Pseudofolliculitis Barbare

Abstract:  24 year old man who has been struggling with pseudofolliculitis barbare (PFB) for 5 years. The following is adapted from notes the patient sent us and is presented here with his consent:

HPI: This type II skin, 24 yo man has been plagued with PFB for 5 years. At age 19 he was treated for one year with minocyclin which he accepted with no noticeable side effects for the first 4 months until he believed his teeth were developing a a gray tinge. During treatment, the sites inflammation diminished but ingrown hairs were still a problem despite not shaving.

For the past year, he has had no treatment other than washing his face daily with oil free "deep action exfoliating scrub" from and moisturizing with Pond's Dry skin cream. He has also been plucking ingrown hairs with tweezers. When cut he uses a topical "triple antibiotic ointment to prevent secondary infection. He has encountered many "hair whorls" which have grown completely under the skin. These hair whorls present as a hard lump with a darker tint under his type II skin. He has been able to remove the hairs which range in size from a half inch to an inch and a half with tweezers.

The ingrown hairs predominate on his jaw line and the hair whorls solely occur under his ear lobe (along his jaw line). The left side of his face is noticeably more affected than his right side. He attributes this to a nervous habit of his in which he scratches and pulls out hairs on his face in the affected region while he is working.

On Exam: He presents with numerous ingrown hairs on his right and left jaw line and in the submental area. The ingrown neck hairs grow about 2 mm in length before growing back into the skin. He also has ingrown hairs that have not surfaced and instead are growing shallowly underneath the surface of the skin. Pustules develop at the sites of the ingrown hairs (both the hairs that have not been able to grow out of the skin and the hairs that have grown back into the skin). 

Clinical Photo (taken by patient)

 Lab and Pathology: Not applicable

Diagnosis: Pseudofolliculitis barbare with pili incarnati

Questions:  Have you found a therapy that is successful in these patients?  Antibiotics?  Topicals? IPL or Alexandrite laser?  Other?

References:

1.  Cook-Bolden FE, Barba A, Halder R, Taylor S . Twice-daily applications of benzoyl peroxide 5%/clindamycin 1% gel versus vehicle in the treatment of pseudofolliculitis barbae.  Cutis. 2004 Jun;73(6 Suppl):18-24.

Abstract: Pseudofolliculitis barbae (PFB) is an inflammatory disorder characterized by the formation of papules, pustules, and hyperpigmentation as a result of ingrown hairs. It is estimated that PFB affects 45% to 83% of black men who shave regularly. In this multicenter, double-blind pilot study, men with 16 to 100 combined papules and pustules on the face and neck were randomized to receive twice-daily benzoyl peroxide 5%/ clindamycin 1% (BP/C) gel (BenzaClin)(n = 47) or vehicle (n = 41) for 10 weeks. Sixty-eight (77.3%) of the participants were black. All patients were required to shave at least twice a week and to use a standardized shaving regimen throughout the study. Clinical evaluations were performed at 2-week intervals. The primary efficacy parameter was the percentage change from baseline in lesion counts. At weeks 2, 4, and 6, mean percentage reductions from baseline in combined papule and pustule counts were significantly greater with BP/C gel compared with vehicle (P < or = .029). Treatment differences in favor of active therapy were more pronounced in the subpopulations of black patients, with least squares mean percentage reductions in papule and pustule counts ranging from 38.2% at week 2 to 63.9% at week 10. Study medication was well tolerated. These positive findings warrant further investigation of BP/C gel in the treatment of patients with PFB.

2.  Tahra M Leheta.  COMPARATIVE EVALUATION OF LONG PULSE ALEXANDRITE LASER AND INTENSE PULSED LIGHT SYSTEMS FOR PSEUDOFOLLICULITIS BARBAE TREATMENT WITH ONE YEAR OF FOLLOW UP.  Indian J Dermatol. 2009 Oct-Dec; 54(4): 364–368.  Free Full Text.

Abstract

Background:

Existing remedies for controlling pseudofolliculitis barbae (PFB) are sometimes helpful; however the positive effects are often short lived. The only definitive cure for PFB is permanent removal of the hair follicle.

Aims:

Our aim was to compare the efficacy of the Alexandrite laser with the intense pulsed light system in the treatment of PFB and to follow up the recurrence.

Methods:

Twenty male patients seeking laser hair removal for the treatment of PFB were enrolled in this study. One half of the face was treated with the long-pulse Alexandrite laser and the other half was treated with the IPL system randomly. The treatment outcome and any complications were observed and followed up for one year.

Results:

All patients exhibited a statistically significant decrease in the numbers of papules. Our results showed that the Alexandrite-treated side needed seven sessions to reach about 80% improvement, while the IPL-treated side needed 10-12 sessions to reach about 50% improvement. During the one year follow up period, the Alexandrite-treated side showed recurrence in very minimal areas, while the IPL-treated side showed recurrence in bigger areas.

Conclusions:

Our results showed that both systems might improve PFB but Alexandrite laser was more effective at reducing PFB than IPL.

Alopecia Areata and Twenty Nail Distrophy

Abstract: 18 year old man with two year history of alopecia areata and six month history of dry lusterless nails.

HPI:  The patient is an 18 year-old college student who has had alopecia areata for the past 2 years.  He is well otherwise and has had no other problems until a number of months ago when he developed a nail dystrophy.   He takes no medications by mouth.  There is no family history of alopecia or autoimmune problems.  Treatment to date for alopecia has been intralesional triamcinlone with regrowth, however, new areas continue to evolve.

O/E:  There are 6 - 7 alopecic areas measuring from 2 to 6 cm in diameter widely scattered over the scalp.  He has some areas of alopepcia on his abdomen.
19 of his nails are dystrophic.  They are lusterless and many are greyish white in color, rough and friable.  One of his toenails appears normal

Clinical Photos:

Lab: All studies have been normal.  Records of these have been requested.

Diagnosis:  Alopecia areata and trachyonychia (aka 20 Nail Dystrophy)

Discussion and Questions: The patient has read about immunotherapy with DNCB and related chemicals.  Has anyone treated a patient with this modality who experienced long-lasting remission?  Do you know of good treatments for his nail dystrophy?

References: 
Alopecia universalis with twenty-nail dystrophy (trachyonychia).

Chien P Jr, Kovich OI.

Dermatol Online J. 2008 May 15;14(5):24.

Department of Dermatology, New York University, USA.

Abstract

A 43-year-old man presented with long-standing trachyonychia of all 20 nails, which worsened after the onset of alopecia universalis 18 months ago. Trachyonychia can be associated with alopecia universalis although the treatment strategies of both conditions differ. The principle of treating trachyonychia may involve regulating the differentiation of keratinocytes and/or reducing inflammation in the nail fold or nail matrix while treatment of alopecia universalis involves immunomodulation.

Free Full Text

Indian J Dermatol Venereol Leprol. 2011 Nov-Dec;77(6):640-5.

Trachyonychia: a comprehensive review.

Gordon KA, Vega JM, Tosti A.

Source

Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, Florida 33136, USA. kagordon@med.miami.edu

Abstract

Trachyonychia or rough nails, may present as an idiopathic disorder of the nails or it can be associated with other dermatological conditions. The dystrophic nail findings seen in trachyonychia are characterized by brittle, thin nails, with excessive longitudinal ridging. The most common histopathologic features associated with trachyonychia are spongiosis and exocytosis of inflammatory cells into the nail epithelia; typical features of lichen planus or psoriasis can also be detected. Determining the cause of trachyonychia is challenging. Treatment is often unsatisfactory, although in general it should be aimed at the underlying cause, if found. In most cases, the nail abnormalities improve spontaneously.  Available full text

Vañó-Galván S, et. al. Sudden hair loss associated with trachyonychia. Cleve Clin J Med. 2008 Aug;75(8):567-8
Department of Dermatology, Ramón y Cajal Hospital, University of Alcalá, Madrid, Spain. sergiovano@yahoo.es  Free Full Text

UTILITY OF DERMOSCOPY IN ALOPECIA AREATA

Mandar Mane, Amiya Kumar Nath, and Devinder Mohan Thappa

Indian J Dermatol. 2011 Jul-Aug; 56(4): 407–411  F  Free Full Text

Twenty-nail dystrophy of alopecia areata.

Horn RT Jr, Odom RB.

Arch Dermatol. 1980 May;116(5):573-4

Abstract

We describe here a patient with dystrophy of all 20 nails, which has persisted for five years after the resolution of alopecia areata. We feel that the term "20-nail dystrophy" is best used to describe a clinical entity that can have several causes.

Incidentaloma

The patient is an 82 man who presented  for evaluation of a facial lesion.  That lesion was a basal cell carcinoma.  However a complete skin exam revealed a suspicious tumor on the right upper back. 

The patient has type III skin.  This "new" lesion on the back is an eight mm in diameter hypopigmented papule with an eccentrically placed  2 mm pigmented papule.

Clinical Photos:

Polarized View

Non-Polarized View

The Power of BLINK:
B = not benign
L = Lonely  1 point
I = Irregular pigment 1 point
N = (pt. unaware of lesion)
K = Known Dermatoscopic abnormalities  1 point
Score = 3  (Mandates biopsy)

The patient is scheduled for an excisional biopsy of the back lesion.  What are your thoughts at this time?

Biopsy shows this to be Malignant Melanoma
Type: unclassified
Thickness: 2.80 mm
Margins: signs of regression at ;ateral margin
Ulceration: absent
Mitoses: > 1/ mm squared
Vascular invasion: absent
Precursor lesion absent

Unusual Pigmentary Process

Presented by Dr. Ana Brasileiro

Central Lisbon Central Hospital

Lisbon, Portugal

Abstract:  51 yo man with 6 year history of pigmentary disorder.

HPI: The patient is a 51 year old male, born in Brazil and living in Portugal for 5 years. He reports that for the last 6 years he has experienced a slowly progressive dermatosis, first affecting the posterior cervical region and the superior part of the trunk, with progressive involvement of all the trunk, arms and legs. The patient denies pruritus or any other symptom. He has no contributory medical past story, and his only medication is occasional omeprazole 20mg. He has no relevant familiar history (neither the parents, brothers or children have any dermatological condition).

O/E:  This shows reticular pattern with hyper and hypopigmentation on the trunk extending to the arms and legs.

Clinical Photos:

Lab: Macrocytic anemia (Hb 11.5 g/L, VGM 106 fL) with normal levels of folic acid, B12 vitamin, as well as normal protein electrophoresis. Iron levels, ferritin and transferin are also normal (although these are not so relevant for a macrocytic anemia) The liver and kidney functions are within the normal range, and the tests for HIV, HCV and syphilis were negative. He has acquired immunity to HBV.

Histopathology: The biopsy showed poikiloderma with amyloid deposits in the superficial dermis, consistent with cutaneous discromic amyloidosis.

Diagnosis: Amyloidosis cutis dyschromica

Questions: What is your opinion about this case?
From your experience, do you know of any case of dyscromic cutaneous amyloidosis starting at the age of 45?
Should we consider other diagnosis?
Do you think that the macrocytic anemia is somehow related with this cutaneous condition?

References: 
1. Amyloidosis cutis dyschromica in two female siblings: cases report; Yang et al. BMC Dermatology 2011, 11:4 “This disorder is characterized by the following features: (i) dotted, reticular hyperpigmentation with hypopigmented spots without papulation almost all over the body; (ii) no or little itchy sensation; (iii) onset before puberty; and (iv) small foci of amyloid closely under the epidermis “

2.Familial amyloidosis cutis dyschromica; Karadag, A.; Simsek, G; Turk J Med Sci 2010; 40 (1): 151-154 “In the literature, there are a few reports of familial cases (…). Cases accompanying autoimmune connective tissue diseases and primary biliary cirrhosis were reported in the other primary cutaneous localized amyloid types, such as macular and lichenoid amyloidosis (7). However, no another systemic and dermatological disease accompany ACD. In the literature there is only one case accompanying generalized morphea (5).”

Hidrotic Ectodermal Dysplasia

Abstract: 44 yo woman with Clouston Syndrome and severe painful kereatoderma

HPI: This 44 year-old disabled woman has the severe stigmata of Clouston Syndrome (CS-HED) (a variant of hidrotic ectodermal dysplasia seen in French Canadian probands).  She has impressive keratoderma of the feet which  make ambulating difficult and painful.  Her father had CS-HED as do three of her daughters.  The presentation in her daughters is variable.  Over the years, her keratoderma has become progressively severe. On a recent visit she said, "Please cut my feet off and feed them to the pigs."  She has been hospitalized in the past for cellulitis secondary to breakdown of plantar keratoderma.  The patient had seen a podiatrist who debrided her feet in theater on occasion, but she moved to another state and her insurance does not cover her there.

O/E:  This is a sad looking 44 year-old woman.  She has alopecia universalis, is edentulous, her nails are dystrophic and she has has abnormal and hyperkeratotic palms.  The most striking feature is massive keratoderma plantaris which tends to break down and become infected.

Clinical Photographs (presented with the patient's consent):

 

Diagnosis and Questions:   This unfortunate woman has massive keratoderma in the setting of Clouston Hidrotic Ectodermal Dysplasia.  Her painful feet make ambulating difficult and intolerable.  This has become more severe over the years and the patient has become discouraged.  She has asked on more than one occasion to have her feet amputated.  My plan at this point is to find a podiatrist or plastic surgeon who can do shave excisions to debride the keratoderma while she is on acitretin which will hopefully forestall the recurrence of her keratoderma.  She had a tubal ligation 22 years ago and is not sexually active at present but will be monitored for pregnancy monthly.  If this approach fails, deep excision of keratoderma with grafting has occasionally been reported.  I plan to start her on 10 mg of acitretin three times a week and increase slowly as tolerated.  

Your suggestions will be appreciated.

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