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Hidradenitis suppurativa

Hidradenitis suppurativa or HS is a poorly studied skin disease that affects areas bearing apocrine sweat glands and hair follicles; such as the underarms, groin and buttocks, and under the breasts in women. more...

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Overview

The disease manifests as clusters of chronic abscesses or boils, sometimes as large as baseballs, that are extremely painful to the touch and may persist for years with occasional to frequent periods of inflammation, culminating in drainage, often leaving open wounds that will not heal. Drainage provides some relief from severe, often debilitating, pressure pain. These flare-ups are often triggered by stress, hormonal changes, or humid heat. Persistent lesions may lead to the formation of sinus tracts, or tunnels connecting the abscesses under the skin. At this stage, complete healing is usually not possible, and progression of the disease in the area is inevitable. Occurrences of bacterial infections and cellulitis (deep tissue inflammation) are likely at these sites. HS pain is difficult to manage.

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone. When they do see a doctor, the disease is frequently misdiagnosed or prescribed treatments are ineffective, temporary and sometimes even harmful. There is no known cure nor any consistently effective treatment.

Although HS is considered a rare disease, its incidence rate is estimated as high as 1 in 300 people.

Other names for HS

  • Acne conglobata
  • Acne Inversa (AI)
  • Apocrine Acne
  • Apocrinitis
  • Fox-den disease
  • Hidradenitis Supportiva
  • Pyodermia sinifica fistulans
  • Velpeau's disease
  • Verneuil's disease

Stages

HS presents itself in three stages:

  1. a few minor sites with rare inflammation; may be mistaken for acne.
  2. frequent inflammations restrict movement and require minor surgery.
  3. inflammation of sites to the size of golf balls, or sometimes baseballs; scarring develops, including subcutaneous tracts of infection (see fistula). Obviously, patients at this stage may be unable to function.

Causes

As this disease is poorly studied, the causes are controvertial and experts disagree. However, potential indicators include:

  • post-pubescent
  • females are more likely than males
  • genetic predisposition
  • plugged apocrine (sweat) gland or hair follicle
  • excessive sweating
  • bacterial infection
  • linked to some immunodeficiency conditions
  • androgen dysfunction
  • genetic disorders that alter cell structure

Research currently implies that people with HS have a tendency towards clogged apocrine glands, which may then become infected with bacteria commonly present on the skin, and the immune system overreacts with excessive inflammation. Attempted treatments can target any of these three aspects of HS.

Read more at Wikipedia.org


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ALA-PDT and blue light therapy for hidradenitis suppurativa - Articles
From Journal of Drugs in Dermatology, 1/1/04 by Michael H. Gold

Abstract

Background: Hidradenitis suppurativa (HS) is a chronic, often suppurative skin condition which affects primarily apocrine glands. A variety of therapies have been used to treat HS, often with unsatisfactory results. Photodynamic therapy (PDT), utilizing topical 20% 5-aminolevulinic acid (ALA) is being used to treat a variety of dermatologic skin concerns, including photorejuvenation and associated actinic keratoses, and acne vulgaris, and other skin tumors.

Objective: The purpose of these case reports is to evaluate the effectiveness of ALA-PDT in treating recalcitrant cases of HS.

Methods: Four patients, not responding to standard HS therapy, underwent short-contact ALA-PDT therapy utilizing a blue light for activation. One to two week intervals between therapies was utilized for 3-4 total treatments and follow-up was for 3 months following the last treatment.

Results: All four of the patients tolerated the therapies well. Clinical improvements from 75-100% were noted n ll of the patients. No adverse effects were seen during the treatments. The treatments were pain free and there was no downtime associated with these ALA-PDT treatments.

Conclusions: HS is a chronic disease which most dermatologists find difficult to treat. The use of ALA-PDT is finding an ever-expanding role in dermatology. These case studies support the use of ALA-PDT in cases of HS. Although all advertising material is expected to conform to ethical and medical standards, inclusion in this publication does not constitute a guarantee or endorsement by the Journal or its staff of the quality or value of such products or of the claims of any manufacturer.

KEYWORDS:

PHOTODYNAMIC THERAPY

HIDRADENTIS SUPPERATIVA

AMINOLEVULINIC ACID

APOCRINE GLANDS

**********

Introduction

Hidradenitis suppurativa (HS) is a chronic, often suppurative cicatricial skin condition which principally affects apocrine gland-bearing skin. (1, 2) The skin of the axillae and inguinal areas are most commonly involved in those that suffer from HS; reports from other apocrine gland areas are also found including the mammary and perianal areas, buttocks, scrotum, and mons pubis. The etiology of HS includes keratinous plugging of the apocrine duct, dilation of the apocrine duct, and severe inflammation of the apocrine gland. (3) A genetic predisposition may play an important role and hormonal influences may be required for the progression of HS. An association with acne vulgaris exists, although some with HS show no signs of acne vulgaris. (4) A variety of treatments have been reported for HS, often with mixed results. These have included both topical and oral antibiotics; a variety of hormonal therapeutics; intralesional corticosteroids; oral retinoids; and a variety of surgical modalities including incision and drainage, wide surgical excision, and C[O.sub.2] laser therapies have been used. (5)

Photodynamic therapy (PDT) refers to the therapeutic use of photochemical reactions. It involves the use of photosensitizing drugs, the application of an appropriate light source to activate the sensitizing drug which leads to the production of singlet oxygen for tissue destruction. The most common photosensitizing drug used for PDT is 20% 5-aminolevulinic acid (ALA). (6, 7) The combination of ALA-PDT is currently FDA approved for the treatment of non-hyperkeratotic actinic keratoses of the face and scalp. (8) Other dermatologic indications for ALA-PDT include basal cell carcinomas, squamous cell carcinomas, Bowen's disease, actinic chelitis, cutaneous T-cell lymphoma, Kaposi's sarcoma, verrucae vulgaris, and molluscum contagiosum. (6, 7) Recently, this author and investigators have described the successful use of

ALA-PDT in treating acne vulgaris and success with the use of the intense pulsed light source and vascular lasers in the photorejuvenation process and associated actinic keratoses. (9, 10)

PDT works by having diseased tissue produce and accumulate protoporphyrin IX (PpIX). PpIX is a potent photosensitizer and has been shown to accumulate in dysplastic and neoplastic dermatologic skin lesions and epidermal appendages and hair follicles which can be induced by the topical application of 5-ALA HCL, the biological precursor of porphyrin. The exogenously induced photosensitization targets the tissue for PDT. During PDT, the porphyrin molecule itself is photoactivated by exposure to visible light which will result in the production of a cytotic oxygen product that will destroy abnormal cells where the PpIX has been concentrated. There is minimal damage to the surrounding unaffected skin making PDT an attractive therapy for dermatologic concerns. (6, 7)

With this background, we identified four individuals with chronic HS who had not responded to traditional therapy with a variety of topical and systemic agents, as well as intralesional corticosteroid therapy. These patients elected to use ALA-PDT therapy as a last resort prior to contemplating a major surgical procedure or C[O.sub.2] laser therapy. All of the patients tolerated the treatments well. There were no adverse effects noted during these treatments. These therapies were pain-free and not associated with any downtime for these patients.

Case #1

The first patient is a 22 year old female with a history of HS involving the axillae and inguinal areas. Previous therapies included systemic and topical antibiotics; systemic, intralesional and topical corticosteroids; and incision and drainage, when appropriate. After receiving full disclosure of the ALA-PDT process and the off-label FDA use of this product for HS, the patient signed an informed consent. Twenty percent 5-ALA, Levulan[R] (Dusa Pharmaceuticals, Wilmington, MA) was applied to the affected areas and allowed to incubate on the skin for 30 minutes prior to light therapy. The light source used was the ClearLight[TM] photoclearing system (Lumenis, Santa Clara, CA), a blue light phototherapy system emitting light in the 407 - 420 nm range. Eighteen minutes of blue light incubation was given to each affected area prior to light therapy. The patient received three total treatments at two-week intervals and achieved 75% clearance with this therapeutic modality. The clearance achieved has been maintained at a three month follow-up visit.

Case #2

The second case report involves a 19-year old female with a history of HS involving the axillary areas. Past treatment has included systemic and topical antibiotics, systemic and intralesional corticosteroids, and incision and drainage of several lesions. After signing an informed consent, the patient underwent four ALA-PDT treatments with the blue light photoclearing system. ALA incubation was for 15 minutes prior to each light therapy. The patient received four blue light/ALA-PDT treatments at 1 week intervals. A 75% clearance rate was achieved which was maintained at a three month follow-up (Figures 1, 2).

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

Case #3

The third case is a 46-year old female with a history of HS involving the inguinal areas. Past therapies have included systemic and topical antibiotics, and systemic and topical corticosteroids. The patient gave and signed an informed consent and received a total of eight blue light/ALA-PDT treatments. The ALA drug incubation was for 30 minutes prior to the blue light therapy. Each therapy was given at 1 week intervals and the patient noted 100% improvement in her HS symptoms. This has remained clear at the three month follow-up visit (Figures 3, 4).

[FIGURE 3 OMITTED]

[FIGURE 4 OMITTED]

Case #4

The fourth case is a 24-year old female with a history of HS in the inguinal area. Past therapies have included systemic and topical antibiotics. The patient signed an informed consent and received four blue light/ALA-PDT treatments at one week intervals. Drug incubation with ALA was for 15 minutes prior to light therapy. The patient noted 75% improvement after the four treatments which persisted at a three month follow-up visit.

Discussion

HS is a chronic skin disease in which a variety of treatment options have been used in an attempt to control the symptoms of the disease. The use of ALA-PDT and an appropriate light source is a novel/new therapeutic option which is receiving a great deal of interest in dermatology as its potential uses are being defined and explored. These case reports describe the first reported cases on the use of ALA-PDT in this predominantly apocrine gland condition.

The exact mechanism of action on how this process works in HS still needs to be determined. It is felt that there is production of PpIX in the affected glandular areas/follicular units which are amenable to ALA-PDT activation with blue light therapy. The patients described in this report achieved clearance rates from 75-100% during the treatment intervals which were able to be maintained at a three month follow-up period. The patient who received the most treatments had the best response which would indicate that continued therapies in the others might yield an even greater clinical response.

ALA-PDT is finding its place in dermatologic therapy. HS can now be added to the list of entities which can be treated with ALA-PDT and light therapy. Perhaps a more invasive surgical procedure, with associated risks and morbidities, can be avoided in these patients by utilizing ALA-PDT.

References

1. Jansen T, Plewig G. Acne inversa. Int J Dermatol 1998; 37:96-100.

2. Brown T, Rosen T, Orengo I. Hidradenitis suppurativa. South Med J 1998; 91:1107-1114.

3. Shelley WB, Cahn MM. Pathogenesis of hidradenitis suppurativa in man: experimental and histologic observations. Arch Dermatol 1955; 72:562.

4. Hurley HJ: axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa and familial benign pemphigus: surgical approach, in Dermatologic Surgery. Principles and Practice, eds Roenigk RA, Roenigk HH, Jr. New York, Marcel Dekker, 1989;735.

5. Lapins J, Sastorius K, Emtestam L. Scanner-assisted carbon dioxide laser surgery: A retrospective follow-up study of patients with hidradenitis suppurativa. J Am Acad Dermatol 2002; 47:280-285.

6. DeRosa FS, Bentley MV. Photodynamic therapy of skin cancers: Sensitizers, clinical studies and future directives. Pharm Res 2000; 17:1447-1455.

7. Szemies RM, Calzavara-Pinton P, Kanar S, et al. Topical photodynamic therapy in dermatology. J Photochem Photobiol 1996; 36:213-216.

8. Gold MH. The evolving role of aminolevulinic acid hydrochloride with photodynamic therapy in photoaging. Cutis 2002; 69:8-13.

9. Gold MH. A single center, open label investigatory study of photodynamic therapy in the treatment of moderate to severe acne vulgaris with aminolevulinic acid topical solution 20% and visible blue light. Poster presentation, American Academy of Dermatology Annual Meeting, March, 2003.

10. Gold MH. A single center, open label investigatory study of photodynamic therapy in the treatment of photoaging with aminolevulinic acid topical solution 20% and intense pulsed light. Poster presentation, American Academy of Dermatology Annual Meeting, March, 2003.

MICHAEL H. GOLD MD

TANCY M. BRIDGES RN, NPC

VIRGINIA LEE BRADSHAW RN, NPC

MOLLY BORING RN, NPC

GOLD SKIN CARE CENTER, NASHVILLE, TN

ADDRESS FOR CORRESPONDENCE:

Michael H. Gold MD

Gold Skin Care Center

2000 Richard Jones Road, Suite 220

Nashville, TN 37215

T; 615-383-2400

F: 615-385-0387

E: goldskin@goldskincare.com

COPYRIGHT 2004 Journal of Drugs in Dermatology
COPYRIGHT 2004 Gale Group

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