Unusual localised Morphoea

presented by

Samer A Dhaher MD

Basra, Iraq

January 27, 2008

Samer A Dhaher MD, Dermatologist, Department of Dermatology, College of Medicine, Basra, Iraq

 
Abstract 20 yo woman with 2 year history of localised morphoea on the scalp and forehead
Patient
20-year-old woman
Duration
2 years
Distribution
forehead and scalp
History

A 20-yr-old woman presented to the Dermatological Department complaining of an atrophic depressed band-like plaque acrossing the forehead & scalp horizontally. The lesion started 2 yrs ago when she underwent a minor surgical proceedure (excision of pilar cyst) at the occipital area. Following the operation, a tightly fitted bandage acrossing the head was applied by the surgeon to "ensure" good haemeostasis and retained in-situ for a further 7 days. Upon removal of the bandage, severe itchy erythematous swelling was erupted at the bandage site. A diagnosis of bandage "allergy" was made & topical steroid was prescribed. With the passage of time, the initial swollen phase gave way to whitish atrophic band acrossing the forehead & scalp with patches of loss of hair

Physical Examination

About 4cm width, atrophic ivory white plaque with slightly raised brawnish-violaceous telangiectatic border was seen at the forehead horizontally, the lesion was extended to involve major parts of the parietal and occipital regions causing scarring alopecia. A diagnosis of morphea was made and a skin biopsy was arranged.

Images

Laboratory Data

Hematology and blood chemistry were normal

Histopathology

Many skin biopsies were taken from different sites and all revealed the following: marked epidermal atrophy, the dermis was "empty looking", no skin appandages were found, there was excessive deposition & hemoginization of collagen fibers, replacement of upper fatty layer by excess collagen fibers and mild to moderate superficial and deep perivascular mononuclear cellular infiltrate.

Diagnosis

Unusual form of morphoea

Reasons Presented

Morphea is rare in the scalp & the hair is shed at an early stage to leave cicatricial alopecia. Trauma may be a provocative factor in some patients e.g. at BCG vaccination, the site of radiotherapy for breast cancer. Borrelial infection is still debatable. Involvement of the frontoparietal region may resemble a sabre cut. However, such particular type has not been reported to follow a "real" trauma.

Questions
  1. Have you ever see morphea like this?
  2. Does this lesion represent " en coup de sabre" but horizontally located or koebnerization phenomenon following trauma?
  3. Is there is place for surgical interference like autologus fat graft that may be useful to improve the cosmetic appearance?
References


Keywords morphoea,
Comments from Faculty and Members

Ian McColl MBChB, FACD, Consultant Dermatologist, John Flynn Medical Centre, Tugun, Queensland, Australia on Jan 27, 2008

I have never seen the scalp involved quite like this. Intralesional steroid into the advancing edge might prevent further extension. Trying to inject fat into this densely sclerotic area seems a bit of a waste of time but as I do not use the technique my opinion is probably worthless!

Steven Chow FRCPI, Senior Consultant Dermatologist, Pantai Medical Centre, Kuala Lumpur, Malaysia on Jan 27, 2008

I have a similar case which turned out to be discoid lupus of the scalp.

Henry Foong FRCP, Consultant Dermatologist, Ipoh Specialist Hospital, Ipoh, Malaysia on Jan 27, 2008

I agree intralesional corticosteroid would be useful to halt the progress of the extension. In addition, I usually check their serology for ANA and put them on hydroxychloroquine 200mg bd too. I doubt autologus fat transfer would be useful in this patient.

Stelios Minas MD, Consultant Dermatologist, Limassol, Cyprus on Jan 27, 2008

I have seen cases like these 3-4 times in Russia. I think it is a type of en coup de sabre. With regards to treatment I usually treat with Penicillin (1.2mili a time for course 20 mili) after that I continue with Plaquenil 200mg bd and Trental 400mg bd for 2-3 months. Topically, I treat with Dermovate cream and Elidel cream. Also I treat every other week with mesotherapy (Trental and Longidasa). I have seen very good effect in some cases. I don't have experience with surgical intervention but would be interested to know the place for surgical intervention.

About penicillin I treat with Extencilline-Benzathine benzylpenicillin (1 200 000 units every 3-4 days I/M for general course 10-20 000 000 units. Personally I like to treat morphea with penicillin for two reasons: first for Borrelia burgdoferi as associating with morphea. The second reason is one of the function of penicillin is destructive synthesis of collagen (we find that penicillin is stronger about destructive synthesis of collagen than Cuprenil) as a pathophysiology of morphea is overproduction of collagen by fibroblasts.

Yes here in Russia elaborate longidasa(www.longidasa.ru-Russian language). I use Longidasa in mesotherapy (injection technique - micropapula) for treatment of morphea and lichen sclerosus et atrophicus with good effects. Usually in combination with Trental. The only negative of Langidasa is very often can give allergic reaction.

Robert Rudolph MD, FACP, Clinical Professor of Dermatology, University of Pennsylvania, Philadelphia, PA, USA on Jan 27, 2008

I've not personally seen such a very peculiar distribution. It certainly seems to follow a pattern which reflects an exogenous trauma or pressure of some kind. I also wonder if any steroids had been administered somewhere, by someone, without your being aware of it.

David Elpern MD, Dermatologist, Skin Clinic, Williamstown, MA, USA on January 28, 2008

If this is morphea, I think it is different from "en coup de sabre." It is most unusual, and I would go along with the person who questioned whether there were some corticosteroid injections which may have caused atrophy. All this being said -- there will always be "experiments of one." Unique cases.

I would like to have the slides read by a dermatopathologist. It would be interesting to hear their differential diagnosis based on a thorough review of the pathology. I am sure Dr. Bhawan at Boston University would be willing to do this. Good luck with this challenging patient!

Shahbaz Janjua MD, Dermatologist, Ayza Skin & Research Center, Lalamusa, Pakistan on Jaunary 28, 2008

This is an interesting case. I have seen a few cases of en coup de sabre linear morphea but not a case of horizontal morphea involving scalp and forehead like this one. I agree with Ian regarding use of intralesional steroids. In my opinion, a combination of UVA1 and topical calcipotriene would be worth trying before any invasive therapy.

Nurul Amin MD, FRCP, Professor of Dermatology, Armed Forces Medical College, Dhaka, Bangladesh on January 28, 2008

No doubt it is an interesting case .I have seen two or three en coup de sabre but not band type in forehead . I can remember one middle-aged lady reported with
localized morphea (5cm X 8cm) on the abdomen, which I treated with topical calcipotriol and topical steroid with good response.

Arash Abtahian MD, Shiraz, Iran on January 28, 2008

This case is interesting for the initiation point of view and I have seen some cases showing en coupe de sabre obliquely or horizontally but not circumferentially. I think for treatment patient should undergo tissue expansion and resection after the disease has stopped its activity.

Joel Bamford MD, Dermatologist Adjunct in Family Practice, Univ Minnesota-Duluth Medical School, Duluth, Minnesota, USA on Jan 28, 2008

  1. No
  2. DLE and steroid atrophy? Pattern on posterior scalp seems to be atypical.
  3. Doubt fat injection helpful here.
    Looking forward to follow-up,

Dermatopathologist review.
Serologic LE studies.
Present to local society.

Elena Pope MD FRCPC, Assistant Professor, University of Toronto, Toronto, Canada on Jan 28, 2008

Very challenging case. I am not sure it is morphea. We had very good results in sclerotic lesions with topical imiquimod applied 5 times a week. It may work better than intralesional steroids which are likely to cause more atrophy.

Meera Mahalingam MD, Associate Professor of Dermatology and Pathology, Boston University, Boston, MA, USA on Jan 28, 2008

Despite the fact that the photomicrographs appear a tad suboptimal one can discern the key pathologic finding i.e. homogenization of dermal collagen. If the mononuclear cell infiltrate included plasma cells, the histopathology would be fine for morphea.

Nico Mousdicas MD, Associate Professor, Department of Dermatology, Indiana University-Purdue Indianapolis, Indianapolis, Indiana, USA on Jan 28, 2008

Very interesting case. Clinically this could be morphea or lupus profundus. If Morphea I concurr with Dr Minas regarding antibiotics. I empiricaly give my patients long term doxycycline or alternantively amoxicillin or erythromycin. I am personally convinced that morphea is caused by a Borrelia spirochete. I note in recent articles in the BMJ where they talk about focal floating microscopy with a sensitivity of up to 98% in picking up Borrelia versus the low 50% pick up rate with PCR. It would not harm to give the patient antibiotics empirically once one is convinced it is morphea.

Omid Zargari MD, Dermatologist, Rasht, Iran on Jan 29, 2008

To me this does not look morphea. What happened here is originated form that "tightly fitted bandage"; I think some degrees of ischemia+infection have made this. Perhaps staged scalp reduction by an expert surgeon will help this poor woman.
I'm very interested to see before-after pictures of Dr. Minas for morphea therapy with meso. Thanks for sharing this case.

Khalid Al Hawsawi M.D., Consultant Dermatologist, King Abdul Aziz Hospital, Makkah, Saudi Arabia on January 30, 2008

Excellent case. I do agree, it is morphea rather than "en coup de sabre".

Jerry Tan M.D., Adjunct Professor, Department of Dermatology, University of Western Ontario, Windsor, Canada on Feb 20, 2008

History, location and appearance suggests scarring secondary to inflammatory response and perhaps infection to tight persistent bandaging (?infected contact dermatitis). Thankfully, posterior scalp lesion may be partially masked by hair - however, scar excision may be worthwhile if still apparent with hair down. Forehead lesion - test site of intra-lesional corticosteroid; would opt primarily for cosmetic camouflage otherwise.



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