| Abstract |
51 yo woman with pemphigus foliaceus developed severe facial hyperpigmentation
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Patient |
51-year-old woman |
Duration |
1 year |
Distribution |
Face |
| History |
A 51 yo housewife presented with painful superficial erosions on her face and trunk in 2007. She was diagnosed to have pemphigus foliceous (PF) with skin biopsies and immunofluoroscence studies and was treated with oral prednisolone. In September 2008, she had a severe relapse and was treated with IV IgG, high dose oral prednisolone and oral methotrexate 12.5 mg weekly.
About a year ago, she developed dusky colored hyperpigmentation on her face. It was asymptomatic except that she could not tolerate bright light - it make her face painful. She was started on topical hydroquinone cream 4% and sunblocks. Despite that, the hyperpigmentation worsened. The possibility of ochronosis was considered and a biopsy done. Biopsy showed pigmentary incontinence but no evidence of ochronosis.
Her present medications include prednisolone 20mg daily, methotrexate 15 mg weekly, oral folic acid 5 mg daily and topical clobetasone butyrate (Eumovate) cream bd. She was not on any other medications or traditional herbs.
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| Physical Examination |
Severe diffuse dusky hyperpigmentation on the face predominantly on the cheeks, forehead and chin. It has a well circumscribed margin on the right cheek. No other significant hyperpigmentation on the rest of the body. Few superficial erosions were noted on the lower back.
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| Images |
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| Laboratory Data |
Blood counts normal
Anti-nuclear Antibody negative |
| Histopathology |
Histopath studies done in March 2007 (Courtesy of Dr Agnes Heng, Hospital Raja Permaisuri Bainun, Ipoh)
Section shows a subcorneal blister containing acantholytic cells, polymorphs and red blood cells. The upper dermis showed perivascular infiltration by polymorphs and mononuclear cells. The deeper dermis is unremarkable. Interpretation: Pemphigus foliaceus
DIF report: Section of patient's skin have been reacted to antibodies IgG, IgM, IgA and C3. The biopsy showed at intercellular depostition of IgG and C3. Interpretation: pemphigus
IIF studies: Blood: Sections of normal human skin were reacted to serial dilutions of the patient's serum. These sections were then incubated with antibodies to IgG, IgA and IgM. The section revealed intercellular staining of IgG mainly in the upper epidermis at a dilution of 1:640. Interpretation: Pemphigus foliaceus
Biopsy of the skin taken from the left temple (April 2009): Normal epidermis with upper dermis infiltrated by melanophages. The deep dermis appeared normal. |
| Diagnosis |
Pemphigus foliaceus. Facial hyperpigmentation - post inflammatory?
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| Reason for presentation |
The severe facial hyperpigmentation is unusual. It appeared to be light-related. Could it have been made worse by topical hydroquinone cream 4%? However, skin biopsy of the lesions did not show any evidence of ochronosis.
Minocycline-induced hyperpigmentation can also cause similar changes but she was not on this medication. A variant of pemphigus foliaceus known as pemphigus erythematosus (Senear Usher Syndrome) have features of LE and PF. However, she did not have the typical malar and butterfly distribution.
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| Questions |
- What is the cause of the facial pigmentation?
- How would you treat her?
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| References |
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| Keywords |
pemphigus foliaceus, hyperpigmentation
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| Comments from Faculty and Members |
Richard Sontheimer M.D., Professor of Dermatology, University of Utah, Salt Lake City, Utah, USA on May 2, 2010
Pemphigus erythematosus typically has a positive circulating antinuclear antibody and a positive lesional lupus band test in addition to intercellular staining on direct immunofluorescence. Consider a methotrexate-induced radiation recall reaction of face leading to post-inflammatory hyperpigmentation. Had this woman earlier in life experienced sunburn reactions on her face?
Robert I.Rudolph, M.D, FACP Clinical Professor of Dermatology, University of Pennsylvania, Philadelphia, PA USA on May 2, 2010
Looks for all the world like an "argyrial pigmentation". Was she using a silver product of some kind either orally or topically before (or even while) she was/is under your care?
My bet is "yes".
Julian Manzur M.D., Professor Enrique Cabrera Faculty of Medicine, Havana, Cuba on May 3, 2010
See on the back near the erosions, some similar dark lesions. Pemphigus foliaceus frequently start on face, leaving macular hyperpigmentation. I am thinking in postinflammatory hyperpigmentation probably as a result of Senear Usher
Samuel Moschella MD, Professor of Dermatology, Emeritus, Harvard Medical School, Burlington, MA, USA on May 6, 2010
When I visited Brazil, they presented several cases of endemic pemphigus foliaceus who developed hyperpigmentation when the disease went into remission. These caucasians resembled mulattoes by developing black or blue-grey tint of the skin. They histologically had an increase number of melanocytes with an increase in melanin.
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