Andrew’s Chapter 33-Part II

1. Andrew’s Chapter 33-Part II • David M. Bracciano D.O.

2. An inflammatory reaction of the hair follicles Leads to cicatricial alopecia Small pustules surround the follicles Erythema, scaling, and smooth shiny depressed scars are apparent Folliculitis Decalvans

3. Pseudopelade When the pustules have healed and scarring remains –pseudopelade occurs Note intact follicles and single hairs growing May occur on axillae and groin as well Folliculitis Decalvans

4. Etiology is unknown Scarring alopecia in a middle-aged man, associated with a hyperkeratotic scale-crust with follicular hyperkeratosis and erythema If gram stain and culture of the exudate reveals coagulase-positive staphylococci, this should be treated Many times no organism is cultured Folliculitis Decalvans

5. * Early lesions show perifollicular inflammation composed of acute and chronic inflammatory cells Folliculitis Decalvans

6. Later lesions shows destruction of follicular epithelium, dense interstitial inflammation, and perifollicular fibrosis, with free hairs in the tissue Folliculitis Decalvans

7. There is perifollicular and interstitial fibrosis accompanied by an interstitial infiltrate of lymphocytes and plasma cells Folliculitis Decalvans

8. Folliculitis Decalvans The eventual loss of follicular epithelium leads to a granulomatous response triggered by free hair shafts

9. Folliculitis Decalvans • TREATMENT: • Cephalosporins, dicloxacillin, and azithromycin and rifampin may be added to therapy for better long-term control • Oral zinc or vitamin C supplementation may enhance response • Chronic inflammation reactions may be helped with topical steroids and by intralesional triamcinolone

10. Thick, asbestos-like (amiantaceous), shiny scales attached to the lower part of the hair shaft, rather like tiles overlapping on a roof Crusting may be localized or, less commonly generalized over the entire scalp There are no structural changes in the hair, but in some patches where the crusting is thick, there may be purulent exudate under the crust and temporary alopecia may occur Tinea Amiantacea

11. Etiology is likely secondary to an infection occurring in seborrheic dermatitis or inverse psoriasis Treatment should be shampoo daily or every other day with selenium sulfide susupension, or a tar shampoo , for a few weeks Prior application of Baker’s P&S liquid is helpful to remove scale and crust Derma-Smoothe and FS shampoo are also effective Tinea Amiantacea

12. Also known as epidemic acne, epidemic follicular eruption, epidemic follicular keratosis, and Brooke’s disease Unknown etiology Occurs in children Keratosis Follicularis Contagiosa

13. Eruption is widespread and symmetrical, affecting chiefly the back of the neck, the shoulders, and the extensor surfaces of the extremities Onset is acute, may affect large numbers of patients in a localized geographic area , and spontaneously involutes over a 3-to-6-week period Keratosis Follicularis Contagiosa

14. There is a horny thickening of these areas, especially pronounced about the follicles, where small black corneous may be discerned Etiology has been hypothesized to be infectious- but not proven Keratosis Follicularis Contagiosa

15. Folliculitis Nares Perforans • Charcterized by small pustules near the tip of the inside of the nose • The lesion becomes crusted, and when the crust is removed it is found that the bulbous end of the affected vibrissa is embedded in the inspissated material • Staphylococccus aureus may at times be cultured from the pustules • Treatment is removal of the hair and topical ointment like mupirocin

16. Charcterized by an asymptomatic eruption of erythematous follicular papules 2 to 8 mm diameter Involving the extenxor ssurfaces of the upper arms, the buttockss, or the upper thighs When small, whitish kerratotic plug is removed from the follicular papule, a small bleeding crater remains Perforating Folliculitis

17. A dilated follicular unit contains a keratotic plug with an admixture of basophilic debris. The follicular epithelium is perforated, and there are degenerated collagen fibers in the adjacent dermis. Perforating Folliculitis

18. Resistant to treatment Topical tretinoin is reported to be effective One report of an HIV-infected man who responded well to thalidomide Perforating Folliculitis

19. Originally termed hyperkeratosis follicularis et paarafollicularis in cutem penetrans Rare disorder Characterized by hyperkeraatosis, forming a horny cone that projects into the dermis, so that when it is removed a pitlike depression remains Usually discrete papules, but may ccoalesce to form circinate plaques Kyrle’s Disease

20. There is a prediliction for the lower extremities, but the upper extremities, head, and neck may be involved Koebner’s phenomenon may also be observed, in which elevated verrucous streaks or plaques are formed The elevated verrucous streaks are only seen in the antecubital and popliteal spaces Kyrle’s Disease

21. Occurs almost exclusively in adults ages 20 to 63 No sex or racial differences seen Has been associated with diabetes mellitus Kyrle’s Disease

22. Histologically: Large keratotic and parakeratotic plugs penetrating through the epidermis into the dermis These plugs cause an inflammatory an foreign-body giant cell reaction about the lower end of the plug in the dermis Mild degenerative changes in the connective tissue with no increase in the elastic tissue also occur Kyrle’s Disease

23. Kyrle’s disease remains stable for years, with possible clearing when the associated illness is controlled Ultraviolet treatment, methotrexate, topical corticosteroids, 5-fluorouracil, and keratolytics are usually ineffective Topical retinoic acid 0.01% cream, isotretinoin, and etretinate have been effective in flattening the lesions Kyrle’s Disease

24. Pinhead-sized, skin-colored papules that grow to 4 to 6 mm and develop a central area of umbilication in which keratinous material is lodged The discrete papules may be numerous and involve sites of frequent trama The lesions reach a maximum size in 4 weeks the slowly regress Reactive Perforating Collagenosis

25. A cup-shaped channel containing degereated collagen bundles and basophilic material Reactive Perforating Collagenosis

26. Trichrome stain : Blue-stained collagen fibers perforating the channel and extending to the surface Reactive Perforating Collagenosis

27. It is believed that this is caused by a peculiar reaction of the skin to superficial trauma Koebnerization is often observed Young children are most frequently affected There is no specific treatment indicated, since the lesions involute spontaneously Reactive Perforating Collagenosis

28. It is reported that between 4% and 10% of dialysis patients develop perforating disorders Lesions are characterized by dome-shaped papules on the legs, or less often on the trunk, neck, arms, or scalp, with variable itchiness Early lesions may be pustular; late lesions resemble prurigo nodularis Perforating Disease of Hemodialysis

29. Perforating Disease of Hemodialysis • The disease may remit promptly after a renal transplant and stopping dialysis • Topical tretinoin may be tried

30. A curious gooseflesh-like follicular hyperkeratosis that may result from persistent pressure and lateral friction of one skin surface on another Such friction is often caused by habitual pressure of elbows, chin or jaw, or neck, often while watching television Two thirds of patients who develop this are atopic Traumatic Anserine Folliculosis

31. A unique erythematous pigmentary disease involving the follicles A reddish brown, sharply demarcated, symmetrical discoloration involving the preauricular and maxillary regions Pigmentation may be blotchy Follicular papules and erythema are present Pityriasiform scaling and slight itching may occur Keratosis pilaris is frequently found on the arms and shoulders It preferentially affects Asian patients Erythromelanosis Follicularis Faciei et Colli

32. A mildly pruritic eruption at times, and is chronic with recurrent exacerbations Characterized by uniform papules, 1 or 2 mm in diameter, and involving all the follicles in the affected areas, which are usually the upper trunk and neck Histologically, the infundibular portion of the follicles is chiefly affected, and the lesions are inflammatory rather than hyperkeratotic Disseminate and Recurrent Infundibulofolliculitis

33. Edema, lymphocytic and neutrophilic infiltration, and slight fibroblastic infiltration surround the affected follicles Treatment with vitamin A has been reported to be effective Isotretinoin or etretinate may also be useful Disseminate and Recurrent Infundibulofolliculitis

34. Disease frequently seen in children Characterized by minute filiform horny spines protruding from follicular openings independent of any papule The spines are discrete and grouped Lichen Spinulosus

35. The lesions appear in crops and are symmetrically distributed over the trunk, limbs, and buttocks There is a predilection for the neck, buttocks, abdominal wall, popliteal fossa, extensor surfaces of the arms A generalized distribution has been reported to occur with HIV infection Lichen Spinulosus

36. Lichen Spinulosus

37. Histology: Shows simple inflammatory changes and follicular hyperkeratosis Treatment: The lesions respond to mild keratolytics like 3% resorcin or salicylic acid ointment, Keralyt gel, Lac-Hydrin lotion, and tretinoin Lichen Spinulosus

38. Palmoplantar Hyperhidrosis(Emotional Hyperhidrosis) * Usually localized to the palms, soles or axillae * May be worse during warm temperature *Can be AD inherited *25% of patients with axillary hyperhidrosis have palmoplantar hyperhidrosis, but patients with palm and sole hyperhidrosis may have axillary hyperhidrosis *Sweating may be intermittent or constant Hyperhidrosis

39. Gustatory Hyperhidrosis Experience excessive sweating of the forehead, upper lip, perioral region, or sternum a few minutes after eating spicy foods, tomato sauce, chocolate, coffee, tea, or hot soups May also be caused by hyperactivity of the sympathetic nerves(Pancoast’s tumor) or postoperatively), sensory neuropathy (diabetes mellitus or subsequent to zoster), parotitis or parotid abscess, and surgery of the parotid gland Hyperhidrosis

40. Other Localized Forms of Hyperhidrosis: Localized sweating can occur over lesions of blue rubber bleb nevus, glomus tumors, hemangiomas and in POEMS syndrome, Gopalan’s syndrome, complex regional pain syndrome, and as a result of spinal cord tumors Hyperhidrosis

41. Generalized Hyperhidrosis May be induced by a hot, humid environment, a febrile illness, or vigorous exercise Hormonal disturbances such as hyperthyroidism, acromegaly, diabetes mellitus, pregnancy, and menopause Other causes include concussion, Parkinson’s disease, pheochromocytoma, hypogycemia, salicylism, and lymphoma, and metastatic tumors transecting the spinal cord Hyperhidrosis

42. Treatment: Treat underlying disorder Topical aluminum chloride or aluminum chlorhydroxide For the axillae, application of a 20% to 25% solution nightly to a very dry axillae Avoid deodorant-use baking soda instead For palmar hyperhidrosis apply 20% aluminum chloride tincture nightly, occluded with plastic gloves Hyperhidrosis

43. May be localized or generalized Generalized anhidrosis occurs in anhidrotic ectodermal dysplasia, quinacrine anhidrosis, miliaria profunda, Sjogren’s syndrome, hereditary sensory neuropathy (type IV) with anhidrosis, some patients with diabetic neuropathy, and multiple myeloma Anhidrosis with pruritis: Present with itching when attempting to sweat Absence of sweat when body temperature rises 0.5degrees C, fine papules appear at each eccrine orifice Cooling completely resolves symptoms Resolution may occur after several years Anhidrosis= absence of sweating

44. Ross syndrome= segmental anhidrosis associated with tonic pupils( Holmes-Adie syndrome) Intolerance and segmental areas of anhidrosis, loss of deep tendon reflexes, and compensatory segmental hyperhidrosis of functionally intact areas Anhidrosis localized to skin lesions over plaques of tuberculoid leprosy Also over ares of segmental vitiligo, in the hypopigmented streaks of incontinentia pigmenti, and on the face of patients with follicular atrophoderma, basal cell carcinomas, and hypotrichosis Anhidrosis

45. Mainly in the axillae Caused by bacterial decomposition of apocrine sweat, producing fatty acids with offensive odors True bromidrosis is usually not recognized by the patient Antibacterial soaps and many comercial deodorants are effective in controlling axillary malodor Frequent bathing, changing of underclothes, shaving, and topical alumuminum chloride Surgical removal of the glands is an option Bromidrosis= fetid sweat

46. Often patient s who complain of offensive axillary sweat actually have psychiatric disorders like delusion, paranoia, phobia, or a CNS lesion Intranasal foreign body and chronic mycotic infection of the sinuses in differential Fish odor syndrome- Caused by excretion of trimethylamine( which smells like rotten fish) in the eccrine seat, urine, saliva, etc. This chemical is produced from carnitine and choline in the diet Caused by an autosomal dominant defect in the ability to metabolize trimethylamine Bromidrosis

47. Rare disorder of the apocrine sweat glands Frequently localized to the face or axillae Most commonly sweat is yellow, less frequently blue, green, or black Colored sweat appears in response to adrenergic stimuli, which cause myoepithelial contractions Colored apocrine sweat fluoresces and is caused by lipofusion Eccrine chromhidrosis is caused by the coloring of clear eccrine sweat by dyes, pigments, or metals on the skin surface Examples of this: blue-green sweat from copper workers, the “red sweat” seen in flight attendants from the red dye in the labels of life-vests Brownish staining from ochronosis Small, round, brown or deep-green macules occur in the palms and soles in patients with excess bile secretion- liver disease Chromhidrosis

48. Fox-Fordyce Disease Rare, occurs in women during adolescence or soon afterward Conical, flesh-colored or grayish, intensely pruritic, discrete follicular papules in areas where the apocrine glands occur

49. Fox-Fordyce Disease

50. Fox-Fordyce Disease Apocrine gland sweating does not occur in areas of involvement Hair density may be decreased 90% occur in women aged 13-35 Pregnancy leads to improvement May occur postmenopausally or in males