory for related skin, eye, neurological and specialized metabolic assessment. The conditions, some infections (e.g. lep- musculoskeletal findings. Genetic word albino is derived from the Latin rosy, tinea versicolor) and vitiligo. counselling should be offered as no cu- word albus,8 meaning white. There are The diagnosis is often self-evident. rative treatment is currently available. several variants to this condition, all Vitiligo has been reported in association with thyroiditis, Grave's disease, Pigmentation with urtication. When having in common autosomal reces- the clinician sees a patient with tan colored macules, papules or nodules, Fig. I he should scratch one or more of these Multiple caf6-au-lait spots of Fig. 2 lesions. A positive reaction (Darier's neurofibromatosis. Extensive neurofibromas. sign) is specific for the diagnosis of cutaneous mastocytosis (urticaria pigmentosa). Mast cells are ubiquitous cells of connective tissue, usually found in small numbers in a perivascu- lar distribution. However, localized accumulation of mast cells may occur in skin and internal organs. These lesions are most common in children (usually appearing in the first three to nine months of life) and may be soli- tary or multiple. They are rarely associated with more ominous involvement of bone, internal organs or the blood stream. Adults have a higher incidence of systemic disease and much less tendency to the spontaneous resolution normally seen in childhood cases. Immunologic and non-immunologic stimuli will induce these cells to release a variety of products, most not- ably histamine. Marked symptoms,

1416 CAN. FAM. PHYSICIAN Vol. 28: AUGUST 1982 Addison's disease, pernicious anemia, Pseudo-acanthosis nigricans nosis. Malignant acanthosis nigricans diabetes mellitus and alopecia areata.9 This is present in many otherwise tends to follow the course of the under- We believe these associations are rare well, obese patients; it is mild and dis- lying tumor and may regress or pro- and treatment of the underlying condi- appears after weight loss. Some dark- gress according to the treatment. tion does not restore skin color. skinned people residing in tropical cli- It should be clear from the afore- There is a localized congenital hy- mates develop a similar condition due mentioned classification that the pres- popigmentation which frequently re- to perspiration and sweating, as do ence of lesions morphologically con- sembles the leaf of the mountain ash some patients receiving cortico- sistent with the diagnosis of acanthosis tree. This is the earliest detectable sign steroids, nicotinic acid and diethylstil- nigricans does not end the physician's of tuberus sclerosis, an autosomal bestrol. work. He must consider many factors, dominant neurocutaneous disease clas- including heredity, body habitus, age sically consisting of seizures, mental Syndromal acanthosis nigricans at onset, drug intake and a detailed retardation and, sooner or later, facial Acanthosis nigricans may occur as a functional inquiry. tumors known as adenoma sebaceum, coincidental finding in at least 16 syn- multiple periungual fibromas and he- dromes1" such as congenital normo- matomas of connective tissue known glycemic and hyperglycemic insulin Pruritus as 'shagreen patches'. As in neurofi- resistance, polycystic ovary disease, Essential pruritus bromatosis, there is a tremendous vari- Wilson's disease and disorders of li- Pruritus is a Latin word used to ability in clinical expression and a high poatrophy. mean itching. Essential pruritis by def- rate of spontaneous mutation. Early inition occurs in the absence of pri- diagnosis in a suspected case may be Benign acanthosis nigricans mary cutaneous lesions and may be a helped by use of a Wood's lamp. This symptom of diagnostic importance. light source emits a wavelength of ul- This is a familial variant, usually of autosomal dominant inheritance, onset The frequency with which essential traviolet light that highlights variation pruritus is associated with internal dis- in epidermal pigmentation. Recently, at birth or early childhood and unasso- ciated with internal disorder. ease varies-5-80% depending on the computerized axial tomography has reported series. The number of indi- been known to confirm an early diag- Malignant acanthosis nigricans vidual diseases anecdotally associated nosis by revealing microcalcification with pruritus is legion. Certain of these in affected brain tissues. This form is seen primarily in men associations have withstood the test of over 40 and is characterized by sudden time and are useful for early diagnosis onset and progressive spread. In some Acanthosis nigricans and even prognosis. Other associa- cases there is extensive involvement of tions, although often quoted, are This is a distinctive velvety, verru- atypical areas such as mucosa, or poorly substantiated, or not terribly cous and hyperpigmented condition palms and soles. The associated ma- useful in disease diagnosis. confined primarily to flexural areas. lignancy is almost always an adenocar- Acanthosis refers to a thickening of the cinoma of intra-abdominal origin, the epidermis. Nigricans is derived from most common primary being the stom- Endocrine disease the Latin verb nigricare, which means ach. Curth10 found that in only 17% of Diabetes. Pruritus occurs in only to turn black. The presence of this con- the cases did the dermatosis precede about 5% of diabetics1 and has been dition may signify a normal variation, the diagnosis of the tumor. This der- related to factors such as dry skin or an ominous underlying disorder as matosis has traditionally been a bad (xerosis), hypohydrosis and misinter- the following classification"'' will il- prognostic sign; the vast majority of pretation of paresthesia as a pruritic lustrate. patients die within one year of diag- sensation. Fig. 3 Tinea versicolor: a common superficial fungus infection resulting in widespread scaly alteration of Fig. 4 pigmentation. Oval areas of hypopigmentation in tuberous sclerosis.

CAN. FAM. PHYSICIAN Vol. 28: AUGUST 1982 1417 Hyperthyroidism. Pruritus occurs in Chronic leukemias. The incidence Miscellaneous. Isolated reports 5-10% 13 of patients and may be related of pruritus is relatively low and more have occurred which relate pruritus to to such factors as heat, vasodilation, common in chronic lymphatic leuke- infestations, collagen diseases, partic- hypermetabolic state and emotional mia. ularly the sicca syndrome, and an factors. Polycythemia rubra vera. Pruritus is array of mental conditions including Hypothyroidism. Itching is seen in seen in 20-50% 12 of cases; it is classi- anxiety, depression, neurosis and psy- approximately 10% of cases and cally worse after bathing. Histamine chosis. Localized pruritus in a pre- usually resolves with treatment of the may be important as circulating levels existing skin lesion such as a nevus underlying disease. are five times normal in this disease. may indicate a malignant transforma- Secondary hyperparathyroidism. Hodgkin's disease. The overall in- tion. We have learned to respect this This has been implicated by some as cidence of pruritus is probably greater symptom. If such a lesion occurs- one of the causes of itching in uremia. than 25%, mainly in older patients and biopsy it! Occasionally, the symptom responds in terminal stages of the disease. The to surgical treatment. frequency and intensity seems to in- Xanthomas Hypoparathyroidism. Ill-defined crease as the disease progresses and Xanthoma is derived from the Greek pruritus has been described with both may be severe. However, pruritus may words xantho (meaning yellow) and precede the actual diagnosis by at least oma (meaning tumefaction). Xan- the idiopathic and post-surgical varie- five years. ties of hypoparathyroidism. thomas are localized infiltrates of lipid Malignant tumors. These tumors, containing phagocytic cells found in including those in the stomach, colon Hepatic Disease the dermis or in tendons and usually and pancreas, have associated itching indicating an underlying disorder of li- Pruritus associated with hepatic dis- in very rare instances, and usually late poprotein metabolism. Circulating ease invariably involves the presence in the disease. plasma lipids are derived from exo- of intra- or extrahepatic cholestasis. It Neurologic disease. Neurologic genous dietary sources and endogen- has been said that pruritus correlates pathways of itching suggest that dis- ously synthesized fats. Abnormal best with cutaneous rather than serum eases of the central nervous system levels of lipoprotein in the blood result levels of bile acids, 14 but recent exper- would often cause itching. This is, in from excessive endogenous produc- imental work15 has questioned the en- fact, rare. Multiple sclerosis has been tion, defective removal or both. Fred- tire theory of bile acid causation. associated with itch, often confined to rickson has grouped the hyperlipopro- Pruritus is the initial symptom of 50% localized areas.18 Of 77 patients with teinemias into five groups based on of patients with primary biliary cirrho- brain tumors, 13 were found to have specific electrophoretic patterns. They sis. In pregnancy, 1-3% of patients ex- pruritus. 19 Six of the patients had may be further divided into primary perience pruritus, which is felt to be pruritus localized to the nostrils with (specific hyperlipoproteinemic dis- secondary to cholestasis, perhaps from tumors involving the floor of the order, often hereditary) and secondary increased estrogen levels. fourth ventricle. Intractable itching (related to underlying diseases such as can be found in a dermatome involved diabetes, hypothyroidism, nephrotic Renal Disease with herpes zoster20 and in tabes dor- syndrome, biliary cirrhosis and pan- salis. The ultimate cause of itching in creatitis). Both hereditary and ac- chronic renal failure is unknown. About 50% of patients have pruritus of Fig. 5 Fig. 6 varying severity. Various attempts to Hyperpigmented papules of Total localized depigmentation of correlate BUN, phosphorus, calcium, urticaria pigmentosa. vitiligo. protein products, abnormal vitamin D metabolism and levels of parathyroid hormone have been unsuccessful. There is an increase in cutaneous mast cells in the skin in chronic renal failure, sweat gland atrophy and neuropathy, all of which may play some role in etiology. Dialysis does not relieve the itching. Some recent reports have suggested ultraviolet B light therapy gives considerable palliation. 16

Blood Disease and Malignancy Iron deficiency anemia. Pruritus has been reported in association with iron deficiency but the evidence is not con- vincing-a Danish study involving 42,000 patients revealed a slight increase in the incidence as compared to controls. 17

1418 CAN. FAM. PHYSICIAN Vol. 28: AUGUST 1982 quired disorders of lipid metabolism Tendenous xanthomas ciated with ischemic heart disease and are able to produce xanthomas which These are almost always associated peripheral vascular insufficiency. may be seen to wax and wane with with high levels of cholesterol and Eruptive xanthomas fluctuations in serum lipoproteins. coronary atherosclerosis. Clinically There are four main clinical variants they appear as deep nodules in ten- These are small yellow papules of xanthoma. dons, ligaments and fascia and can be 2-4 mm in diameter with an erythema- tous halo. They occur in crops, pri- Planar xanthomas confused wtih gouty tophi and rheu- matoid nodules. There are two rare cir- manly over extensor surfaces of arms, The most common of these is the cumstances when these lesions are as- legs and buttocks and appear exclu- xanthelasma which is seen on the eye- sociated with normal serum lipids. In sively in patients with high concentra- lid and adjacent skin. Only 25% of pa- cerebrotendenous xanthomatosis22 (a tions of triglycerides in the plasma. tients with this finding will have a lipid storage disease), a lipid known as Preliminary investigation for xan- serum lipid abnormality.1 If present, cholestanol accumulates in tendons, thomas should include a 12-hour fast- the cholesterol is elevated. This find- lung and cerebellar white matter, re- ing determination of cholesterol and ing is significant primarily in patients sulting in mental deficiency and cere- triglycerides. If these are increased, a under 40. Xanthoma striatum pal- bellar ataxia. An occasional patient lipoprotein electrophoresis should be mare,21 another form of plane xanth- will develop tendenous xanthomas obtained. Evaluation of blood sugar, oma, appears as flat linear lesions of from deposition of certain plant thyroid, liver and renal function will the palm and volar finger creases. sterols23 which are not normally ab- diagnose many of the secondary dis- Conditions producing high levels of sorbed in significant amounts. eases. In suspected paraproteinemias, cholesterol and triglyceride can be as- serum and urine protein electro- sociated with these lesions. A rare Tuberous xanthomas phoresis, chest X-ray and bone mar- variant is that occurring with parapro- These lesions are yellow-red nod- row examination will be necessary. teinemias such as multiple myeloma ules with a predilection for extensor where extensive yellow-orange lesions surfaces such as elbows, knees, Photosensitivity infiltrate the face, neck and upper knuckles and buttocks. They indicate This may be defined as an abnormal trunk. These patients may or may not significant elevation of cholesterol or adverse reaction to sunlight energy have high lipid levels. and/or triglycerides. They are asso- in the skin. Fig. 7 Characteristic location of acanthosis nigricans Fig. 8 showing velvety hyperpigmentation. Xanthalasma.

Fig. 9 Tuberous xanthomata in a characteristic location.

1419 CAN. FAM. PHYSICIAN Vol. 28: AUGUST 1982 In many sections of our society a toses requires common sense and an ery, parsley, figs, dills) containing good suntan is equated with health and index of suspicion, based on. lesional furocoumarins, fluorescein derivatives wealth. Sunbathing has become almost distribution. Facial prominences and some coal tar preparations may an obsession. The sun emits a wide (nose, cheeks, chin, upper ears) and produce similar reactions. The photo- spectrum of radiation. For practical the extensor aspects of the arms, hands toxicity is dose-dependent, but the purposes we will consider primarily and legs are the most commonly in- mechanism is poorly understood. wavelengths of 290-400 nanometers, volved areas, but almost any pattern as these are involved in most cutan- can be produced, depending on the pa- Photoallergic eous reactions. Shorter wavelengths tient's state of dress or undress. In photoallergic reactions, an agent do not reach the earth's surface be- Shaded areas such as beneath the chin reaches the skin via topical or systemic cause of absorption by atmospheric and in skin folds will, of course, be use and is altered by light to form an gases, including the ozone layer. relatively spared. allergen. There is a delay between ex- Wavelengths between 290-320 nano- Acute adverse cutaneous reactions posure to light and appearance of the meters (known as UVB) are the prime to sunlight may be grouped in the fol- eruption which is usually eczematous producers of suntan, sunburn and skin lowing classifications: in morphology. A reaction occurs in cancer. Wavelengths of 320-400 nano- only a small minority of those ex- meters (known as UVA) augment Physiologic posed. Topical antibacterials including these reactions and are responsible for We are all familiar with physiologic hexachlorophene, dichlorophene and most drug and chemical-induced pho- sunburn reactions and those in whom certain carbanilides are among those tosensitivity reactions. Wavelengths of skinned agents implicated in photoallergy.24 visible they are likely to occur. Fair 400-800 nanometers (known as people with blue eyes, a tendency to light) rarely produce photosensitivity freckling and little propensity to tan Congenital reaction, with a few notable excep- to be affected. 320 are the most likely A variety of genetic disorders result tions. Wavelengths longer than in undue acute and chronic reactions to nanometers penetrate window glass. Phototoxic sunlight. Some of these are associated The prime protection from sunlight with characteristic syndromes is the pigment melanin, which is pro- Phototoxic reactions are a non- Rothmund- on exaggerated sunburn re- (Bloom's, Cockayne's, duced by epidermal melanocytes immunologic Thomson). Xeroderma pigmentosa is a the basis of genetic characteristics and sponse. They occur with commonly DNA such as thiazide rare group of disorders of repair various endogenous and exogenous used medications in aging of the stimuli. Individual differences in sun diuretics, sulfonamides, oral hypogly- resulting premature the deriva- skin and early development of multiple sensitivity depend not only on cemic agents, phenothiazine aggressive cutaneous tumors. amount of melanin present but also on tives, griseofulvin and some antibiot- its packaging and distribution within ics (doxycycline, dimethychlor- the epidermis. tetracycline, nalidixic acid). Topical Metabolic Recognition of sun-induced derma- agents or plants (parsnips, liver, cel- Porphyrins are metabolic intermedi-

Fig. 10 Eruptive xanthomata in patient with high serum triglycerides.

Fig. 1 1 Phototoxic reaction to oral medication. E _ --

*os. _.

Vol. 28: AUGUST 1982 1420 CAN. FAM. PHYSICIAN ates in the production of heme, a mole- lengths (i.e. porphyria at approx- heavy metal induced hyperpigmentation. S cule produced by the liver and during imately 405 nm). Photoallergic reac- American Academy of Dermatology 1981; only 5:1-18. erythropoiesis. Porphyrins are the tions often occur in the UVA wave- 4. Whitehouse D: Diagnostic value of the well established endogenous photosen- lengths (320-400 nm). Carcinogenesis cafe au lait spot in children. Arch Dis sitizers. When they accumulate as a re- and sunburn is felt to reside primarily Child 1966; 41:316-319. sult of a specific enzyme defect, clini- in the UVB wavelength (290-320 5. Crowe FW, Schull WJ: Diagnostic im- cal disease may result. The pattern will nm). portance of the cafe au lait spot in neurofibromatosis. Ann Intern Med 1963; depend on the metabolic intermediate Sunscreens may be divided into 91:758-766. present in excess. Porphyria cutanea three groups according to the type of 6. Crowe FW: Axillary freckling as a tarda is the most common form and is protection they offer.24 diagnostic aid in neurofibromatosis. Ann not infrequently seen by dermatolo- 1. Short UV sunscreens (290- Intern Med 1962; 61:1142-1143. as excess These are formulations con- 7. Soter NA, Austen KF, Wasserman SI: gists. It presents clinically 320 nm). Oral disodium cromoglycate in the treat- friability of skin and the propensity to taining para aminobenzoic acid ment of systemic mastocytosis. N Engl J form vesicles, milia and hyperpigmen- (PABA) or its esters. Med 1979; 301:465469. tation on sun-exposed skin. There is 2. Full spectrum UV sunscreens (290- 8. Leder M, Rosenblum M: A Dermatolo- often increased hair growth in the tem- 400 nm). These are usually benzo- gic Dictionary. West Haven, CT., Dome Laboratories, 1976. poral area, and liver disease is invari- phenone derivatives and, although 9. Fitzpatrick TB: Dermatology in Inter- ably present. A latent enzyme defect they are not as effective screeners as nal Medicine. New York, McGraw Hill of uroporphyrinogen decarboxylase PABA, they offer some protection in Book Co., 1976, pp. 575-582. may become manifest by the intake of the UVA range (320-400 nm). 10. Curth HO: Skin lesions and internal carcinoma in cancer of the skin, in An- estrogens, iron or alcohol. 3. Full spectrum ultraviolet and visi- drade R, Gumport JL, Popkin GL, et al The most famous of the porphyrias, ble light sunscreens (290-700 nm). (eds): Cancer ofthe Skin: Biologies, Diag- known as acute intermittent porphyria, These include preparations containing nosis and Management. Philadelphia, does not have cutaneous manifesta- titanium oxide, red petrolatum and W.B. Saunders Co., 1976. tions. Complete investigation of the zinc oxide. This group of screens is 11. Schwartz RA: Acanthosis nigricans, florid cutaneous papillomatosis and the porphyrias requires laboratory assess- most protective but is the least cosmet- sign of Leser-Trelat. Cutis 1981; 28:319- ment of urine, stool and red blood cell ically acceptable. 331. porphyrins.2 12. Winkelman RK, Muller S: Pruritus. Disorders in which a deficiency of Annu Rev Med 1964; 15:53-64. Conclusion 13. Barnes HM, Sarkany I, Calnan CD: nicotinic acid may develop (pellegra, Pruritus and thyrotoxicosis. Trans St. Hartnup's disease, Kwashiorkor, car- All the cutaneous signs of internal John's Hospital Dermatological Society cinoid syndrome) are associated with disease are far too numerous to be 1974; 60:59-62. dermatitis on sun exposed skin. completely covered in a paper of this 14. Stiehl A: Bile and bile acid sulphates Connective tissue diseases such as scope. We have tried to discuss prob- in the skin of patients suffering from cho- lems encountered in daily practice. A lestasis and pruritus. Z Gastroenterol lupus erythematosis and dermatomyo- 1974; 12:121-124. sitis often develop photo-induced der- complete history and physical assess- 15. Moschella SM: Dermatology Update. matoses. Lupus patients not infre- ment, combined with selective ancil- New York, Elsevier, 1982, pp. 185-196. quently experience exacerbation of lary investigation, are essential in pa- 16. Gilchrest BA, Rowe JW, Brown RS, et tients suspected of suffering from a al: Relief of uremic pruritus with ultravio- their internal disease precipitated by let photo therapy. N Engl J Med 1977; sunlight, thus emphasizing the need systemic disease on the basis of cutan- 297:136-138. for vigorous photoprotection. eous findings. 17. Tahkunen H: Iron deficiency pruritus. JAMA 1978; 239:1394. 18. Osterman PO: Paroxysmal itching in Acknowledgements multiple sclerosis. Br J Dermatol 1976; Idiopathic 95:555-558. We would like to thank Dr. Brenda 19. Andreev UC, Petrou I: Skin manifesta- Disorders recognized clinically as Moroz, Chief of Dermatology at the as solar urti- tions associated with tumours of the brain. sun induced entities, such Montreal Children's Hospital, for the Br J Dermatol 1975; 92 :675-678. caria and a 'grab bag' group of dis- use of several photographs, and De- 20. Liddell K: Post herpetic pruritus. Br orders known as polymorphous light borah Stewart for typing the manu- Med J 1974; 4:165. eruption, presently lack good explana- script. 21. Polano MD: Xanthomatosis and hy- further perlipoproteinemia. Dermatologica 1974; tion. It is hoped with investiga- 149:1. tion specific etiologies will be estab- 22. Philippart M, Van Bogaert L: Choles- lished. References tanolosis (cerebiotendonis xanthomatosis). The treatment of sun induced der- 1. Callen JP: Cutaneous Aspects of Inter- Arch Dermatol 1969; 21:603-610. matoses requires reduction of sun ex- nal Disease. Chicago, Year Book Medical 23. Bhaitcharhya AK, Connor WE: B- Publishers, Inc., 1981. sitosterolemia and xanthomatosis. J Clin posure, physical protection (hats, long 2. Braverman IM: Skin Signs of Systemic Invest 1974, 53:1033-1043. sleeves) and the rational use of sun- Disease. Philadelphia, W.B. Saunders 24. Hurwitz S: Clinical Podiatric Derma- screens. Many photosensitivity dis- Company, 1981. tology. Philadelphia, W.B. Saunders Co., orders are caused by specific wave- 3. Grandstein RD, Sober AJ: Drug and 1981, pp. 69-82.

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