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Last Updated: Oct 12th, 2010 - 01:53:59 |
Title: Painful Eyrthematous Papules
Presenter: Chris Buatti D.O.
Dermatology Program: Genesys Regional Medical Center
Program Director: Kimball Silverton D.O.
Submitted on:
Jun 16, 2010
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CHIEF COMPLAINT:
A 48 year old African American male presented with new onset acne like lesions on his face.
CLINICAL HISTORY:
Signs and symptoms:
These erythematous painful papules started two months prior, and the patient complained primarily of pruritis(fig 1, 2).
He was recently diagnosed with HIV after being hospitalized for pneumonia last June. His PCP started him on antiretrovirals, and bactrim. He also denied constitutional symptoms such as fever, chills, weight loss, myalgias or arthralgia.
Previous Treatment:
None
Other information:
None
PHYSICAL EXAM:
1-5 mm painful, puritic papules of the cheek, forehead and neck. Some excoriated follicular papules present. Oral mucosa, hair and nails were not affected. There was no cervical, axillary, or inguinal adenopathy.
LABORATORY TESTS:
A shave biopsy was obtained from one of the lesions and sent for histological analysis. The patient CBC, ANA, CD4, CD8, and TSH levels drawn. His absolute CD4 count was 67 cells/mm3, well below the threshold of 200 cells/mm3 usually seen in patients with EPF (4), and he was anemic with a hemoglobin level of 9.8. All other labs were within normal limits.
DERMATOHISTOPATHOLOGY:
A biopsy was taken from his left forehead with a differential of EPF versus granuloma rosacea. The pathology report came back showing abundant eosinophils and superficial granulomatous infiltrate. The diagnoses of sarcoid and granulomatous rosacea were entertained, but neither of these disease processes present with eosinophils. EPF findings are usually identified after serial biopsies are performed with careful analysis of every hair follicle (5).
Histological, an infiltrate of mononuclear cells and eosinophils is seen around the upper portion of the hair follicle at the level of the sebaceous gland. As lesions evolve, eosinophils and lymphocytes enter the follicular structure and the sebaceous glands.
The diagnosis of EPF is extremely difficult to make, and frequently becomes clear after the diagnosis of HIV is finally made.
DIFFERENTIAL DIAGNOSIS:
1. Acne Vulgaris
2. Rosacea
3. Drug Reaction
4. Pityrosporum Folliculitis
5. Eosinophilic (pustular) Folliculits
SCROLL DOWN FOR ANSWER AND DISCUSSION.
CORRECT DIAGNOSIS:
Eosinophilic (pustular) Folliculitis
DISCUSSION:
Dermatologic conditions are often a presenting clue in immunosuppresed patients dealing with the HIV infection. One such condition is eosinophilic (pustular) folliculitis (EPF), a part of the immune restoration syndrome. EPF is a reflection of abnormal Th2/Th1 immune response. This unique culture-negative HIV related disorder often manifests itself late in the disease process when CD4 counts drop below 200 cells/mm3, and may be the only source of morbidity for the patient (1, 2). Most patients with EPF are men in their third and forth decade of life (3), and they complain of a chronic, edematous, follicular eruption involving the trunk and face (2). Pustular lesions are uncommon; pustules are usually smaller than in bacterial folliculitis and represent end-stage lesions.
Initial treatment of eosinophilic pustular folliculitis is topical steroids and antihistamines. If the patients fails to respond, phototherapy (UVB or PUVA) or intraconazole 200 mg twice a day may be effective. Some patients repeated applications of permetherin every other night for 6 weeks may be of benefit. This therapy is directed at Demodex mites, which may be the antigenic trigger of this condition.
Isotretinoin is can also be effective often after a few months, in a dose of about .5mg to 1mg/kg/day. Prednisone can be used at an initial dose of 70 mg PO and tapered over two weeks, but lesions frequently reoccur once it is stopped. Other treatment options are dapsone, indomethacin, and tacrolimus which remains to be fully explored and tested.
TREATMENT:
The patient was started on Claritin, Doxycycline 100 mg bid and Desonide crm applied bid to decrease inflammation (6). During the course of treatment other therapies were discussed, but no treatment is considered uniformly effective. The difficulty lies with pathophysiology of the disease. Some believe EPF is caused by an immune mediated hypersensitivity to the fungus Malassezia. Our biopsy was negative with PAS stain, but a course of ketoconazole was discussed (7). Multiple bacteria have been blamed, but treatment with antistaphylococcal antibiotics have not been shown to be effective (6).
In the absence of improvement of the underlying immunodeficiency, EPF with HIV infection may be recalcitrant to therapy and severity can spontaneously fluctuate.
HIV/AIDS continues to be on the rise throughout the world. HIV related dermatosis is being seen at all levels of care, and its unique presentation and treatment should be explored.
REFERENCES:
1. Ofuji S, Ogino A, Horio T. Eosinophilic pustular folliculitis. Acta Dermatologica Kyoto 1970;50:195-203.
2. Ofuji S. Eosinophilic pustular folliculitis. Dermatologica 1987;174:53-6.
3. Ellis E, Scheinfeld N. Eosinophilic pustular folliculitis: a comprehensive review of treatment options. Am J Clin Dermatol 2004;208:229-30.
4. Rosenthal D, LeBoit, Klimpp L. Human immunodeficiency virus –associated eosinophilic folliculitis. A unique dermatosis associated with advanced HIV infection. Arch Dermatol 1991;127:206-9.
5. Piantanida EW, Turiansky GW, Kenner JR. Immunosuppression-associated eosinophilic folliculitis: diagnosis by transverse histologic sections. J Am Acad Dermatol 1998;197:178-80.
6. Fearfield LA, Rowe A, Francis N, et al. Itchy folliculitis and human immunodeficiency virus infection: clinicopathological and immunological features, pathogenesis and treatment. Br J Dermatol 1999;141:3-11.
7. McCalmont TH, Altemus D, Murer T, et al. Eosinophilic folliculitis. The histologic spectrum. Am J Dermatopathol 1995;17:439-46.
Additional Comment:
Please see attached photos
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